UNIT 3

Diabetes Insipidus DI

can be classified into two types—nephrogenic DI and central DI. Nephrogenic DI can be transmitted genetically (e.g., sex-linked, autosomal dominant, or autosomal recessive forms) or be acquired due to chronic renal disease, hypercalcemia, hypokalemia, or use of certain drugs such as lithium, amphotericin, methicillin, and rifampin (Breault & Majzoub, 2016a). This variant of DI is not associated with the pituitary gland and is related to decreased renal sensitivity to antidiuretic hormone (ADH). Therapeutic management for nephrogenic DI involves diuretics, high fluid intake, restricted sodium intake, and a high-protein diet. Desmopressin acetate (DDAVP) is usually ineffective in the treatment of nephrogenic DI. Central DI is a disorder of the posterior pituitary gland and is the most common form of DI (Children's Hospital of Boston, 2019). Therefore, central DI will be the focus of the remainder of this discussion. Central DI is characterized by excessive thirst (polydipsia) and excessive urination (polyuria) that is not affected by decreasing fluid intake. Typically, this disorder occurs in children as a result of complications from head trauma or after cranial surgery to remove hypothalamic-pituitary tumors such as craniopharyngioma. Some cases can be hereditary; however, 10% of central DI cases in children are idiopathic (Breault & Majzoub, 2016a). Other causes include genetic mutations, granulomatous disease, infections such as meningitis or encephalitis, vascular anomalies, congenital malformations, infiltrative disease such as leukemia, or administration of certain drugs that are associated with inhibition of vasopressin release, such as phenytoin (Breault & Majzoub, 2016a). DI is usually permanent and requires treatment throughout life. Pathophysiology Central DI results from a deficiency in the secretion of ADH. This hormone, also known as vasopressin, is produced in the hypothalamus and stored in the pituitary gland. ADH is involved in concentrating the urine from the kidneys by stimulating reabsorption of water in the renal collecting tubules through increased membrane permeability. This conserves water and maintains normal osmolality. With a deficiency in ADH, the kidney loses massive amounts of water and retains sodium in the serum. Therapeutic Management Unless a tumor is present (in which case it is removed by surgery), the usual treatment of central DI involves a low solute diet (low sodium and low protein), daily replacement of ADH, and/or use of a thiazide diuretic (Bichet, 2017). The drug of choice for home treatment is DDAVP, a long-acting vasopressin analog (Breault & Majzoub, 2016a). In children, it is typically given intranasally. However, it can also be administered subcutaneously, orally, or buccally. The drug is given every 8 to 12 hours. The dose depends on the child's age, urine output, and urine specific gravity. Treatment of DI and the use of DDAVP in infants and small children are challenging and complicated due to their inability to access fluids and articulate thirst (Bichet, 2017). In neonates and young infants, the treatment is often solely fluid therapy due to their large volume requirements of nutritive fluid (i.e., the drive behind an infant's fluid intake is hunger rather than thirst) (Breault & Majzoub, 2016a). However, research has shown that subcutaneously administered DDAVP may be more effective than oral or intranasal therapy in infants and small children related to variable absorption and the challenge of administering accurate doses via these routes (Bichet, 2017). In the hospital, the child may receive aqueous vasopressin, 8-arginine vasopressin (Pitressin), intravenously (Breault & Majzoub, 2016a). This is a short-acting drug, so the dosage can be adjusted quickly. Both the long- and short-acting forms of the medication decrease urinary output and thirst, and the dosages of both forms of these drugs need to be titrated to achieve the desired effect. Health History Nursing assessment involves obtaining a history of any conditions that led to the development of the disorder. This review includes information about the neonatal period as well as a current history of infections such as meningitis, diseases such as leukemia, or familial patterns. Although most symptoms of endocrine disorders develop slowly, the onset of this disorder is abrupt. The health history usually elicits the cardinal symptoms as well as complaints representing the early signs of dehydration. The most common initial symptoms reported are polyuria and polydipsia (Breault & Majzoub, 2016a; Bichet, 2017). Except for unconscious children, the child typically maintains adequate perfusion by drinking water. The parent or child may report frequent trips to the bathroom, nocturia, or enuresis. When the child cannot compensate for the excessive loss of water by increasing fluid intake, other symptoms will be reported, such as weight loss or signs of dehydration. For example, irritability may be due to the early signs of dehydration or the frustration the child feels at being unable to quench his or her thirst. Other signs may be intermittent fever, vomiting, and constipation. Physical Examination Observation and inspection may reveal weight loss or failure to thrive in the young infant. Inspection may also reveal signs of dehydration, such as dry mucous membranes or decreased tears. The child may excrete greater than 3 L/m2 of urine per day. On auscultation, tachycardia or increased respiratory rate may be signs of compensation for the decrease in fluid volume. Palpation reveals slightly depressed fontanels or decreased skin turgor. Laboratory and Diagnostic Testing Diagnostic tests used to evaluate DI include: Radiographic studies such as CT scan, MRI, or ultrasound of the skull and kidneys can determine whether a lesion or tumor is present. Urinalysis: urine is dilute, osmolality is less than 3,000 mOsm/L, specific gravity is less than 1.005, and sodium is decreased. Serum osmolality is greater than 300 mOsm/L. Serum sodium is elevated. Fluid deprivation test measures vasopressin release from the pituitary in response to water deprivation. Normal results will show decreased urine output, increased urine specific gravity, and no change in serum sodium. Maintain fluid intake regimens as ordered. Monitor fluid status by measuring vital signs, fluid intake and output, and daily weights (using the same scale at the same time of day). If fluids are stopped too soon, the child may become hypernatremic, which can lead to seizures. Feed infants more frequently, since they excrete more dilute urine, consume larger volumes of free water, and secrete lower amounts of vasopressin than older children. Monitor for signs and symptoms of dehydration during the fluid deprivation test as well as when starting the treatment regimen. Educating the Family Involve the family in development of the fluid intake regimens. A journal or daily log is essential in maintaining the fluid regimen and identifying problems. Children with intact thirst centers can self-regulate their need for fluids, but if this is not the situation, help the family develop a plan for 24-hour fluid replacement. This may require instruction on nasogastric or gastrostomy feedings. Infants will need fluid intake at night. Educate the family about the symptoms of water intoxication (drowsiness, listlessness, headache, confusion, sudden weight gain, and anuria) and dehydration. Help the family develop a plan to inform the school and other individuals in the child's life about the need for liberal bathroom privileges and extra fluids to prevent accidents or dehydration. Teaching Guidelines 26.1 gives tips on educating the family about the medication regimen. Recommend that the family obtain a medical ID alert bracelet or necklace for the child. Encourage compliance with follow-up appointments, which will probably be every 6 months. TEACHING GUIDELINES 26.1 DDAVP Intranasal Administration Keep DDAVP in the refrigerator at all times (if directed, some products no longer require refrigeration—refer to product insert). Clear the nostrils (the medication may be poorly absorbed if the child has nasal congestion). Insert the measuring tube into the bottle. Fill to proper dosage and hold the top of the tube closed while inserting the medication-filled end into the nostril. Blow the liquid out of the tubing into the nostril. When using metered nasal spray, spray must be primed before first use. If the child sneezes, repeat the dosage. Measure urine specific gravity to monitor effectiveness of the drug. Monitor for signs and symptoms of overdosage such as confusion, headache, drowsiness, and rapid weight gain due to fluid retention.

Autism spectrum disorder (ASD)

has its onset in infancy or early childhood and affects 1 in 68 children (Goldson & Reynolds, 2018). The spectrum of autism disorder ranges from mild to severe. Many children with autism are intellectually disabled, requiring lifelong supervision, though the majority of children with ASD display normal to high intelligence levels (Buchanan, 2019). Autistic behaviors may be first noticed in infancy as developmental delays or between the age of 12 and 36 months when the child regresses or loses previously acquired skills. Parental concerns about development may be sensitive indicators of the development of autism. Pathophysiology Though the exact etiology of autism continues to be unknown, genetics has been well studied in these children and ASD is mainly considered to be a genetic disorder, though there may also be issues with brain connectivity (Buchanan, 2019). Children with ASD display impaired social interactions and communication as well as perseverative or stereotypic behaviors. They may fail to develop interpersonal relationships and experience social isolation. Therapeutic Management There are no medications or treatments available to cure autism. The goal of therapeutic management is for the child to reach optimal functioning within the limitations of the disorder. Each child's treatment is individualized; behavioral and communication therapies are very important. Children with ASD respond very well to highly structured educational environments, so early, intensive behavioral interventions are necessary. Stimulants may be used to control hyperactivity, and antipsychotic medications are sometimes helpful in children with repetitive and aggressive behaviors. Many families are drawn to the use of complementary and alternative medical therapies in attempts to treat their autistic child. They may use vitamins and nutritional supplements, herbs or restrictive diets, music therapy, art therapy, and sensory integration techniques. To date, these therapies have not been scientifically proven to improve autism (Volkmar, 2019). See Evidence-Based Practice Box 28.1. Nursing Assessment Elicit the health history, noting delay or regression in developmental skills, particularly speech and language abilities. Failure to point at objects and to gaze at an object jointly with another by 18 months are concerning signs. The most common early characteristics are a consistent failure to orient to one's name, regard people directly, use gestures, and to develop speech (Goldson & Reynolds, 2018). The child may be mute, utter only sounds (not words), or repeat words or phrases over and over. The parent may report that the infant or toddler spends hours in repetitive activity and demonstrates bizarre motor and stereotypic behaviors. The infant may resist cuddling, lack eye contact, be indifferent to touch or affection, and have little change in facial expression. Toddlers may display hyperactivity, aggression, temper tantrums, or self-injury behaviors, such as head banging or hand biting. The history may also reveal hypersensitivity to touch or hyposensitivity to pain. Assess the child's functional status, including behavior, nutrition, sleep, speech and language, education needs, and developmental or neurologic limitations. Assist with screening, using an approved autism screening tool such as the Modified Checklist for Autism in Toddlers-Revised (M-CHAT-R), which is recommended for administration at 18 months of age, and then again at 24 to 30 months of age (refer to for a link to the M-CHAT-R). Additional screening tools include the Social Communication Questionnaire (SCQ) and the Pervasive Developmental Disorders Screening Test-II (PDDST-II). Perform a thorough physical examination. Observe the infant or toddler for lack of eye contact, failure to look at objects pointed to by the examiner, failure to point to himself or herself, failure to let his or her needs be known, perseverative play activities, and unusual behavior such as hand flapping or spinning. Measure growth parameters, in particular noting head circumference (macrocephaly or microcephaly may be associated with ASD). Note the presence of large, prominent, or posteriorly rotated ears. Examine the skin for hypo- or hyperpigmented lesions. Note asymmetry of nerve function or palsy, hypertonia, hypotonia, alterations in deep tendon reflexes, toe-walking, loose gait, or poor coordination. Obtain hearing screening results and ascertain that lead screening has been performed. Nursing Management When children are initially diagnosed with autism, provide parents with an extensive amount of emotional support, professional guidance, and education about the disorder while they are attempting to adjust to the diagnosis. Assess the fit between the child's developmental needs and the treatment plan. Help parents overcome barriers to obtain appropriate education, developmental, and behavioral treatment programs. Ensure that the child younger than 36 months of age receives services via the local early intervention program and children 3 years and older have an IEP in place if enrolled in the public school system. Stress the importance of rigid, unchanging routines, as children with ASD often act out when their routine changes (which is likely to occur if the child must be hospitalized for another condition). Many special schools exist for children with significant developmental disorders, though some are extremely expensive. Assess the parents' need for respite care and make referrals accordingly. Provide positive feedback to parents for their perseverance in working with their child.

Hodgkin disease,

malignant B lymphocytes grow in the lymph tissue, usually starting in one general area of lymph nodes. The presence of Reed-Sternberg cells (giant transformed B lymphocytes with one or two nuclei) differentiates Hodgkin disease from other lymphomas. As the cells multiply, the lymph nodes enlarge, compressing nearby structures, destroying normal cells, and invading other tissues. The cause of Hodgkin disease is still being researched, but there appears to be a link to Epstein-Barr virus infection (Hackney, Welch, & Schwartz, 2017). Hodgkin disease is rare in children younger than 5 years of age and is most common in adolescents and young adults; in preadolescents, it is more common in boys than girls (Hackney et al., 2017). In addition to the traditional staging (I through IV, depending on the amount of spread of the cancer; Table 24.8), Hodgkin is also classified as A (asymptomatic) or B (presence of symptoms of fever, night sweats, or weight loss of 10% or more). Prognosis depends on the stage of the disease, tumor bulk, and A or B classification (disease classified as A generally carries a better prognosis). Overall, children with Hodgkin disease have a 5- to 10-year survival rate of over 90% (Craddock et al., 2018). Complications of Hodgkin disease include liver failure and secondary cancer such as acute nonlymphocytic leukemia and NHL. Chemotherapy, usually with a combination of drugs, is the treatment of choice for children with Hodgkin disease. Radiation therapy may also be necessary. In the child with disease that does not go into remission or in the child who experiences relapse, HSCT may be an option. Nursing Assessment Explore the health history for common signs and symptoms, which may include recent weight loss, fever, drenching night sweats, anorexia, malaise, fatigue, or pruritus. Elicit the health history, determining risk factors such as prior Epstein-Barr virus infection, family history of Hodgkin disease, genetic immune disorder, or HIV infection. Evaluate respiratory status, as the presence of a mediastinal mass may compromise respiration. Palpate for enlarged lymph nodes; they may feel rubbery and tend to occur in clusters (most common sites are cervical and supraclavicular) (Fig. 24.16). Palpate the abdomen for hepatomegaly or splenomegaly, which may be present with advanced disease. The chest radiograph may reveal a mediastinal mass. The CBC may be normal or reflect anemia. Tissue sampling will reveal Reed-Sternberg cells.

Shock

may be defined as an inability for blood flow and oxygen delivery to meet the metabolic demands of tissue (van der Jagt, 2017). If shock is left untreated, cardiopulmonary arrest will result. Shock, which may be classified as compensated or decompensated, is due to a variety of clinical problems. Compensated shock occurs when poor perfusion exists without a decrease in BP. In decompensated shock, inadequate perfusion is accompanied by a drop in BP. Unchecked decompensated shock leads to cardiac arrest and death. The principles of PALS stress the early evaluation and management of children in compensated shock with the goal of preventing decompensated shock (de Caen et al., 2015). Once the child in shock is hypotensive, organ perfusion is dramatically impaired and a dire clinical scenario ensues. Pathophysiology Shock is the result of dramatic respiratory or hemodynamic compromise. Impaired CO, impaired systemic vascular resistance (SVR), or a combination of both causes shock. CO is equal to HR times ventricular stroke volume (SV) (CO = HR × SV). SV is how much blood is ejected from the heart with each beat. SV is related to left ventricular filling pressure, the impedance to ventricular filling, and myocardial contractility. Left ventricular filling pressure is also known as preload, and the impedance to ventricular filling is commonly called afterload. Young children and infants have relatively small SVs compared to older children and adults. Therefore, infants and young children differ from their adult counterparts in that their CO depends on their heart rate, not their SV. Clinically, in cases of circulatory compromise and compensated shock in infants and children, the heart rate is increased. The exception to this is a paradoxical phenomenon in neonates, who may have bradycardia rather than tachycardia. SVR or afterload is the impediment to the heart's ventricular ejection. Increased SVR will result in a decrease in blood flow unless the ventricular pressure increases. Increased vascular resistance is a common problem in shock. In children who have shock-related increased SVR, CO will fall unless the ventricle can compensate by increasing pressure. In cardiac insufficiency, the child's heart will have impaired ability to compensate for the increased afterload. Altered microcirculatory status is common in all types of shock. Compensatory mechanisms are activated in response to decreased blood flow. Sympathetic nervous system response results in marked contraction of larger-vessel sphincters and arterioles. This compression results in dramatically impaired capillary blood flow. Blood is redirected away from less important body systems, such as the skin and the kidneys, to the vital organs (the heart and brain). During compensated shock, the body can maintain some level of blood flow to the vital organs. Peripheral vasoconstriction, the body's compensatory response to diminished blood flow, often results in the child's ability to maintain a normal or near-normal BP. As shock continues, capillary beds become obstructed by cellular debris, and platelets and white blood cells aggregate. Endothelial damage occurs as a result of capillary congestion. Poor blood flow to the capillaries results in anaerobic metabolism. Lactic acid accumulates, and this can lead to acidosis. In addition, children with septic shock sustain marked endothelial damage as a result of exposure to bacterial toxins. The cumulative effect of capillary obstruction and dramatically impaired blood flow is tissue ischemia. As tissue ischemia progresses, the child will show signs of altered perfusion to vital organs. For example, as blood flow to the brain is diminished, the child will demonstrate an altered level of consciousness. Altered blood flow to the kidneys will result in decreased urine output or absence of urine output (oliguria). Commonly, the heart rate will increase in the early stages of shock, but as the heart becomes compromised as a result of poor perfusion, the child will become bradycardic. The child will demonstrate an increased respiratory rate in the initial phase of shock. Tachypnea is seen in septic shock as well. In fact, the child may demonstrate marked hyperventilation in an effort to blow off carbon dioxide in response to the acidosis that is associated with septic shock. Types of Shock The most common types of shock are hypovolemic, septic, cardiogenic, and distributive. Hypovolemic shock, the most common type of shock in children, occurs when systemic perfusion decreases as a result of inadequate vascular volume (Peterson & Lee, 2019). Children commonly have hypovolemic shock that occurs in association with fluid losses. For example, hypovolemic shock may occur with gastroenteritis that results in vomiting and diarrhea, medications such as diuretics, and heat stroke. Other causes of hypovolemia in children include blood loss, such as from a major injury, and third spacing of fluid, such as with burns. Septic shock is related to a systemic inflammatory response in which there may be increased CO with a low SVR, known as warm shock. More commonly in children, septic shock results in a decrease in CO with an increase in SVR, known as cold shock. Cardiogenic shock results from an ineffective pump, the heart, with a resultant decrease in SV. Children with structural heart disease and resultant arrhythmia are at risk for cardiogenic shock (Peterson & Lee, 2019). Distributive shock is the result of a loss in the SVR. A relative hypovolemia occurs, most often with neurogenic injury-related shock and anaphylaxis. In relative hypovolemia, the vascular compartment expands due to systemic vasodilation. This results in a relatively larger vasculature requiring more fluid to maintain CO despite no actual loss of fluid. Finally, toxic drug ingestions may also lead to shock. Health History In shock, the health history is based on the child's presentation. Children with shock are critically ill and require emergent intervention. Therefore, the history is obtained as life-saving interventions are provided. Determine when the child first became ill and treatments that have been given thus far. Inquire about sources of volume loss, such as: Vomiting Diarrhea Decreased oral intake Blood loss Ask when the child last urinated. Investigate for other related symptoms such as behavioral changes or lethargy. Has the child had a fever or rash, complained of headache, or been exposed to anyone with similar symptoms? Inquire about day care attendance and whether the family has recently traveled outside of the country. Determine if the child has a history of a congenital heart defect or other heart condition or if the child has severe allergies. Ask the parent about accidental ingestion of medications or other substances and, for the older child or adolescent, about the possibility of illicit substance use. Physical Examination The key to successful shock management is early recognition of the signs and symptoms. Obtain vital signs, noting any alterations. Measure BP, although this is not a reliable method of evaluating for shock in children. Children tend to maintain a normal or slightly less than normal BP in compensated shock while sacrificing tissue perfusion until the child suffers a cardiopulmonary arrest. Therefore, other components of the circulatory evaluation will be more valuable when assessing a child. As with any emergency, determine the presence of a central pulse; if not present begin compressions. Evaluate the airway; is it patent? Then determine if the child is breathing. The child in shock will often demonstrate signs of respiratory distress, such as grunting, gasping, nasal flaring, tachypnea, and increased work of breathing. Auscultate breath sounds to determine the adequacy of air entry and airflow. If the child shows signs of respiratory distress, manage the airway and breathing problem first, as discussed earlier in the chapter. Assess the skin color. Palpate the skin temperature and determine quality of pulses. Except in special cases, such as distributive shock, the child in shock will generally have darker and cooler extremities with delayed capillary refill. Note the line of demarcation if present. This refers to the point on the distal extremity where cool temperature begins (the proximal portion of the extremity may continue to be warm). In distributive shock, the initial assessment will reveal full and bounding pulses and warm, erythemic skin. Evaluate the pulse quality. Distal pulses will likely be weaker than central pulses. Evaluate the child's hydration state and check skin turgor. Decreased elasticity is associated with hypovolemic states, though this is usually a late sign. Observe the child's face; in compensated shock the child may be awake but obtunded and demonstrate signs of distress. The child in decompensated shock may have his or her eyes closed and may be responsive only to voice or other stimulation. Evaluate pupillary responses. Determine urinary output, which will be decreased in the child with shock. After having evaluated and provided initial life-saving management for airway, breathing, and circulation, evaluate the child's entire body for other disabilities. Injuries warrant vigilant evaluation for ongoing blood loss, although they may also produce internal blood loss (e.g., a femur fracture). Look for signs of malformation, swelling, redness, or pain of the extremities, which may suggest internal blood loss. Also inspect for any open wounds and active sites of bleeding. Children with abdominal injuries also may lose copious amounts of blood internally. Inspect the abdomen for redness, skin discoloration, or distention. Auscultate for bowel sounds in all four quadrants. Laboratory and Diagnostic Testing As the child is being resuscitated, laboratory tests and radiographs will be ordered and obtained. However, no diagnostic test should replace the priority of respiratory support, vascular access, and fluid administration. Laboratory results will guide ongoing management. Common laboratory and diagnostic tests used for children with shock include: Blood glucose levels: usually performed at the bedside using a glucose meter to obtain a rapid result Electrolytes: to evaluate for electrolyte abnormalities CBC with differential: to assess for viral or bacterial infection (septic shock) and to evaluate for anemia and platelet abnormalities Blood culture: to evaluate for sepsis; preliminary results will not be available for 1 to 2 days C-reactive protein: to evaluate for infection Arterial blood gases: to assess oxygen and carbon dioxide levels and to provide information about acid-base balance Toxicology panel (if ingestion is suspected) Lumbar puncture: to evaluate the cerebrospinal fluid for meningitis Urinalysis: to evaluate for glucose, ketones, and protein; concentration (specific gravity) is increased in dehydration states Urine culture: to evaluate for urinary tract or kidney infection Radiographs: to evaluate heart size; to evaluate the lungs for pneumonia or pulmonary edema (present with cardiogenic shock) Managing the Child's ABCs Always evaluate and manage the airway and breathing and check for pulses. Initiate CPR if the child is pulseless. All children who have signs and symptoms of shock should receive 100% oxygen via mask. If the child has poor respiratory effort or is apneic, administer 100% oxygen via BVM or ET tube (refer to the section on respiratory arrest for more specific information about management of airway and breathing). As part of ongoing monitoring, institute cardiac and apnea monitoring and assess oxygen saturation levels via pulse oximetry. Obtaining Vascular Access Once the airway and breathing are addressed, nursing management of shock focuses on obtaining vascular access and restoring fluid volume. Children with signs of shock should receive generous amounts of isotonic IV fluids rapidly. However, obtaining vascular access in critically ill children can be challenging. Vascular access must be obtained using the quickest route possible in children whose condition is markedly deteriorated, such as those in decompensated shock. Various forms of vascular access available for the management of the critically ill child include: Peripheral IV route: a large-bore catheter is used to give large amounts of fluid. This route may not be feasible in children with significant vascular compromise. Central IV route: central lines can be inserted into the jugular vein and threaded into the superior vena cava. The femoral route is best for obtaining central venous access while CPR is in progress because the insertion procedure will not interfere with life-saving interventions involving the airway and cardiac compressions. The subclavian vein, located under the clavicle, is an alternative route for central access. Saphenous vein: the saphenous vein (found in the ankle) is an alternative route for venous access that is obtained using a surgical incision. Intraosseous access: intraosseous access, obtained by cannulating the bone marrow, is recommended in cases of decompensated shock or cardiac arrest if IV access cannot be attained rapidly. The preferred site is the anterior tibia. Special intraosseous needles are used (generally a 15-gauge needle for older children, 18-gauge for younger children). The needle is inserted using a firm twisting motion slightly away from the growth plate. Any medications or fluids that can be administered using an IV site can be given using this route. Alternative sites include the femur, the iliac crest, the sternum, and the distal tibia. Restoring Fluid Volume Administer IV isotonic fluids, such as Ringer's lactate or normal saline (the isotonic fluids of choice) rapidly. Administer 20 mL/kg of the prescribed fluid as a bolus, infusing the fluid as rapidly as possible. In general, a large-bore syringe, such as a 35- to 60-mL syringe attached to a three-way stopcock, is the preferred method for rapid fluid delivery in children. Infusing the fluid via gravity is too slow. The fluid bolus may be repeated up to two times (for a total of three times) if required. Children in septic shock will often require larger volumes of fluid as a result of the increased capillary permeability. Children in shock due to trauma will usually receive a colloid, such as blood, when there is an inadequate response to crystalloid isotonic fluid. After each fluid bolus, reassess the child for signs of positive response to the fluid administration. Insert an indwelling urinary catheter to allow for accurate and frequent measurement of urine output. Indicators of improvement include: Improved cardiovascular status: the central and peripheral pulses are stronger. The line of demarcation of extremity coolness is diminishing and capillary refill is improved (time is decreased). BP is improved. Improved mental status: the child is more alert. For example, the child's eyes are open and watching personnel. If the child is younger, he or she may be pulling at the IV line. Improved urine output: this may not be noted initially but should be noted over the next few hours; the goal is 1 to 2 mL/kg/hr. The process of fluid resuscitation involves giving the fluid, assessing and reassessing the child, and documenting findings. Children in shock may require as much as 100 to 200 mL/kg of resuscitative fluid during the initial hours of shock management. Most children in shock need and can tolerate this large volume of fluid. Continued reassessment will determine if the child is beginning to experience fluid overload in the form of pulmonary edema (this is rare but may occur in children with pre-existing cardiac conditions or severe chronic pulmonary disease) (AHA, 2016; van der Jagt, 2017). Administering Medications In some circumstances, such as septic shock or distributive shock, fluid alone does not adequately improve the child's status and adjunctive medications may be ordered. Vasoactive medications are used either alone or in combination to improve CO and to increase or decrease SVR. The selection of medications is dictated by the child's cardiac and vascular status. For example, dobutamine is a medication with significant β-adrenergic effects and thus can improve cardiac contractility. Epinephrine, which affects the heart muscle, is also a powerful vasoconstrictor. Dopamine affects the heart at lower doses but increasingly affects the vasculature with increased doses. These medications may be given as a loading dose, followed by a continuous infusion. When vasoactive drugs are administered, monitor for improvement in heart rate, BP, perfusion, and urine output. Refer to Drug Guide 29.1 for additional information

Secondary immunodeficiency

may occur as a result of chronic illness, malignancy, use of immunosuppressive (lowering the immune response) medication, malnutrition or protein-losing state, prematurity, or HIV infection. This discussion will focus on HIV infection.

Learning Disabilities

About 10% of children and adolescents have learning disabilities and in children with chronic illness, learning disabilities are two times more common than in the general population (von Hahn, 2019a). The essential characteristic of learning disability is an innate cognitive difficulty resulting in lower academic achievement than would be expected for the child's intellectual potential (von Hahn, 2019a). Learning disabilities become evident when a child of average intelligence has difficulty mastering basic academic skills. Learning disabilities can affect the child's ability to listen, speak, read, write, and perform mathematics. For example: Children with dyslexia have difficulty with reading, writing, and spelling. Children with dyscalculia have problems with mathematics and computation. Children with dyspraxia have problems with manual dexterity and coordination. Children with dysgraphia have difficulty producing the written word (composition, spelling, and writing). Therapeutic Management Therapeutic management may involve remedial or compensatory approaches or may use interventions directed toward social-emotional problems. The focus of the remedial approach is to improve specific skills. The compensatory approach helps the child to compensate for the disability, rather than attempting to directly correct it. Social-emotional problems may result from frustration or low self-esteem related to capabilities. These may respond to supportive interventions and improvement in coping. Nursing Assessment Elicit the health history, noting risk factors such as a family history of learning disability, problems during pregnancy or birth, prenatal alcohol or drug use, low birthweight, premature or prolonged labor, head injury, poor nutritional status or failure to thrive, or lead poisoning. Obtain detailed information about the educational difficulties the child is experiencing (e.g., he or she seems to do fine in math but always reverses letters when reading). A thorough physical examination may reveal clues to comorbid (existing simultaneously) conditions. Ensure that the child has undergone a comprehensive education evaluation with assessment testing to diagnose the specific learning disability. Testing may be performed by a school, educational, developmental, or clinical psychologist; occupational therapist; speech and language therapist; or other developmental specialist, depending on the areas of learning with which the child is experiencing difficulty. Nursing Management Ensure that families are aware of their child's rights under the Individuals with Disabilities Act (IDEA), which was reapproved in 2004 (108th Congress, 2004). IDEA offers protection from discrimination and the right to assistance in the school or workplace. Each child will need an individualized education plan (IEP) that reflects his or her particular needs, which then must be provided for through the school system. Offer encouragement and support to families as they advocate for their child. Follow up at subsequent healthcare visits to determine that the child is receiving the services he or she needs to optimize his or her potential for success. Refer families for additional resources through the National Center for Learning Disabilities, Learning Disabilities Online, or the Center for Learning Differences (links to these resources are provided on ).

Casting

Application of plaster or fiberglass material to form a rigid apparatus to immobilize a body part Fracture reduction, dislocations, correction of deformities Assess frequently for neurovascular compromise, skin impairment at cast edges. Protect cast from moisture. Teach family how to care for cast at home. After the cast or splint is applied, drying time will vary based on the type of material used. Splints and fiberglass casts usually take only a few minutes to dry and will cause a very warm feeling inside the cast, so warn the child that it will begin to feel very warm. Plaster requires 24 to 48 hours to dry. Take care not to cause depressions in the plaster cast while drying, as those may cause skin pressure and breakdown. Instruct the child and family to keep the cast still, positioning it with pillows as needed. Caring for the Child With a Cast Perform frequent neurovascular checks of the casted extremity to identify signs of compromise early. These signs include: Increased pain Increased edema Pale or blue color Skin coolness Numbness or tingling Prolonged capillary refill Decreased pulse strength (or absence of pulse) Notify the physician or nurse practitioner of changes in neurovascular status or odor or drainage from the cast. Fiberglass casts usually have a soft fabric edge, so they usually do not cause skin rubbing at the edges of the cast. On the other hand, plaster casts require special treatment of the cast edge to prevent skin rubbing. This may be accomplished through a technique called petaling: cut rounded-edge strips of moleskin or another soft material with an adhesive backing and apply them to the edge of cast, as shown in Figure 22.9. Home Cast Care For the first 48 hours, elevate the extremity above the level of the heart and apply cold therapy for 20 to 30 minutes, then off 1 to 2 hours, and repeat. Take your prescribed pain medication for at least the first 48 hours. Assess for swelling, and have the child wiggle the fingers or toes frequently (hourly while awake). For itching inside the cast: Never insert anything into the cast for the purposes of scratching. Blow cool air in from a hair dryer set on the lowest setting or tap lightly on the cast. Do not use lotions or powders. Do not pull padding out from the inside of the cast. Protect the cast from wetness. Apply a plastic bag around cast and tape securely for bathing or showering. Continue to avoid placing the cast directly in water (unless it is Gore-Tex lined). Waterproof cast covers are available through medical supply stores (still remain cautious about submerging cast with water). Cover it when your child eats or drinks. If a cast become soiled it can be wiped clean with a slightly damp clean cloth. If the cast gets wet, dry it with a blow dryer on the cold setting (if warm setting is used the child could get burned). Use of a vacuum cleaner with a hose attachment to pull air through may speed drying; be careful to avoid skin. If the child has a large cast, change position every 2 hours during the day and while sleeping change position as often as possible. Check the skin for irritation. Press the skin back around edges of the cast. Use a flashlight to look for reddened or irritated areas. Feel for blisters or sores. Call the physician or nurse practitioner if: The casted extremity is cool to the touch, pale, blue, or very swollen. The child cannot move the fingers or toes. Severe pain occurs when the child attempts to move the fingers or toes. Persistent numbness or tingling occurs. Drainage or a foul smell comes from under the cast. Severe itching occurs inside the cast. The child runs a fever greater than 101.5°F for longer than 24 hours. Skin edges are red and swollen or exhibit breakdown. Child complains of rubbing or burning under cast. The cast gets wet and does not dry or is cracked, split, or softened. TEACHING GUIDELINES 22.2 Skin Care After Cast Removal Brown, flaky skin is normal and occurs as dead skin and secretions accumulate under the cast. New skin may be tender. Soak with warm water daily. Wash with warm soapy water, avoiding excessive rubbing, which may traumatize the skin. Discourage the child from scratching the dry skin. Apply moisturizing lotion to relieve dry skin. Encourage activity to regain strength and motion of extremity.

Syndrome of Inappropriate Antidiuretic Hormone

"Low and wet" Decreased urination Hyponatremia Serum osmolality <280 mOsm/kg Urine specific gravity >1.030 Increased urine osmolality Fluid retention, weight gain, and hypertension

POISONING

Emergency care of the pediatric poisoning victim consists of rapid nursing assessment and prompt management. Nursing Assessment Nursing assessment of the poisoning victim focuses on a thorough health history, followed by physical examination and laboratory and diagnostic testing. Health History Obtain the health history from the parents or caregiver or, in the case of an older child or teenager, from the child. Inquire about the approximate time of poisoning and the nature of the toxin. Was the toxin ingested, inhaled, or applied to the skin? In the case of pill ingestion, does the caregiver have the medication bottle? Did the child experience nausea, vomiting, anorexia, abdominal pain, or neurologic changes such as disorientation, slurred speech, or altered gait? Determine the progression of the symptoms. Did the parent or caregiver call the National Poison Control Center Hotline? Has any treatment been given? In the case of older children and teens, inquire about any history of depression or threatened suicide. Physical Examination Ingestion of medications or chemicals may result in a wide variety of clinical manifestations. Perform a thorough physical examination, noting alterations that may occur with particular ingestions, such as: Hyper- or hypotension Hyper- or hypothermia Respiratory depression or hyperventilation Miosis (pupillary contraction) or mydriasis (pupillary dilatation) Pay particular attention to the child's mental status, skin moisture and color, and bowel sounds (Velez, Shepherd, & Goto, 2019). Laboratory and Diagnostic Testing The suspected poison may direct the laboratory and diagnostic testing. A variety of blood tests may be performed: Chemistry panel: to detect hypoglycemia or metabolic acidosis and assess renal function ECG: to identify arrhythmias or conduction delay Liver function tests: to assess for liver injury Urine and blood toxicology screens (available for a limited number of medications; may vary per institution) Specific drug levels if the substance ingested is known or highly suspected Nursing Management When poisoning occurs, give priority to the child's ABCs. Treat alterations as discussed earlier in this chapter. Monitor vital signs frequently and provide supportive care. Few specific antidotes are available for medications or other toxins. Activated charcoal may be administered to bind with the chemical substance in the bowel. Alternatively, whole bowel irrigation with polyethylene glycol electrolyte solutions may be necessary. Occasionally, dialysis is required to lower the level of toxin in the bloodstream. The intervention is based on the source of the ingestion. For example, activated charcoal is an effective method for preventing the absorption of many medications but is not effective in the case of an iron overdose. If opiate or other narcotic ingestion is suspected, administer naloxone to reverse the respiratory depression or altered level of consciousness. Treatment of seizures and alterations in thermoregulation may also be needed. Specific treatment of the poisoning will be determined when the toxin is identified and poison control is queried. Maintain ongoing assessment of the poisoned child because many toxins exhibit very late effects.

Respiratory Arrest

Respiratory emergencies may lead to respiratory failure and eventual cardiopulmonary arrest in children. Infants and young children are at greater risk for respiratory emergencies than adolescents and adults because they have smaller airways and underdeveloped immune systems, resulting in a diminished ability to combat serious respiratory illnesses. Young children often lack coordination, making them susceptible to choking on foods and small objects, which may also lead to cardiopulmonary arrest. In addition, sudden infant death syndrome (SIDS) is a leading cause of cardiopulmonary arrest in young infants and thus is the leading cause of postneonatal mortality in the United States (Graham & Peoples, 2019). For these reasons, nurses must be skilled at recognizing the signs of pediatric respiratory distress so they can prevent progression to cardiopulmonary arrest. Table 29.2 lists some of the more common causes of pediatric respiratory arrest. Nursing Assessment If the child has severe respiratory compromise, obtain a brief history while simultaneously providing respiratory interventions. To obtain the history, use the following questions as a guide: When did the symptoms begin and when do they occur? Did the symptoms have a sudden onset (as with a foreign- body aspiration)? How have the symptoms progressed? Is the cough continual, intermittent, or worse at night or with exercise? Has there been any stridor? (Stridor is heard upon inhalation and may be associated with swelling of the trachea [as with croup] or with a foreign body in the upper airway.) Is there wheezing? If so, is the wheezing on inspiration or on expiration, or both? What makes the symptoms better and what makes them worse? Does drinking from a bottle induce the symptoms (as with gastroesophageal reflux-induced aspiration)? Is the child taking any medication for the symptoms? Does the child or do any members of the immediate family have a history of chronic respiratory disease, such as asthma? Are the child's immunizations up to date? Was the child born prematurely? If so, did the child require mechanical ventilation? For how long? Were there any respiratory problems during the first few days of life? When did the child last eat? (This question is important because a recent meal will increase the child's risk of aspiration in the event of a respiratory arrest. In addition, the presence of food in the stomach will increase the risk of aspiration during tracheal intubation.)Physical Examination In an emergency, physical examination is often limited to inspection, observation, and auscultation. First, quickly survey the respiratory status. Determine if the child is breathing. INSPECTION AND OBSERVATION Establish if the airway is patent, maintainable, or unable to be maintained. The child with a patent airway is breathing without signs of obstruction. The maintainable airway remains patent independently by the child or with interventions such as a towel roll under an infant's neck or the insertion of a nasal trumpet. The airway that cannot be maintained does not remain patent unless a more aggressive intervention, such as the insertion of a tracheal tube, is performed. Look at the child's posture. Is the child sitting up, leaning forward, and drooling, as with epiglottitis? Observe the child's face: Does he or she appear anxious or relaxed? Children in respiratory distress often appear anxious. Look at the nose and mouth. Are the nares patent? Is there noticeable nasal congestion or mucus coming from the nose? Note nasal flaring or mouth breathing. Observe for head bobbing. Listen for audible expiratory grunting or inspiratory stridor. Note the child's color. Does the child appear pale, mottled, dusky, or cyanotic? Children may appear mottled in response to poor oxygenation, hypothermia, or stress. Children with severe respiratory compromise may appear dusky. Look for cyanosis around the mouth or on the trunk. Cyanosis is a late and often ominous sign of respiratory distress. Central cyanosis is more likely to be associated with respiratory or cardiac compromise. In contrast, peripheral cyanosis is more likely to be associated with circulatory alteration. Evaluate the pattern and quality of respiration, noting the respiratory rate. Tachypnea (increased respiratory rate) is often noted in children in respiratory distress. However, seriously ill children grunt and may have normal or subnormal respiratory rates. Hypoventilation, a decrease in the depth and rate of respirations, is noted in very ill children or in children who have central respiratory depression secondary to narcotics. If the child is a young infant (younger than 2 months) or premature, periodic breathing may occur. Periodic breathing is regular breathing with occasional short pauses (brief periods of apnea). After the apneic pause, the infant will breathe rapidly (up to 60 breaths per minute) for a short period and then will resume a normal respiratory rate. In general, the infant who has periodic breathing looks pink and has a normal heart rate (HR). Observe for the use of accessory muscles in the neck or retractions in the chest, determining the extent and severity of the retractions. AUSCULTATION Auscultate the lungs with the diaphragm of the stethoscope. Breath sounds over the tracheal region are higher pitched and are described as vesicular, while breath sounds over the peripheral lung fields tend to be lower pitched, known as bronchial. Instruct the child to take deep breaths with the mouth open. To encourage the young child to exhale strongly, instruct him or her to "blow out" the penlight (as with a candle) or to blow on a tissue. Encourage the child not to breathe more rapidly than normal (to prevent hyperventilation [increased depth and rate of respirations]) and to avoid making any noises with the mouth. Auscultate the child's chest systematically. Listen in all anterior, axillary, and posterior regions, comparing the left to the right sides. Note any decreased or absent breath sounds, which may be the result of bronchial obstruction (as with mucous infection) or air trapping (as in children with asthma). Unilateral absent breath sounds are associated with foreign-body aspiration and pneumothorax. Note the presence and location of adventitious breath sounds such as crackles, wheezes, or rhonchi. Document the presence of a pleural friction rub (a low-pitched, grating sound), a sound resulting from inflammation of the pleura. PALPATION Palpate the chest for any abnormalities. In the older, less severely ill, and cooperative child, assess for tactile fremitus. Using the palm of the hand, palpate over the lung regions in the same manner as for auscultation and percussion while the child says "ninety-nine." Increased vibrations elicited during this maneuver are associated with consolidating conditions, such as pneumonia. PERCUSSION Percuss the interspaces of the chest between the ribs in the same systematic fashion as with auscultation. Normally, percussion over an air-filled lung reveals resonant sounds. Note the presence of hyperresonance, which may indicate an acute problem such as a pneumothorax or a chronic disease such as asthma. In contrast, percussion sounds will be dull over a lobe of the lung that is consolidated with fluid, infectious organisms, and blood cells, as in the case of pneumonia. Laboratory and Diagnostic Testing Use continuous pulse oximetry if respiratory status is a concern. Note and report oxygen saturation levels below 95%. (See Chapter 18 for additional information about use of the pulse oximeter.) Additional tests may reveal: Arterial or capillary blood gases: hypoxemia, hypercarbia, altered pH Chest radiograph: alterations in normal anatomy or lung expansion, or evidence of pneumonia, tumor, or foreign body Metal detector: evidence of coins (Gilger & Jain, 2019) Nursing Management The basic principle of pediatric emergency care and PALS is prevention of cardiopulmonary arrest (van der Jagt, 2017). Therefore, the nurse must rapidly assess and appropriately manage children who have signs of respiratory distress. Nursing management of the child in respiratory distress involves maintaining a patent airway, providing supplemental oxygen, monitoring for changes in status, and in some cases assisting ventilation. In addition to providing these life-saving measures and monitoring the child's progress, offer support and education to the child and family. Maintaining a Patent Airway When a child exhibits signs of respiratory distress, make a quick decision about whether it will be safe to allow the child to stay with the parent or whether the child must be placed on the examination table or bed. For example, in the case of croup, the child will often breathe more comfortably and experience less stridor while in the comfort of the parent's lap. Many children in respiratory distress often are most comfortable sitting upright, as this position helps to decrease the work of breathing by allowing appropriate diaphragmatic movement. In contrast, a child with a decreasing level of consciousness may need to be placed in the supine position to facilitate positioning of the airway. The infant may benefit from a small towel folded under the shoulders or neck (Fig. 29.3). Avoid neck flexion or hyperextension, which may completely occlude the infant's airway. In children older than age 1 year, the optimal method for opening the airway is to hyperextend the neck, as recommended by the AHA (Atkins et al., 2015). If a cervical spine injury is not suspected, use the head tilt-chin lift technique to open the airway. If the child has suffered head or neck trauma and cervical spine instability is a concern, use the jaw-thrust maneuver by placing three fingers under the child's lower jaw and lifting the jaw upward and outward (Fig. 29.4). In either case, never place the hand under the neck to open the airway. Often the nurse encounters an acutely ill child who cannot maintain an airway independently but may be able to do so with some assistance. For example, sometimes simply opening the airway and moving the tongue away from the tracheal opening is all that is required to regain airway patency. In certain conditions, a nasopharyngeal or oropharyngeal airway may be necessary for airway maintenance. Comparison Chart 29.1 provides additional information about these types of airways. Assisting Ventilation Using a Bag-Valve-Mask The child in respiratory distress may ventilate poorly, hypoventilate, or tire and become apneic. In this case, the child may require assistance with ventilation through bag-valve-mask (BVM) ventilation. Refer to Table 29.3 for additional information on BVM ventilation, tracheal intubation (the insertion of a tube into the trachea for the purposes of ventilation), the use of an anesthesia bag or flow-inflating ventilation system, and laryngeal mask airways. BVM ventilation is used in the management of children who cannot ventilate or oxygenate effectively on their own. This technique is a more efficient way of ensuring ventilation than using only supplemental oxygen. In addition, resuscitating a child in this manner is superior to mouth-to-mouth resuscitation as it provides higher oxygen concentrations and protects the nurse from exposure to oral secretions (Atkins et al., 2015). However, this technique requires proper training and practice. The proper procedure involves appropriate opening of the airway followed by providing breaths with the BVM. Ventilation with the BVM may be performed with either one or two rescuers. First, choose an appropriate- sized bag and a corresponding face mask that fits the infant or child. Self-inflating bags are usually available in neonatal, infant, child, and adult sizes. Corresponding masks are available. Choose a face mask that properly fits the child's face and that provides a seal over the nose and mouth and excludes the eyes, thus preventing any pressure on the eyes (Fig. 29.5). Connect the BVM via the tubing to the oxygen source and turn on the oxygen. When resuscitating infants and children, set the flow rate at approximately 10 L/min. For an adolescent who is adult sized, set the flow rate at 15 L/min or higher to compensate for the larger-volume bag. Check to make sure that the oxygen is flowing through the tubing to the bag. Self-inflating bags do not provide free-flow oxygen out of the face mask; manual pumping of the bag is necessary. However, the bags have a corrugated plastic tail that allows oxygen to freely flow. Therefore, check over the tail for oxygen flow through the bag. After opening the airway appropriately (see above), place the mask over the child's face. When one rescuer is providing ventilation (commonly referred to as "bagging"), the person must provide a seal with the mask over the child's face with one hand and use the other hand to manipulate the resuscitator bag. The hand used to provide the mask seal will simultaneously maintain the airway in an open position. Generally, use the left thumb and index finger to hold the mask on the child's face. While maintaining a good seal with the mask, use upward pressure on the jaw angle while pressing downward on the mask below the child's mouth to keep the mouth open (Fig. 29.6). Take care not to put pressure on the neck with the fourth and fifth fingers.Compress the bag to deliver breaths at the amount recommended in infants and children. Initially, provide two rescue breaths and observe for a chest rise. Rescue breaths should not overinflate the lungs. Breaths should be delivered over 1 second. After the first two rescue ventilations, perform rescue breathing at a rate of one breath every 3 to 5 seconds, or about 12 to 20 breaths per minute. Delivering each breath should be a steady, one-inhalation-to-one-exhalation ratio. This means that the amount of time delivering the inspiratory ventilation is equal to the amount of time that expiration is allowed. While ventilating the infant or child, work with, not against, any spontaneous respiratory effort; in other words, if the child is breathing out, do not attempt to force air in at the same time. MONITORING EFFECTIVENESS OF VENTILATION During the resuscitation, continually reassess the child's response to the resuscitative efforts, noting: Adequacy of chest rise Absence or minimal presence of abdominal distention Improved HR and pulse oximetry readings Improved color Capillary refill less than 3 seconds with strengthening pulses If the child's status deteriorates and he or she becomes pulseless, then CPR must be started. In addition, periodically and briefly stop ventilating to evaluate for spontaneous respirations. PREVENTING COMPLICATIONS RELATED TO BAG-VALVE-MASK VENTILATION During resuscitation, healthcare personnel usually exhibit high-energy levels, a normal physiologic response that facilitates resuscitative efforts as the rescuers act quickly. However, this heightened state can lead to overzealousness while ventilating an infant or child. Healthcare providers may inadvertently ventilate the child too rapidly using too much tidal volume, leading to excessive ventilation volume and increased airway pressure. This poor technique can be detrimental to the child, causing: Reduced cardiac output (CO) (due to increased intrathoracic pressure and increased cardiac afterload) Air trapping Barotrauma (trauma caused by changes in pressure) Air leak (thus reducing the oxygen delivered to the child) Thus, nurses must be mindful of their technique during bagging, not exceeding the recommended respiratory rate or providing too much tidal volume to the child. Ventilate the child in a controlled and uniform manner, providing just enough volume to result in a chest rise. Assisting With Ventilation Using Tracheal Intubation Endotracheal intubation is needed if the infant or child does not have a maintainable airway or will require artificial ventilation for a prolonged time (see Table 29.3). Intubation of infants and children is a procedure that requires great skill and therefore should be performed by only the most qualified and experienced personnel. Children are most commonly intubated orally, rather than nasally, in acute situations. Nurses are an essential part of the intubation team, usually assisting a physician, nurse practitioner, respiratory therapist, or physician's assistant during the intubation procedure (Nursing Procedure 29.1). The nurse may set up the equipment, prepare and administer intubation medications, or assist with suctioning the oral secretions and preparing the tape to secure the endotracheal tube (ET) tube. In a child in full arrest, the nurse might be responsible for performing ongoing chest compressions while other team members manage the child's airway. SETTING UP EQUIPMENT Appropriate setup and preparation of equipment is essential (Table 29.4). The ET tube size used depends on the child's size. To calculate ET tube size, divide the child's age by 4 and add 4. The resulting number will indicate the size of the ET tube in millimeters. For example, if the child is 2 years old, the proper-sized tube would be 4.5 ([2/4] + 4 = 4.5). Always have one size smaller ready also, so have a 4.0 and a 4.5 ET tube for this child. NURSING PROCEDURE 29.1 Assisting With Endotracheal Intubation Prepare equipment and supplies. Draw up medications (for rapid sequence intubation). Turn up the volume on the cardiac monitor so that members of the team can easily hear the audible QRS indication of the child's heart rate and note any bradycardia with the procedure. Turn on the suction. Make sure that suction is working by placing your hand over the tubing before you attach the suction catheter. Continue to ventilate the child with the bag-valve-mask (BVM) and 100% oxygen as the team prepares to intubate the child. When there is no suspected cervical spine injury, in the child older than age 2 years, place a small pillow under the child's head to facilitate opening of the airway; this step is unnecessary in children younger than age 2 due to the prominence of their occiput. When assisting with the intubation, stand beside the child's head and prepare to assist with suctioning of oral secretions, providing BVM ventilation as needed, and assisting with securing the tube with tape. Before the initial intubation attempt and after each subsequent attempt to intubate, provide several inhalations of 100% oxygen via the BVM ventilation method (optimally for a few minutes). Administer premedication and medications for sedation. Administer paralyzing medication. Observe as the healthcare professional who is intubating the child follows the recommended procedure for intubation using the laryngoscope to visualize the vocal cords. ADMINISTERING MEDICATIONS Several medications are often used to facilitate intubation of children. Premedicating a child before passing an ET tube aids in the following: Reducing pain and anxiety (consistent with the concept of atraumatic care) Minimizing the effects of passing the ET tube down the airway (vagal stimulation leading to bradycardia [decrease in HR]) Preventing hypoxia Reducing intracranial pressure Preventing airway trauma and aspiration of stomach contents

Retinoblastoma

is a congenital, highly malignant tumor that arises from embryonic retinal cells. It accounts for 5% of cases of blindness in children (Craddock et al., 2018). Most children are diagnosed by age 5, and the 5-year survival rate is 90% when the tumor is confined to the retina (Craddock et al., 2018). Retinoblastoma may be hereditary or nonhereditary. Nonhereditary retinoblastoma may be associated with advanced paternal age and always presents with unilateral involvement. Hereditary retinoblastoma is inherited via the autosomal dominant mode. These cases may be unilateral or bilateral. The tumor may grow forward into the vitreous cavity of the eye or extend into the subretinal space, causing retinal detachment. The tumor may extend into the choroid, the sclera, and the optic nerve. Complications include spread to the brain and the opposite eye, as well as metastasis to lymph nodes, bone, bone marrow, and liver. Secondary tumors, most often sarcomas, may also occur in children who have been treated for retinoblastoma. Table 24.12 explains the classification of retinoblastoma. The goals of treatment are to eradicate the tumor, preserve vision, and provide a good cosmetic outcome. Retinoblastoma may be treated with radiation, chemotherapy, laser surgery, cryotherapy, or a combination of these treatments. Moderate vision may be preserved for most children without advanced disease. In advanced disease or in the case of a massive tumor with retinal detachment, enucleation (removal of the eye) is necessary. Nursing Assessment Parents are often the first to notice the "cat's eye reflex" or "whitewash glow" to the child's affected pupil. Obtain the health history, determining when other associated symptoms such as strabismus, orbital inflammation, vomiting, or headache began. Inquire about risk factors such as a family history of retinoblastoma or other cancer, or the presence of chromosomal anomalies. Assess pupils for size and reactivity to light. Note presence of leukocoria ("cat's eye reflex," a whitish appearance of the pupil) in the affected eye (Fig. 24.19). Assess the eyes for associated signs, which may include erythema, orbital inflammation, or hyphema. Diagnostic evaluation includes an ophthalmologic examination under anesthesia. CT, MRI, or ultrasound of the head and eyes will help to visualize the tumor. The infant or toddler may also undergo lumbar puncture and bone marrow aspiration to determine the presence and extent of metastasis. Nursing Management Provide routine postoperative care to the infant or toddler. If the eye is enucleated, observe the large pressure dressing on the eye socket for bleeding. Dressing changes to the socket may include sterile saline rinses and/or antibiotic ointment application. If disease occurs outside of the eye or if metastasis is present, inform the parents that chemotherapy will be necessary. Monitor for side effects of chemotherapy (see the Nursing Process Overview section). Follow-up will include eye examinations every 3 to 6 months until age 6 and then annually to check for further tumor development. If the eye is enucleated, a prosthetic eye will be fitted several weeks after removal. Teach families use of the prosthetic eye; it does not require daily removal. Provide parents with support and encouragement. Refer the family for genetic counseling. Children with a family history of retinoblastoma need ophthalmologic examination shortly after birth, then every 2 to 4 weeks, and then at increasing intervals until age 5 years (Craddock et al., 2018).

Diabetes Insipidus

"High and dry" Increased urination Hypernatremia Serum osmolality >300 mOsm/kg Urine specific gravity <1.005 Decreased urine osmolality Dehydration, thirst

Guillain-Barré syndrome (GBS)

(also called acute immune-mediated polyneuropathies) is a diverse group of syndromes with several forms. The disorder occurring most often is a one in which an immune response within the body attacks the peripheral nervous system but does not usually affect the brain or spinal cord. GBS results in inflammation and demyelinization of the peripheral nerves. Weakness and paralysis occur in a progressive fashion. Progression is usually complete in 2 to 4 weeks, followed by a stable period leading to the recovery phase, which lasts for a few weeks to months in most cases but can take years. Severity of the disorder ranges from mild weakness to total paralysis. Though not fully understood, it is believed to be an autoimmune condition that most commonly is triggered by a previous viral or bacterial infection, usually described as an upper respiratory tract infection or an acute gastroenteritis with fever. In rare cases it has occurred after the child has had an immunization or surgery. GBS is more commonly seen in adults than in children (Ryan, 2017). Therapeutic Management Treatment of GBS is symptomatic and focuses on lessening the severity and speeding recovery. Management may include plasma exchange and administration of IVIGs, especially in severe cases. The goal of treatment is to keep the body functioning until the nervous system recovers. GBS is a life-threatening condition, and some children will die during the acute phase due to respiratory failure. Most children will make a full recovery, but a few may have residual damage. Nursing Assessment Early diagnosis and prompt treatment are essential since the disorder can quickly lead to respiratory failure and death from muscle paralysis. For a full description of the assessment phase of the nursing process, refer page 903. Assessment findings pertinent to GBS are discussed below. Health History Elicit a description of the present illness and chief complaint. The clinical presentation of GBS is fairly similar in children and adults. Note onset of symptoms within a few days or weeks after the causative infection or event. Determine presence of muscle weakness and paresthesias such as numbness and tingling which have a quick onset. Classically GBS initially affects the legs and progresses in an ascending manner, but occasionally it affects the arms or face first and proceeds in a descending manner. Ask about presence of paralysis, ataxia, or sensory disturbances. Physical Examination and Laboratory and Diagnostic Tests Note decreased or absent tendon reflexes, facial weakness, difficulty swallowing, or paralysis. Cerebrospinal fluid (CSF) analysis may reveal an increased level of protein, but this may not be evident until after the first week of the illness. Electrodiagnostic studies, such as electromyelogram (EMG) and nerve conduction velocity, can assist in the diagnosis of GBS. Nursing Management Nursing management is supportive. In severe cases the child may require intensive nursing care along with mechanical ventilation. Observe the child closely for the extent of paralysis and monitor for respiratory involvement. Nursing care focuses on the same concerns as in any child with extreme immobility or paralysis. Prevention of complications associated with immobility is a central concern and involves maintaining skin integrity, preventing respiratory complications and contractures, maintaining adequate nutrition, and managing pain. Turn and/or reposition the child every 2 hours, perform range-of-motion exercises, assess the skin for redness or breakdown, keep the skin clean and dry, encourage fluid intake to maintain hydration status, and encourage coughing and deep breathing every 2 hours and as needed. Provide enteral feeding or parenteral nutrition if swallowing becomes impaired. Perform physical therapy exercises as prescribed to help prevent complications and promote motor skill recovery. Provide support and education to the parent and child. Support the family as the rapid onset and long recovery can be difficult, causing strain on the family and its finances. If residual disability occurs, assist the family to adjust and to care for their child. Nursing Assessment Note history of fatigue and weakness; difficulty chewing, swallowing, or holding up the head; or pain with muscle fatigue. In the verbal child, note complaints of double vision. Observe the child for ptosis (droopy eyelids) or altered eye movements from partial paralysis. Note increased work of breathing. Laboratory testing may involve the edrophonium (Tensilon) test, in which a short-acting cholinesterase inhibitor is used. Acetylcholine receptor (AchR) antibodies may be present in elevated quantities in the serum. Nursing Management The goals of nursing management include prevention of respiratory problems and providing adequate nutrition. Administer anticholinergic or other medications as ordered, teaching children and families about the use of these drugs. Anticholinergic drugs should be given 30 to 45 minutes before meals, on time and exactly as ordered. Encourage families to seek prompt medical treatment for suspected infections. Encourage appropriate stress management and avoidance of extreme temperatures. Teach families that physical activities should be performed during times of peak energy; rest periods are needed for energy conservation. Teach families to call their neurologist immediately if signs and symptoms of myasthenic crisis or cholinergic crisis, which results from overmedication with anticholinergic medications, appear. Myasthenic crisis and cholinergic crisis have a similar presentation: rapidly increasing muscle weakness with resultant respiratory distress. Encourage children to wear a medical alert bracelet.

Addison disease

(deficiency in the adrenal steroids, glucocorticoids [cortisol], and mineralocorticoids [aldosterone]) It results from damage or destruction of the adrenal glands caused by infections such as tuberculosis, fungal infections, or HIV-related infections; hemorrhage or surgical removal of both glands; or dysfunction of the hypothalamus or pituitary gland. Generally, the etiology in children is an autoimmune process that is familial or sporadica Hyponatremia Hyperkalemia Water loss, dehydration, muscular weakness, fatigue, weight loss, anorexia, syncope, nausea, vomiting, and diarrhea Hypoglycemia Hypotension Hyperpigmentation of skin Adrenal crisis, also referred to as addisonian crisis, can occur (refer to section on CAH for more information) Similar to that of congenital adrenal insufficiency.

Hypoparathyroidism

(deficiency of PTH)Most common is accidental removal or destruction of the parathyroid gland during thyroidectomy or radial neck dissection; may also be congenital (result of aplasia or hypoplasia of the parathyroid gland) Hypocalcemia Hyperphosphatemia Hyperexcitability of neuromuscular function, uncontrolled spasms, and hypocalcemic tetany (general muscular hypertonia); positive Chvostek sign (facial muscle spasm elicited by tapping the facial nerve); positive Trousseau sign (carpopedal spasm that results from oxygen deficiency) Laryngeal spasm, stridor Poor eating Lethargy Administer intravenous calcium gluconate for acute or severe tetany, then intramuscular or oral calcium as prescribed. Monitor the child for the development of cardiac arrhythmias. Ensure that the intravenous site is patent; if extravasation occurs, tissue damage or cardiac arrhythmias may result. Monitor fluid and electrolyte status, weigh the child daily, and measure urinary calcium excretion to prevent nephrocalcinosis. Institute seizure precautions and reduce environmental stimuli (e.g., loud or sudden noises, bright lights, or stimulating activities). Observe for signs and symptoms of laryngospasm (e.g., stridor, hoarseness, or a feeling of tightness in the throat). Teach the child and family about the need for continuous daily administration of calcium salts and vitamin D. Have the family observe for vitamin D toxicity by observing for signs such as weakness, fatigue, lassitude, headache, nausea and vomiting, and diarrhea.

Cushing syndrome

(excess levels of one or all the hormones [glucocorticoids, mineralocorticoids, and adrenal androgens] but most commonly glucocorticoid excess) Usually, this condition is due to a small ACTH-producing pituitary adenoma. The most common cause in older children is prolonged or excessive use of corticosteroid therapyb Note history of rapid weight gain, decreased velocity of linear growth, muscle weakness, fatigue, irritability, sleep disturbance, and hypertension History of long-term corticosteroid use, water retention, poor wound healing, frequent infections, and in teenage girls, missed menstrual periods Refer to Figure 26.5 Skin may be thin and fragile; acne may be present Management varies depending on the cause. The goal is to restore hormone balance and reverse Cushing syndrome. If the cause is an adrenal or pituitary tumor, then surgical removal of the tumor alone or the entire adrenal gland is performed. If the cause is long-term steroid therapy, then the corticosteroid dose is reduced to the lowest dose that is effective in treating the underlying disorder. Cortisol synthesis-inhibiting medications may be used. Counsel the family that the cushingoid appearance is reversible with appropriate treatment. Be alert for signs of adrenal insufficiency if the child has surgery or if corticosteroid withdrawal occurs quickly.

Hyperparathyroidism

(hypersecretion of PTH) Parathyroid adenoma is the most common cause; secondary hyperparathyroidism is primarily due to renal failure Hypercalcemia Hypophosphatemia Depression of neuromuscular function, child may trip and drop objects, general fatigue, failure to thrive, headaches, poor school performance, and irritability, somnolence, stupor, or difficulty concentrating. Irregular heart rate, possibly related to cardiac dysrhythmias. Skeletal pain, fractures, formation of bone tumors, or flank pain related to renal calculi Administer IV fluids and diuretics as prescribed to increase urinary excretion of calcium in children not in renal failure. Administer prescribed medication to treat hypercalcemia, such as oral phosphate (antihypercalcemic agent), pamidronate, calcitonin, or etidronate disodium (by inhibiting bone resorption of calcium). Increase the child's fluid intake to minimize renal calculi formation. Provide fruit juices to maintain low urinary pH, acidity of body fluids, and calcium absorption. Strain the urine for renal casts. Dietary calcium is restricted. Monitor for safety by assessing the child's level of muscular weakness, preventing falls or injury, and checking for fractures. If the child develops renal rickets (osteodystrophy), long-term braces may be required, so provide family education and encourage compliance. Surgery may be performed to remove abnormal parathyroid tumor. Keep the diet low in phosphorus and watch for hypocalcemia and onset of tetany after surgery.

phenotype

(the outward characteristics of the individual).

Congenital hypothyroidism

, also known as cretinism, usually results from failure of the thyroid gland to migrate during fetal development (Lafranchi & Huang, 2016). This results in malformation or malfunction of the thyroid gland, which leads to insufficient production of the thyroid hormones that are required to meet the body's metabolic and growth and development needs. Congenital hypothyroidism leads to low concentrations of circulating thyroid hormones (triiodothyronine [T3] and thyroxine [T4]). Congenital hypothyroidism occurs in 1:2,000 to 1 in 4,000 live births (LaFranchi, 2018a). It occurs over a wide range of ethnic groups, though less among African Americans, and is more common in girls than boys (Lafranchi, 2018a). Complications include intellectual disability if untreated, short stature, growth failure, and delayed physical maturation and development (Lafranchi & Huang, 2016). Congenital hypothyroidism is one of the most common preventable causes of intellectual disability. The later it is diagnosed, the greater the disability is (LaFranchi, 2018a). Most newborns have few if any symptoms and the occurrence is sporadic, not typically hereditary; therefore, most cases of congenital hypothyroidism are detected via newborn screening programs. Pathophysiology Congenital hypothyroidism is due to a defect in the development of the thyroid gland in the fetus due to a spontaneous gene mutation, an inborn error of thyroid hormone synthesis resulting from an autosomal recessive trait, pituitary dysfunction, or failure of the CNS-thyroid feedback mechanism to develop. Transient primary hypothyroidism may also occur; it results from transplacental transfer of maternal medications, maternal thyroid-blocking antibodies, iodine deficiency, and fetal or neonatal exposure to excessive iodines (such as use of iodine antiseptics during delivery or procedures or excess ingestion of iodine by the mother) (Lafranchi, 2018a). Therapeutic Management To prevent intellectual disability and restore normal growth and motor development, thyroid hormone replacement with sodium L-thyroxine (Synthroid, synthetic thyroxine, or Levothroid) is given. The recommended starting dosage is 10 to 15 μg/kg/day (American Academy of Pediatrics, 2011; LaFranchi, 2018b). There are no adverse effects with physiologic doses, but thyroid function tests are performed initially every 2 weeks to closely monitor for effects and to ensure proper dosing. Since thyroid hormone is vital to the infant's developing CNS, the goal is to normalize thyroid function as quickly as possible. This treatment will be needed lifelong to maintain normal metabolism and promote normal physical and mental growth and development. Health History Inquire whether the neonatal metabolic screening test was performed and results were obtained. Determine if the test was done less than 24 to 48 hours after birth. If so, a repeat test may be warranted (see Laboratory and Diagnostic Testing below). Inquire about maternal history that may indicate a connection to hypothyroidism, such as maternal exposure to iodine. Additional history findings may include sensitivity to cold, constipation, feeding problems, or lethargy. Since parents like babies to sleep well, the parents may not complain that the baby is sleeping too much; rather, they may remark that it is difficult to keep the baby awake. Physical Examination Most infants are asymptomatic until the first month, when they begin to develop clinical signs. Inspection and observation reveal a lethargic baby or a child with hypotonia, hypoactivity, and a dull expression. A combination of lethargy and irritability may exist, with an overall delayed mental responsiveness. Measurements of weight and height may reveal delayed growth. Other findings may include a persistent open posterior fontanel, coarse facies with short neck and limbs, periorbital puffiness, enlarged tongue, and poor sucking response (Fig. 26.3). The skin may appear pale with mottling or yellow from prolonged jaundice, or it may be cool, dry, and scaly to the touch, with sparse hair development on the older child. Auscultation of the chest might reveal bradycardia. Signs of respiratory distress and decreased pulse pressure may also be present. On palpation of the abdomen, there may be evidence of an umbilical hernia or a mass due to constipation. Laboratory and Diagnostic Testing Every infant should have a newborn screen for thyroid hormone levels before discharge from the hospital or 2 to 4 days after birth (American Academy of Pediatrics, 2006, reaffirmed 2011; LaFranchi, 2018b). When the test is performed within the first 24 to 48 hours along with other metabolic screenings, the result may be inaccurate because of the immediate increase in thyroid-stimulating hormone (TSH) shortly after birth (American Academy of Pediatrics, 2006, reaffirmed 2011). Radioimmunoassay is used to measure levels of T4, which accurately reflect the child's thyroid status. If the T4 level is low, then a second confirming laboratory test is performed, as well as determining whether the TSH is elevated. A thyroid scan may also be used to check for the absence or ectopic placement of the gland. In addition to serum measurement of T4, other diagnostic tests include serum T3, radioiodine uptake, thyroid-bound globulin, and ultrasonography. Nursing Management The overall goal of nursing management of the infant or child with congenital hypothyroidism is to establish a normal growth pattern without complications such as intellectual disability or failure to thrive. Individualize the nursing plan of care based on the infant's responses to the illness. Promoting Appropriate Growth Measure and record growth at regular intervals. Thyroid levels are measured at recommended intervals, such as every 2 weeks until the target range is reached on a stabilized dose of medication, then every 1 to 3 months until the child is 1 year old, every 2 to 3 months until the child is 3 years old, and becoming less frequent as the child gets older (Lafranchi, 2018b). A trial off the medication may be performed around the age of 3, under physician or nurse practitioner supervision, to confirm the diagnosis (Lafranchi, 2018b). Monitor for signs of hypo- or hyperfunction, including changes in vital signs, thermoregulation, and activity level. Provide adequate rest periods and meet thermoregulation needs. If the infant's tongue is unusually large, observe feeding ability, prevent airway obstruction, and position the infant on the side. Fluid restrictions or a low-salt diet may be ordered. Educating the Family Since many infants are asymptomatic, the diagnosis may be unexpected, so reassure and convey realistic expectations to the family. Developmental screening may be required if the child showed any symptoms initially or as the child gets older, to ensure that drug therapy is appropriate. Educate the family about the disorder, the medication and method of administration, and adverse effects such as increased pulse rate (which may indicate an overdose of thyroid hormone). L-Thyroxine is an oral medication and is not available in a liquid form. The pill form must be crushed for infants and young children. It can be mixed with a small amount of formula or breast milk and placed in the nipple, but it should not be placed in a full bottle of formula or breast milk because the infant will not ingest all the medication if he or she does not finish the bottle. The medication can also be mixed with a small amount of liquid and given with a dropper. Medication absorption is affected by soy-based formulas, fiber, and iron preparations (American Academy of Pediatrics, 2006, reaffirmed 2011; LaFranchi, 2018b). Therefore, carefully evaluate the formula the infant is on before administering L-thyroxine. Inform the family that this medication will be needed throughout the child's life. Explain that missed doses may lead to developmental delays and poor growth. Tell them that frequent blood tests will be needed to evaluate thyroid function and the child's growth rate; genetic counseling may be needed. Clinical examination, including growth and development assessment, should occur every few months until the child is 3 years old. Serum T4 and TSH should be evaluated often, and more frequent monitoring may be needed if noncompliance occurs, if abnormal values occur, or with any changes in medication dosage or treatment regimen. The nurse may need to help the family to find a laboratory nearby or to handle financial issues related to the therapy. Educate the family about infant stimulation programs if the child shows cognitive problems, retarded physical growth, or slow intellectual development. Some information may need to be reinforced during school-age or adolescent stages of development. Finally, encourage the family to obtain a medical ID bracelet or necklace for the child.

Polycystic ovary syndrome (PCOS)

, also referred to as functional ovarian hyperandrogenism or ovarian androgen excess, is an endocrine disorder that produces a variety of symptoms in adolescent girls and women. The exact cause is unknown. Testosterone production by the ovaries and adrenal cells is excessive, causing hirsutism, balding, acne, increased muscle mass, and decreased breast size. Polycystic ovaries may or may not be present. There is a genetic predisposition for PCOS along with nonhereditary factors, the most common being insulin resistance and obesity (Rosenfield, 2018). Complications of excess androgen production in women include infertility, insulin resistance, and hyperinsulinemia, leading to diabetes mellitus (DM), increased risk for endometrial carcinoma, and cardiovascular disease. Therapeutic management involves the administration of oral contraceptives for their hormonal effects, as well as insulin-sensitizing medications such as metformin (Glucophage). Nursing Assessment Explore the health history for oligomenorrhea (irregular, infrequent periods) or amenorrhea. Symptoms typically emerge at or soon after puberty but often go undiagnosed. Note weight in relation to standardized growth charts and calculate body mass index (BMI) to determine whether the girl is overweight or obese. Inspect the skin for acne, acanthosis nigricans (darkened, thickened pigmentation, particularly around the neck or in the axillary region), and hirsutism (excess body hair growth). Assist with collection of timed blood specimens for glucose and insulin levels (which will often show unexpectedly elevated insulin levels in relation to the glucose level). Laboratory tests may show elevated levels of free testosterone and other androgenic hormones. Nursing Management One of the most important functions of the nurse in relation to PCOS is to assist with early recognition and treatment. Educate the adolescent girl about the use of oral contraceptives to normalize hormone levels, which will decrease androgenic effects. Support the teen in her efforts to diet and exercise to lose weight. Oral insulin-sensitizing drugs such as metformin (Glucophage) may be prescribed. Encourage the teen to comply with the medication regimen. Routinely measure weight to determine progress with weight loss. Measure blood pressure to screen for hypertension, which may develop as a complication of PCOS. Online support groups and education are available. Links to several resources are provided on .

Lymphomas

, or tumors of the lymph tissue (lymph nodes, thymus, spleen), account for about 10% to 15% of cases of childhood cancer (Craddock et al., 2018). Lymphomas may be divided into two categories—Hodgkin disease (or Hodgkin lymphoma) and non-Hodgkin lymphoma (NHL), which includes more than a dozen types. Hodgkin disease tends to affect lymph nodes located closer to the body's surface, such as those in the cervical, axillary, and inguinal areas, whereas NHL tends to affect lymph nodes located more deeply inside the body.

Four or more new episodes of acute otitis media in 1 year Two or more episodes of severe sinusitis in 1 year Treatment with antibiotics for 2 months or longer with little effect Two or more episodes of pneumonia in 1 year Failure to thrive in the infant Recurrent deep skin or organ abscesses Persistent oral thrush or skin candidiasis after 1 year of age History of infections requiring IV antibiotics to clear Two or more serious infections such as sepsis Family history of primary immunodeficiency

10 Warning Signs of Primary Immunodeficiency

Tyrosinemia

: deficiency in an enzyme essential in the metabolism of tyrosine; accumulation of the by-products results in liver and kidney damage Symptoms usually appear in the first months of life: fever, failure to thrive, poor weight gain, vomiting, diarrhea, cabbage-like odor, enlarged liver and spleen, increased bleeding tendency, distended abdomen, jaundice, cirrhosis, and liver failure Diet low in phenylalanine and tyrosine www.liverfoundation.org: American Liver Foundation

Milieu therapy

A specially structured setting designed to promote the child's adaptive and social skills. A safe and supportive environment for those at risk for self-harm or those who are very ill or very aggressive.

Food Allergies

A true food hypersensitivity or allergy is defined as an immunologic reaction resulting from the ingestion of a food or food additive. This type of reaction is an IgE- mediated response to a particular food. Food allergy affects approximately 8% of children and can lead to significant medical complications (Covar et al., 2018). During the first few years of life, the most common food allergens are milk, eggs, peanuts, tree nuts, fish and shellfish, wheat, and soy. Older children and adults most often demonstrate allergy to peanuts, tree nuts, fish, and shellfish (Volkman & Chiu, 2019). Most reactions occur within minutes of exposure, but they may occur up to 2 hours after ingestion. Signs and symptoms of a food allergy reaction include hives, flushing, facial swelling, mouth and throat itching, and runny nose. Many children also have a gastrointestinal reaction, including vomiting, abdominal pain, and diarrhea. In extreme cases, swelling of the tongue, uvula, pharynx, or upper airway may occur. Wheezing can be an ominous sign that the airway is edematous. Rarely, cardiovascular collapse occurs. Though the risk for anaphylaxis is small, parents, caregivers, and physicians and nurse practitioners should be vigilant when caring for children with food allergies. Therapeutic Management Therapeutic management involves verifying the food allergy, avoiding the allergen, and treating the reaction with medications, including antihistamines and epinephrine (in the case of an anaphylactic reaction). To verify the food allergy, a trial elimination diet may be indicated. If symptoms resolve without the food, true allergy may be present. On an elimination diet, the child stops eating all suspicious foods for 1 to 2 weeks and then retries the foods one at a time, over a period of several days, to see whether a similar reaction occurs. Oral challenge testing and retrying of foods after an elimination diet are often done in the physician's or nurse practitioner's office or hospital setting if severe reactions have occurred in the past. Food avoidance is recommended for those who have a highly predictive reaction to testing or a history of anaphylactic response. Prevention of food allergies is also important (see Evidence-Based Practice Box 25.1). Discerning a true food allergy from intolerance to certain foods is an important part of therapeutic management. "Food intolerance" is a general term that describes an abnormal physiologic response to an ingested food or food additive that has not been proven to be immunologic. Often a milk allergy is confused with lactose intolerance. Therefore, a detailed dietary history is important when distinguishing a true allergy versus intolerance. Nursing Assessment It is important to accurately assess children with food allergy reactions. In the initial nursing assessment, immediately assess the child for airway, breathing, or circulation problems (see Chapter 29). If the child's condition is stable, finish the assessment. Make sure that the health history includes a detailed food history and documentation of the reaction, including the food suspected of causing the reaction, the quantity of food ingested, the length of time between ingestion and development of symptoms, the symptoms, what treatment has been administered, and the subsequent response. Note gastrointestinal symptoms such as: Burning in the mouth or throat Bloating Nausea Diarrhea Assess for risk factors such as previous exposure to the food, history of poorly controlled asthma, or an increase in atopic dermatitis flare-ups in relation to food intake. Inspect the skin for color, rash, hives, or edema. Auscultate the heart and lungs to determine heart rate and to assess for wheezing. Allergy skin-prick tests and radioallergosorbent blood tests (RASTs) are used widely by physicians and nurse practitioners to look for allergic reactions. Food-specific IgE testing is recommended if the child has a history of food allergy. If the child has episodic symptoms, an oral challenge in a controlled setting may be appropriate. For an oral challenge, the child slowly eats a serving of the offending food over the period of 1 hour. Record vital signs and note the presence or absence of allergic symptoms. Nursing Management Initial nursing management is aimed at stabilizing the child's condition if an acute reaction to a food allergen is present (see Chapter 29). As stated above, medications used in the treatment of a food allergy reaction include histamine blockers and, in anaphylactic reactions, epinephrine. Teach the child (if appropriate) and the parents how and when to use these medications during an allergic reaction. The child who has been prescribed an EpiPen should carry the pen with him or her at all times. Since these reactions can be so sudden (unknown ingestion of allergen) and severe, it is helpful for the family to have a written emergency plan in case of a reaction. Managing the Child's Diet Aim dietary teaching at educating the child and family on how to avoid the offending foods. Families should be extremely careful when reading food labels. A dietitian may be helpful in this teaching process. Teaching Guidelines 25.1 gives information about hidden allergens in food. Teach the parents what "safe" foods can be substituted for offensive ones (Box 25.5). Children with peanut allergy should also avoid tree nuts. Having a child with a food allergy can be very anxiety producing for parents; they often live in fear that the child may accidentally ingest an allergen. Educate the child and family about allergic reactions to help decrease their anxiety. Teach the child and family how to recognize the signs and symptoms of an allergic reaction. It may be necessary to provide information to day care providers as well as schoolteachers, staff, and camp counselors. Refer families to the Food Allergy & Anaphylaxis Network. A link to this website can be found on CoursePoint.

Testicular Cancer

Although uncommon in teens, testicular cancer is most frequently diagnosed in young adult males (NCI, n.d.). It is one of the most curable cancers if diagnosed early. To get into the habit of screening for testicular lumps, encourage adolescent boys to begin performing testicular self-examinations monthly (Teaching Guidelines 24.5). See Healthy People 2030. TEACHING GUIDELINES 24.5 Testicular Self-Examination Perform the examination once a month, after a shower. Be familiar with the size and weight of your testicles. Roll the testicle between your fingers. The small rope-like structure is the epididymis; this is normal. Report any lump, swelling, or heaviness of one testicle to your physician or nurse practitione

GENOMIC IMPRINTING

Another nontraditional inheritance pattern results from a process called genomic imprinting. Genomic imprinting plays a critical role in fetal growth and development and placental functioning. It is a phenomenon by which the expression of a gene is determined by its parental origin. In genomic imprinting both the maternal and paternal alleles are present, but only one is expressed; the other is inactive. Genomic imprinting does not alter the genetic sequence itself, but affects the phenotype observed. In these cases, the altered genes in a certain region of the genome have very different expressions depending on whether they were inherited from the mother or the father. Several human syndromes are known to be associated with defects in gene imprinting. Disorders that result from a disruption of imprinting usually involve a growth phenotype and include varying degrees of developmental problems. Common examples include Prader-Willi syndrome (a condition resulting in severe hypotonia and hyperphagia, leading to obesity and intellectual disability), Angelman syndrome (a neurodevelopmental disorder associated with intellectual disability, jerky movements, and seizures), and Beckwith-Wiedemann syndrome (characterized by somatic overgrowth, congenital malformations, and a predisposition to embryonic neoplasia) (Scott & Lee, 2016c).

AUTOSOMAL DOMINANT INHERITANCE

Autosomal dominant inheritance occurs when a single gene in the heterozygous state is capable of producing the phenotype. In these cases, the abnormal or mutant gene overshadows the normal gene and the individual will demonstrate signs and symptoms of the disorder. The affected person usually has one affected parent. However, there are varying degrees of presentation among individuals in a family. For example, a parent with a mild form of the disorder could have a child with a more severe form (termed variable expression). In some autosomal dominant disorders there may be no history of an affected family member. This can be due to the child representing a new mutation or the result of incomplete or reduced penetrance, which means that a person with the genetic mutation does not develop phenotypic features of the disorder. Incomplete or reduced penetrance may result from a combination of genetic, environmental, and lifestyle factors; age; and gender. Offspring of an affected parent will have a 50% chance of inheriting two normal genes (disorder free) and a 50% chance of inheriting one normal and one abnormal gene (and, thus, the disorder) (Scott & Lee, 2016c) (Fig. 27.1). Females and males are equally affected by autosomal dominant disorders and an affected male can pass the disorder on to his son (Scott & Lee, 2016c). This male-to-male transmission is important in distinguishing autosomal dominant inheritance from X-linked inheritance. Common types of genetic disorders that follow the autosomal dominant pattern of inheritance include neurofibromatosis, Huntington disease, Marfan syndrome, and osteogenesis imperfecta type 1 (Genetics Home Reference, 2019e).

AUTOSOMAL RECESSIVE INHERITANCE

Autosomal recessive inheritance occurs when two copies of the mutant or abnormal gene in the homozygous state are necessary to produce the phenotype. In other words, two abnormal genes are needed for the individual to demonstrate signs and symptoms of the disorder. Both parents of the affected person must be heterozygous carriers of the gene (clinically normal, but carriers of the gene). Offspring of two carriers of the abnormal gene have a 25% chance of inheriting two normal genes; a 50% chance of inheriting one normal gene and one abnormal gene (carrier); and a 25% chance of inheriting two abnormal genes (and, thus, the disorder) (Scott & Lee, 2016c) (Fig. 27.2). Affected individuals are usually present in only one generation of the family. Females and males are equally affected, and a male can pass the disorder on to his son (Scott & Lee, 2016c). The chance that any two parents will both be carriers of the mutant gene is increased if the couple has consanguinity (relationship by blood or common ancestry) (Scott & Lee, 2016c). Common types of genetic disorders that follow the autosomal recessive inheritance pattern include cystic fibrosis and sickle cell disease (Genetics Home Reference, 2019e).

Set limits with the child, holding him or her responsible for his or her behavior. Do not argue, bargain, or negotiate about the limits once established. Provide consistent caregivers (unlicensed assistive personnel and nurses for the hospitalized child) and establish the child's daily routine. Use a low-pitched voice and remain calm. Redirect the child's attention when needed. Ignore inappropriate behaviors. Praise the child's self-control efforts and other accomplishments. Use restraints only when necessary.

Behavior management techniques include the following:

MITOCHONDRIAL INHERITANCE

Certain diseases result from mutations in the mitochondrial DNA. Mitochondria (the part of the cell responsible for energy production) are inherited almost exclusively from the mother. Therefore, mitochondrial inheritance is usually passed from the mother to the offspring, regardless of the offspring's sex (differentiating mitochondrial inheritance from X-linked recessive inheritance). These mutations are often deletions and abnormalities and are often seen in one or more organs, such as the brain, eye, and skeletal muscle. They are often associated with energy deficits in cells with high energy requirements, such as nerve and muscle cells. These disorders tend to be progressive and the age of onset can vary from infancy to adulthood. There is an extreme amount of variability in symptoms within a family. Examples of disorders that follow mitochondrial inheritance include Kearns-Sayre syndrome (a neuromuscular disorder) and Leber hereditary optic neuropathy (which causes progressive visual impairment) (Scott & Lee, 2016c).

Tachyarrhythmias

Children normally have faster heart rates than adults, and fever, fear, and pain are common explanations for significant increases in the heart rate of a child (tachycardia). This normal elevation in heart rate is known as sinus tachycardia. However, once the fever is reduced, the child is comforted, or the pain is managed, the heart rate should return close to the child's baseline. Hypoxia and hypovolemia are pathologic reasons for tachycardia in the child. If the child has sinus tachycardia that results from any of these causes, the focus is on the underlying cause. It is inappropriate and dangerous to treat sinus tachycardia with medications aimed at decreasing the heart rate or with a defibrillation device. Tachyarrhythmias in children that are associated with cardiac compromise have unique characteristics that present differently from sinus tachycardia. Examples of these include supraventricular tachycardia (SVT) and ventricular tachycardia. SVT is a cardiac conduction problem in which the heart rate is extremely rapid and the rhythm is very regular, often described as "no beat-to-beat variability." Comparison Chart 29.3 explains the differences between SVT and sinus tachycardia. The most common cause of SVT is a re-entry problem in the cardiac conduction system. Commonly, SVT is the result of a genetic cardiac conduction problem such as Wolff-Parkinson-White syndrome. SVT may also be associated with medications such as caffeine and theophylline. Children often can tolerate the characteristically higher heart rate that is associated with SVT for short periods of time. However, the increased demand that is placed on the cardiovascular system usually overtaxes the child and results in signs of congestive heart failure if the SVT continues unchecked for a prolonged time.Ventricular tachycardia is a rhythm involving an elevation of the heart rate and a wide QRS (greater than 0.08 seconds) that is the result of an abnormal, rapid firing of one or both of the ventricles. Ventricular tachycardia is a rare arrhythmia in children and usually is associated with a congenital or acquired cardiac abnormality. In addition, prolonged QT syndrome is a conduction abnormality that can result in ventricular tachycardia and sudden death in children. Less commonly, ingestion of medications and toxins, acidosis, hypocalcemia, abnormalities of potassium, and hypoxemia have been associated with the development of ventricular tachycardia in children.Managing Tachyarrhythmias The tachyarrhythmias include SVT (stable or unstable) and ventricular tachycardia with a pulse. Examine the ECG to determine if the child is experiencing ventricular tachycardia or SVT. Clinically, determine whether the child in SVT is showing signs that require emergent intervention or if the child is stable. In compensated SVT, the child will appear to be alert, breathing comfortably, and well perfused. The child who is demonstrating signs of compromise, such as a change in consciousness, respiratory status, and perfusion, is considered to be in uncompensated SVT. Uncompensated SVT requires emergent intervention. The child who has ventricular tachycardia with a pulse will have poor perfusion and also requires immediate intervention. The evaluation and approaches to the tachyarrhythmias are discussed in Table 29.6.

Hypnosis

Deep relaxation with suggestibility remarks.

Play therapy

Designed to change emotional status. Encourages the child to act out feelings of sadness, fear, hostility, or anger.

Provide sufficient calories and good nutrition for normal growth and development. The diet should be low in saturated fats and concentrated carbohydrates. Learn to identify carbohydrate, protein, and fat foods. Make adjustments during periods of rapid growth and for issues such as travel, school parties, and holidays. Consult a dietitian with expertise in diabetes education as needed. Provide three meals per day and midafternoon and bedtime snacks. Consistency of intake can help prevent complications and maintain near-normal blood glucose levels. Encourage the child to exercise routinely to help the body use insulin efficiently, thus reducing the insulin requirement. Encourage the child to participate in age-appropriate sports. When exercising, monitor insulin dose and nutritional and fluid intake, and observe for hypoglycemic reactions. Add an extra snack containing 15 to 30 g carbohydrate for each 45 to 60 minutes of exercise. Avoid exercising excessively when insulin is peaking.

Diet and Exercise for Children With Diabetes Mellitus

Family therapy

Exploration of the child's emotional issue and its effect on family members. Helps the family to focus in more constructive ways.

Dialectical behavioral therapy

Group and individual sessions to treat chronic suicidal thoughts in borderline personality disorder. Individuals learn responsibility for their problems and better deal with negative emotions.

Acquired Hypothyroidism

Hypothyroidism also occurs as an acquired condition. This disorder most commonly results from an autoimmune chronic lymphocytic (Hashimoto) thyroiditis (LaFranchi & Huang, 2016; Lafranchi, 2018c). As a genetic condition, antibodies develop against the thyroid gland, causing the gland to become inflamed, infiltrated, and progressively destroyed. It occurs more often in girls during childhood and adolescence (Lafranchi, 2018c). Less common etiologies include hypothyroidism associated with pituitary or hypothalamic disease or exposure to drugs or substances such as antithyroid medications, anticonvulsants, lithium and amiodarone that interfere with thyroid hormone synthesis, thyroid injury such as radiation, thyroidectomy, and hemangiomas, and iodine deficiency or excess (LaFranchi & Huang, 2016; Lafranchi, 2018c). Therapeutic management is the same as for congenital hypothyroidism. Management involves oral sodium L-thyroxine, which is given at 2 to 6 μg/kg/day based on age to maintain T4 in the upper half of the normal range and to suppress TSH. Nursing Assessment Interview the family and child to determine activity tolerance and behavior changes. The symptoms may develop over a period of time and may be subtle. Note vague complaints of fatigue, weakness, weight gain, cold intolerance, constipation, and dry skin. The severity of symptoms depends on the length of time that the hormone deficiency has existed and its extent. Reviewing the growth pattern may reveal a slowed or arrested growth rate (height) and increased weight. Physical examination may reveal a goiter (enlargement of the thyroid gland). Deep tendon reflexes may be sluggish and the face, eyes, and hands may be edematous. Note thinning or coarse hair, muscle hypertrophy with muscle weakness, and signs of delayed or precocious puberty. The diagnostic evaluation involves serum thyroid function studies (TSH, T3, and T4) as well as serum thyroid antibodies to confirm autoimmune thyroiditis. MRI and a thyroid uptake test and scan may also be necessary. Nursing Management Work with the family to establish a daily schedule for administering L-thyroxine, which should be taken 30 to 60 minutes before a meal for optimal absorption. Explain to the family that growth is related to the child's response to the treatment, and there are no specific strategies to aid in this growth. The family should understand the diagnosis, should be able to recognize signs and symptoms of thyroid hypo- and hyperfunction, and should know when to notify the physician or nurse practitioner. The family and child may need assistance in accepting the therapy as well as the experience of catch-up growth that may occur at the beginning of therapy. The child with chronic or severe hypothyroidism may be at risk for adverse effects such as restlessness, insomnia, or irritability. The child's thyroid levels should be evaluated at recommended intervals such as every 3 to 6 months by a pediatric endocrinologist.

Prevention of cardiac arrest and injuries Early CPR Early access to emergency response system Early advanced care (pediatric advanced life support [PALS]) Integrated post-cardiac arrest care (van der Jagt, 2017)

In contrast, the pediatric chain of survival is:

Chromosomal Abnormalities

In some cases of genetic disorders, the abnormality occurs due to problems with the chromosomes. Chromosomal abnormalities do not follow straightforward patterns of inheritance. Although some chromosomal disorders can be inherited, most others occur due to random events during the formation of reproductive cells or in early fetal development. Sperm and egg cells each have 23 unpaired chromosomes. When they unite during conception they form a fertilized egg with 46 chromosomes. Sometimes before pregnancy begins, an error has occurred during the process of cell division, leaving an egg or sperm with too many or too few chromosomes. If this egg or sperm cell joins with a normal egg or sperm cell, the resulting embryo has a chromosomal abnormality. Chromosomal abnormalities can also occur due to an error in the structure of the chromosome. Small pieces of the chromosome may be deleted, duplicated, inverted, misplaced, or exchanged with part of another chromosome. Most chromosomal abnormalities occur due to an error in the egg or sperm. Therefore, the abnormality is present in every cell of the body. Some abnormalities can happen after fertilization, during mitotic cell division, and result in mosaicism. Mosaicism or the mosaic form is when the chromosomal abnormalities do not show up in every cell; only some cells or tissues carry the abnormality. In mosaic forms of the disorder the symptoms are usually less severe than if all the cells were abnormal. Chromosomal abnormalities occur in about 1% to 2% of live births (Bacino & Lee, 2016). There is a much higher frequency of chromosomal abnormalities in spontaneous abortions and stillbirths (Bacino & Lee, 2016). Congenital anomalies and intellectual disability are often associated with chromosomal abnormalities (Bacino & Lee, 2016). These abnormalities occur on autosomal or nonsex chromosomes as well as sex chromosomes and can result from abnormalities of either chromosome number or chromosome structure. A karyotype is a pictorial analysis of chromosomes. It depicts a systematic arrangement of chromosomes of a single cell by pairs (Fig. 27.5). Karyotyping is often used in prenatal testing to diagnose or predict genetic diseases.

Retinopathy: Type 1 diabetes: eye examination by ophthalmologist (with expertise in diabetes) once child is 10 years old or puberty has started (whichever is earlier) and has had diabetes for 3 to 5 years; eye examinations every 1 to 2 years unless different recommendation by professional Type 2 diabetes: eye examination by ophthalmologist (with expertise in diabetes) shortly after diagnosis; annual examinations unless different recommendation by professional Nephropathy: Type 1 diabetes: annual screening for microalbuminuria (which occurs when the kidneys leak small amounts of albumin into the urine) once child is 10 years old or puberty has started and has had diabetes for 5 years Type 2 diabetes: screen at diagnosis and annually thereafter for microalbuminuria Neuropathy: Type 1 diabetes: annual foot examination once child has reached puberty or ≥10 years old and has had diabetes for 5 years Type 2 diabetes: foot examination at diagnosis and annually Dyslipidemia: Type 1 diabetes: obtain a lipid profile in children ≥10 years old at time of diagnosis (once glucose level has been stabilized); if normal, repeat every 3 to 5 years Type 2 diabetes: obtain fasting lipid panel at diagnosis (once glucose level has been stabilized), then annually Hypertension: blood pressure measured at each routine visit In addition, children with type 1 diabetes should be screened for additional autoimmune disorders such as celiac disease (screen after diagnosis and then after 2 years and again after 5 years, screen more often if symptoms or family history present) and hypothyroidism (screen after diagnosis, once glucose level has been stabilized, and every 1 to 2 years or sooner if symptoms are present) Assess for psychosocial and diabetes-related distress generally starting around 7 to 8 years old Screen for eating disorders starting at 10 to 12 years old. Refer to Evidence-Based Practice 26.1

Management of Complications Another important aspect of therapeutic management includes monitoring and managing complications. The American Diabetes Association (2019) has developed recommendations for standards of medical care to help monitor complications and reduce risk. These include:

Multifactorial Inheritance

Many of the common congenital malformations, such as cleft lip, cleft palate, neural tube defects, pyloric stenosis, clubfoot, congenital hip dysplasia, and cardiac defects, are attributed to multifactorial inheritance (Scott & Lee, 2016c). These conditions are thought to be caused by multiple gene and environmental factors. That is, a combination of genes from both parents, along with unknown environmental factors, produces the trait or condition. An individual may inherit a predisposition to a particular anomaly or disease. The anomalies or diseases vary in severity and often a sex bias is present. For example, pyloric stenosis is seen more often in males, while congenital hip dysplasia is much more likely to occur in females. Multifactorial conditions tend to run in families, but the pattern of inheritance is not as predictable as with single-gene disorders. The chance of recurrence is also less than in single-gene disorders, but the degree of risk is related to the number of genes in common with the affected individual. The closer the degree of relationship, the more genes an individual has in common with the affected family member, and thus the higher risk that the individual's offspring will have a similar defect. In multifactorial inheritance the likelihood that both identical twins will be affected is not 100%, indicating that there are nongenetic factors involved

Group therapy

May be conducted in a school, hospital, treatment facility, or neighborhood center. Feelings are expressed and participants gain hope, feel a part of something, and benefit from role modeling. Takes advantage of peer relationships as developmental focus in preteen and teen groups.

Hyperthyroidism

Nervousness/anxiety Diarrhea Heat intolerance Weight loss Smooth, velvety skin

Blood Glucose Monitoring

Obtain glucose levels before meals and bedtime snacks. Perform monitoring more often during prolonged exercise, if you are ill, if you have eaten more food than usual, or if you suspect nighttime hypoglycemia. Use the manufacturer's recommendations and perform quality control measures as directed. Look for patterns. For example, 3 to 4 days of a consistent pattern of glucose values above 200 mg/dL before dinner indicates a need to adjust the insulin dose. Blood glucose measurements are the best way to determine daily insulin dosages. Normal levels are as follows: nondiabetics: 70 to 110 mg/dL; (target levels should be individualized), children and adolescents with type 1 DM: before meals 90 to 130 mg/dL, at bedtime 90 to 150 mg/dL (American Diabetes Association, 2019).

Diet and Exercise

Other therapies involve diet and exercise protocols. Medical nutrition therapy (MNT) can be initiated to prevent type 2 diabetes in children showing signs of prediabetes, to help glycemic control in existing diabetes, and to help slow the development of complications associated with diabetes. MNT can be complex and must be individualized to each child incorporating the child's food preferences, activity level, cultural preferences, and family habits and schedule. Enlisting the help of a registered dietician who has expertise in diabetic management is recommended (American Diabetes Association, 2019). The appropriate diet for a child or adolescent with diabetes is a balanced, healthy diet that meets the child's growth and development needs. The child and family need to understand the effect that food has on the child's glucose levels. Monitoring carbohydrate intake is an important component of diet management and assists with glycemic control. Nutritional recommendations for a child with diabetes or prediabetes include the following: limit sweets, ensure consistent food intake (eat often and try to avoid skipping meals), monitor carbohydrate intake, eat whole grains and plenty of fruits and vegetables, and limit fat. It has been shown that regular exercise can improve glycemic control and can prevent the development of type 2 diabetes (American Diabetes Association, 2019). Also, exercise has an important influence on the hypoglycemic effects of insulin (by causing the release of glucagon, which will result in increased blood glucose). Therefore, it is important for the child to maintain or increase his or her activity levels. Exercise can lead to both hyperglycemia or hypoglycemia; therefore, frequent glucose monitoring before, during, and after exercise is important (American Diabetes Association, 2019). If the child is taking insulin, the family must know how to adjust the medication dosage or add food to maintain blood glucose control. The child needs to have access to rapid-acting carbohydrates and the child and family should ensure pre-exercise blood glucose levels of 90 to 250 (American Diabetes Association, 2019). Children with type 2 DM are often overweight, so the exercise plan is very important in helping the child to lose weight as well as assisting with the hypoglycemic effects of the medications.

Mendelian or Monogenic Laws of Inheritance

Principles of inheritance of single-gene disorders are the same that govern the inheritance of other traits, such as eye and hair color. These are known as Mendel's laws of inheritance, named for Gregor Mendel, an Austrian naturalist who conducted genetic research. These patterns occur because a single gene is defective and the disorders that result are referred to as monogenic or, sometimes, mendelian disorders. If the defect occurs on the autosome, the genetic disorder is termed autosomal; if the defect is on the X chromosome, the genetic disorder is termed X linked. The defect also can be classified as dominant or recessive. Monogenic disorders include autosomal dominant, autosomal recessive, X-linked dominant, and X-linked recessive.

Cervical Cancer

Risk factors for cervical cancer include young age at first intercourse, infection with a sexually transmitted disease, and a history of multiple sex partners. More and more teenagers are presenting with these risk factors. Cervical cancer may be prevented through use of the human papillomavirus (HPV) vaccine, which is recommended to be given as a three-vaccine series to all girls and boys beginning at age 11 to 12 years (AAP, 2019). Despite the availability of the vaccine, not all girls and boys will receive it. Counsel all sexually active adolescents to seek reproductive care, which is available without parental consent in most states. The screening Papanicolaou (Pap) smear is efficient and reliable at determining abnormal cervical cells and is a key part of screening for cervical cancer (if cancer is present, the parent will have to be notified). Cervical cancer has a very high response to therapy and rate of cure if treated in its early stages. Therefore, encourage teenage girls to be responsible for their sexual health by seeking appropriate examination and screening. See Healthy People 2030

Sickle Cell Disease

SCD is a group of inherited hemoglobinopathies in which the RBCs do not carry the normal adult hemoglobin, but instead carry a less effective type. In the United States, the most common types of SCD are hemoglobin SS disease (termed sickle cell anemia [SCA]), hemoglobin SC disease, and hemoglobin sickle-β-thalassemia. SCD is most common in individuals of African, Mediterranean, Middle Eastern, and Indian decent (Centers for Disease Control and Prevention [CDC], 2017). The focus of this discussion will be on hemoglobin SS disease. Instead of Hgb AA, individuals with SCA have Hgb SS (Hgb A refers to adult hemoglobin, HgB S refers to sickle hemoglobin). In hemoglobin S, glutamic acid is replaced with valine in the hemoglobin molecule. This results in an elongated RBC with a shortened lifespan. The elongated cell is more rigid than a normal cell and becomes sickled in shape (Fig. 24.7). One in 325 African American newborns has SCD (CDC, 2017). Persons with heterozygous representation (Hgb AS) are said to have sickle cell trait and are carriers for the disorder; about 1 in 13 African American newborns have sickle cell trait (CDC, 2017). Generally, persons with sickle cell trait have only minimal health problems. SCA is transmitted via an autosomal recessive inheritance pattern. The recessive genes for sickle cell are passed on from both parents who have the gene for Hgb AS (sickle cell trait). Refer to Chapter 27 for further information on autosomal recessive gene transmission. Figure 24.8 illustrates the inheritance probability with each reproductive event. Infants with SCA are usually asymptomatic until 3 to 4 months of age because Hgb F protects against sickling. Complications of SCA include recurrent vaso-occlusive pain crises, stroke, sepsis, ACS, splenic sequestration, reduced visual acuity related to decreased retinal blood flow, chronic leg ulcers, cholestasis and gallstones, delayed growth and development, delayed puberty, and priapism (the sickled cells prevent blood from flowing out of an erect penis). Children with SCA have an increased incidence of enuresis because the kidneys cannot concentrate urine effectively (Brandow & Scott, 2019). As children reach adulthood, multiple organ dysfunction is common. Pathophysiology Significant anemia may occur when the RBCs sickle. Sickling may be triggered by any stress or traumatic event, such as infection, fever, dehydration, physical exertion, excessive cold exposure, or hypoxia (Borhade & Kondamudi, 2019). As the cells sickle, the blood becomes more viscous because the sickled cells clump together and prevent normal blood flow to the tissues of that area. The sickle-shaped RBCs cannot pass through the smaller capillaries and venules of the circulatory system (Fig. 24.9). This vaso-occlusive process leads to local tissue hypoxia followed by ischemia and may result in infarction. Pain results as circulation is decreased to the area. Pain can occur in any part of the body but is most common in the joints. Pain causes increased metabolic need by resulting in tachycardia and sometimes tachypnea, which leads to further sickling. Clumping of cells in the lungs (acute chest syndrome [ACS]) results in decreased gas exchange, producing hypoxia, which leads to further sickling. ACS and multiorgan failure are the leading common causes of death in children with SCD (Fields, Vichinsky, & DeBaun, 2018). Sequestration of blood in the spleen leads to splenomegaly and abdominal pain. Hemolysis follows sickling and leads to further anemia. The increased activity of the spleen related to RBC hemolysis leads to splenomegaly, then fibrosis and atrophy. Functional asplenia develops in early childhood (Fields et al., 2018). Therapeutic Management The therapeutic management of children with SCA focuses on preventing vaso-occlusive episodes and infection as well as other complications. Functional asplenia (decrease in the ability of the spleen to function appropriately) places the child at significant risk for serious infection with Streptococcus pneumoniae or other encapsulated organisms. Prophylactic antibiotics in the young child and appropriate immunization in all children with SCA can reduce the risk of serious infection (Fields et al., 2018). Treatment of vaso-occlusive episodes focuses on pain control. Oxygen administration is necessary during episodes of crisis to prevent additional cell sickling. Adequate hydration with intravenous fluids is critical. Close monitoring of Hgb, Hct, and reticulocytes determines the point at which transfusion of PRBCs becomes necessary. Electrolyte analysis is also necessary to ensure that appropriate amounts of electrolytes are present in the serum. When RBCs are administered, there is the potential for hemolysis of the cells, thus increasing the potassium level in the serum. Antibiotic therapy is necessary when infection is present. Box 24.3 describes additional medical treatments that are needed in some children. INSPECTION AND OBSERVATION Inspect the conjunctivae, palms, and soles for pallor and the skin for pallor, lesions, or ulcers. Note jaundice of the skin or scleral icterus. Document color and moisture of oral mucosa. Measure temperature to evaluate for infection (which can precipitate a sickling crisis). Note blood pressure, which may be decreased with severe anemia or increased with sickle cell nephropathy. Determine baseline mental status. Perform a neurologic assessment frequently, as about 11% of children with SCA will experience an overt stroke (Ambruso, Nuss, & Wang, 2018). Common laboratory and diagnostic studies ordered for the assessment of SCA include: Hemoglobin: baseline is usually 7 to 10 mg/dL; will be significantly lower with splenic sequestration, ACS, or aplastic crisis Reticulocyte count: greatly elevated Peripheral blood smear: presence of sickle-shaped cells and target cells Platelet count: increased Erythrocyte sedimentation rate: elevated Abnormal liver function tests with elevated bilirubin Prevention or Early Recognition of Vaso-Occlusive Events Seek immediate attention for ANY febrile illness. Obtain vaccinations and penicillin prophylaxis. Encourage adequate fluid intake daily Avoid temperatures that are too hot or too cold Avoid overexertion or stress Have 24-hour access to physician, nurse practitioner, or facility familiar with sickle cell care Contact medical provider promptly if you suspect a pain crisis is developing. Seek medical attention immediately if any of the following develop: Child is pale and listless Abdominal pain Limp or swollen joints Cough, shortness of breath, chest pain Increasing fatigue Unusual headache, loss of feeling, or sudden weakness Sudden vision change Painful erection that won't go down (priapism)

Contact the physician or nurse practitioner. Perform SMBG more often. Check for ketones in the urine, especially if blood glucose is elevated. Use a sliding scale to calculate the insulin dosage.

Sick-day instructions may include the following FOR DIABETES

Cognitive behavioral therapy

Teaches children to change reactions so that automatic negative thought patterns are replaced with alternative ones.

Individual therapy

The child and therapist work together to resolve the conflicts, emotions, or behavior problems. Trust is central. Structured based on the child's developmental level (e.g., may use play therapy for a younger child).

Behavioral therapy

Uses stimulus and response conditioning to manage or alter behavior. Reinforces desired behaviors, replacing the inappropriate ones. Consistency is of utmost importance.

TRAUMA

The leading cause of death in children and adolescents is unintentional injuries (Centers for Disease Control and Prevention [CDC], 2019). Automobile accidents continue to be a top cause of death in all child age groups (CDC, 2019). Childhood trauma also results from pedestrian accidents, sporting and bicycling injuries, and firearm use. Children of varying ages are susceptible to various forms of injury due to their developmental level as well as their environmental exposure. Young children rely on their caregivers to promote their safety. Young children also are not developmentally equipped to be able to recognize dangerous situations. Because pediatric injury is so common, nurses must become adept at assessment and intervention in the pediatric trauma victim. Nursing Assessment The trauma survey includes a brief health history as the child is being assessed and life-saving measures are being instituted. Health History Begin the health history by asking when the injury happened. If the child sustained a motor vehicle-related injury, ask how fast the vehicle was going. Determine if the child was appropriately restrained in the automobile. If the child was riding a bicycle, was skateboarding, or using in-line skates, was he or she wearing a helmet, kneepads, and wrist guards? Determine what interventions were performed at the scene. Was the child immobilized on a backboard to protect the cervical spine? If the child is bleeding, ask the person who transported the child to estimate the amount of blood lost. If the child experienced a fall, ask if the fall was witnessed and the height from which the child fell. Did the child fall onto a hard surface such as concrete? How did the child land: on the head or back, or did the child catch himself or herself with the hands? Younger children and boys are at higher risk for injuring their head. Did the child lose consciousness at the scene? What kind of behavior did the child exhibit after the fall? Since the fall, has the child complained of a headache or been vomiting? While obtaining a detailed history about the fall, think about the child's developmental stage. For example, does it seem plausible that a toddler might fall down the stairs? In contrast, what is the likelihood that a 2-month-old would suffer a fractured femur from a fall? Keep in mind the possibility of child abuse. Critically evaluate the reported circumstances and try to determine if the history, developmental stage of the child, and type of injury sustained match. In addition, evaluate the type of injury that the child sustained and the history given by the caregiver. For example, children who fall from significant heights often suffer skeletal fractures, but abdominal and chest injuries rarely result from falling from significant heights. Physical Examination Physical examination of the child with a traumatic injury should be approached with an evaluation of the ABCs (primary survey) first. Assess the patency of the airway and establish the effectiveness of breathing (as discussed earlier in the chapter). Examine the child's respiratory effort, breath sounds, and color. Next, evaluate the circulation. Note the pulse rate and quality. Observe the color, skin temperature, and perfusion. If bleeding has occurred, the child's circulation may become compromised. After assessing and intervening for the child's ABCs, proceed to the secondary survey. Assess for disability (D). Rapidly assess critical neurologic function. Determine the level of consciousness, pupillary reaction, and verbal and motor responses to auditory and painful stimuli. If the child is a young infant, palpate the anterior fontanel: a full and bulging fontanel signals increased intracranial pressure. The traumatized child's neurologic status may range from completely normal to comatose. Following the ABCs and D (disability) is E (exposure). Expose the child to observe the entire body for signs of injury, whether blunt or penetrating. Perform a systematic, thorough inspection of the child's body. Note active bleeding and extremity deformity, as well as any lacerations and abrasions. Observe for movement and any complaints of immobility or pain with movement. Inspect the abdomen for redness, skin discoloration, or distention. Auscultate for bowel sounds in all four quadrants. If the child is verbal, ask if he or she has any pain in the stomach. If the child is younger, ask, "Do you have a tummy ache?" If the child reports abdominal pain, ask the child to point to where it hurts. Note any guarding of the abdomen, which is an indication of abdominal pain. If bowel injury is a possibility, only light palpation is acceptable. Always assess the least tender areas first and palpate the more sensitive areas last. Laboratory and Diagnostic Testing As in other pediatric emergencies, never delay life-saving measures to wait for laboratory or diagnostic test results. In addition to routine laboratory tests, common laboratory and diagnostic tests for the pediatric trauma victim include: Type and cross-match: to assess the child's blood type before blood products are given Prothrombin time and partial thromboplastin time: to evaluate for clotting dysfunction Amylase and lipase: to identify pancreatic injury Liver function tests: to assess for liver injury Pregnancy test (in any female who has reached puberty) CT scan, ultrasound, or MRI of the head, abdomen, or extremities: to evaluate the extent of the injury Nursing Management Nursing management of the pediatric trauma victim focuses initially on the ABCs. Providing Immediate Care If head or spinal injury is suspected, open the airway using the jaw-thrust maneuver with cervical spine stabilization (see Fig. 29.4). The guidelines for basic life support recommend that if the airway cannot be opened using the jaw-thrust maneuver, it may be opened using the head tilt-chin lift maneuver since opening the airway is a priority (Atkins et al., 2015). The head and neck of a trauma victim should be stabilized manually. Clear the airway of obstruction using a large-bore suction device such as a Yankauer. If the child is breathing on his or her own, give oxygen at the highest flow possible (such as with a nonrebreathing mask). If the child is not breathing on his or her own, intervene with basic life support discussed earlier in this chapter (Atkins et al., 2015). If a BVM device is available, connect it to the oxygen source and use the bag to ventilate the child. Observe the chest rise and be careful not to overventilate, as this results in abdominal distention. Deliver breaths at a rate of one breath every 3 seconds (Atkins et al., 2015). Do not hyperventilate. In the not-too-distant past, head injury in children was managed using hyperventilation. This resulted in hypocapnia (decreased amounts of carbon dioxide in the blood). The physiologic effect of hypocapnia is the induction of vasoconstriction, which in turn results in tissue ischemia. Therefore, current management of head injury in children does not use hyperventilation. The only exception to this rule is in an acute situation, if the child is showing signs of a possible brain stem herniation, hyperventilation may be used initially and briefly. Assess the child for a strong central pulse. If the child has no pulse, initiate CPR immediately. When perfusion is compromised, administer IV fluid resuscitation. Trauma victims are more likely to require colloids or blood products due to blood loss from the injury.

Using soft wrist restraints if necessary to prevent the child from removing the ET tube Providing sedative and/or paralyzing medications Using caution when moving the child for radiographs, changing linens, and performing other procedures MONITORING THE CHILD WHO IS INTUBATED Provide ongoing and frequent monitoring of the intubated child to determine adequacy of oxygenation and ventilation as noted earlier. Once the child is intubated, the ventilatory support being provided should result in improvement in oxygen saturation and vital signs. If the child begins to exhibit signs of poor oxygenation, perform a quick assessment. Auscultate the lungs for equal air entry and determine the HR. Are the breath sounds equal? Is the HR normal for age? Perform a quick survey of the equipment and look for any disconnected tubes or kinks in the tubing. Determine oxygen saturation levels via pulse oximeter and evaluate the end-tidal CO2 color (see Box 29.1). Use the mnemonic "DOPE" for troubleshooting when the status of a child who is intubated deteriorates: D = Displacement. The ET tube is displaced from the trachea. O = Obstruction. The ET tube is obstructed (e.g., with a mucous plug). P = Pneumothorax. Usually a pneumothorax results in a sudden change in the child's assessment. The signs of a pneumothorax include decreased breath sounds and decreased chest expansion on the side of the pneumothorax. Subcutaneous emphysema may be noted over the chest. In the case of tension pneumothorax, there may be a sudden drop in HR and blood pressure. E = Equipment failure. Relatively simple problems as previously discussed, such as a disconnected oxygen supply, can cause the child to deteriorate. Culprits such as a leak in the ventilator circuit or a loss of power are other types of equipment failure that may be responsible (van der Jagt, 2017). Make sure all equipment is appropriately connected and functional. When obstruction with secretions is suspected, suction the ET tube. If the ET tube is displaced from the trachea, remove the tube if it remains in the child's mouth and begin BVM ventilation. In the case of pneumothorax, prepare to assist with needle thoracotomy. PREPARING THE INTUBATED CHILD FOR TRANSPORT Once the child is stabilized with a secure ET tube in place, prepare to transport the child. The child will be moved by stretcher to an ICU in the acute care facility or by air or land ambulance to another facility that specializes in the care of acutely ill children. Make sure that all tubes are taped securely. During transport, use portable oxygen and ventilate manually with the BVM. As the sending nurse, ensure that all laboratory results are obtained and provided to the receiving nurse. If the child is going to another facility, complete a detailed summary of the resuscitation or provide a copy of the nurse's and/or progress notes. Complete the appropriate transfer forms as determined by the institution. If the child is being transported by ambulance, the parents may not be able to accompany their child. In this case, find out as much as possible about the transport and assist the parents by giving directions to the receiving institution.

The nurse plays a key role in ensuring that the ET tube remains taped securely in place by doing the following:

Maternal age 35 years or older when the baby is born Paternal age 50 years or older Previous child, parents, or close relatives with an inherited disease, congenital anomalies, metabolic disorders, developmental disorders, or chromosomal abnormalities Consanguinity or incest Pregnancy screening abnormality, including α-fetoprotein, triple/quadruple screen, amniocentesis, or ultrasound Stillborn with congenital anomalies Two or more pregnancy losses Teratogen exposure or risk Concerns about genetic defects that occur frequently in their ethnic or racial group (e.g., those of African descent are most at risk for having a child with sickle cell anemia) Abnormal newborn screening Child born with one or more major malformations in a major organ system Child with abnormalities of growth Child with developmental delay, intellectual disability, blindness, or deafness

Those Who May Benefit From Genetic Counseling

Hypothyroidism

Tiredness/fatigue Constipation Cold intolerance Weight gain Dry, thick skin; edema of face, eyes, and hands Decreased growth

CARDIAC ARRHYTHMIAS AND ARREST

Unlike adults, in whom cardiopulmonary arrest is most often caused by a primary cardiac event, children typically have healthy hearts and thus rarely experience primary cardiac arrest. More commonly they experience cardiopulmonary arrest from gradual deterioration of respiration and/or circulation (Peterson & Lee, 2019). In particular, children experiencing a respiratory emergency or shock may deteriorate and eventually demonstrate cardiopulmonary arrest. Thus, the standard of care for managing a child in this situation is vastly different from that for an adult. Nurses should be skilled in evaluating and managing respiratory alterations and shock in children, as discussed in previous sections. Overwhelming evidence suggests that if primary respiratory compromise or shock is identified and treated in the critically ill child, a secondary cardiac arrest can be prevented. Rare exceptions do exist, however. For example, electrolyte abnormalities and toxic drug ingestions are primary insults to the cardiovascular system that may lead to a sudden cardiac arrest rather than a gradual progression. Other exceptions in which the child is at risk for a primary and sudden cardiac arrest include: History of a serious primary congenital or acquired cardiac defect Potentially lethal arrhythmias, such as prolonged QT syndrome Hypertrophic cardiomyopathy Traumatic cardiac injury or a sharp blow to the chest, known as "commotio cordis" (e.g., when a high-velocity ball hits the chest) The overwhelming majority of children rarely experience cardiac arrhythmias, so it is beyond the scope of this chapter to discuss the myriad possible complex rhythm disturbances. Therefore, this discussion will be limited to the management of emergent cardiac conditions that are more typically found in childrenPathophysiology The AHA (2016) has simplified the nomenclature used to describe pediatric cardiac compromise and has established three major categories of cardiac rhythm disturbances: Slow: bradyarrhythmias Fast: tachyarrhythmias Absent: pulseless, cardiovascular collapse The pathophysiology, causes, and therapeutic management of each of the categories of rhythm disturbances are discussed below.

SUBMERSION INJURY

Water can be a great source of fun and exercise for children and adolescents, but drowning is the second-leading cause of preventable death in children and adolescents in the United States and worldwide (World Health Organization [WHO], 2018). In warm-weather states where swimming pools are more common, drowning is the primary cause of death in young people. Most drowning deaths are preventable, and the WHO (2018) notes that "lapse in adult supervision is the single most important contributory cause for drowning." Survival and neurologic outcome of drowning depend on early and appropriate resuscitation. In recent years, with appropriate resuscitation efforts and treatment, children have demonstrated better neurologic outcomes (Chandy & Weinhouse, 2019). Pathophysiology Typically, a child who is drowning will struggle to breathe and eventually will aspirate water. Aspiration of relatively small amounts of water leads to poor oxygenation, with retention of carbon dioxide. Alveolar surfactant is depleted during the drowning event and pulmonary edema commonly occurs. Hypoxemia results in increased capillary permeability and resultant hypovolemia. Even small amounts of aspirated water may lead to pulmonary edema within an 8-hour period after the drowning episode (Chandy & Weinhouse, 2019). A drowning survivor is also at risk for renal complications due to altered renal perfusion during the hypoxemic state. Nursing Assessment Nursing assessment of the drowning survivor is crucial and must take place quickly and accurately. Health History Obtain the history rapidly while providing life-saving interventions. Ask about the circumstances of the event: Where did the incident occur? Was the child in a lake, river, ocean, or swimming pool? Was the child submerged in a toilet, bucket, or bathtub? Did someone witness the child's entry into the water? Was the water fresh or salty? Cold or warm? Is it likely the water was contaminated? Were there any extenuating circumstances, such as a diving or automobile accident, associated with the near drowning? What was the approximate length of time of the submersion? Was the child conscious or unconscious when rescued? What was done at the scene? Was CPR initiated? If so, when? If a cervical spine injury was suspected, was the cervical spine immobilized? Was an AED used? When did the child last eat (to prepare for possible intubation)? Physical Examination Evaluate airway patency and breathing. Auscultate all lung fields for signs of pulmonary edema, such as coarseness or crackles. Evaluate the heart rate, pulses, and perfusion. Note the cardiac rhythm on the monitor and report evidence of arrhythmias. Evaluate the child's neurologic status. Use a pen light to determine pupillary reaction. Use the pediatric coma score to further assess the neurologic status. Does the child open the eyes spontaneously, to stimuli, or not at all? Is there any spontaneous movement? Is the younger child crying? Can the older child speak? Measure the child's temperature, as hypothermia often occurs with near drowning. Laboratory and Diagnostic Testing While awaiting laboratory and diagnostic testing results, continue resuscitative efforts as addressed below. Laboratory and diagnostic tests typically include the following: Arterial blood gases: hypoxemia, acidosis ECG: cardiac arrhythmias Chest radiography: pulmonary edema, infiltrates Serum electrolytes: imbalance related to development of shock Nursing Management Because of the potentially devastating effects that drowning-related hypoxia has on the child's brain, airway interventions must be initiated immediately after retrieving a child from the water. Every second counts. Initial interventions for a drowning victim are always focused on the ABCs; commonly, resuscitative efforts have begun before the child arrives at the acute care facility. If a cervical spine injury is suspected (as in the case of a diving accident), provide stabilization either manually or with a cervical collar. As with any suspected neck injury, do not remove the cervical collar until injury to the cervical spine has been ruled out through a radiograph and clinical evaluation. Suction the airway to ensure airway patency. The child may have aspirated particles from a contaminated water source or emesis, a relatively common complication associated with drowning. A large-bore suction catheter (e.g., Yankauer) is an effective tool for clearing the upper airway. Administer supplemental oxygen at 100%. Children who have poor or absent respiratory effort most likely will require intubation. Insert an orogastric or nasogastric tube to decompress the stomach and prevent aspiration of stomach contents. Initiate chest compressions if a pulse is not present. Usually, the child exhibits some degree of hypothermia and will require warming. Generally, the core body temperature should be raised slowly, as warming a drowning victim too quickly may have deleterious effects. Remove any wet clothing, dry the child, and cover him or her with warmed blankets. Warm IV fluids and use other warming methods as prescribed.

HIV Infection

Worldwide, 1.8 million children younger than 18 years old are living with HIV infection and about 400 become infected with HIV each day (UNAIDS, 2016). Children acquire HIV either vertically or horizontally. Vertical transmission refers to perinatal (in utero or during birth) transmission or via breast milk. Vertical transmission may also be referred to as mother to child transmission. Horizontal transmission refers to transmission via nonsterile needles (as in intravenous drug use or tattooing) or via intimate sexual contact. With nationwide screening of blood products, HIV transmission via transfused blood products has become rare (McFarland, 2018). HIV infection in children may be further classified depending on severity of immune suppression. This classification may serve to guide healthcare planning. Infants primarily become infected through their mothers, whereas adolescents primarily contract HIV infection through sexual activity or intravenous drug use. In the United States, perinatal transmission of HIV infection has declined dramatically due to improved maternal detection and treatment, as well as newborn treatment (McFarland, 2018). Currently, there is no cure for HIV infection, though survival has improved since the advent of antiretroviral therapy (ART), particularly when used in combination). In addition to improved survival, improved growth, neurodevelopment, and immune function occur with ART (National Institutes of Health [NIH], 2019). Pathophysiology HIV affects immune function via alterations mainly in T-cell function, but it also affects B cells, natural killer cells, and monocyte/macrophage function. HIV infects the CD4 (T-helper) cells. The virus replicates itself via the CD4 cell and renders the cell dysfunctional. Immune deficiency results as the number of normal, functioning CD4 cells drops. Initially, as CD4 counts decrease, the T-suppressor (CD8) counts increase, but as the disease progresses, CD8 counts also fall. The helper T-cell function declines even in asymptomatic infants and children who have not experienced significant decreases in the CD4 cell count. The T cells lose response to recall antigens, and this loss is associated with an increased risk of serious bacterial infection (Kronman, Crowell, & Vora, 2019). Without appropriate T cell, B cell, natural killer cell, monocyte, and macrophage function, the infant's or child's immune system cannot fight infections it ordinarily could. Recurrent infection with ordinary organisms occurs more frequently in children with HIV infection, and the infections are more severe than in noninfected children. Opportunistic infections also occur in HIV-infected children, similar to those in adults with HIV infection. Current guidelines related to opportunistic infection prevention emphasize ART for prevention as well as insuring appropriate immunization and antibiotic prophylaxis for certain organisms (Kronman et al., 2019). Physical Examination Perform a thorough and complete physical examination on the child with suspected or known HIV infection. Note presence of fever. Measure weight, height or length, and head circumference (in children younger than 3 years old) and plot this information on standard growth charts, noting whether the measurements fall within the average or below the lower percentiles. Perform a developmental screening test to detect developmental delay. Inspect the oral cavity for candidiasis. Observe work of breathing (may be increased if pneumonitis or pneumonia is present). Determine level of consciousness (may be depressed if HIV encephalopathy is present). Auscultate the lungs, noting adventitious breath sounds associated with pneumonia or pneumonitis. Palpate for the presence of enlarged lymph nodes (lymphadenopathy) or swollen parotid glands. Palpate the abdomen, noting hepatosplenomegaly. Laboratory and Diagnostic Tests Common laboratory and diagnostic studies ordered for the assessment of HIV infection include: RNA or DNA—nucleic acid (NAT) or polymerase chain reaction (PCR) test: positive in infected infants who are not breastfed at 1 month of age, and in all infected infants at 6 months of age. Box 25.2 gives information on timing of testing. CD4 counts (low in HIV infection) Nursing Management Nursing care of the child with HIV infection is directed at avoiding infection, promoting compliance with the medication regimen, promoting nutrition, providing pain management and comfort measures, educating the child and caregivers, and providing ongoing psychosocial support. Children with HIV infection may access health services through funding provided by the Ryan White Comprehensive AIDS Resources Emergency Act (Health Resources and Services Administration, the HIV/AIDS Program, 2019). This federal funding provides for primary health care and other services to persons with HIV infection. The nursing process overview section beginning on page 903 lists appropriate nursing diagnoses and interventions. In addition, nursing management specific to HIV infection is covered below

Neuroblastoma,

a tumor that arises from embryonic neural crest cells, is the most common extracranial solid tumor in children (Russell & McLean, 2019). It most frequently occurs in the abdomen, mainly in the adrenal gland, but it may occur anywhere along the paravertebral sympathetic chain in the chest or retroperitoneum. When diagnosed past infancy or early toddlerhood, by the time of diagnosis, the neuroblastoma has usually already metastasized. Neuroblastoma is the second most frequently occurring solid tumor in children; 90% of cases are diagnosed before the age of 5 years (Craddock et al., 2018). Staging of the tumor at diagnosis determines the course of treatment and prognosis. Table 24.10 discusses the staging of neuroblastomas. Survival rates range from about 40% for stage 3 to 80 to 100% for stages 1, 2, and 4S (Shohet & Nuchtern, 2019). Prognosis depends on the tumor stage, age at diagnosis, location of tumor, and location of metastasis. Metastasis to the bone is a worse prognostic factor than metastasis to the skin, liver, or bone marrow. Children who relapse after initial treatment also tend to have a dismal prognosis. In addition to metastasis, complications may include nerve compression, resulting in neurologic deficits. The neuroblastoma must be removed surgically. Radiation and chemotherapy are administered to all children with neuroblastoma except those with stage I disease, in whom the tumor is completely resected. Health History Presenting signs and symptoms of neuroblastoma depend on the location of the primary tumor and the extent of metastasis. Often parents are the first to notice a swollen or asymmetric abdomen. Elicit the health history, documenting bowel or bladder dysfunction, especially watery diarrhea, neurologic symptoms (brain metastasis), bone pain (bone metastasis), anorexia, vomiting, or weight loss. Physical Examination Note neck or facial swelling, bruising above the eyes, or edema around the eyes (metastasis to skull bones). Inspect the skin for pallor or bruising (bone marrow metastasis) and document cough or difficulty breathing. Auscultate the lungs for wheezing. Palpate for lymphadenopathy, especially cervical. Palpate the abdomen, noting a firm, nontender mass. Palpate for and note hepatomegaly or splenomegaly if present. Laboratory and Diagnostic Testing Laboratory and diagnostic testing may reveal the following: CT scan or MRI to determine site of tumor and evidence of metastasis Chest radiograph, bone scan, and skeletal survey to identify metastasis Bone marrow aspiration and biopsy to determine metastasis to the bone marrow 24-hour urine collection for homovanillic acid (HVA) and vanillylmandelic acid (VMA); levels will be elevated.

Intellectual disability

refers to a functional state in which significant limitations in intellectual status and adaptive behavior (functioning in daily life) develop before the age of 18 years. Intellectual disability occurs in about 1% of the population (Pivalizza & Lalani, 2018). Although intellectual disability includes the definition of intellectual quotient (IQ) less than 70 to 75, the range of impairments associated with the low IQ is variable. Impairments in the adaptive domains of conceptual, social, or practical assist with determining the severity of intellectual disability (from mild to profound) (Pivalizza & Lalani, 2018). Long ago persons with intellectual disability (formerly termed mentally retardation) were confined to institutions and were thought to be harmful to society. In the early 21st century, most children with intellectual disability are receiving their education in public schools with their peers and living at home with their families or elsewhere in the community. Only the most severely affected individuals require separate classrooms or schools. Pathophysiology In many instances of intellectual disability the exact cause remains unknown. Prenatal errors in central nervous system development may be responsible. Other potential causes include an insult or damage to the brain during the prenatal, perinatal, or postnatal period. Prenatal exposure to alcohol or other drugs may impact cognitive development as well. Motor problems such as hyper- or hypotonia, tremor, ataxia, or clumsiness, or visual motor problems may occur concomitantly with intellectual disability. In addition, functioning at a higher level may be prevented when a learning disability or sensory processing impairment is also present. Intellectual disability may be categorized according to severity of impairment across domains. See Table 28.2. Therapeutic Management The primary goal of therapeutic management of children with intellectual disability is to provide appropriate educational experiences that allow the child to achieve a level of functioning and self-sufficiency needed for existence in the home, community, work, and leisure settings. A multidisciplinary approach may be used and the child's conceptual, social, practical, and intellectual abilities will drive school placement and the focus of the educational experience. The majority of individuals with intellectual disability require only minimal support in the school or home setting, and these individuals are able to achieve some level of self-sufficiency. Only a few children and adults with intellectual disability require extensive support and require long-term caretaking. Nursing Assessment Perform developmental screening at each healthcare visit to identify developmental delays early. Elicit the health history, determining the mental and adaptive capacities of the child's parents and other family members. Obtain a detailed pregnancy and birth history. Document sequence and age of attainment of developmental milestones. Note history of motor, visual, or language difficulties. Assess the child's health history for risk factors such as preterm or postterm birth, low birthweight, birth injury, prenatal or neonatal infection, prenatal alcohol or drug exposure, genetic syndrome, chromosomal alteration, metabolic disease, exposure to toxins (e.g., lead), head injury or other trauma, nutritional deficiency, cerebral malformation, and other brain disease or mental health disorder. Note history of or concomitant seizure disorder, orthopedic problems, speech problems, or vision or hearing deficit. For the child with known intellectual disability, assess language, sensory, and psychomotor functioning. Determine the child's ability to toilet, dress, and feed himself or herself. Ask the parents about involvement with school and community services and support. On physical examination note dysmorphic features (possibly very mild) consistent with certain syndromes (e.g., fetal alcohol syndrome; see Box 28.2). Evaluate the newborn or metabolic screening results. Computed tomography or magnetic resonance imaging of the head may be performed to evaluate the brain structure. Thyroid function tests may be ordered to rule out thyroid problems leading to developmental delay. Fetal Alcohol Syndrome Results from in utero alcohol exposure Typical facial features include low nasal bridge with short upturned nose, flattened midface, long philtrum with narrow upper lip Poor coordination, skeletal abnormalities Microcephaly Failure to thrive Hearing loss Nursing Management When children with intellectual disability are admitted to the hospital (usually for some other physical or medical condition), it is important for the nurse to continue the child's usual home routine. Follow through with feeding and motor supports that the child uses. Ensure that the child is closely supervised and remains free from harm. Allow parents time to verbalize frustrations or fears. For some families the caretaking burden is extensive and lifelong; arrange for respite care as available. Support the child's strengths and assist the child and family to follow through with therapy or treatment designed to enhance the child's functioning. Assist with the development of the child's IEP as appropriate.

Muscular dystrophy

refers to a group of inherited conditions that result in progressive muscle weakness and wasting. The muscles affected are primarily the skeletal (voluntary) muscles. Nine types of muscular dystrophy exist. All include muscle weakness over the lifetime; it is progressive in all cases but more severe in others. The various muscular dystrophies are most often diagnosed in childhood and affect a variety of muscle groups. The inheritance pattern for muscular dystrophy differs for each type but may be X-linked, autosomal dominant, or recessive. The genetic mutation in muscular dystrophy results in absence or decrease of a specific muscle protein that prevents normal function of the muscle. The skeletal muscle fibers are affected, yet there are no structural abnormalities in the spinal cord or the peripheral nerves. Table 22.3 gives specifics related to the various types of muscular dystrophy. Duchenne muscular dystrophy, the most common neuromuscular disorder of childhood, results in a shortened life expectancy (Darras, 2018). Due to advances in medical care, such as improvements in noninvasive mechanical ventilation, better management of cardiac dysfunction using angiotensin-converting enzymes (ACE) inhibitors and the use of steroids, survival into their 30s, with some cases into their 40s or 50s, is becoming more common (Darras, 2018). The incidence is about 1 in 3,600 live male births (Sarant, 2016b). For these reasons, this discussion will focus on Duchenne muscular dystrophy. Pathophysiology The gene mutation in Duchenne muscular dystrophy results in the absence of dystrophin, a protein that is critical for maintenance of muscle cells. The gene is X-linked recessive, meaning that mainly boys are affected and they receive the gene from their mothers (women are carriers but have no symptoms). Absence of dystrophin leads to generalized weakness of voluntary muscles, and the weakness progresses over time. The hips, thighs, pelvis, and shoulders are affected initially; as the disease progresses, all voluntary muscles as well as cardiac and respiratory muscles are affected. Boys with Duchenne muscular dystrophy are often late in learning to walk. As toddlers, they may display pseudohypertrophy (enlarged appearance) of the calves. During the preschool years they fall often and are quite clumsy. The affected child has difficulty climbing stairs and running and cannot get up from the floor in the usual fashion. The school-age child walks on the toes or balls of the feet with a rolling or waddling gait. Balance is disturbed significantly, and the child's belly may stick out when the shoulders are pulled back to stay upright and keep from falling over. During the school-age years it also becomes difficult for the child to raise his or her arms. Sometime between the ages of 7 and 12 years nearly all boys with Duchenne muscular dystrophy lose the ability to ambulate, and by the teen years any activity of the arms, legs, or trunk requires assistance or support (Darras, 2018). Most boys with Duchenne muscular dystrophy have some degree of intellectual impairment although intelligence level is often normal, but many may exhibit a specific learning disability (Sarant, 2016b). Therapeutic Management There is no cure for Duchenne muscular dystrophy. However, the use of corticosteroids may slow the progression of the disease (Muscular Dystrophy Association [MDA], 2019b). It is thought that prednisone helps by protecting muscle fibers from damage to the sarcolemma (defective in the absence of dystrophin). The side effects of corticosteroids are many, including weight gain, osteoporosis, and mood changes (MDA, 2019b; Sarant, 2016b). Calcium supplements and vitamin D are prescribed to prevent osteoporosis, and antidepressants may be helpful when depression occurs related to the chronicity of the disease and/or as an effect of corticosteroid use (Darras, 2018). Medications to decrease the workload of the heart, such as beta-blockers and ACE inhibitors may be prescribed. Braces or orthoses and mobility and positioning aids are necessary. As the muscles deteriorate, joints may become fixated, resulting in contractures. Contractures restrict flexibility and mobility and cause discomfort. Sometimes contractures require surgical tendon release. Spinal curvatures result over time. The boy with Duchenne muscular dystrophy who can still walk may develop lordosis. More frequently, scoliosis or kyphosis develops with this disorder. Surgical spinal fixation with rod implantation is often required by adolescence (MDA, 2019b). Additional complications include pulmonary, urinary, or systemic infections; depression; learning or behavioral disorders; aspiration pneumonia (as oropharyngeal muscles become affected); cardiac dysrhythmias; and, eventually, respiratory insufficiency and failure (as weakness of the chest muscles and diaphragm progresses) INSPECTION AND OBSERVATION Observe the child's ability to rise from the floor. A hallmark finding of Duchenne muscular dystrophy is the presence of the Gowers sign: The child cannot rise from the floor in standard fashion because of increasing weakness (Fig. 22.24). Observe the child's gait. Determine effectiveness of cough. AUSCULTATION AND PALPATION Auscultate the heart and lungs. Note tachycardia, which develops as the heart muscle weakens. Note adequacy of breath sounds, which may diminish with decreasing respiratory function. Note muscle strength with resistance testing. Palpate muscle tone. Laboratory and Diagnostic Tests Electromyography (EMG) demonstrates that the problem lies in the muscles, not in the nerves. Serum creatine kinase levels are elevated early in the disorder, when significant muscle wasting is actively occurring. Muscle biopsy provides definitive diagnosis, demonstrating the absence of dystrophin. DNA testing reveals the presence of the gene. Promoting Mobility Administer corticosteroids and calcium supplements as ordered. Encourage at least minimal weight bearing in a standing position to promote improved circulation, healthier bones, and a straight spine. Boys with Duchenne muscular dystrophy may use a standing walker or standing frame to maintain an upright position. Perform passive stretching or strengthening exercises as recommended by the physical therapist. These exercises preserve mobility and may help to prevent muscle atrophy. Use orthotic supports such as hand braces or AFOs to prevent contractures of joints. Schedule activities during the part of the day when the child has the most energy. Teach parents the use of positioning, exercises, orthoses, and adaptive equipment. Use of a wheelchair full time typically occurs by age 12 (MDA, 2019b).

Osteosarcoma

accounts for 60% of bone cancer in children, occurring most frequently in adolescents, males, and Whites (Craddock et al., 2018). It presumably arises from the embryonic mesenchymal tissue that forms the bones. The most common sites are in the long bones, particularly the proximal humerus, proximal tibia, and distal femur. Complications include metastasis, particularly to the lungs and other bones, and recurrence of disease within 3 years, primarily affecting the lungs. Surgical removal of the tumor is necessary. Chemotherapy is often administered before surgery to decrease the size of the tumor; it is usually administered after surgery to treat or prevent metastasis. Radiation is not helpful. The type of surgery performed depends on the tumor size, extent of disease outside of the bone, distant metastasis, and skeletal maturity. Radical amputation may be performed, but often teens undergo a limb-sparing procedure (Craddock et al., 2018). Nursing Assessment Obtain the health history, ascertaining when pain, limp, or limitation of motion was first noticed. Dull bone pain may be present for several months, eventually progressing to limp or gait changes. Inspect the affected limb for erythema and swelling. Palpate the affected area for warmth and tenderness and to determine the size of the soft tissue mass, if also present. As with other pediatric cancers, a thorough physical examination is warranted to detect other abnormalities that may indicate metastasis. Laboratory and diagnostic testing may include: CT scan or MRI to determine the extent of the lesion and to identify metastasis Bone scan to determine the extent of malignancy Nursing Management The adolescent will generally be quite anxious about the possibility of amputation and even about the limb salvage procedure. Present preoperative teaching at the adolescent's developmental level and ensure that he or she is included in planning treatment. Regardless of the type of surgery performed, provide routine orthopedic postoperative care. Educate the adolescent and parents on the care of the stump, if amputation is necessary, and ensure that the teen becomes competent in crutch walking. A prosthesis may be ordered. The adolescent will need time to adjust to these significant body image changes and may benefit from talking with another teen who has undergone a similar procedure. Support the teen in choosing clothing that may camouflage the prosthesis while still allowing the teen to appear fashionable. Provide emotional support, as the teen's maturity level allows him or her to understand the severity of the disease. Peer support groups are often helpful, as teens value their peers' opinions and enjoy being part of a group. Examples of comprehensive online support groups are Melissa's Living Legacy Foundation/Helping Teens Live with Cancer and The Wellness Community. Links to these resources are provided on .

Maple sugar urine disease:

affects the metabolism of amino acids. A deficiency in the enzyme that metabolizes leucine, isoleucine, and valine, which are components of protein often referred to as the branch chain amino acids. These amino acids then accumulate in the blood and cause damage to the brain No symptoms at birth, but if untreated newborns soon begin to show neurologic signs, vomiting, poor feeding, increased reflex action, and seizures. Lower intake of protein (as occurs with breastfeeding) may delay presentation of symptoms. If untreated, can lead to life-threatening neurologic damage Special low-protein diet, will vary based on severity of symptoms; limited natural protein requires a medical food product supplements such as BCAA-free Thiamine supplements may be given Diet must be continued throughout life Liver transplant has been performed with good results (child on normal diet posttransplant) www.msud-support.org: Maple Syrup Urine Disease Family Support Group

GH deficiency,

also known as hypopituitarism or dwarfism, is characterized by poor growth and short stature. GH is vital for postnatal growth. It is released throughout the day, with most secreted during sleep. GH stimulates linear growth, bone mineral density, and growth in all body tissues. GH deficiency occurs in approximately 1 in 4,000 children (Parks & Felner, 2016). Often, this condition is first identified when the physician or nurse practitioner assesses growth patterns. Children may start with a normal birth weight and length, but within a few years, the child is less than the third percentile on the growth chart. Possible complications related to GH deficiency and its treatment include altered carbohydrate, protein, and fat metabolism; hypoglycemia; glucose intolerance/ diabetes; slipped capital femoral epiphysis; pseudotumor cerebri; leukemia; recurrence of central nervous system (CNS) tumors; infection at the injection site; edema; and sodium retention. Pathophysiology GH deficiency is generally a result of the failure of the anterior pituitary or hypothalamic stimulation on the pituitary to produce sufficient GH. This lack of GH impairs the body's ability to metabolize protein, fat, and carbohydrates. Primary causes of GH deficiency include injury to, or destruction of, the anterior pituitary gland or hypothalamus. Causes include a tumor (e.g., craniopharyngioma), infection, infarction, CNS irradiation, abnormal formation of these organs in utero, or damage or trauma during birth or after. It may also be part of a genetic syndrome, such as Prader-Willi syndrome or Turner syndrome, or the result of a genetic mutation or deletion. In some cases, the cause may be idiopathic, such as nutritional deprivation or psychosocial issues, and reversible. Psychosocial dwarfism results from emotional deprivation that causes suppression of production of the pituitary hormones, resulting in decreased GH. The child is withdrawn, has bizarre eating and drinking habits such as drinking from toilets, and has primitive speech. The treatment involves removing the child from the dysfunctional environment and providing normal dietary intake. With normalized eating and behavioral habits, pituitary secretion is restored and the child dramatically catches up in growth parameters. Therapeutic Management Treatment of primary GH deficiency involves the use of supplemental GH. Secondary GH deficiency requires removal of any tumors that might be the underlying problem, followed by GH therapy. Biosynthetic GH, derived from recombinant DNA, is given by subcutaneous injection. The dosage is 0.16 to 0.24 mg/kg/week, divided into equal doses given once daily for best growth (Rogol & Richmond, 2019). Treatment continues until near-final height is achieved. This can be determined by the child deciding he or she is tall enough, a growth rate of less than 0.8 to 1 in/year, or bone age greater than 16 years in boys and greater than 14 years in girls (Parks & Felner, 2016; Rogol & Richmond, 2019). Health History The health history may reveal a familial pattern of short stature or a prenatal history of maternal disorders such as malnutrition. The past history may be significant for birth history of intrauterine growth retardation or past history of severe head trauma or a brain tumor such as craniopharyngioma. Evaluate previous and current growth patterns. Note history of chronic illness such as cardiac, kidney, or intestinal disorders that may contribute to a decreased growth pattern. Assess the child's feelings about being short. Physical Examination In addition to the linear height being at or below the third percentile on standard growth charts, the physical assessment findings may show that the child has a higher weight-to-height ratio (Fig. 26.2). Other physical findings may include prominent subcutaneous deposits of abdominal fat; a child-like face with a large, prominent forehead; a high-pitched voice; delayed sexual maturation (e.g., micropenis and undescended testes in boys); delayed dentition; delayed skeletal maturation; and decreased muscle mass. Laboratory and Diagnostic Testing The child will undergo laboratory tests to rule out chronic illnesses such as renal failure or liver and thyroid dysfunction. Laboratory and diagnostic tests used in children with suspected GH deficiency include: Bone age (as shown by radiographs) will be two or more deviations below normal. CT or MRI scans rule out tumors or structural abnormalities. Pituitary function testing confirms the diagnosis. This test consists of providing a GH stimulant such as glucagon, clonidine, insulin, arginine, or L-dopa to stimulate the pituitary to release a burst of GH. Peak GH levels below 7 to 10 ng/mL in at least two tests confirm the diagnosis. Promoting Growth The goal of growth promotion is for the child to demonstrate an improved growth rate, as evidenced by at least 3 to 5 in in linear growth in the first year of treatment without complications. With early diagnosis and treatment, the child has a better prognosis for reaching a normal adult height. Growth is usually excellent in the first year of therapy compared to later years (Parks & Felner, 2016). Treatment stops when the epiphyseal growth plates fuse. At the beginning of treatment, monitor for height increase and possible side effects related to the medications. Measure the child's height at least every 3 to 6 months and plot growth over time on standardized growth charts. Provide information to the child and family about normal development and growth rates, bone age, and growth potential. Explore with the family and child the expectations and their understanding of what is normal so they will have realistic expectations of treatment. Consult a dietitian if the child and family need assistance in providing adequate nutrition for growth and development. Educating the Family GH is available as a powder that is mixed with packaged diluents. Most are available in multidose pen delivery systems, with some systems not requiring reconstitution (Rogol & Richmond, 2019). Explain how to prepare the medication and give the correct dosage. Encourage rotation of sites in the subcutaneous tissue to prevent skin irritation. Have the family provide a return demonstration to make sure they understand correct dilution and administration of GH. Continue to provide periodic evaluation and ongoing support. Instruct the family to report headaches, rapid weight gain, increased thirst or urination, or painful hip or knee joints as possible adverse reactions. Explain to the family that the child will need to visit the pediatric endocrinologist every 3 to 6 months to monitor for growth, for potential adverse effects, and for compliance with therapy. Stress the importance of complying with the GH replacement therapy and frequent supervision by a pediatric endocrinologist. Emphasize that the success of the treatment is dependent on adherence to the regimen prescribed. Educate the family about the financial costs of therapy, which may be high; the family may need help in obtaining assistance and require referral to social services. Guide the family and child in setting realistic goals and expectations based on age, personal abilities and strengths, and the effectiveness of the GH replacement therapy. For example, the family may want to encourage the child to choose sports that are not dependent on height. Encourage the family to dress the child according to age, not size.

Oral diabetic medications,

also referred to as hypoglycemic, antidiabetic, or antihyperglycemic medications, are used in DM type 2 if glycemic control cannot be achieved by diet and exercise. Oral diabetic medications work in a variety of ways. Sulfonylureas such as glipizide [Glucotrol] and glyburide [DiaBeta]), meglitinides (such as repaglinide [Prandin]), and nateglinide (Starlix) stimulate insulin secretion by increasing the response of β cells to glucose. Another group, the biguanides, reduces glucose production from the liver. Metformin is an example of a biguanide and is an effective initial therapy unless significant liver or kidney impairment is present. Insulin sensitizers are used to help decrease insulin resistance and improve the body's ability to use insulin in the liver and skeletal tissues. α-Glucosidase inhibitors are used to slow digestion of starch in the small intestines so that glucose from the starch enters the bloodstream more slowly and can be matched more effectively with the impaired insulin response of the body. Combination agents are also available. Common adverse effects of these oral diabetic medications include headache, dizziness, flatulence and GI distress, edema, and liver enzyme elevation. If the oral hypoglycemics fail to maintain a normal glucose level, then insulin injections will be required to manage type 2 DM.

The HGP,

an international effort to produce a comprehensive sequence of the human genome, was coordinated by the U.S. Department of Energy and the National Institutes of Health. It began in October 1990 and was completed in May 2003. Its goals included: Identify all of the approximately 20,500 genes in human DNA. Determine the sequences of the 3 billion chemical base pairs that make up human DNA. Store this information in databases to make it accessible for further study. Improve tools for data analysis. Transfer related technologies to the private sector. Address the ethical, legal, and social implications of this discovery (U.S. Department of Energy Genome Programs, 2019). One goal of the HGP was to translate the findings into new and more effective strategies for the prevention, diagnosis, and treatment of genetic disorders. Current and potential applications for the HGP to health care include rapid and more specific diagnosis of disease, with hundreds of genetic tests available in research or clinical practice; earlier detection of genetic predisposition to disease; less emphasis on treating the symptoms of a disease and more emphasis on seeking the fundamental causes of the disease; new classes of drugs; avoiding environmental conditions that may trigger disease; and repair or replacement of defective genes using gene therapy. This knowledge, along with the commercialization of the technology, can change both professional and parental understanding of genetic disorders. The potential benefits of these discoveries are vast, but so is the potential for misuse. These advances challenge all healthcare professionals to consider the many ethical, legal, and social ramifications of genetics in human lives. In the near future, risk profiling based on an individual's unique genetic makeup will be used to tailor prevention, treatment, and ongoing management of health conditions. This profiling will raise issues associated with privacy and confidentiality related to workplace discrimination and access to health insurance. Issues of autonomy are equally problematic as society considers how to address the injustices that will inevitably surface when disease risk can be determined years in advance of its occurrence. Nurses will play an important role in developing policies and providing direction and support in this arena. In order to fulfill this important role, nurses need a basic understanding of genetics, including inheritance and inheritance patterns. (Visit for links to resources on the ethical, social, and legal issues surrounding human genetic research and advances.)

Inborn errors of metabolism

are a group of hereditary disorders. They are collectively common, but individually rare with most having an incidence of less than 1 in 100,000 (Sutton, 2019). Most follow an autosomal recessive inheritance pattern. They are caused by gene mutations that result in abnormalities in the synthesis or catabolism of proteins, carbohydrates, or fats. The body cannot convert food into energy as it normally would. Most inborn errors are due to a defect in an enzyme or transport protein that results in a block in the metabolic pathway. The blocked metabolic pathway allows for accumulation of the damaging by-product of the impaired metabolic process or may be responsible for a deficiency or absence of a necessary product. Presentation can occur at any time, even in adulthood, but many affected individuals exhibit signs in the newborn period or shortly after. Most inborn errors of metabolism presenting in the neonatal period are lethal if specific treatment is not initiated immediately. Newborn screening is used to detect these disorders before symptoms develop. It began in the early 1960s with screening for phenylketonuria (PKU). Technical advances and developments in screening techniques (such as tandem mass spectrometry) now allow dozens of metabolic disorders to be detected from a single drop of blood (Kemper, 2018). A child who tests positive will require additional testing to confirm the diagnosis (see Chapter 9 for more information on newborn screening for inborn errors of metabolism). Therapeutic management of these disorders varies depending on the cause of the error of metabolism, but dietary management is often a key component. The diagnostic workup usually requires a variety of specific laboratory studies and may include: Glucose: may be elevated Ammonia: may be elevated Blood gases: may have low bicarbonate and low pH, metabolic acidosis (respiratory alkalosis may also be seen, especially when high ammonia levels are present) Early diagnosis is the key to saving and improving the lives of these children.

Neurofibromatoses

are neurocutaneous genetic disorders of the nervous system that primarily affect the development and growth of neural cell tissues. There are distinct types: neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis (Korf, 2018).Neurofibromatosis 1 (von Recklinghausen disease) is the more common type and is discussed here (Korf, 2018). This disorder causes tumors to grow on nerves and produce other abnormalities such as skin changes and bone deformities. Although many affected persons inherit the disorder, nearly half of the cases are due to a new mutation (Korf, 2018). The inheritance pattern is autosomal dominant; therefore, offspring of affected individuals have a 50% chance of inheriting the altered gene and presenting with symptoms. Neurofibromatoses are due to a mutation of the neurofibromin gene on chromosome 17. The estimated prevalence is 1 in 2,600 to 3,000 live births (Korf, 2018). Complications associated with neurofibromatosis include headaches; hydrocephalus; scoliosis; cardiac defects; hypertension; seizures; vision and hearing loss; neurocognitive deficits, including learning disabilities, attention deficit disorder, fine and gross motor delays, ASD, and behavior and psychosocial issues; abnormalities of speech; and a higher risk for neoplasms. There is no cure for neurofibromatosis. Therapeutic management is aimed at controlling symptoms and managing complications. Surgical intervention can help reduce some of the bone malformations and remove painful or disfiguring tumors. These children should have a yearly physical, including blood pressure and cardiovascular examination, scoliosis screening, ophthalmology examination, developmental screening, and a neurologic examination. The disease is progressive and symptoms usually worsen over time, but it is difficult to predict the course. Most affected individuals develop mild to moderate symptoms, with non-life-threatening complications, and live a normal, productive life. Nursing Assessment On assessment the nurse may find café-au-lait spots (light-brown macules), which are the hallmark of neurofibromatosis (Korf, 2018) (Fig. 27.14). These are usually present at birth but can appear during the first year of life and usually increase in size, number, and pigmentation. They are present all over the body, particularly the trunk and extremities, while usually sparing the face. Pigmented nevi, axillary freckling, and slow-growing cutaneous, subcutaneous, or dermal neurofibromas, which are benign tumors, are other signs of neurofibromatosis. Many children with neurofibromatosis have larger than normal head circumference and are shorter than average. The severity of symptoms varies greatly, but the diagnosis is made if two or more of the clinical signs in Box 27.5 are present. Clinical Signs of Neurofibromatosis Diagnosis is made if two or more of the following are present: Six or more café-au-lait macules (light-brown spots) >5 mm in diameter in children and >15 mm in diameter in adolescents and adults Two or more neurofibromas (benign tumors) or one plexiform neurofibroma (tumor that involves many nerves) Freckling in the armpit or groin Presence of an optic glioma (a tumor on the optic nerve) Two or more growths on the iris of the eye (Lisch nodules or iris hamartomas) Abnormal development of the spine (scoliosis), the temple bone of the skull, or the tibia A first-degree relative (parent, sibling, or child) with neurofibromatosis

Brain tumors

are the most common form of solid tumor and the second most common type of cancer in children (Craddock et al., 2018). Slightly more than half of brain tumors arise in the posterior fossa (infratentorial); the rest are supratentorial in origin. The cause of brain tumors in children is unknown. Some tumors are localized (low grade), while others are of higher grade and more invasive. The prognosis depends on the location of the tumor and extent of tumor. Low-grade tumors and those that are fully resectable have a better prognosis than tumors that are located deeper within the brain or are more invasive, making them difficult to resect (Craddock et al., 2018). There are many different types of childhood brain tumors; Table 24.9 explains the most common ones. Complications of brain tumors include hydrocephalus, increased intracranial pressure, brain stem herniation, and negative effects of radiation such as neuropsychological, intellectual, and endocrinologic sequelae (Lau & Teo, 2017). Pathophysiology Though the cause of brain tumors is generally not known, the effects of brain tumors are predictable. As the tumor grows within the cranium, it exerts pressure on the brain tissues surrounding it. The tumor mass may compress vital structures in the brain, block cerebrospinal fluid flow, or cause edema in the brain. The result is an increase in intracranial pressure. Presenting symptoms vary according to location and type of tumor. Therapeutic Management The type of tumor may be identified at the time of surgery. The location of the tumor within the brain will determine the extent to which it can safely be resected. Children with hydrocephalus may require a ventriculoperitoneal shunt (see Chapter 16 for further information on hydrocephalus). Radiation is reserved for children older than age 3 years because it can have long-term neurocognitive effects (Lau & Teo, 2017). Chemotherapy is being used increasingly in the treatment of pediatric brain tumors in an attempt to avoid the use of radiation therapy. Health History Elicit a description of the present illness and chief complaint. Common signs and symptoms reported during the health history might include: Nausea or vomiting Headache Unsteady gait Blurred or double vision Seizures Motor abnormality or hemiparesis Weakness, atrophy Swallowing difficulties Behavior or personality changes Irritability, failure to thrive, or developmental delay (in very young children) Explore the child's current and past medical history for risk factors such as history of neurofibromatosis, tuberous sclerosis, or prior treatment for CNS leukemia. Physical Examination Observe for strabismus or nystagmus, "sunsetting" eyes, head tilt, alterations in coordination, gait disturbance, or alterations in sensation. Note alteration in gag reflex, cranial nerve palsy, lethargy, or irritability. Note the child's posture. Check pupillary reaction, noting size, equality, reaction to light, and accommodation. Measure blood pressure, which may decrease with increasing intracranial pressure. In the infant, palpate the anterior fontanel for bulging. Assess deep tendon reflexes, noting hyperreflexia. Laboratory and Diagnostic Tests Common laboratory and diagnostic studies ordered for the assessment of CNS tumors include: CT, magnetic resonance imaging (MRI), or positron emission tomography (PET) will demonstrate evidence of the tumor and its location within the intracranial cavity. Lumbar puncture with cerebrospinal fluid cell evaluation may show tumor markers or the presence of α-fetoprotein or human chorionic gonadotropin, which may assist in the diagnosis Providing Preoperative Care Preoperatively, care focuses on monitoring for additional increases in intracranial pressure and avoiding activities that cause transient increases in intracranial pressure. Administer dexamethasone as prescribed to decrease intracranial inflammation. Prevent straining with bowel movements by use of a stool softener. Assess the child's pain level as well as level of consciousness, vital signs, and pupillary reaction to determine subtle changes as soon as possible. Provide a tour of the intensive care unit, which is where the child will wake up after the surgery. Instruct the child and family about the possibility of intubation and ventilation in the postoperative period. If a ventriculoperitoneal shunt will be placed for the treatment of hydrocephalus caused by the tumor, provide education about shunts to the child and family (see Chapter 16). Shave the portion of the head as determined by the neurosurgeon. Some children may choose to have the entire head shaved. Sometimes children with long hair may feel better about losing it if they donate it to Locks of Love, an organization that provides hairpieces for financially disadvantaged children who have long-term medical hair loss. A link to this resource is provided on . Providing Postoperative Care Regulate fluid administration, as excess fluid intake may cause or worsen cerebral edema. Administer mannitol or hypertonic dextrose to decrease cerebral edema. Assess vital signs frequently, along with checking pupillary reactions and determining level of consciousness. Extreme lethargy or coma may be present for several days postoperatively. Increases in temperature may indicate infection or may be caused by cerebral edema or disturbance of the hypothalamus. Treat hyperthermia with antipyretics such as acetaminophen and with sponge baths, as increases in temperature increase metabolic need. Reduce the temperature slowly. Monitor for signs of increased intracranial pressure. Headache is common in the postoperative period. Assess pain level and provide analgesics as prescribed. Minimize environmental stimuli, providing a calm and quiet atmosphere. Check the head dressing for cerebrospinal fluid drainage or bleeding. Assess for and document the extent of head, face, or neck edema. Administer eye lubricant if edema prevents complete closure of the eyelids. Apply cool compresses to the eyes to decrease swelling. As the child begins to regain consciousness, he or she may be confused or combative. Restrain the child if needed to keep him or her in bed and prevent dislodging of tubes and lines.

Anxiety disorders

are the most commonly diagnosed psychiatric conditions among children and adolescents (Bennett & Walkup, 2019). Anxiety often occurs together with other mental health disorders, especially depression. Normal children experience fear, worry, and shyness. Infants fear loud noises, being startled, and strangers. Toddlers are afraid of the dark and of separation. Preschoolers fear imaginary creatures and body mutilation. School-aged children worry about injury and natural events, whereas adolescents are anxious about school and social performance. These normal fears produce a certain level of anxiety that is tolerated by most children, but it is important to distinguish normal developmentally appropriate anxiety from an anxiety disorder. Anxiety is considered to be a reaction to a perceived or actual threat. The threat may or may not be distorted by the child, and the emotional distress leads to behavioral responses. Types of Anxiety Disorders Generalized anxiety disorder (GAD) is characterized by unrealistic concerns over past behavior, future events, and personal competence. Social phobia is a disorder characterized by the child or teen demonstrating a persistent fear of speaking or eating in front of others, using public restrooms, or speaking to authorities. Selective mutism refers to a persistent failure to speak. Separation anxiety is more common in children than adolescents. In this disorder, the child may need to remain close to the parents, and the child's worries focus on separation themes. OCD is characterized by compulsions (repetitive behaviors such as cleaning, washing, or checking something), which the child performs to reduce anxiety about obsessions (unwanted and intrusive thoughts). Posttraumatic stress disorder (PTSD) is an anxiety disorder that occurs after a child experiences a traumatic event, later experiencing physiologic arousal when a stimulus triggers memories of the event. Pathophysiology Anxiety disorders are thought to occur as a result of disrupted modulation within the central nervous system. Underactivation of the serotonergic system and overactivation of the noradrenergic system are thought to be responsible for dysregulation of physiologic arousal and the resulting emotional experience. Disruption of the γ-aminobutyric acid (GABA) system may also play a role. Genetic factors may also play a role in the development of anxiety disorders, as may family and environmental influences. Additionally, abnormal thoughts or behaviors may have been learned through observation or conditioning (Bennett & Walkup, 2019). Therapeutic Management Therapeutic management of anxiety disorders generally involves the use of pharmacologic agents and psychological therapies. Anxiolytics or antidepressants are the most common pharmacologic approaches. Cognitive- behavioral therapy; individual, family, or group psychotherapy; and other behavioral interventions such as relaxation techniques may also be useful. Nursing Assessment Children and adolescents do not usually express anxiety directly. Therefore, it is very important for the nurse to evaluate somatic complaints and perform a careful health history. Health History Explore the child's current and past medical history for risk factors such as depression, anxious temperament, family history of anxiety disorders, certain environmental or life experiences (such as parental dysfunction or significant stressful event or trauma), or unstable parental attachment. Elicit the health history, noting history of social inhibition, panic, or "heart racing." Young children may display overactivity, acting out, sleep difficulties, or separation issues. Older children may describe feelings of nervousness, anger, fear, or tension and may display disruptive behavior. Ask the child to choose a number on a scale from 0 to 10 to describe how much he or she worries about things. Have the parent rank the child's worry in the same fashion and ask the parent what the child worries about most. Determine frequency of headaches and stomachaches. Use a standardized screening tool such as the Multidimensional Anxiety Scale for Children (MASC), Spence Children's Anxiety Scale (SACS), Preschool Anxiety Scale, and Beck Anxiety Inventory for Youth. Links to each of these screening tools are available on . Physical Examination Perform a complete physical examination to rule out physiologic causes of the child's symptoms. Note patches of hair loss that occur with repetitive hair twisting or pulling associated with anxiety. Evaluate for evidence of nail biting, sucking blisters, or skin erosion from finger rubbing. Inspect the entire body for signs of self-injury, which may or may not be present. Nursing Management Screen children at well-child or other healthcare visits, as well as upon admission to the hospital, for anxiety symptoms. If an anxiety disorder is suspected, refer the child to the appropriate mental health provider for further evaluation. When the child is diagnosed with an anxiety disorder and medication is prescribed, teach families about medication administration and adverse effects. Encourage and praise them for follow-through related to cognitive and behavioral therapy or psychotherapy. Provide emotional support to the child and family. Assess the family for the presence of parental anxiety or insecure attachment. Note parenting style and parent-child interactions. Not only the child but also the family will benefit from interventions that improve parent-child relationships, decrease parental anxiety, and foster parenting skills that promote autonomy in the child. Thus, refer the child and family to concurrent family therapy if needed.

X-linked inheritance disorders

are those associated with altered genes on the X chromosome. They differ from autosomal disorders. If a male inherits an X-linked altered gene, he will express the condition. This is because a male has only one X chromosome and, therefore, all the genes on his X chromosome will be expressed (the Y chromosome carries no normal allele to compensate for the altered gene). Because females inherit two X chromosomes, they can be either heterozygous or homozygous for any allele. Therefore, X-linked disorders in females express similarly to autosomal disorders.

Collapsed Rhythms (Pulseless Rhythms)

as defined by PALS, is one that produces cardiac arrest with no palpable pulse and no signs of perfusion (cardiac arrest) (AHA, 2016). Typically, the most common pulseless arrest rhythms in children are asystole and pulseless electrical activity (PEA). Asystole occurs when there is no cardiac electrical activity, commonly referred to as "a straight line" on the ECG. The child with PEA has some appreciable rhythm on the ECG but no palpable pulses. PEA may be caused by hypoxemia, hypovolemia, hypothermia, electrolyte imbalance, tamponade, toxic ingestion, tension pneumothorax, or thromboembolism. Ventricular tachycardia may also present as pulseless. Ventricular fibrillation, once thought to be rare in children, occurs in serious cardiac conditions in which the ventricle is not pumping effectively. It may develop from ventricular tachycardia. Ventricular fibrillation (VF) is characterized by variable, high-amplitude waveforms (coarse VF) or a finer, lower-amplitude waveform with no discernible cardiac rhythm (fine VF). In either case, CO is insufficient. Health History Obtain a brief health history of the child with a cardiac emergency while simultaneously assessing the child and providing life-saving interventions. Key areas to inquire about include: History of cardiac problems, asthma, chromosomal anomaly, delayed growth Symptoms such as syncope, dizziness, palpitations or racing heart, chest pain, coughing, wheezing, increased work of breathing Activity tolerance with play or feeding: Does the child get out of breath, turn blue, or squat during play? Can the child keep up with playmates? Does the infant tire with feedings? Precipitating illness, fever, unexplained joint pains, ingested medications Participation in a sport before the cardiac event occurred or injury to the chest Family history of cardiac problems, sudden death from a cardiac condition, heart attacks at a young age, chromosomal abnormalities Treatment measures performed at the scene: Was CPR initiated? Was an AED used? INSPECTION AND OBSERVATION Assess the child's airway patency and efficiency of breathing. Observe the child's color, noting circumoral pallor or duskiness or central pallor, mottling, duskiness, or cyanosis. Note any increased work of breathing, grunting, head bobbing, or apnea. Inspect the chest for barrel shape, which may be associated with chronic pulmonary or cardiac disease. Observe the pericardium for the presence of lifts or heaves. Note diaphoresis, anxious appearance, or dysmorphic features (almost 50% of children with Down syndrome also have a congenital cardiac defect [Marion & Levy, 2019]). Determine if neck vein distention is present. Inspect the fingertips for clubbing, which is indicative of chronic tissue hypoxemia. AUSCULTATION Auscultate the breath sounds, noting any crackles or wheezes. Auscultate the heart rate. If the child does not have an adequate pulse, initiate CPR. If the child has a strong, perfusing pulse, complete the cardiac assessment. Auscultate with the diaphragm of the stethoscope first and then listen with the bell. Evaluate all of the auscultatory areas, listening first over the second right interspace (aortic valve) and then over the second left interspace (pulmonic valve); next move to the left lower sternal border (tricuspid area); and finally auscultate over the fifth interspace, midclavicular line (mitral area). Evaluate the rate and rhythm of the heart. Listen for any extra sounds or murmurs. Note and describe the quality, intensity, and location of any cardiac murmurs. PERCUSSION AND PALPATION Percuss between the costal interspaces and note the heart's size. Palpate the heart to find the point of maximal impulse (PMI) and to evaluate for an associated thrill. A thrill feels like a fluttering under the fingers and is associated with cardiac pathology. Palpate and note the quality of the pulses. Evaluate each of the pulses bilaterally and note whether they are absent, faint, normal, or bounding. Compare the quality of pulses on each side of the body and also those of the upper and lower body. Note the skin temperature and evaluate the capillary refill. Nursing Management Provide oxygen at 100%. Institute cardiac monitoring and assess oxygen saturation levels via pulse oximetry. Obtain the child's preprinted code drug sheet or use the Broselow tape to obtain the child's height to estimate the ET tube sizes and medication dosages that are appropriate for the child. Always remember to intervene in this order: first airway, then breathing, then circulation. The remainder of this discussion will assume that the nurse has initiated interventions for airway and breathing as discussed earlier in the chapter.Managing Collapsed Rhythms As in any pediatric emergency, support the ABCs. Manage the airway, provide oxygen, and give fluids. In addition, if the child is pulseless or has a heart rate less than 60 bpm, initiate cardiac compressions (see "Providing Cardiopulmonary Resuscitation" earlier in the chapter). In addition, some children may require medications and/or defibrillation or synchronized cardioversion. The pulseless rhythms include ventricular tachycardia, ventricular fibrillation, asystole, and PEA. ECG characteristics and management of these rhythms are summarized in Table 29.7. Also treat the underlying causes of the arrhythmia, if known.The AHA emphasizes the importance of cardiac compressions in pulseless individuals with arrhythmias (de Caen et al., 2015). Give compressions before and immediately after defibrillation (see defibrillation section earlier in the chapter). Administer medications such as epinephrine, lidocaine, or amiodarone as ordered. In the past it was recommended that individuals who required defibrillation be given three shocks in a row, but recent research findings have shown that the individual should be defibrillated only once, followed by five cycles of CPR. For defibrillation to be most effective, cardiac compressions must be performed effectively with minimal interruptions (de Caen et al., 2015).

Tay-Sachs:

caused by insufficient activity of an enzyme called hexosaminidase A, which is necessary for the breakdown of certain fatty substances in brain and nerve cells Occurs more frequently among persons of Ashkenazi Jewish descent (with 1 in every 25 being is a carrier) Infants appear normal and healthy for the first few months of life. Then, as harmful quantities of the fatty substances (called gangliosides) build up in tissues and nerve cells and cause damage, mental and physical deterioration occur. The child becomes blind, deaf, and unable to swallow; muscles begin to atrophy; and paralysis sets in. Dementia, seizures, and an increased startle reflex may be seen. There is a late-onset type of Tay-Sachs seen in persons in their 20s and early 30s, but this is much rarer No treatment or cure. Medical management will focus on managing symptoms and maintaining comfort Anticonvulsants may be given to control seizures Death usually occurs in early childhood, by age 4 or 5 Carriers can be identified by a blood test and prenatal testing is available www.ntsad.org: National Tay-Sachs & Allied Diseases Association

Tourette syndrome

consists of multiple motor tics and one or more vocal tics occurring either simultaneously or at different times. Children are not tic-free for longer than 3 months. Tics are defined as sudden rapid recurrent stereotypical movements and/or sounds over which the child appears to have no control. Tourette syndrome affects about 0.5% of children with onset before 21 years of age (Jankovic, 2019). Comorbid (existing simultaneously) conditions such as ADHD, obsessive-compulsive disorder (OCD), and others may occur in up to 60% of children with Tourette syndrome (incidence depending upon the comorbid condition) (Jankovic, 2019). The exact pathophysiologic mechanism of Tourette syndrome has yet to be identified, though genetics does seem to play a part. Therapeutic management is highly individualized and involves psychopharmacology and behavioral therapies. Habit reversal training may help in some children. Nursing Assessment Evaluate the health history for the occurrence of tics. The child may be embarrassed or ashamed about the tics and the parents may feel fearful, angry, or guilty. Determine the presence of symptoms of comorbid conditions. Elicit the child's past health history, noting a family history of tics. Assess the child's psychosocial history to determine the extent to which the tics interfere with friendship, school performance, and self- esteem. Observe the child for simple or complex motor tics. Vocal tics such as sniffling, grunting, clicking, or word utterance may occur. Perform a thorough physical examination, which is usually normal. Nursing Management Inform families that the tics become more noticeable or severe during times of stress and less pronounced when the child is focused on an activity such as watching TV, reading, or playing a video game. Help the family to build on the child's functional behaviors and adaptive skills to improve the child's self-esteem. Encourage the family to pursue classroom accommodations such as allowing for "tic breaks," taking untimed tests or tests in another room, or using note takers or tape recording. Support the family's decisions related to medication use and therapy and provide appropriate education about the particular drugs and therapies. "Teaching the Tiger" by M. P. Dornbush and S. K. Pruitt (Hope Press) is useful for teachers of the child with Tourette syndrome. For additional support, refer families to Tourette Syndrome Association, Tourette Syndrome Foundation of Canada, or Tourette Syndrome Plus (links to these resources are provided on ).

Phenylketonuria (PKU):

deficiency in a liver enzyme leading to inability to process the essential amino acid phenylalanine properly. Phenylalanine accumulation can lead to brain damage unless PKU is detected soon after birth and treated No symptoms at birth. Most cases are identified before symptoms are present due to newborn screening (PKU is screened for in all states) If undiagnosed, most common sign is developmental delay along with vomiting, irritability, eczema-like rash, mousy odor to urine, microcephaly, seizures and behavioral abnormalities Low-phenylalanine diet Phenylalanine is found mostly in protein-containing foods such as meat and milk (including breast milk and formula) www.pkunetwork.org: Children's PKU Network www.pkunews.org: National PKU News

Homocystinuria:

deficiency in the enzyme needed to digest a component of food called methionine (an amino acid) Typically, no symptoms at birth. In the first few months of life symptoms including vomiting, poor feeding, failure to thrive, hypotonia. If undetected and untreated can lead to intellectual disability, psychiatric disturbances, developmental delays, displacement of the lens of the eye, abnormal thinning and weakness of bones, and formation of thrombi in veins and arteries that can lead to life-threatening complications such as stroke Vitamin B6 and B12 supplements and possibly other supplements, such as betaine and folic acid; Methionine-restricted diet and cystine supplements; Aspirin and dipyridamole to decrease thromboembolic events www.rarediseases.org: National Organization for Rare Disorders

Galactosemia:

deficiency in the liver enzyme needed to convert galactose, the breakdown product of lactose, which is commonly found in dairy products, into glucose. Galactose accumulation leads to damage to vital organs No symptoms at birth. If undiagnosed, newborn will have jaundice, feeding intolerance, diarrhea, and vomiting and will not gain weight. Signs and symptoms of sepsis and cataracts are often seen. If untreated, can lead to liver disease, blindness, severe intellectual disability, and death Ingestion of galactose can produce sepsis in an affected child; therefore, septic workup and antibiotics may be necessary in a child if galactose ingestion has occurred Elimination of galactose and lactose from the diet is the only treatment. Therefore, milk and dairy products will be eliminated for life www.galactosemia.org: Parents of Galactosemic Children

Mood disorders

in children include depressive disorders and bipolar disorder. It is difficult to quantify the incidence of depression in children under age 5 years, due to lack of sophistication of communication skills. In prepubertal children, about 1% to 3% are diagnosed with depression, compare to 8% in adolescents (Burstein et al., 2018). Children may experience major depressive disorder or dysthymic disorder. Girls are twice as likely to be affected as boys, particularly during the teenage years. Bipolar disorder refers to a condition of alternating manic and depressive episodes and it's incidence in children is unknown (Weeks & Spratling, 2018). During the manic episode, mood is significantly elevated and the child displays excess energy. Depression may cause significant alterations in school performance and social relationships. Anxiety disorders and disruptive behavior may occur together with depression. Substance abuse may also occur concurrently with depression. Divorce and serious family issues may contribute to the development of depression because of the ongoing stress they place on the child and their strong psychological impact. Children and adolescents experiencing depressive episodes may harm themselves purposefully (without intent to kill themselves). They may hit, cut, or burn themselves (Young, Simonton, Key, Barczyk, & Lawson, 2017). Additionally, depressed children are at risk for suicide. The Centers for Disease Control and Prevention (CDC) Youth Risk Behavior Surveillance 2017 Report revealed that 17.2% of teens had seriously considered suicide, 13.6% had a plan, and 7.4% had attempted suicide (CDC, 2018). Pathophysiology Depression in children is likely multifactorial in nature. It may result from neuroendocrine changes (particularly serotonin), genetic transmission, adverse early life events, and/or family factors. Family factors include abuse, parental early-onset mood disorder, parental substance abuse or criminality, or lack of family cohesion and increased incidence of discord (Brent & Maalouf, 2019). Therapeutic Management Children with mood disorders usually benefit from psychotherapy, often paired with pharmacologic antidepressants (Brent & Maalouf, 2019). This helps the child to deal with the psychosocial consequences of his or her behavior on his or her interpersonal relationships with others. Crisis management, parental counseling, and individual, group, or family therapy may be useful. Bipolar disorder may be treated with mood stabilizers or atypical antipsychotics, best combined with psychotherapy (Weeks & Spratling, 2018). Nursing Assessment Children with untreated depression are at high risk for suicide as well as the development of comorbid disorders such as anxiety disorders, substance abuse, eating disorders, self-harm, and disruptive behavioral disorders (such as conduct disorder or ADHD) (Burstein et al., 2018). The nurse must screen all children for the development of depression. Health History Obtain a health history from the child and separately from the parent. Evaluate the child for history of recent changes in behavior, changes in peer relationships, alterations in school performance, withdrawal from previously enjoyed activities, sleep disturbances, changes in eating behaviors, increase in accidents, or sexual promiscuity. If possible, use a standardized depression screening questionnaire; there are many available. Ask about potential stressors such as school concerns, conflicts with parents, dating issues, and abuse (physical or sexual). When bipolar disorder is suspected, the history may reveal rapid, pressured speech; increased energy; decreased sleep; flamboyant behavior; or irritability during manic episodes. Note history of weight loss, failure to thrive, or increased incidence of infections in the infant. For the toddler, note delay or regression in developmental skills, increase in nightmares, or parental reports of clinginess. The preschooler may have a history of loss of interest in newly acquired skills; manifest encopresis, enuresis, anorexia, or binge-eating; or make frequent negative self-statements. The parents of a school-aged child may report that he or she has a depressed, irritable, or aggressive mood. Assess for risk factors for suicide, which include: Previous suicide attempt Change in school performance, sleep, or appetite Loss of interest in formerly favorite school or other activities Feelings of hopelessness or depression Statements about thoughts of suicide Physical Examination Observe the infant for weepiness, withdrawn behaviors, or a frozen facial expression. Note a sad or expressionless face in the toddler or preschooler. In any age child, observe for apathy. Inspect the entire body surface for self-inflicted injuries (such as cuts or burns), which may or may not be present. The remainder of the physical examination is generally normal unless the depressed child also has a chronic medical condition. Educating and Supporting the Child and Family Teach families that mood disorders are biologic conditions, not personality flaws. Teach families how to administer antidepressant medication and to monitor for adverse effects. Encourage and praise the child's and family's efforts at following through with cognitive and behavioral therapies. Support the family throughout the process, as sometimes treatment may be lengthy. Refer parents to local support resources or to the Depression and Bipolar Support Alliance or the Child and Adolescent Bipolar Foundation. Links to these resources are provided on . Preventing Depression and Suicide Establish a trusting relationship with the children and adolescents with whom you interact, particularly in the primary care setting, school, or chronic illness clinic. This trusting relationship may encourage children or adolescents to confide feelings or problems earlier than they may do with their parents. Screen all healthy and chronically ill preteens and teens for the development of depression (Burstein et al., 2018). Use standardized screening tools such as those listed in Box 28.4. When a potential problem is identified, immediately refer the child for mental health assessment and intervention. It is important to identify depression early so that treatment can start. When a grief-inducing event is impending (such as the death of a family member), begin preventive intervention to help the child to deal with it. Provide appropriate observation for any child exhibiting suicidal ideation. See Healthy People 2030.

Eating disorders

include pica, rumination, anorexia nervosa, and bulimia. They affect a significant number of children, especially adolescents. Pica, which occurs most frequently in 2- to 3-year-olds, is an eating disorder in which the child ingests (over at least a 1-month period) a nonnutritive material such as paint, clay, or sand. Rumination is an eating disorder occurring in infants in which the baby regurgitates partially digested food or formula and expels or swallows it. The numbers of children affected by pica and rumination is not known. This discussion will focus on anorexia nervosa and bulimia, as they are more commonly encountered. Anorexia nervosa and bulimia are common eating disorders affecting primarily adolescents, though younger children may also be affected. In American society being thin is highly valued, which compounds the problem. The lifetime prevalence rate for anorexia nervosa and bulimia is about 0.3% to 0.9% each, yet these problem often arise in childhood, particularly adolescence (Yager, 2018). Anorexia nervosa is characterized by dramatic weight loss as a result of decreased food intake and sharply increased physical exercise. Bulimia refers to a cycle of normal food intake, followed by binge-eating and then purging. Typically, the adolescent with bulimia remains at a near-normal weight. Complications of anorexia and bulimia include fluid and electrolyte imbalance, decreased blood volume, cardiac arrhythmias, esophagitis, rupture of the esophagus or stomach, tooth loss, and menstrual problems. Therapeutic management may occur in either the inpatient or outpatient setting. In either case, a multidisciplinary approach including individual and family therapy as well as nutritional therapy is needed for the best chance at successful treatment. Typically, medications are not an initial or primary treatment for eating disorders (Yager, 2019). Nursing Assessment Determine the health history, noting risk factors such as family history, female gender, Caucasian race, preoccupation with appearance, obsessive traits, or low self- esteem. Adolescents with anorexia may have a history of constipation, syncope, secondary amenorrhea, abdominal pain, and periodic episodes of cold hands and feet. Parents usually note the chief complaint as weight loss. Note history of depression in the child with bulimia. Evaluate the child's self-concept, noting multiple fears, high need for acceptance, disordered body image, and perfectionism. Perform a thorough physical examination. The anorexic is usually severely underweight, with a body mass index (BMI) of less than 17. Note cachectic appearance, dry sallow skin, thinning scalp hair, soft sparse body hair, and nail pitting. Measure vital signs, noting low temperature, bradycardia, or hypotension. Auscultate the heart, noting murmur as a result of mitral valve prolapse (occurs in about one third of adolescents with anorexia). The adolescent with bulimia will be of normal weight or slightly overweight. Inspect the hands for calluses on the backs of the knuckles and split fingernails. Inspect the mouth and oropharynx for eroded dental enamel, red gums, and inflamed throat from self-induced vomiting. Careful laboratory and diagnostic evaluation of serum electrolytes and an electrocardiogram are needed in adolescents with anorexia because severe electrolyte disturbances and cardiac arrhythmias often occur. Nursing Management Most children with eating disorders can be treated successfully on an outpatient basis, though this treatment may require many months. Those with anorexia who display severe weight loss, unstable vital signs, food refusal, or arrested pubertal development or who require enteral nutrition will need to be hospitalized. Refeeding syndrome (cardiovascular, hematologic, and neurologic complications) may occur in the severely malnourished adolescent with anorexia if rapid nutritional replacement is given. Therefore, slow refeeding is essential to avoid complications. Give phosphorus supplements as ordered. Assess vital signs frequently for orthostatic hypotension, irregular and decreased pulse, or hypothermia. Consult the nutritionist for assistance with calculating caloric needs and determining an appropriate diet. Aim for a weight gain goal of 0.5 to 2 lb per week. Instruct the child and family to keep a daily journal of intake, bingeing (excessive consumption) and purging (forced vomiting) behaviors, mood, and exercise. The journal may be used as an assessment tool as well as to document progress toward recovery. Assist the child and family to plan a suitably structured routine for the child that includes meals, snacks, and appropriate physical activity. Use the physical findings associated with anorexia to educate the child about the consequences of malnutrition and how they can be remedied with adequate nutrient intake. Refer the adolescent, as appropriate, to behavior or group therapy. Assess the child's need for medical intervention for concomitant depression or anxiety (some anorexics also require psychotropic medications). Provide emotional support and positive reinforcement to the child and family. Refer the family to local support groups or online resources such as the Academy for Eating Disorders or the National Eating Disorders Association (links to these resources are provided on ).

Child maltreatment

includes physical abuse, sexual abuse, emotional abuse, and neglect. Physical abuse refers to injuries that are intentionally inflicted on a child and result in morbidity or mortality. Sexual abuse refers to involvement of the child in any activity meant to provide sexual gratification to an adult. Emotional abuse may be verbal denigration of the child or occur as a result of the child witnessing domestic violence. Neglect is defined as failure to provide a child with appropriate food, clothing, shelter, medical care, and schooling (Chiesa & Sirotnak, 2018). Statistics related to family violence as well as child physical and sexual abuse is difficult to determine, as the perpetrator usually forces the victim into silence. Children usually do not want to admit that their parent or relative has hurt them, partly from feelings of guilt and partly because they do not want to lose that parent. In 2015, 4 million referrals to child protective services were made alleging child maltreatment in 7.2 million children, yet this may be an underestimate of the prevalence of child abuse (Chiesa & Sirotnak, 2018). Abuse and violence occur across all socioeconomic levels but are more prevalent among the poor, and the largest percentage of those affected are under 3 years of age. Despite the lack of adequate statistics, it is well known that the problem of abuse and violence is widespread. Parents or caregivers are the most frequent perpetrators of abuse against children (Chiesa & Sirotnak, 2018). A history of childhood abuse is associated with the development of anxiety and depressive disorders, suicidal ideation and attempts, and alcohol and drug use. Child maltreatment may result in significant physical injury, poor physical health, and, in some cases, impaired brain development. Being a victim of abuse places children at risk for developmental and behavioral problems, decreased cognitive functioning, poor academic achievement, and deficits in relationships (Kleinschmidt, 2018). Therapeutic management of victims of abuse and violence involves physical treatment of the injury, palliative care in some cases, and intervention to preserve or restore the child's mental well-being as well as family functioning. To protect children, all states require by law that healthcare professionals report suspected cases of child abuse or neglect (Child Welfare Information Gateway, 2019). Nursing Assessment Elicit the health history, noting the chief complaint and timing of onset. Assess for appropriateness of the parent-child attachment (often altered in the case of neglect). Pay particular attention to statements made by the child's parent or caretaker. Is the history given consistent with the child's injury? Identify abuse and violence by screening all children and families using these questions: Questions for children: Are you afraid of anyone at home? Who could you tell if someone hurt you or touched you in a way that made you uncomfortable? Has anyone hurt you or touched you in that way? Questions for parents: Are you afraid of anyone at home? Do you ever feel like you may hit or hurt your child when frustrated? Assess for risk factors in children and parents or caretakers. Risk factors for abuse in children include poverty, prematurity, cerebral palsy, chronic illness, or intellectual disability. Risk factors for being abusers in parents or caretakers include a history of being abused themselves, alcohol or substance abuse, or extreme stress. Determine if the child has a history of hurting self or others (e.g., cutting), running away, attempting suicide (taking one's own life), or being involved in high-risk behaviors. Note inappropriate sexual behavior for developmental age, such as seductiveness, as this may indicate sexual abuse. Note history of chronic sore throat or difficulty swallowing, which may occur with forced oral sex or sexually transmitted infections. Document history of genital burning or itching (associated with sexual abuse). Note nonspecific symptoms of emotional abuse such as low self-confidence, sleep disturbance, hypervigilance, headaches, or stomachaches. Physical Examination Perform a gentle but thorough physical examination, using a soft touch and calm voice. Observe the parent- child interaction, noting fear or an excessive desire to please. Note the infant's level of consciousness. Vigorous shaking in the infant leads to intracranial hemorrhage and shaken baby syndrome. Inspect the skin for bruises, burns, cuts, abrasions, contusions, scars, and any other unusual or suspicious marks. Current or healed scratches or cuts may be found on parts of the body ordinarily covered by clothing in the child who self-mutilates. Burns that occur in a stocking or glove pattern, or only to the soles or palms, are highly suspicious for inflicted burns. Injuries in various stages of healing are also indicative of abuse. Bruises on the chest, head, neck, or abdomen are suspicious for abuse. Nonambulatory children infrequently experience bruises or fractures. Figure 28.1 shows injury sites usually indicative of abuse; Figure 28.2 is a photograph of a child who was beaten with an electric cord. Observe for inflammation of the oropharynx (may occur with forced oral sex). Inspect the anus and penis or vaginal area for bleeding or discharge (which may indicate sexual abuse). Laboratory and Diagnostic Tests Common laboratory and diagnostic studies ordered for the assessment of abuse include: Radiographic skeletal survey or bone scan may reveal current or past fractures. Computed tomography scan of the head may reveal intracranial hemorrhage. Rectal, oral, vaginal, or urethral specimens may reveal sexually transmitted infections such as gonorrhea or chlamydia. Nursing Management Refer suspected cases of neglect or abuse to the local child protection agency. When abusive activity is identified in the hospital, notify the social services and risk management departments. In addition to physical or palliative care needed for the injuries, abused children need to redevelop a sense of trust in adults. Provide consistent care to the abused child by assigning a core group of nurses. Child abuse requires a multidisciplinary approach that may include psychological therapy for the child and family. Role model appropriate caretaking activities to the parent or caregiver. Call attention to normal growth and development activities noted in the infant or child, as sometimes parents have expectations of child behavior that may be unrealistic based on the child's age, leading to the abuse. Praise parents and caretakers for taking appropriate steps toward getting help and for providing appropriate care to the child. Refer parents to Parents Anonymous, an organization dedicated to the prevention of child abuse through strengthening of the family (see https://parentsanonymous.org). When it is determined by the child protective team that the child would be in danger to continue living in the current situation, the child may be removed from the home. If the child is removed from the family temporarily or permanently, provide the foster or adoptive family with education necessary to assume the child's care.

X-linked dominant

inheritance occurs when a male has an abnormal X chromosome or a female has one abnormal X chromosome. All of the daughters and none of the sons of an affected male will inherit the condition, while both male and female offspring of an affected woman have a 50% chance of inheriting the condition (Scott & Lee, 2016c) (Fig. 27.4). Males are more severely affected than females. Many X-linked dominant disorders have lethal results in males (Scott & Lee, 2016c). In females, even though the gene is dominant, having a second normal X gene offsets the effects of the dominant gene to some extent, resulting in decreasing severity of the disorder. X-linked dominant disorders are rare; examples include hypophosphatemic (vitamin D-resistant) rickets and fragile X syndrome (Genetics Home Reference, 2019e).

Psoriasis

is a chronic inflammatory skin disease with periods of remission and exacerbation; control is possible with conscientious therapy. It is an immune-mediated disorder occurring in persons with a genetic predisposition. While psoriasis only affects about 2% of the adult population, between 30% and 45% of all psoriasis cases are diagnosed in childhood (Reeves, Hinds, & Antaya, 2018). Hyperproliferation of the epidermis occurs, with a rash developing at sites of mechanical, thermal, or physical trauma. Therapeutic management includes skin hydration with emollient creams, use of tar preparations, topical steroids, and ultraviolet light, among others. Narrow-band ultraviolet light has been used with some success in children with severe psoriasis. Nursing Assessment Note family history of psoriasis. Determine onset and progression of rash, as well as treatments used and the response to treatment. Question the child about pruritus, which is usually absent with psoriasis. Inspect the skin for erythematous papules that coalesce to form plaques, most frequently found on the scalp, elbows, genital area, and knees (Fig. 23.17). Facial plaques may also occur and are more common in children than adults. The plaques have a silvery or yellow-white scale and sharply demarcated borders. Layers of scale may be present, which, when removed, result in pinpoint bleeding (referred to as the Auspitz sign). Plaques on the scalp may result in alopecia. Examine the palms and soles, noting fissures and scaling. Skin biopsy, though rarely needed for diagnosis, will show hyperplastic epidermis, with thinning of the papillary dermis. Exposure to sunlight may promote healing but take care not to allow the child to become sunburned. Apply skin moisturizers or emollients daily to prevent dry skin and flare-ups. Apply topical anti-inflammatory creams as prescribed during flare-ups. Apply tar shampoos or skin preparations. Use mineral oil and warm towels to soak and remove thick plaques.

Turner Syndrome

is a common abnormality of the sex chromosome. The phenotype is female. It occurs in about 1 in 2,500 live female births (Bacino, 2019c). The abnormality is due to a loss of all or part of one of the sex chromosomes. About half of the affected individuals have only one X chromosome; the other half have a variety of abnormalities of one of their sex chromosomes and may present with the mosaic form. There is no cure for Turner syndrome. Therapeutic management will focus on managing the health issues associated with the syndrome. Children with Turner syndrome are more prone to cardiovascular problems, kidney and thyroid problems, skeletal disorders such as scoliosis and osteoporosis, hearing and eye disturbances, learning disabilities, and obesity (Bacino & Lee, 2016; Backelijauw, 2019). Infertility is usually present, but a few spontaneous pregnancies have been reported (Backelijauw, 2019). Growth hormone administration is a standard of care and usually begins when the child's height falls below the fifth percentile for healthy girls. Hormone replacement therapy may also be given to initiate puberty and complete growth. Nursing Assessment On assessment, note patterns of growth; short stature and slow growth will be a characteristic finding and often the first indication. Other physical characteristics include a webbed neck, low posterior hairline, wide-spaced nipples, edema of the hands and feet, amenorrhea, no development of secondary sex characteristics, sterility, and perceptual and social skill difficulties (Fig. 27.11). Turner syndrome can be suspected prenatally by ultrasound findings such as fetal edema or redundant nuchal skin (Backelijauw, 2019). It can be diagnosed by chromosomal analysis, either prenatally or after birth. Most children are diagnosed at birth or in early childhood when slow growth or growth failure is noted. Some cases will not be diagnosed until the pubertal growth spurt does not occur. Nursing Management Nursing management is mainly supportive. Provide education and support to the family; they need to understand that short stature and infertility are likely. Explain that intellectual disability is unlikely, but some learning disabilities may be present. Emphasize that with medical supervision and support, girls with Turner syndrome may lead healthy, satisfying lives. Counseling about infertility is important. Parents may be upset that their daughter will not be able to reproduce, so explain that many alternatives for reproduction, such as in vitro fertilization and adoption, are available. Providing resources for the family is an important nursing function. The Turner Syndrome Society of the United States provides assistance, support, and education to individuals with Turner syndrome and their families. A link to resources is provided on .

DIABETES MELLITUS DM

is a common chronic disease seen in children and adolescents. In DM, carbohydrate, protein, and lipid metabolism are impaired. The cardinal feature of DM is hyperglycemia. The major forms of diabetes are classified as: Type 1, which is caused by a deficiency of insulin secretion due to pancreatic β-cell damage Type 2, which is a consequence of insulin resistance that occurs at the level of skeletal muscle, liver, and adipose tissue with different degrees of β-cell impairment (Svoren & Jospe, 2016) Other types of diabetes secondary to certain conditions such as cystic fibrosis, glucocorticoid use (as in Cushing syndrome), infections, and certain genetic syndromes such as Down syndrome, Klinefelter syndrome, and Turner syndrome (Svoren & Jospe, 2016) Gestational diabetes (diabetes during pregnancy) The discussion for this chapter will focus on type 1 and type 2 diabetes as these are the most common types seen in children. Every year, approximately 18,000 children and adolescents are diagnosed with type 1 DM and approximately 5,000 with type 2 DM (Centers for Disease Control and Prevention [CDC], 2017). Historically, childhood DM was assumed to be type 1 and type 2 DM occurred mostly in adults. However, in recent years, type 2 DM has been reported in U.S. children and adolescents at an increasing rate (American Diabetes Association, 2019). This increase in incidence of type 2 DM among children and adolescents may be attributed to the rise in obesity and decreased physical activity in young people along with exposure to diabetes in utero. Many children with type 2 DM have a relative with type 2 DM and/or are overweight. Certain minority ethnic and racial groups, such as Hispanic and African-American children, have a higher rate of type 2 DM (CDC, 2017). See Healthy People 2030. Care of children with diabetes differs from that of adults due to physiologic and developmental differences. In children, insulin sensitivity varies as the child grows and goes through sexual maturation. Children are dependent on others for their care, and self-management ability varies among children based on age, developmental level, and individual differences. Care will be needed in a variety of settings such as school, day care, and extracurricular activities. Therefore, teaching and education will need to involve parents and other caregivers throughout childhood and adolescence. Refer to Table 26.4, which discusses developmental issues related to DM. Pathophysiology Type 1 DM is an autoimmune disorder that occurs in genetically susceptible individuals who may also be exposed to one of several environmental or acquired factors, such as chemicals, viruses, or other toxic agents implicated in the development process. As the genetically susceptible individual is exposed to environmental factors, the immune system begins a T-lymphocyte-mediated process that damages and destroys the β cells of the pancreas, resulting in inadequate insulin secretion. This deficiency of insulin leads to an inability of cells to take up glucose. The end result is hyperglycemia, glucose accumulation in the blood, and the body's inability to use its main source of fuel efficiently. The kidneys try to lower blood glucose, resulting in glycosuria and polyuria, and protein and fat are broken down for energy. The metabolism of fat leads to a buildup of ketones and acidosis (see discussion of diabetic ketoacidosis below). In type 2 DM, the pancreas usually produces insulin but the body is resistant to the insulin or there is an inadequate insulin secretion response (the body can produce insulin but not enough to meet the body's needs). Eventually, insulin production decreases (resulting from the pancreas working overtime to produce insulin), with a result similar to type 1 DM. If DM goes unrecognized or is inadequately treated (especially type 1 DM), diabetic ketoacidosis (DKA) or fat catabolism develops (a deficiency or ineffectiveness of insulin results in the body using fat instead of glucose for energy), resulting in anorexia, nausea and vomiting, lethargy, stupor, altered level of consciousness, confusion, decreased skin turgor, abdominal pain, Kussmaul respirations and air hunger, fruity (sweet-smelling) or acetone breath odor, presence of ketones in urine and blood, tachycardia, and, if left untreated, coma and death. Prolonged exposure to high blood glucose levels results in damage to blood vessels and nerves. Long-term complications of DM include failure to grow, delayed sexual maturation, poor wound healing, recurrent infections (especially of the skin), retinopathy, neuropathy, vascular complications, nephropathy, cerebrovascular disease, peripheral vascular disease, and cardiovascular disease. Consistent, well-controlled blood glucose levels can prevent these complications from developing for many years. On the other hand, poorly controlled DM can lead to complications much earlier. Therapeutic Management Treatment for DM must occur as part of a multidisciplinary healthcare team, with the family and child as a central part of that team. In the past, the child would be admitted to the hospital for 3 to 5 days for stabilization and education, but today the trend is toward treating children on an outpatient basis. Established glucose control is essential in reducing the risk of long-term complications associated with DM. Therefore, general goals for therapeutic management include: Achieving normal growth and development Promoting optimal serum glucose control, including fluid and electrolyte levels and near-normal hemoglobin A1c or glycosylated hemoglobin (which is hemoglobin that glucose is bound to and it monitors long-term control of blood sugars and diabetes) levels Preventing complications Promoting positive adjustment to the disease, with ability to self-manage in the home Self-monitoring of blood glucose (SMBG) at home is essential to improve glycemic control, to provide self-management of this disease, and to help to prevent complications such as severe hypo/hyperglycemia. The child and caregiver need to be aware of the importance of checking blood glucose regularly and more frequently when needed. Documenting blood glucose values is necessary to provide information on glucose control. This allows their physician or nurse practitioner to evaluate the effectiveness of their treatment regimen. Accuracy of SMBG is dependent on proper user technique; therefore, assessment of technique and education reinforcement are important at each visit (see Teaching Guidelines 26.2). Real-time continuous glucose monitoring system should be considered for children with type 1 DM. This may be helpful in children with hypoglycemic unawareness or frequent hypoglycemic episodes. A sensor is placed under the skin that measures interstitial glucose.Health History and Physical Examination During the initial diagnosis of DM, obtain a detailed history of family patterns and problems in school related to some of the mental and behavior changes that may occur in a hyperglycemic state (e.g., weakness, fatigue, mood changes). The child or parent may report unusual or excessive thirst (polydipsia) coupled with frequent urination (polyuria). The child may also complain of blurred vision, headaches, or bedwetting. The child with type 1 DM may have a history of poor growth. Comparison Chart 26.3 gives information about common history and physical examination findings in children with type 1 DM versus type 2 DM. In the child who is known to have DM, the health history includes any problems with hyperglycemia or hypoglycemia, diet, activity and exercise patterns, types of medications (insulin or oral diabetic medications) and dose and times of administration, ability to monitor blood glucose levels, and ability to administer insulin. Perform a thorough physical examination, noting any abnormal findings. Laboratory and Diagnostic Testing The American Diabetes Association (2019) currently recommends the use of fasting plasma glucose levels, 2-hour postprandial glucose levels, and/or hemoglobin A1c as reliable sources to diagnose diabetes (refer to Common Laboratory and Diagnostic Tests 26.1). A fasting glucose level greater than or equal to 126 mg/dL, a 2-hour plasma glucose level greater than or equal to 200 mg/dL during an oral glucose tolerance test, a random glucose level greater than or equal to 200 mg/dL (accompanied by typical symptoms of diabetes), or a hemoglobin A1c greater than 6.5% are laboratory criteria for the diagnosis of DM (Levitsky & Misra, 2019a). With each of these tests, if hyperglycemia is not explicit, the results should be confirmed with a repeat test on a different day (Levitsky & Misra, 2019a). Other laboratory and diagnostic tests include serum measurements of islet cell antibodies. Serum levels of urea nitrogen, creatinine, calcium, magnesium, phosphate, and electrolytes such as potassium and sodium may be drawn. Additional tests include a complete blood count, urinalysis, and immunoassay to measure levels of C-peptides after a glucose challenge to verify endogenous insulin secretion. The American Diabetes Association (2019) recommends screening for type 2 DM if a child presents with being overweight or obese after the onset of puberty or ≥10 years old and also has one of the following risk factors: Family history: a parent or relative with type 2 DM Ethnic background: Native American, African American, Latino, Asian American, or Pacific Islander Conditions associated with insulin resistance such as acanthosis nigricans, hypertension, dyslipidemia, or PCOS History of maternal diabetes or mother with gestational diabetes when child was in utero Typically, in children with type 1 DM and sometimes in cases of type 2 DM, glucose is regulated by subcutaneous insulin via injection or insulin pump. Often, the regimen consists of three injections of intermediate-acting insulin, with the addition of rapid-acting insulin before breakfast and dinner or three injections of a short-acting insulin with a long-acting injection at bedtime. Insulin doses are typically ordered on a sliding scale related to the serum glucose level and how the insulin works. Insulin doses and frequency are based on the needs of the child utilizing information gained from blood glucose testing. Regulating glucose can be challenging in children due to continual growth, onset of puberty, varying activity levels with unpredictable schedules, unpredictable eating habits, and the inability to always verbalize the way they are feeling. Thus, close monitoring of changing glucose levels through SMBG is essential in determining adjustments needed in insulin therapy, food intake, and activity level. Adjustment of insulin dosing based on carbohydrate intake is essential to manage blood sugar levels. The use of carbohydrate counting can help children enjoy more freedom to choose their type or amount of food and can allow them to vary their meal and snack times. It allows them to predict the rise in blood sugar that will occur after eating a specific amount or type of carbohydrate and allows them to take into account recent or expected activity level. It requires knowledge of carbohydrate amounts and calculations with each dose of short-acting insulin. Each scale will vary per child as the insulin per carbohydrate serving is calculated on an individual basis. See the Dosage Calculation Question under Developing Clinical Judgment at the end of the chapter for an example. Parents will need extensive education and continual follow-up in order to ensure successful use of this method. Teach the child and family to use proper subcutaneous injection techniques to avoid injecting into muscle or vascular spaces. Figure 26.8 shows appropriate sites for subcutaneous injection of insulin. Teach the child and family to rotate sites to avoid adipose hypertrophy (fatty lumps that absorb insulin poorly). If the child is using an insulin pump, additional education will be needed. If the child has a severe hypoglycemic reaction, administer glucagon (a hormone produced by the pancreas and stored in the liver) either subcutaneously or intramuscularly. Children under 20 kg receive 0.5 mg; children over 20 kg receive 1 mg (Svoren & Jospe, 2016). Dextrose (50%) may be given intravenously if needed. If the child is not having a severe reaction and is coherent, glucose paste or tablets may be used. Offer 10 to 15 g of a simple carbohydrate such as orange juice if the child feels some symptoms of low blood glucose and glucose monitoring indicates a drop in blood glucose level. Follow this with a more complex carbohydrate such as peanut butter and crackers to maintain the glucose level. The child with severe hyperglycemia resulting in DKA is usually treated in the pediatric intensive care unit. In the case of a child presenting with DKA to the hospital, monitor the glucose level hourly to prevent it from falling more than 100 mg/dL/hr. A too-rapid decline in blood glucose predisposes the child to cerebral edema. Fluid therapy is given to treat dehydration, correct electrolyte imbalances (sodium and potassium due to osmotic diuresis), and improve peripheral perfusion. Administration of regular insulin, given intravenously, is preferred during DKA (only regular insulin may be given intravenously [IV]). Challenges related to educating children with DM include: Children lack the maturity to understand the long-term consequences of this serious chronic illness. Children do not want to be different from their peers; having to make lifestyle changes may result in anger or depression. Poor families may not be able to afford appropriate food, medication, transportation, and telephone service. Families may demonstrate unhealthy behaviors, making it difficult for the child to initiate change because of the lack of supervision or role modeling. Family dynamics are affected because management of diabetes must occur all day, every day. Among the topics to include when teaching children and their families about diabetes management are: Self-measurement of blood glucose (Fig. 26.9) Urine ketone testing Medication use (Fig. 26.10) Oral diabetic agents Subcutaneous insulin injection or insulin pump use Subcutaneous site selection and rotation When to alter insulin dosages Use of glucagon to treat severe hypoglycemia Signs and symptoms of hypoglycemia and hyperglycemia (refer to Comparison Chart 26.4) Treatment for hypoglycemia and hyperglycemia at home or other setting such as school Monitoring for and managing complications (see above) Sick-day instructions Laboratory testing and follow-up care Diet and exercise as part of DM management (see above) Good glucose control is dependent on accurate monitoring and medication administration by the child or caregiver. Assessment of the child's or parent's technique and review of procedure and instructions should occur with each visit. Treatment of hypoglycemia and hyperglycemia may have to occur at home or in another setting such as school. In either case, someone that is trained and able to check the child's blood glucose level needs to be available. In the case of hypoglycemia, early recognition is key. Therefore, all caregivers need to be educated on the causes of hypoglycemia (such as increased physical activity, delayed meals or snacks, insulin, oral diabetic medication, illness, stress, and hormonal fluctuations) along with the signs and symptoms. The child also needs access to glucose tablets or a rapidly absorbing carbohydrate such as orange juice, as well as a snack with complex carbohydrates and protein within 30 to 60 minutes of the hypoglycemic episode. Injectable glucagon needs to be available in the case that the hypoglycemia is severe and the child is unconscious. In the event of hyperglycemia, the child needs immediate access to rapid-acting insulin injection.

Delayed puberty

is a condition of delayed secondary sexual development. In girls, it exists if the breasts have not developed by age 12 and in boys, it exists when no testicular enlargement or scrotal changes have occurred by age 14 (Crowley & Pitteloud, 2018). The most common cause for delayed puberty is a hereditary pattern of growth and development known as constitutional delay (or a "late bloomer") (Crowley & Pitteloud, 2018). In these cases, there is a family pattern of late-onset puberty. These teens will usually develop normally, just at a later time than their peers. Hypogonadism also may result when there is decreased stimulation of the gonads due to dysfunction or tumors in the hypothalamus or pituitary gland. Other causes include irradiation, infection, trauma, or genetic syndromes such as the Turner or Klinefelter syndrome. Another common cause is a chronic condition such as anorexia or cystic fibrosis. Therapeutic management involves administering testosterone (males) or estradiol-conjugated estrogen (females) in low dosages if there is no underlying situation to address. This is usually necessary for only a short time to get puberty started.

Trisomy 21 (Down syndrome)

is a genetic disorder caused by the presence of all or part of an extra 21st chromosome. It is the most common chromosomal abnormality associated with intellectual disability (Ostermaier, 2019b). One in 730 live births results in trisomy 21 (Bacino & Lee, 2016; Weremowicz, 2018). The incidence at conceptions is more than twice that but many trisomy 21 conceptions result in spontaneous abortion (Bacino & Lee, 2016). Trisomy 21 is seen in all ages, races, and socioeconomic levels, but a higher incidence is found with a maternal age older than 35 years (Bacino & Lee, 2016). This is partly explained by the fact that 90% of cases with an extra chromosome 21 originates from the mother (Weremowicz, 2018). The likelihood of having a baby with Down syndrome is around 1 in 1,000 in women younger than age 30, 1 in 350 at age 35, 1 in 85 at age 40, and 1 in 35 at age 45 (Weremowicz, 2018). Trisomy 21 is associated with some degree of intellectual disability, characteristic facial features (e.g., slanted eyes and depressed nasal bridge), and other health problems (e.g., cardiac defects, visual and hearing impairment, intestinal malformations, and an increased susceptibility to infections). The severity of these problems varies. The prognosis has been improving over the past few decades. Fundamental changes in the care of these children have resulted in longer life expectancy (around 55 to 56 years of age) and an improved quality of life (Ostermaier, 2019a). Pathophysiology Trisomy 21 is a disorder caused by nondisjunction or translocation before, at, or after conception. Each egg and sperm cell normally contain 23 chromosomes. When they join, this results in 23 pairs or 46 chromosomes. Sometimes an extra chromosome originates in the development of either the egg or the sperm, resulting in an embryo with three chromosome 21s in all cells (Fig. 27.7). This results in the characteristic features and birth defects of Down syndrome. This type and timing of nondisjunction, resulting in the presence of three chromosome 21s in all cells, is responsible for 95% of the cases of Down syndrome (Bull & the Committee on Genetics, 2011, reaffirmed 2018).In approximately 1% to 2% of cases of Down syndrome, the nondisjunction occurs after fertilization and a mixture of two cell types is seen (Bull & the Committee on Genetics, 2011, reaffirmed 2018). In these cases some cells have 47 chromosomes (due to three chromosome 21s), while others have the normal 46 chromosomes (with the normal two chromosome 21s present). This is referred to as the mosaic form of Down syndrome. Children with mosaic Down syndrome may have a milder form of the disorder, but this is not a general finding. About 3% to 4% of Down syndrome cases involve a translocation, in which part of the number 21 chromosome breaks off during cell division before or at conception and attaches (or translocates) to another chromosome (usually chromosome 14) (Bull & the Committee on Genetics, 2011, reaffirmed 2018). The cells will remain with 46 chromosomes, but this extra portion of the number 21 chromosome results in the clinical findings of Down syndrome. Cases of translocation are not associated with advanced maternal age, as is the situation with nondisjunction errors (Weremowicz, 2018). Therapeutic Management Management of Down syndrome will involve multiple disciplines, including a primary physician; specialty physicians such as a cardiologist, ophthalmologist, and gastroenterologist; nurses; physical therapists; occupational therapists; speech therapists; dietitians; psychologists; counselors; teachers; and, of course, the parents. There is no standard treatment for all children, and there is no prevention or cure. Treatment is mainly symptomatic and supportive. The overall focus of therapeutic management will be to promote the child's optimal growth and development and function within the limits of the disease. MANAGING COMPLICATIONS Children with Down syndrome need the usual immunizations, well-child care, and screening recommended by the American Academy of Pediatrics. In addition, medical management will focus on complications associated with Down syndrome. Congenital heart disease occurs in 40% to 50% of children with Down syndrome (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Cardiac problems vary from minor defects that respond to medication therapy to major defects that require surgical intervention. Children with Down syndrome also have an increased incidence of gastrointestinal disorders (Bull & the Committee on Genetics, 2011, reaffirmed 2018). These disorders vary from those that can be managed by dietary manipulation, such as celiac disease and constipation, to intestinal malformations such as Hirschsprung disease and imperforate anus, which require surgical intervention. Hearing and vision impairments also are common. More than 75% of children with Down syndrome have a hearing loss (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Otitis media is a common problem, inflicting 50% to 70% of children with Down syndrome and is often the cause for hearing loss (Ostermaier, 2019b). Sixty percent of children with Down syndrome have an eye disease (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Therefore, regular evaluation of vision and hearing is essential. Obstructive sleep apnea is present in 50% to 75% of children with Down syndrome (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Often parents are unaware their child is having sleep disturbances, so baseline testing in young children may be warranted. Children with Down syndrome have a higher incidence of thyroid disease, which can affect growth and cognitive function (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Most of these children have hypothyroidism (an underactive thyroid), but sometimes hyperthyroidism (an overactive thyroid) occurs. Periodic thyroid testing may be warranted. Children with Down syndrome are also at a higher risk for obesity and delayed dental eruptions or hypodontia (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Some studies have found an increased risk of type 1 diabetes in children with Down syndrome (Ostermaier, 2019b). Children with Down syndrome are at an increased risk for atlantoaxial instability (increased mobility of the cervical spine at the first and second vertebrae) (Bull & the Committee on Genetics, 2011, reaffirmed 2018). In most cases these children are asymptomatic, but symptoms may appear if spinal cord compression occurs. Screening for atlantoaxial instability may be appropriate, especially if the child is involved in sports. Children with Down syndrome are at an increased risk for certain hematologic problems, such as anemia, transient leukemia (mostly during the newborn period), leukemia (later onset) and polycythemia during infancy (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Children with Down syndrome also have a higher susceptibility to infection and a higher mortality rate from infectious diseases (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Precautions to prevent and monitor for infection are needed. Other potential complications include alopecia, communication disorders, and seizures. EARLY INTERVENTION THERAPY Children with Down syndrome progress through the same developmental stages as typical children, but they do so on their own timetable (refer to Table 27.3 for the average age of skill acquisition in children with Down syndrome). For example, children with Down syndrome will learn to walk, but the average child with Down syndrome walks at 24 months (vs. 12 months for a child without Down syndrome). Conditions such as hypotonia, ligament laxity, decreased strength, enlarged tongue, and short arms and legs are common in children with Down syndrome, and early intervention can help in the development of gross and fine motor skills, language, and social and self-care skills. Parents also benefit from early intervention programs in terms of support, encouragement, and information. Early intervention programs teach parents how to interact with their child while meeting the child's specific needs and encouraging development. Common Clinical Manifestations of Down Syndrome Hypotonia Short stature Flattened occiput Small (brachycephalic) head Flat facial profile Depressed nasal bridge and small nose Oblique palpebral fissures (an upward slant to the eyes) Brushfield spots (white spots on the iris of the eye) Low-set, small ears Abnormally shaped ears Small mouth Protrusion of tongue; tongue is large compared to mouth size Arched, narrow palate Hands with broad, short fingers A single deep transverse crease on the palm of the hand (simian crease) Congenital heart defect Short neck, with excessive skin at the nape Hyperflexibility and looseness of joints (excessive ability to extend the joints) Dysplastic middle phalanx of fifth finger (one flexion furrow instead of two) Epicanthal folds (small skin folds on the inner corner of the eyes) Excessive space between large and second toe Laboratory and Diagnostic Tests Down syndrome risk screening can be calculated incorporating maternal age prenatally between 11 and 14 weeks using ultrasound and blood tests (nuchal translucency and pregnancy-associated plasma protein A [PAPP-A] and human chorionic gonadotropin [hCG]), and around 16 and 18 weeks using triple/quadruple blood tests to detect AFP, hCG, estriol, and/or inhibin A levels (Bull & the Committee on Genetics, 2011, reaffirmed 2018). Ultrasound and amniocentesis or chorionic villi sampling (CVS) to detect chromosomal abnormalities can also occur prenatally. Down syndrome can be confirmed after birth using chromosome analysis (see Common Laboratory and Diagnostic Tests 27.1). Common laboratory and diagnostic studies ordered for the diagnosis and assessment of complications associated with Down syndrome include: Echocardiogram: to detect cardiac defects Vision and hearing screening: to detect vision and hearing impairments Thyroid hormone level: to detect thyroid disease Cervical radiographs: to assess for atlantoaxial instability Ultrasound: to assess for gastrointestinal malformations Promoting Growth and Development Children with Down syndrome tend to grow more slowly, learn more slowly, have shorter attention spans, and have trouble with reasoning and judgment. Their personality tends to be one of genuine warmth and cheerfulness along with patience, gentleness, and a natural spontaneity. Growth and developmental milestones for children with Down syndrome have been developed as a guide for physicians and nurse practitioners. Table 27.3 gives examples of the average age range at which these children reach selected milestones versus typical children. Promoting Nutrition Children with Down syndrome may have difficulty sucking and feeding due to lack of muscle tone. They tend to have small mouths; a smooth, flat, large tongue; and due to the underdeveloped nasal bone, chronically stuffy noses. This may lead to poor nutritional intake and problems with growth. These problems usually improve as the child gains tongue control. Use of a bulb syringe, humidification, and changing the infant's position can lessen the problem. Breastfeeding a baby with Down syndrome is usually possible, and the antibodies in breast milk can help the infant fight infections. The caregiver's hand can be used to provide additional support of the chin and throat. Speech or occupational therapists can work on strengthening muscles and assisting in feeding accommodations. Other feeding problems and failure to thrive can be related to cardiac defects and usually improve after medical management is initiated or corrective surgery performed.

Congenital Adrenal Hyperplasia CAH

is a group of autosomal recessive inherited disorders in which there is an insufficient supply of the enzymes required for the synthesis of cortisol and aldosterone. More than 90% of the cases of CAH are caused by a deficiency of 21-hydroxylase (21-OH) enzyme (White, 2016b). It is the most common type of adrenocortical insufficiency seen in children with an incidence of about 1 in 15,000 to 20,000 live births (White, 2016b). Therefore, our discussion will focus on this type. This condition can be life threatening and requires prompt diagnosis and treatment after birth (White, 2016b). Complications of CAH include hyponatremia, hyperkalemia, hypotension, shock, hypoglycemia, short adult stature, and adult testicular tumor in males. Pathophysiology Classic 21-OH enzyme deficiency results in blocking the production of adrenal mineralocorticoids and glucocorticoids. A reduction of cortisol occurs, which leads to increased adrenocorticotropic hormone (ACTH) production by the anterior pituitary to stimulate adrenal gland production. Prolonged oversecretion of ACTH causes enlargement or hyperplasia of the adrenal glands and excess production of androgens, leading to male characteristics appearing early or inappropriately. In males, the enzyme deficiency of 21-OH with excessive androgen secretion leads to a slightly enlarged penis, which may become adult sized by school age, and a hyperpigmented scrotum. Males do not have obvious signs at birth but may enter puberty by 2 to 3 years of age. The female fetus develops male secondary sexual characteristics; thus, CAH causes ambiguous genitalia in girls (White, 2016b). The clitoris is enlarged and may resemble the penis, the labia have a rugated appearance, and the labial folds are fused, but the internal reproductive organs, including the ovaries, fallopian tubes, and uterus, are normal.A milder form of 21-OH deficiency becomes evident later (genitals are normal at birth), in the toddler or preschool years, with premature adrenarche (early sexual maturation), pubic hair development, accelerated growth velocity, advanced bone age, early closure of the epiphyseal plates resulting in short stature as an adult, acne, and hirsutism (excessive body hair growth). Males usually have normal fertility while females may have lower fertility. Aldosterone insufficiency also leads to fluid and electrolyte imbalances, such as hyponatremia, hyperkalemia, and hypotension due to depletion of extracellular fluid. Cortisol insufficiency leads to hypoglycemia. Therapeutic Management The goal of treatment is to stop excessive adrenal secretion of androgens while maintaining normal growth and development. Most children with 21-OH deficiency will take a glucocorticoid such as hydrocortisone and the mineralocorticoid fludrocortisone (Florinef) for life. Infants may also require sodium supplementation. When the medications are taken at physiologic doses, there are no adverse effects, but if the drug levels become elevated, hypertension, growth impairment, and acne become a problem. Regular follow-up care and appropriate titration maintain the dose at appropriate levels to allow normal growth and development.Usually when girls are born with ambiguous genitalia, standard medical treatment is to correct the external genitalia and establish adequate sexual functioning. Gender can be determined by karyotyping chromosomes. Typically, a reduction of the clitoris and opening of the labial folds is done within 2 to 6 months of life, with further surgeries at puberty (White, 2016b). Some argue that surgery should be delayed until the child is old enough to decide what kind of correction (if any) should be performed (White, 2016b). The decision to intervene immediately or to delay treatment is a complex one that raises many concerns for the family. The medical team needs to be sensitive to this and provide psychosocial support to the parents and family (Merke, 2018). Health History and Physical Examination Obtain the health history, noting history of abnormal genitalia at birth in the infant. In the toddler or preschooler, note history of accelerated growth velocity and signs of premature adrenarche. Upon inspection of the infant's genitalia, note a large penis in the boy and ambiguous genitalia in the girl (Fig. 26.6). When observing the toddler or preschooler, note pubic hair development, acne, and hirsutism. Laboratory and Diagnostic Testing The most common type of CAH, 21-OH enzyme deficiency, is detected by newborn metabolic screening. If this test has not been done or the results are unavailable, obtain random hormone levels or levels associated with ACTH stimulation. Radiographs reveal advanced bone age and premature closure of epiphyseal plates of the long bones. Nursing Management In addition to the common interventions associated with endocrine disorders in childhood (see Nursing Process Overview earlier in the chapter), nursing management of the infant or child with CAH focuses on preventing and monitoring for acute adrenal crisis, helping the family to understand the disease, providing education to the child and family about the importance of maintaining hormone supplementation, and providing emotional support to the family. Preventing and Monitoring for Acute Adrenal Crisis Providing ongoing assessment of the ill or hospitalized child with a history of CAH is crucial in order to recognize the development of life-threatening acute adrenal crisis. Signs and symptoms of acute adrenal crisis include persistent vomiting, dehydration, hyponatremia, hyperkalemia, hypotension, tachycardia, and shock. Monitor children with CAH closely and notify the physician or nurse practitioner if adrenal crisis is suspected. If signs and symptoms of adrenal crisis develop, the child will receive intravenous steroids, such as hydrocortisone, and aggressive fluid resuscitation, often using 5% dextrose in normal saline (D5NS), to correct electrolyte imbalances. Educating the Family About the Medication Regimen Medication will be required throughout the child's life because cortisone is necessary to sustain life. Teach the family the appropriate oral dosages of hydrocortisone and fludrocortisone. It is critical to maintain tight control over the levels of these medications in the bloodstream. Either underdosing or overdosing may lead to short adult stature. Low levels of the hormones may also result in adrenal crisis (as discussed above). These drugs are usually given orally but in some instances will need to be given via intramuscular injections. Teach families how to give hydrocortisone intramuscularly if the child is vomiting and cannot keep down oral medication. If the child becomes ill, is under stress, or needs surgery, additional doses of medications may be required. Encourage the family to obtain a medical ID bracelet or necklace for the child. Providing Family Support Make sure the family of a newborn with ambiguous genitalia feels comfortable asking questions and exploring their feelings. There are many issues to consider, such as whether the family will reassign the child's sex or raise the child with the original assignment at birth. The birth certificate may pose a problem if the state requires identification of sex. Cultural attitudes, the parents' expectations, and the extent of family support influence the family's response to the child and the decision-making process related to sex assignment and surgical correction. If corrective surgery is immediately decided upon, then typical surgical concerns for newborns will need to be addressed. In general, laypeople do not understand adrenal function and what this diagnosis may mean to the family. Provide families with privacy to discuss these issues, and offer emotional support. When referring to the infant, use terms such as "your baby" instead of the pronouns "he," "she," or "it" and describe the genitals as "sex organs" instead of "penis" or "clitoris." Refer families to the CARES Foundation (Congenital Adrenal Hyperplasia Research, Education, and Support) and the Magic Foundation for additional support and resources. Links to these r

Bradyarrhythmias

is a heart rate significantly slower than the normal heart rate for that age. Bradycardia in children is most commonly sinus bradycardia. In other words, there is not a cardiac nodal abnormality associated with the slowed heart rate. In sinus bradycardia, the P waves and QRS complex remain normal on the ECG. Brief dips in heart rates can be normal, such as when the child sleeps. Children are also susceptible to brief drops in heart rate that are associated with vagal stimulation. For example, passing an orogastric tube down the esophagus of a young infant may induce a temporary bradycardic response. These normal decreases in the child's heart rate should recover with or without stimulation and are not normally associated with signs of altered perfusion. Less commonly, children manifest bradycardia as a result of cardiac abnormalities and heart block. Infants with bradycardia related to heart block may exhibit poor feeding and tachypnea, whereas older children may demonstrate fatigue, dizziness, and syncope. Comparison Chart 29.2 compares the causes of sinus bradycardia and heart block in children. In contrast, the child with a serious and possibly life-threatening bradyarrhythmia will have a heart rate below 60 bpm, with signs of altered perfusion. The most common causes of profound bradycardia in children are respiratory compromise, hypoxia, and shock. Sustained bradycardia is commonly associated with arrest. It is an ominous sign and should be taken seriously.Managing Bradyarrhythmias The management of sinus bradycardia is focused on remedying the underlying cause of the slow heart rate. Since hypoxia is the most common cause of sustained bradycardia, oxygenation and ventilation are necessary. The newborn is particularly susceptible to bradycardia in relation to hypoxemia. Continue to reassess the child to determine if the bradycardia improves with adequate oxygenation and ventilation. If bradycardia persists, administer epinephrine and/or atropine as ordered. Epinephrine is the drug of choice for the treatment of persistent bradycardia. Other causes of bradycardia such as hypothermia, head injury, and toxic ingestion are managed by addressing the underlying condition. Warming the hypothermic child may restore a normal sinus rhythm. Children with head injury may have bradycardia without any cardiac involvement, and with successful management of the head injury, the bradycardia will resolve. Antidotes to toxins may be necessary in children whose bradycardia is the result of a toxic ingestion.

Osteogenesis imperfecta

is a genetic bone disorder that results in low bone mass, increased fragility of the bones, and other connective tissue problems such as joint hypermobility, resulting in instability of the joints. All of these contribute to fracture occurrence. Dentinogenesis imperfecta may also occur. This is characterized by the tooth enamel wearing easily and brittle and discolored teeth. The disorder usually occurs as a result of a defect in the collagen type 1 gene, usually through an autosomal dominant inheritance pattern but some types are inherited in a recessive manner (Butler, 2019). The types of osteogenesis imperfecta range from mild to severe connective tissue and bone involvement (Table 22.2). Subtypes A and B exist depending on (A) the absence or (B) the presence of dentinogenesis imperfecta (Marini, 2016). In children with moderate to severe disease, fractures are more likely to occur, and short stature is common. In addition to multiple fractures, additional complications include early hearing loss, acute and chronic pain, scoliosis, and respiratory problems. Therapeutic Management The goal of medical and surgical management is to decrease the incidence of fractures and maintain mobility. Bisphosphonate administration is used for moderate to severe disease. Fracture care is often required. Physical therapy and occupational therapy prevent contractures and maximize mobility. Standing with bracing is encouraged. Lightweight splints or braces may allow the child to bear weight earlier. Severe cases may require surgical insertion of rods into the long bones. Nursing Assessment Elicit a health history, which may reveal a family history of osteogenesis imperfecta, a pattern of frequent fractures, or screaming associated with routine care and handling of the newborn. Inspect the eyes for sclerae that have a blue, purple, or gray tint. Note abnormalities of the primary teeth. Inspect skin for bruising and note joint hypermobility with active range of motion. Laboratory tests may include a skin biopsy (which reveals abnormalities in type 1 collagen) or DNA testing (locating the genetic mutation). Nursing Management Handle the child carefully and teach the family to avoid trauma (Teaching Guidelines 22.3). Refer families to the Osteogenesis Imperfecta Foundation (a link to which can be found on ), which provides access to multiple resources as well as clinical trials. The site includes an online store with excellent books and booklets. Encourage safe mobility. Reinforce physical and occupational therapists' recommendations for promotion of fine motor skills and independence in activities of daily living, as well as use of adaptive equipment and appropriate promotion of mobility. Adapted physical education is important to promote mobility and maintain bone and muscle mass. If the child is ambulatory, even with adaptive equipment use, walking is a good form of exercise. Swimming and water therapy are appropriate, allowing independent movement with little fracture risk. Preventing Injury in Children With Osteogenesis Imperfecta Never push or pull on an arm or leg. Do not bend an arm or leg into an awkward position. Lift a baby by placing one hand under the legs and buttocks and one hand under the shoulders, head, and neck. Do not lift a baby's legs by the ankles to change the diaper. Do not lift a baby or small child from under the armpits. Provide supported positioning. If fracture is suspected, handle the limb minimally.

Ewing sarcoma

is a highly malignant bone tumor. It is rarer than osteosarcoma, accounting for about 30% of childhood bone tumors (Craddock et al., 2018). It occurs most frequently in the pelvis or femur (Russell & McLean, 2019). About 25% of children demonstrate metastasis; the lungs, bone, and bone marrow are the most common sites (Craddock et al., 2018). The prognosis for Ewing sarcoma depends on the extent of metastasis. Radiation, chemotherapy, and surgical excision are usually used in combination. Treatment varies depending on the site of the primary tumor and the extent of metastasis at diagnosis. Myeloablative chemotherapy (which destroys the child's marrow) may be used for metastatic disease, followed by a stem cell rescue transplant. Nursing Assessment Explore the history for intermittent pain that progressively worsens. Note a possible history of fever. Eventually the pain becomes constant and severe, sometimes interrupting sleep. Note the presence of swelling or erythema at the tumor site. CT scan or MRI of the affected area will reveal the extent of the tumor. Biopsy is necessary to establish the diagnosis. CT scan of the chest, bone scan, and bilateral bone marrow aspiration with biopsy determine the extent of metastasis. Nursing Management Before treatment begins, discourage active play or weight bearing on the affected extremity to avoid pathologic fracture at the tumor site. Nursing management focuses on addressing the adverse effects of treatment (refer to the Nursing Process Overview section). Give honest and direct answers to teens with Ewing sarcoma who ask questions about their disease. These children will undergo intensive therapy and spend a great deal of time in the hospital. Depending on the age of the child, fantasy play, art or pet therapy, drama, writing, humor, and/or music may help the child to work through the psychological impact of this disease. Refer to the Nursing Process Overview section earlier in the chapter for additional interventions, which should be individualized depending on the child's and family's response to the disease process and treatment

Systemic lupus erythematosus (SLE)

is a multisystem autoimmune disorder that affects both humoral and cellular immunity. SLE can affect any organ system, so the onset and course of the disease are quite variable. Only 5% of SLE cases present in children and it is rarely diagnosed before age 9 years, primarily being diagnosed around the age of puberty (Haftel, 2019). SLE is more common in non-Caucasians, and young people have a greater relative risk of death from SLE (Klein-Gitelman, 2019). Pathophysiology In SLE, autoantibodies react with the child's self-antigens to form immune complexes. The immune complexes accumulate in the tissues and organs, causing an inflammatory response resulting in vasculitis. Injury to the tissues and pain occur. Since SLE may affect any organ system, the potential for alterations or damage to tissues anywhere in the body is significant. In some cases, the autoimmune response may be preceded by a drug reaction, an infection, or excessive sun exposure. In children, the most common initial symptoms are hematologic, cutaneous, and musculoskeletal in origin. The disease is chronic, with periods of remission and exacerbation (flares). Common complications of SLE include ocular or visual changes, cerebrovascular accident (CVA), transverse myelitis, immune complex-mediated glomerulonephritis, pericarditis, valvular heart disease, coronary artery disease, seizures, and psychosis. Therapeutic Management Therapeutic management focuses on treating the inflammatory response. Nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and antimalarial agents are often prescribed for the child with mild to moderate SLE. The child with severe SLE or frequent flare-ups of symptoms may require high-dose (pulse) corticosteroid therapy or drugs. When end-stage renal failure develops as a result of glomerulonephritis, dialysis becomes necessary. Health History Elicit a description of the present illness and chief complaint. Common signs and symptoms reported during the health history are history of fatigue, fever, weight changes, pain or swelling in the joints, numbness, tingling or coolness of extremities, or prolonged bleeding. Assess for risk factors, which include female sex; family history; African, Native American, or Asian descent; recent infection; drug reaction; or excessive sun exposure. Physical Examination Measure temperature and document the presence of fever. Observe the skin for malar rash (a butterfly-shaped rash over the cheeks); discoid lesions on the face, scalp, or neck; changes in skin pigmentation; or scarring (Fig. 25.3). Document alopecia. Inspect the oral cavity for painless ulcerations and the joints for edema. Measure blood pressure, as hypertension may occur with renal involvement. Auscultate the lungs; adventitious breath sounds may be present if the pulmonary system is involved. Palpate the joints, noting tenderness. Palpate the abdomen and note areas of tenderness (abdominal involvement is more common in children with SLE than in adults). Box 25.4 lists common clinical findings in SLE. Nursing Management Nursing management of the child or adolescent with SLE is long term and supportive. Management focuses on preventing and monitoring for complications. Educate the child and family about the importance of a healthy diet, regular exercise, and adequate sleep and rest. Administer NSAIDs, corticosteroids, and antimalarial agents as ordered for the child with mild to moderate SLE and pulse corticosteroid therapy or immunomodulators to the child with severe SLE or frequent flare-ups. Assist families to deal with this chronic illness and adolescents with their struggles with body image and independence. Refer families to support services such as the Lupus Alliance of America and the Lupus Foundation of America, links to which can be found on CoursePoint. Preventing and Monitoring for Complications Teach families to apply sunscreen (minimum SPF 15) to their child's skin daily to prevent rashes resulting from photosensitivity. Instruct the child and family to protect against cold weather by layering warm socks and wearing gloves when outdoors in the winter. If the child is outside for extended periods during the winter months, inspect the fingers and toes for discoloration. Watch for the development of nephritis by evaluating blood pressure, serum BUN and creatinine levels, and urine output and monitoring for hematuria or proteinuria. Ensure that yearly vision screening and ophthalmic examinations are performed to preserve visual function should changes occur.

Leukemia

is a primary disorder of the bone marrow in which the normal elements are replaced with abnormal WBCs. Normally, lymphoid cells grow and develop into lymphocytes, and myeloid cells grow and develop into RBCs, granulocytes, monocytes, and platelets. Leukemia may develop at any time during the usual stages of normal lymphoid or myeloid development. Leukemia may be classified as acute or chronic, lymphocytic or myelogenous. Acute leukemias are rapidly progressive diseases affecting the undifferentiated or immature cells; the result is cells without normal function. Chronic leukemias progress more slowly, permitting maturation and differentiation of cells so that they retain some of their normal function. Acute leukemias, including ALL and acute myelogenous leukemia (AML), occur much more commonly in children and adolescents than do chronic leukemias (Russell & McLean, 2019). Therefore, they will be the focus of the discussion below. Complications of leukemia include metastasis (spread of cancer to other sites) to the blood, bone, CNS, spleen, liver, or other organs and alterations in growth. Late effects include problems with neurocognitive function and ocular, cardiovascular, or thyroid dysfunction. With advances in treatment over the past 50 years, most cases of childhood leukemia are curable. However, children who experience relapse or present with advanced disease have a poorer prognosis (Russell & McLean, 2019).

Severe combined immune deficiency (SCID)

is a rare X-linked or autosomal recessive disorder; it can occur in girls or boys. SCID is characterized by absent T-cell and B-cell function. There are at least five types of SCID, classified according to the exact genetic defect. SCID is a potentially fatal disorder requiring emergency intervention at the time of diagnosis. Gene therapy provides some promise for the future treatment of SCID, but until then hematopoietic cell transplantation is necessary (Heimall, 2019). IVIG infusions may help decrease the number of infections until bone marrow or stem cell transplantation can be done (Heimall, 2019). Certain children with SCID (adenosine deaminase enzyme deficiency) may benefit from lifelong subcutaneous adenosine deaminase enzyme replacement. In addition, long-term antibiotic therapy helps to contain chronic infections in some children with SCID. Nursing Assessment Note history of chronic diarrhea and failure to thrive. Note history of severe infections beginning early in infancy. Inspect the mouth for persistent thrush. Auscultate the lungs, noting adventitious sounds related to pneumonia. Laboratory findings include very low levels of all of the immunoglobulins. Nursing Management Preventing infection is critical. Teach the family to practice good hand washing. The child must not be exposed to persons outside the family, particularly young children. Instruct families to administer prophylactic antibiotics if prescribed. Educate families that the child should not receive live vaccines. Encourage adequate nutrition; supplemental enteral feedings may be necessary in the child with poor appetite. Administer IVIG infusions as prescribed and monitor for adverse reactions (refer to the nursing management section for hypogammaglobulinemia for further information related to IVIG administration). If the child receives a bone marrow transplant (human leukocyte antigen [HLA]-matched sibling is preferred), provide posttransplant care as outlined in Chapter 24. Teach the family that severe cutaneous human papillomavirus infection may occur after stem cell transplantation (even years later). Refer the family for genetic counseling. Provide ongoing support; this is a difficult disease for families to cope with and the therapy required is lifelong.

Rhabdomyosarcoma

is a soft tissue tumor that usually arises from the embryonic mesenchymal cells that would ordinarily form striated muscle. The most common locations for the tumor are the head and neck, genitourinary tract, and extremities (Fig. 24.17). The tumor is highly malignant and spreads via local extension or through the venous or lymphatic system, with the lung being the most common site for metastasis. Diagnosis is usually made between 2 and 5 years of age, with 70% of all rhabdomyosarcomas diagnosed by age 10 years (Craddock et al., 2018). The prognosis is based on the stage of the disease at diagnosis. Box 24.4 explains the staging of rhabdomyosarcoma. Prognosis is generally favorable for stage I disease (up to 95% long-term survival, yet drops to a 25% to 30% 5-year survival rate for those with metastatic disease) (Hackney et al., 2017). Complications of rhabdomyosarcoma include metastasis to lung, bone, or bone marrow and direct extension into the CNS, resulting in brain stem compromise or cranial nerve palsy. Nursing Assessment The child or parent will often discover an asymptomatic mass and seek medical attention at that time. Obtain a health history, noting recent illness, when the mass was discovered, and whether it has changed since first noted. Examine the history for risk factors such as parental smoking, exposure to environmental chemicals, family history of cancer, or neurofibromatosis. Note respiratory effort and cough and auscultate the lungs for adventitious sounds. Palpate for lymphadenopathy. Palpate the abdomen for a mass or hepatosplenomegaly. Abnormalities found on physical examination depend on the location of the rhabdomyosarcoma (Table 24.11). Laboratory and diagnostic testing may include: CT scan or MRI of primary lesion and the chest for metastasis Open biopsy of the primary tumor for definitive diagnosis Bone marrow aspiration and biopsy, bone scan, and skeletal survey to determine metastasis

Latex allergy

is an IgE-mediated response to exposure to latex, a natural rubber product used in many common items (especially gloves in the healthcare setting). The pathophysiology of latex allergy is similar to that of food allergy. Avoidance of latex products is recommended for those who are allergic to it. An immediate allergic reaction may occur if a latex-allergic child comes into contact with latex. Latex allergy can also result in anaphylaxis (refer to section above on anaphylaxis). Nursing Assessment Screen all children who visit a healthcare facility of any kind for latex allergy. Ask if the child is allergic to rubber gloves or has ever developed hives after exposure to them. Ask the parent if the child has symptoms such as coughing, wheezing, or shortness of breath after glove exposure. Has the child ever had swelling in the mouth or complained that the mouth itched after a dental examination? Determine whether the child has ever had allergic symptoms after eating foods with a known cross-reactivity to latex, such as pear, peach, passion fruit, plum, pineapple, kiwi, fig, grape, cherry, melon, nectarine, papaya, apple, apricot, banana, chestnut, carrot, celery, avocado, tomato, or potato. For the child who has come into contact with latex, assess for symptoms of a reaction such as hives; wheeze; cough; shortness of breath; nasal congestion and rhinorrhea; sneezing; nose, palate, or eye pruritus; or hypotension. Nursing Management Nursing management of latex allergy focuses on preventing exposure to latex products. Instruct children and their families to avoid foods with a known cross-reactivity to latex such as those listed above. If the child is exposed to latex, remove the irritating substance and cleanse the area with soap and water. Assess for the need for resuscitation and perform it if needed. Become familiar with your institution's latex allergy policy. Know which products contain latex and which do not. Document latex allergy on the chart, the child's identification band, the medication administration record, and the physician's order sheet. Refer families to resources for persons with latex allergy. Some helpful links are provided on

Wiskott-Aldrich syndrome

is an X-linked genetic disorder that results in immunodeficiency, eczema, and thrombocytopenia. It affects males only. The defective gene responsible for this disorder is called the Wiskott-Aldrich syndrome protein (WASp). Complications include autoimmune hemolytic anemia, neutropenia, skin or cerebral vasculitis, arthritis, inflammatory bowel disease, and renal disease (Ochs, 2018). Autoimmune disease may require high-dose steroids, azathioprine, or cyclophosphamide. Splenectomy may be performed to correct thrombocytopenia. The only cure is hematopoietic cell transplantation, though gene therapy is currently under investigation. Nursing Assessment Note history of petechiae, bloody diarrhea, or bleeding episode in the first 6 months of life. Note any history of hematemesis or intracranial or conjunctival hemorrhages. Observe the skin for eczema, which usually worsens with time and tends to become secondarily infected (Fig. 25.2). Laboratory findings include low IgM concentration, elevated IgA and IgE concentrations, and normal IgG concentrations. Nursing Management Administer IVIG as ordered to help decrease the frequency of bacterial infections. Perform good skin care and frequently assess eczematous areas to detect secondary infection (refer to Chapter 23 for care of eczema). If the child undergoes splenectomy, in addition to providing routine postoperative care, be aware of the additional risk for development of infection in the asplenic child. Refer to Chapter 24 for information related to hematopoietic cell transplantation.

Cerebral palsy

is a term used to describe a range of nonspecific clinical symptoms characterized by abnormal motor pattern and postures caused by nonprogressive abnormal brain function. The majority of causes occur before delivery (80%), but can also occur in the natal and postnatal periods (Box 22.1) (Barkoudah & Glader, 2018b; Johnston, 2016). Many times, no specific cause can be identified (Barkoudah & Glader, 2018b). Cerebral palsy is the most common movement disorder of childhood; it is a lifelong condition and one of the most common causes of physical disability in children (Johnston, 2016). The incidence is about 2 in every 1,000 live births and is higher in premature and low-birthweight infants (Barkoudah & Glader, 2018b). See Healthy People 2030. Most affected children will develop symptoms in infancy or early childhood. There is a large variation in symptoms and disability. For some children it may be as mild as a slight limp; for others it may result in severe motor and neurologic impairments. Primary signs include motor impairments such as spasticity, muscle weakness, and ataxia, which is lack of coordination of muscle movements during voluntary movements such as walking or picking up objects. Complications include mental impairments, seizures, growth problems, impaired vision or hearing, abnormal sensation or perception, and hydrocephalus. Most children can survive into adulthood, but function and quality of life can vary from near normal to substantial impairments (Barkoudah & Glader, 2019a).Pathophysiology Cerebral palsy is a disorder caused by abnormal development of, or damage to, the motor areas of the brain, resulting in a neurologic lesion. It is difficult to establish an exact location of the neurologic lesion, but it causes a disruption in the brain's ability to control movement and posture. The lesion itself does not change over time; thus, the disorder is considered nonprogressive since the brain injury does not progress. However, the clinical manifestations of the lesion change as the child grows. Some children may improve, but many either plateau in their attainment of motor skills or demonstrate worsening of motor abilities because it is difficult to maintain the ability to move over time. Cerebral palsy is classified in several ways. One common way is by the type of movement disturbance (Table 22.5). Therapeutic Management Management of cerebral palsy involves multiple disciplines, including a primary physician, specialty physicians such as a neurologist and an orthopedic surgeon, nurses, physical therapists, occupational therapists, speech therapists, dietitians, psychologists, counselors, teachers, and parents. There is no standard treatment for all children. The overall focus of therapeutic management will be to assist the child to gain optimal development and function within the limits of the disease. Treatment is mainly preventative, symptomatic, and supportive. Spasticity management will be a primary concern and will be determined by clinical findings. Medical management is focused on promoting mobility through the use of therapeutic modalities and medications. Surgical management is often required and is used to correct deformities related to spasticity. PHYSICAL, OCCUPATIONAL, AND SPEECH THERAPY The use of therapeutic modalities such as physical therapy, occupational therapy, and speech therapy will be essential in promoting mobility and development in the child with cerebral palsy. The earlier the treatment begins, the better chance the child has of overcoming developmental disabilities PHARMACOLOGIC MANAGEMENT Various pharmacologic options are available to manage spasticity (see Drug Guide 22.1). Oral medications used to treat spasticity include baclofen, dantrolene sodium, and diazepam. Children with dyskinetic/athetoid cerebral palsy may be given anticholinergics to help decrease abnormal movements. Anticholinergic agents, such as scopolamine (also known as hyoscine) or glycopyrrolate, decrease saliva and are used to help control drooling. Parenterally administered medications such as botulin toxins and baclofen are also used to manage spasticity. Botulinum toxin is injected into the spastic muscle to balance the muscle forces across joints and to decrease spasticity. It is useful in managing focal spasticity in which the spasticity is interfering with function, producing pain, or contributing to a progressive deformity. Botulin toxin injection is performed by the physician or nurse practitioner and can be done in the clinic or outpatient setting. Phenol block, a neurolytic agent that provides a temporary reduction in spasticity, may be used in conjunction with botulinum toxin or alone if botulinum toxin has been proved to be ineffective or contraindicated for the child (Barkoudah & Glader, 2019b). SURGICAL MANAGEMENT Many children will require surgical procedures to correct deformities related to spasticity. Multiple corrective surgeries may be required; they usually are orthopedic or neurosurgical. Surgery may be used to correct contractures that are severe enough to cause movement limitations. Common orthopedic procedures include tendon lengthening procedures, correction of hip and adductor muscle spasticity, and fusion of unstable joints to help improve locomotion, correct bony deformities, decrease painful spasticity, and maintain, restore, or stabilize a spinal deformity. Neurosurgical interventions may include placement of a shunt in children who have developed hydrocephalus, or surgical interventions to decrease spasticity. Selective dorsal root rhizotomy is used to decrease spasticity in the lower extremities by reducing the amount of stimulation that reaches the muscles via the nerves. Common signs and symptoms reported during the health history of the undiagnosed child might include: Intrauterine infections Prematurity with intracranial hemorrhage Difficult, complicated, or prolonged labor and delivery Multiple births History of possible anoxia during prenatal life or birth History of head trauma Delayed attainment of developmental milestones Muscle weakness or rigidity Poor feeding Hips and knees feel rigid and unbending when pulled to a sitting position Seizure activity Subnormal learning Abnormal motor performance, scoots on back instead of crawling on abdomen, walks or stands on toes Physical Examination Observe general appearance. Pay close attention to the neurologic assessment and motor assessment. Assess for delayed development, size for age, and sensory alterations such as strabismus, vision problems, and speech disorders. Abnormal postures may be present. While lying supine, the infant may demonstrate scissor crossing of the legs with plantar flexion. In the prone position the infant may raise his or her head higher than normal due to arching of the back, or the opisthotonic position may be noted. The infant may also abnormally flex the arms and legs under the trunk. Primitive reflexes may persist beyond the point at which they disappear in a healthy infant. Evolution of protective reflexes may be delayed. Watch the infant or child play, crawl, walk, or climb to determine motor function and capability. Note any movement disorder. Infants with cerebral palsy may demonstrate abnormal use of muscle groups such as scooting on their back instead of crawling or walking. Assess active and passive range of motion. Pay particular attention to muscle tone. Though an increased or decreased resistance may be noted with passive movements, hypertonicity is most often seen. Increased resistance to dorsiflexion and passive hip abduction are the most common early signs. Sustained clonus (muscular spasm) may be present after forced dorsiflexion. Lift the child by placing your hands in the infant's or child's axillary area to assess shoulder girdle function and tone. Infants with cerebral palsy often demonstrate prolonged standing on their toes when supported in an upright standing position in this fashion. Lift the young child off the ground while the child holds your thumbs to test hand strength. Observe for presence of limb deformity, as decreased use of an extremity (as in the case of hemiparesis) may result in shortening of the extremity compared to the other one. Common supplementary laboratory and diagnostic tests ordered for the diagnosis and assessment of cerebral palsy include: Electroencephalogram: usually abnormal but the pattern is highly variable Cranial radiographs or ultrasound: may show cerebral asymmetry MRI or CT: may show area of damage or abnormal development but may be normal Screening for metabolic defects and genetic testing may be performed to help determine the cause of cerebral palsy Promoting Mobility Mobility is critical to the development of the child with cerebral palsy. Treatment modalities to promote mobility include physiotherapy, pharmacologic management, and surgery. Surgical procedures are discussed above. Physical or occupational therapy as well as medications may be used to address musculoskeletal abnormalities, to facilitate range of motion, to delay or prevent deformities such as contractures, to provide joint stability, to maximize activity, and to encourage the use of adaptive devices. The nurse's role in relation to the various therapies is to provide ongoing follow-through with prescribed exercises, positioning, or bracing. When casting, splinting, or orthotics are used, assess skin integrity frequently. Pain management may also be necessary. Nursing management of children receiving botulin toxin focuses on assisting with the procedure and providing education and support to the child and family. Nursing interventions related to baclofen include assisting with the test dose and providing pre- and postoperative care if a pump is placed, as well as providing support and education to the child and family. Teaching Guidelines 22.5 gives information related to baclofen pump insertion

Anaphylaxis

is an acute IgE-mediated response to an allergen that involves many organ systems and may be life-threatening. In addition to nuts, shellfish, eggs, and bee or wasp stings, drugs such as penicillin and NSAIDs, radiopaque dyes, and latex are the leading causes of anaphylaxis (Volkman & Chiu, 2019). The reaction is severe and usually starts within 5 to 10 minutes of exposure, though delayed reactions are possible. Histamines and secondary mediators are released from the mast cells and eosinophils in response to contact with an allergen. Cutaneous, cardiopulmonary, gastrointestinal, and neurologic symptoms occur. Vasodilation results in a rapid decrease in plasma volume, leading to the risk of circulatory collapse. Prolonged resuscitation may be needed, and death may occur. Therapeutic management focuses on assessment and support of the airway, breathing, and circulation. Epinephrine is usually required, and intramuscular or intravenous diphenhydramine is used secondarily. Late-onset reactions can be prevented with corticosteroids. Nursing Assessment Assess patency of the airway and adequacy of breathing. Determine if circulation is sufficient. Note level of consciousness. Obtain a brief history, inquiring specifically about allergen exposure. Determine whether the child has received any medication (e.g., epinephrine or diphenhydramine) since the onset of the reaction and what effect the medication had on the symptoms. Table 25.3 gives additional signs and symptoms of anaphylaxis. Nursing Management Nursing management initially focuses on supporting the airway, breathing, and circulation. Provide supplemental oxygen by mask or bag-valve-mask ventilation. Administer epinephrine as ordered to reverse the allergic process. Ensure that bronchodilator inhalation treatment (albuterol) is given if bronchospasm is present. Administer intravenous fluids to provide volume expansion. Observe the child for 4 to 6 hours in case of recurrent attack (Covar et al., 2018). Preventing and Managing Future Episodes It is critical to educate the family about preventing and managing future episodes. Teach the family how to use injectable epinephrine in case of subsequent allergen exposure. Intramuscular epinephrine may be given via the EpiPen or EpiPen Jr. Dosage is based on the child's weight. The child should carry the pen with him or her at all times. Explain to the child and family that the gray safety release on the EpiPen should never be removed until just before use. In addition, teach the child and family that the thumb, fingers, or hand should not be placed over the black tip. Nursing Procedure 25.1 gives further instructions related to EpiPen use. Instruct the child and family to call 911 and seek immediate medical attention after using the EpiPen. Warn the child that the epinephrine may make him or her feel as if the heart is racing.

Juvenile dermatomyositis

is an autoimmune disease that results in inflammation of the muscles or associated tissues. It occurs more often in girls and is generally diagnosed between the ages of 5 and 10 years (Hutchinson & Feldman, 2017). The cause remains unclear but it may be an autoimmune response triggered by exposure to a virus or to certain medications (Hutchinson & Feldman, 2017). As with other autoimmune diseases, a genetic predisposition is present. The inflammatory cells of the immune system cause a vasculitis that affects the skin, muscles, kidneys, retinas, and gastrointestinal tract. Therapeutic management involving the use of high-dose glucocorticoid or other immunosuppressants is necessary to prevent the complications of painful calcium deposits under the skin, as well as joint contractures. Methotrexate, IVIG, and cyclosporine may also be used. With appropriate treatment, children may recover completely, though some children experience relapses (Hutchinson & Feldman, 2019). Nursing Assessment Elicit a health history, which commonly includes fever, fatigue, and rash, usually followed by muscle pain and weakness. Determine onset and progression of muscle weakness. Inspect the skin for the presence of rash involving the upper eyelids and extensor surfaces of the knuckles, elbows, and knees. The rash is initially a reddish- purplish color, and then progresses to scaling with resulting roughness of the skin. Test muscle strength, particularly noting weakness in the pelvic and shoulder girdles. Laboratory and diagnostic testing may include muscle enzyme levels, a positive ANA test, and an EMG to distinguish muscular weakness from other causes. Nursing Management Administer medications as ordered and teach families about their use; instruct them to monitor for side effects. Educate the family about the importance of maintaining the medication regimen in order to prevent calcinosis (calcium deposits) and joint deformity in the future. Encourage compliance with physical therapy regimens. Ensure that children are excused from physical education classes while the disease is active.

Juvenile idiopathic arthritis

is an autoimmune disorder in which the autoantibodies mainly target the joints. Inflammatory changes in the joints cause pain, redness, warmth, stiffness, and swelling. Stiffness usually occurs after inactivity (as in the morning, after sleep). Some forms also affect the eyes or other organs. Table 25.2 explains the three types. Juvenile idiopathic arthritis is a chronic disease; the child may experience healthy periods alternating with flare-ups (Soep, 2018). Juvenile idiopathic arthritis was formerly termed "juvenile rheumatoid arthritis," but unlike adult rheumatoid arthritis, few types of juvenile arthritis actually demonstrate a positive rheumatoid factor. Therapeutic management focuses on inflammation control, pain relief, promotion of remission, and maintenance of mobility. NSAIDs, corticosteroids, and antirheumatic drugs such as methotrexate and etanercept are prescribed, depending on the type and severity of the disease. NSAIDs are helpful with pain relief, but disease-modifying (antirheumatic) drugs are necessary to prevent disease progression (several of which are approved for use in children). Nursing Assessment Note history of irritability or fussiness, which may be the first sign of this disease in the infant or very young child. Note complaints of pain, though children do not always communicate this. Document history of withdrawal from play or difficulty getting the child out of bed in the morning (joint stiffness after inactivity). Inquire about history of fever (above 39.5°C for 2 weeks or more in systemic disease). Measure temperature (fever is present with systemic disease). Inspect skin for evanescent, pale red, nonpruritic macular rash, which may be present at diagnosis of systemic disease. Observe the gait, noting limping or guarding of a joint or extremity. Document growth, which may be delayed. Inspect and palpate each joint for edema, redness, warmth, and tenderness (Fig. 25.4). Note positioning of joints (usually flexed in position of comfort). Mild to moderate anemia and an elevated erythrocyte sedimentation rate are common. Young children with the pauciarticular form may demonstrate a positive ANA, and adolescents with polyarticular disease may have a positive rheumatoid factor. Nursing Management Nursing management focuses on managing pain, maintaining mobility, and promoting a normal life. Refer the child to a pediatric rheumatologist to ensure that he or she receives the most up-to-date treatment. Administer disease-modifying medications and teach children and families how to do so. Refer families Childhood Arthritis & Rheumatology Research Alliance for clinical research trial information. Encourage regular eye examinations and vision screening to allow for early treatment of visual changes and to prevent blindness. Managing Pain and Maintaining Mobility Administer medications as prescribed to control inflammation and prevent disease progression. Refer to Drug Guide 25.1 for information related to NSAIDs, corticosteroids, and disease-modifying antirheumatic drugs. Maintain joint range of motion and muscle strength via exercise (physical or occupational therapy). Swimming is a particularly useful exercise to maintain joint mobility without placing pressure on the joints. Teach families appropriate use of splints prescribed to prevent joint contractures. Monitor for pressure areas or skin breakdown with splint or orthotic use. Promote sleep with a warm bath at bedtime and warm compresses to affected joints or massage. To prevent social isolation, encourage the child to attend school and ensure that teachers, the school nurse, and classmates are educated about the child's disease and any limitations on activity. Having two sets of books (one at school and one at home) allows the child to do homework without having to carry heavy books home. Modifications such as allowing the child to leave the classroom early in order to get to the next class on time may seem small but can have a significant impact on the child's life.

A gene

is the basic unit of heredity of all traits. Genes occupy a specific location on a chromosome (a long, continuous strand of DNA that carries genetic information) and determine the organism's physical and mental characteristics. In humans each somatic cell (a cell forming the body of an organism) has 46 chromosomes: 22 pairs of nonsex chromosomes (autosomes) and 1 pair of sex chromosomes.

Insulin replacement therapy

is the cornerstone of management of type 1 DM. Insulin is administered daily by subcutaneous injections into adipose tissue over large muscle masses using a traditional insulin syringe or a subcutaneous injector (Fig. 26.7). U-100 insulin may also be administered using a portable insulin pump (see discussion below). The frequency, dose, and type of insulin are based on how much the child needs to achieve a normal, average blood glucose concentration and to prevent hypoglycemia. Typically, two to four daily injections are commonly used, with dosage depending on the needs of the child. The dose may need to be increased during the pubertal growth spurt as well as during times of illness or stress. An insulin pump is a device that administers a continuous infusion of rapid-acting insulin. Studies have established it to be a safe and effective way to improve glycemic control and reduce episodes of severe hypoglycemia in young people (American Diabetes Association, 2019). It consists of a computer, a reservoir of rapid-acting insulin, thin tubing through which the insulin is delivered, and a small needle inserted into the abdomen. Insulin pumps attempt to mimic the physiologic insulin release by delivering small continuous infusions of insulin with additional bolus units administered at mealtimes, for planned carbohydrate intake, and if glucose testing results show it is needed. Advantages of this kind of therapy include: There are fewer injections and less trauma. Children's food intake can be unpredictable, so insulin delivery can occur after a meal and be adjusted based on actual intake. Children can be sensitive to insulin and require only minute doses, which the pump can deliver with precision. The pumps can store different basal rates for different times during the day and days of the week. For example, a higher basal rate may be needed in the morning when the child is sitting at his or her desk and a lower rate may be necessary during the afternoon when the child is more active with recess and physical education classes. In addition, rates can be programmed differently for school days versus weekend days, when the child may sleep later and have differing activity levels. Types of insulin include rapid acting, short acting, intermediate acting, and long acting (Table 26.5). Each type works at a different pace and most children will use more than one type. In some cases, premixed combinations of intermediate acting and short or rapid acting, such as 70% NPH and 30% regular, may be used. Again, this depends on the needs of the child. Insulin can be kept at room temperature (insulin that is administered cold may increase discomfort with injection) but should be discarded 1 month after opening even if refrigerated. Any extra, unopened vials should be stored in the refrigerator.

Acute Lymphoblastic Leukemia ALL

is the most common form of cancer in children. Eighty-five percent of cases of ALL occur in children between 2 and 10 years of age (Craddock et al., 2018). It is more common in white children than in other races. ALL is classified according to the type of cells involved—T cell, B cell, early pre-B cell, or pre-B cell. Most children will achieve initial remission if appropriate treatment is given. The overall cure rate of ALL is over 70% (Craddock et al., 2018). Prognosis is based on the WBC count at diagnosis, the type of cytogenetic factors and immunophenotype, the age at diagnosis, and the extent of extramedullary involvement. Generally, the higher the WBC count at diagnosis, the worse the prognosis. Children between 1 and 9 years of age and with a WBC count less than 50,000 at diagnosis have the best prognosis. When a child experiences a relapse, the prognosis becomes poorer. Complications include infection, hemorrhage, poor growth, and CNS, bone, or testicular involvement. Pathophysiology The exact cause of ALL remains unknown. Genetic factors and chromosome abnormalities may play a role in its development. In ALL, abnormal lymphoblasts abound in the blood-forming tissues. The lymphoblasts are fragile and immature, lacking the infection-fighting capabilities of the normal WBC. The growth of lymphoblasts is excessive and the abnormal cells replace the normal cells in the bone marrow. The proliferating leukemic cells demonstrate massive metabolic needs, depriving normal body cells of needed nutrients and resulting in fatigue, weight loss or growth arrest, and muscle wasting. The bone marrow becomes unable to maintain normal levels of RBCs, WBCs, and platelets, so anemia, neutropenia, and thrombocytopenia result. As the bone marrow expands or the leukemic cells infiltrate the bone, joint and bone pain may occur. The leukemic cells may permeate the lymph nodes, causing diffuse lymphadenopathy, or the liver and spleen, resulting in hepatosplenomegaly. With spread to the CNS, vomiting, headache, seizures, coma, vision alterations, or cranial nerve palsies may occur (Craddock et al., 2018). Therapeutic Management Therapeutic management of the child with ALL focuses on giving chemotherapy to eradicate the leukemic cells and restore normal bone marrow function. Treatment is divided into three stages. CNS prophylaxis is provided at each stage in order to prevent metastasis to the CNS (Craddock et al., 2018). The length of treatment and choice of medications are based on the child's age, risk category, and subtype determined by bone marrow analysis. Table 24.7 discusses the stages of leukemia treatment. For relapsed or less responsive leukemia, HSCT may be necessary. Health History Elicit a description of the present illness and chief complaint. Common signs and symptoms reported during the health history might include: Fever (may be persistent or recurrent, with unknown cause) Recurrent infection Fatigue, malaise, or listlessness Pallor Unusual bleeding or bruising Abdominal pain Nausea or vomiting Bone pain Headache (Russell & McLean, 2019) Explore the child's current and past medical history for risk factors such as: Male gender Age 2 to 5 years Caucasian race Down syndrome (and many other genetic syndromes) Sibling with leukemia Radiation exposure Previous chemotherapy treatment (Russell & McLean, 2019) Physical Examination Take the child's temperature (fever may be present), and look for petechiae, purpura, or unusual bruising (due to decreased platelet levels). Inspect the skin for signs of infection. Auscultate the lungs, noting adventitious breath sounds, which may indicate pneumonia (present at diagnosis or due to immunosuppression during treatment). Note location and size of enlarged lymph nodes. Palpate the liver and spleen for enlargement. Document tenderness on abdominal palpation. Laboratory and Diagnostic Tests Common laboratory and diagnostic studies ordered for the assessment of ALL include: CBC: abnormal findings include low hemoglobin and hematocrit, decreased RBC count, decreased platelet count, and elevated, normal, or decreased WBC count Peripheral blood smear may reveal blasts. Bone marrow aspiration: stained smear from bone marrow aspiration will show greater than 25% lymphoblasts. Bone marrow aspirate is also examined for immunophenotyping (lymphoid vs. myeloid, and level of cancer cell maturity) and cytogenetic analysis (determines abnormalities in chromosome number and structure). Immunophenotyping and cytogenetic analysis are used in the classification of the leukemia, which helps guide treatment. Lumbar puncture will reveal whether leukemic cells have infiltrated the CNS. Liver function tests and blood urea nitrogen (BUN) and creatinine levels determine liver and renal function, which, if abnormal, may preclude treatment with certain chemotherapeutic agents. Chest radiography may reveal pneumonia or a mediastinal mass. Nursing Management Nursing care of children with ALL focuses on managing disease complications such as infection, pain, anemia, bleeding, and hyperuricemia and the many adverse effects related to treatment. Many children require blood product transfusion for the treatment of severe anemia or low platelet levels with active bleeding. Individualize nursing care based on the diagnoses, interventions, and outcomes presented in Nursing Process Overview section earlier in the chapter, depending on the child's response to the disease and chemotherapy. Refer to the Nursing Process Overview section for further information related to managing the adverse effects of chemotherapy. Reducing Pain Children and teens with leukemia suffer pain related to the disease as well as the treatment. Chemotherapy drugs commonly used in leukemia may cause peripheral neuropathy and headache. Lumbar puncture and bone marrow aspiration, which are periodically performed throughout the course of treatment, also cause pain. The most common areas of pain are the head and neck, legs, and abdomen (probably from protracted vomiting with chemotherapy). Use distraction techniques, such as listening to music, watching TV, or playing games, to help take the child's mind off the pain. Administer mild analgesics such as acetaminophen for acute episodes of pain. Using EMLA cream prior to venipuncture, port access, lumbar puncture, and bone marrow aspiration may decrease procedure-related pain events. In addition, applying heat or cold to the painful area is usually acceptable. Administer narcotic analgesics, as prescribed, for episodes of acute severe pain or for palliation of chronic pain.

Fragile X syndrome

is the most common inherited cause of intellectual disability (Van Esch, 2018). It is the outcome of a mutation of a gene (FMR1 [fragile X mental retardation]) on the X chromosome. This mutation essentially "turns off" the gene, triggering fragile X syndrome. Males and females are both affected, but it is more commonly seen in males, and affected females usually have milder symptoms (Genetics Home Reference, 2019d). The incidence is approximately 1 in 8,000 females and 1 in 4,000 males (Genetics Home Reference, 2019d). The inheritance of fragile X is complex and is less straight forward than single-gene or mendelian inheritance. Some carrier females are affected, and not all males with the gene abnormality show symptoms. Males and females are both fertile and can transmit the disorder to their offspring, so genetic counseling is appropriate. The prognosis for individuals with fragile X is good, and they tend to live a normal life span. There is no cure for fragile X syndrome. Therapeutic management will be multidisciplinary and aimed at interventions to improve cognitive, emotional, and behavioral impairments. Nursing Assessment During childhood, clinical manifestations are subtle, with minor dysmorphic features and developmental delay. Problems with sensation, emotion, and behavior often are the first signs. A delay in attaining developmental milestones will most likely be the first clue found on assessment. Intellectual impairment can range from subtle learning disabilities to severe intellectual disability and autistic-like behaviors. In adolescence, boys tend to present with characteristic features such as an elongated face; prominent jaw; large, protruding ears; large size; macroorchidism (large testes); and a range of behavioral abnormalities and cognitive deficits (Fig. 27.13). There is a characteristic pattern to the cognitive deficits, with problems in abstract reasoning, sequential processing, and mathematics. Typical behavior problems include attention deficits, hand flapping and biting, hyperactivity, shyness, social isolation, low self-esteem, and gaze aversion. In females the clinical manifestations are similar but are more varied and often present in a milder form. Diagnosis is confirmed by molecular genetic testing. Fragile X can be diagnosed prenatally if inheritance is suspected. Nursing Management Nursing management will be mainly supportive. Early diagnosis and intervention with developmental therapies and an individualized education plan are ideal. Care of these children will be the same as care of other children with intellectual disability (see Chapter 28 for further information on intellectual disability). Provide education and support to the family. The National Fragile X Foundation provides education and emotional support and works to increase awareness and advance research for fragile X. Links to resources are provided on .

ADHD

is the most common neurodevelopmental disorder of childhood, affecting 8% to 11% of school-aged children (Krull, 2019). ADHD is characterized by inattention, impulsivity, distractibility, and hyperactivity. Three subtypes of ADHD exist: hyperactive-impulsive, inattentive, and combined. The child with ADHD has a disruption in learning ability, socialization, and compliance, placing significant demands on the child, parents, teachers, and community. Children with ADHD often have a comorbidity (disorder accompanying the primary illness) such as oppositional defiant disorder, conduct disorder, an anxiety disorder, depression, a less severe developmental disorder, an auditory processing disorder, or learning or reading disabilities (Krull, 2019). Comparison Chart 28.1 gives information about oppositional defiant disorder and conduct disorder to distinguish them from ADHD. Pathophysiology Though the exact cause of ADHD remains unidentified, an alteration in the catecholamine neurotransmitter system may responsible, but genetics, environmental exposures, and structural brain abnormalities may play a role (Krull, 2019). The symptoms of impulsivity, hyperactivity, and inattention begin before 7 years of age and persist longer than 6 months. Symptoms exist in the school and home settings, impairing family and social interactions. Children and teens with ADHD experience frustration, labile moods, emotional outbursts, peer rejection, poor school performance, and low self-esteem. They may also have poor metacognitive abilities such as organization, time management, and the ability to break a project down into a series of smaller tasks. They are not lazy or unmotivated, but simply have poor skills in these areas. Box 28.3 provides criteria for the diagnosis of ADHD (Goldson & Reynolds, 2018).Diagnosis of Attention-Deficit/ Hyperactivity Disorder Presence of six or more of the following in the child 17 years of age and younger: Failure to pay close attention Careless mistakes on schoolwork Difficulty paying attention to tasks or play Doesn't listen Doesn't follow through Doesn't complete tasks Doesn't understand instructions Poorly organized Avoids, dislikes, or fails to engage in activities requiring mental effort Loses things needed for task completion Easily distracted Forgetful Fidgety or squirmy Often out of seat Activity inappropriate to the situation Cannot engage in quiet play Always on the go Talks excessively Blurts out answers Has difficulty waiting his or her turn Often interrupts or intrudes on others Additionally, symptoms have been present in two or more settings, at least two of the symptoms occurred prior to age 12, persistence of symptoms beyond 6 months and to a degree inconsistent with developmental level or negatively interferes with social or academic performance, and symptoms are not associated with purely oppositional behavior or as a component of a psychotic disorder and cannot be explained by the diagnosis of a different mental health disorder. Therapeutic Management Medication management of ADHD includes the use of psychostimulants, nonstimulant norepinephrine reuptake inhibitors, and/or α-agonist antihypertensive agents. These medications are not a cure for ADHD but help to increase the child's ability to pay attention and decrease the level of impulsive behavior. The child's activity level is not usually affected. Behavior therapy and classroom restructuring may be useful as part of the therapeutic management plan. Concomitant disorders, such as anxiety, should also be treated (see discussion of anxiety disorders below). Nursing Assessment For a full description of the assessment phase of the nursing process, refer to the assessment section of the nursing process overview beginning on page 1008. Assessment findings pertinent to ADHD are discussed below. Health History Elicit a description of the behavioral issue or school performance problem. Explore the child's history for risk factors such as head trauma, lead exposure, cigarette smoke exposure, prematurity, or low birthweight. The past history may also reveal a larger than usual number of accidents. Determine if there is a family history of ADHD. Question the parent about school behavior. The school-aged child may be unable to stay on task, talks out of turn, leaves his or her desk frequently, and either neglects to complete in-class and homework assignments or forgets to turn them in. The adolescent may be inattentive in school, poorly organized, and forgetful. Several behavioral checklists are available that may assist in the diagnosis of ADHD. They may be completed by the child's teacher and/or parent and focus on behavior patterns related to conduct or learning problems, social competence, anxiety, activity level, and attention. Obtain the completed behavioral checklists (usually one from the parent and one from the teacher) as well as any school records or testing performed. Physical Examination Perform vision and hearing screening to rule out vision or hearing impairment as the cause of poor school performance. Observe the preschool child's behavior, noting quickness, agility, fearlessness, and the desire to touch or explore everything in the room. The older child or adolescent may have difficulty staying on task during the examination or change the subject frequently while conversing. Laboratory and Diagnostic Tests No definitive laboratory or diagnostic test is available for the identification of ADHD. A complete blood count may be performed to rule out anemia, and thyroid hormone levels may be drawn to determine whether they are normal. Nursing Management Having a child with ADHD can be frustrating as the child's inattention, high activity level, impulsivity, and distractibility are often very difficult to deal with. Parents may doubt their ability to be effective parents or may view their child as somehow defective. Children with ADHD may also feel they are bad, faulty, stupid, or intellectually challenged. Provide emotional support, allowing enough time for the family to air their concerns. Work with the child and family to develop goals such as completion of homework, improved communication, or increasing independence in self-care. Assist the family to advocate for their child's needs through the public school system. The child is entitled to a developmentally appropriate education via an IEP as necessary (refer to Chapter 12 for additional information about special education). The IEP should be updated as needed. Ensure coordination of health and school services. Flag the child's chart and set up a schedule for systematic communication with the family and school. Teach families and school personnel to use behavioral techniques such as time-out, positive reinforcement, reward or privilege withdrawal, or a token system. The token system rewards appropriate behavior with a token and results in a token being taken away if inappropriate behavior occurs. At the end of a specified period of time, the tokens may be exchanged for a prize or privilege. Refer families to local support groups and the national ADHD support group, links to which are provided on . Explain that stimulant medications should be taken in the morning to decrease the adverse effect of insomnia. Some children may experience decreased appetite, so giving the medication with or after the meal may be beneficial. The child may feel "different" from his or her peers if he or she has to visit the school nurse for a lunchtime dose of ADHD medication; this may lead to noncompliance and a subsequent increase in ADHD symptoms, with deterioration in schoolwork. In this situation, encourage the family to explore with their physician or nurse practitioner the option of one of the newer extended-release or once-daily ADHD medications. See Dosage Calculation Box 28.1

Wilms tumor

is the most common renal tumor, the second most common abdominal solid tumor in children, and most commonly occurs between the ages of 2 and 5 years (Hackney et al., 2017). It usually affects only one kidney (Fig. 24.18). The etiology is unknown, but some cases occur via genetic inheritance. Associated anomalies may occur with Wilms tumor. Wilms tumor demonstrates rapid growth and is usually large at diagnosis. Metastasis occurs via direct extension or through the bloodstream. Wilms tumor most commonly metastasizes to the perirenal tissues, liver, diaphragm, lungs, abdominal muscles, and lymph nodes. Staging of Wilms tumor is provided in Box 24.5. The tumor is also additionally designated as having favorable histology (FH) or unfavorable histology (UH). UH is noted by the presence of anaplasia (focal or diffuse giant polypoid nuclei). The prognosis depends on staging at diagnosis and the extent of metastasis, with survival in children with stages I to III (with FH) being 91% (Hackney et al., 2017). Complications include metastasis or complications from radiation therapy such as liver or renal damage, female sterility, bowel obstruction, pneumonia, or scoliosis. Therapeutic Management Surgical removal of the tumor and affected kidney (nephrectomy) is the treatment of choice and also allows for accurate staging and assessment of tumor spread. Radiation or chemotherapy may be administered either before or after surgery. Health History Parents typically initially observe the abdominal mass associated with Wilms tumor and then seek medical attention. Elicit the health history, noting when the mass was discovered. Note abdominal pain, which may be related to rapid tumor growth. Document history of constipation, vomiting, anorexia, weight loss, or difficulty breathing. Determine risk factors such as hemihypertrophy of the spine, Beckwith-Wiedemann syndrome, genitourinary anomalies, absence of the iris, or family history of cancer. Physical Examination Measure blood pressure; hypertension occurs in 25% of children with Wilms tumor (Craddock et al., 2018). Inspect the abdomen for asymmetry or a visible mass. Observe for associated anomalies as noted above. Auscultate the lungs for adventitious breath sounds associated with tumor metastasis. Palpate for lymphadenopathy. Laboratory and Diagnostic Testing Laboratory and diagnostic testing may include: Renal or abdominal ultrasound to assess the tumor and the contralateral kidney CT scan or MRI of the abdomen and chest to determine local spread to lymph nodes or adjacent organs, as well as any distant metastasis CBC, BUN, and creatinine: usually within normal limits Urinalysis: may reveal hematuria or leukocytes 24-hour urine collection for HVA and VMA to distinguish the tumor from neuroblastoma (levels will not be elevated with Wilms tumor)

Klinefelter syndrome

is the most common sex chromosomal abnormality (Bacino & Lee, 2016). The karyotype and phenotype are male, but one or more extra X chromosomes is present. The abnormality is usually caused by nondisjunction during meiosis, but mosaic forms do present. The incidence of Klinefelter syndrome is 1 in 500 to 1,000 males (Bacino, 2019c). Males present with some female-like physical features that are caused by testosterone deficiency. The risk of recurrence in future pregnancies is not increased. There is no cure for Klinefelter syndrome. Therapeutic management will focus on interventions to enhance masculine characteristics, such as testosterone replacement. Early recognition and hormonal treatment are important to improve quality of life and prevent serious consequences. Cosmetic surgery may be performed to minimize female characteristics such as gynecomastia (increased breast size). Nursing Assessment Due to nonspecific findings during childhood, the diagnosis is not usually made until adolescence or adulthood. Prenatal diagnosis is rare unless amniocentesis was performed for genetic testing. Many males with Klinefelter syndrome reach adulthood without being diagnosed (Bacino & Lee, 2016). The diagnosis is confirmed by chromosomal analysis. On assessment, lack of development of secondary sex characteristics may be found. The individual may have decreased facial hair, gynecomastia, decreased pubic hair, and hypogonadism or underdeveloped testes, which leads to infertility. The individual may be taller than average by 5 years of age, with long legs and a short torso (Fig. 27.12). Intellectual disability is not present, but cognitive impairments of varying degree, such as motor delay, speech or language difficulties, attention deficits, and learning disabilities, may be found. Nursing Management Nursing management will be mainly supportive. Provide education and support to the family. Links to The American Association for Klinefelter Syndrome and the Association for X and Y chromosome variations are provided on . Counseling about infertility is important. Educate children and families that marriage and sexual relations are possible. Parents may be upset that their son will not be able to reproduce, so explain that many alternatives for reproduction are available and technology is advancing in the field of infertility.

Hyperthyroidism

is the result of hyperfunction of the thyroid gland. This leads to excessive levels of circulating thyroid hormones. This condition is uncommon in children. The peak incidence in children occurs in adolescence as a result of Graves disease (Huang & LaFranchi, 2016). Graves disease is an autoimmune disorder that causes excessive amounts of thyroid hormone to be released in response to human thyroid stimulator immunoglobulin (TSI). It occurs five times more often in girls than in boys (Huang & LaFranchi, 2016). A goiter usually develops in this condition. There is a genetic marker in individuals affected by Graves disease, with the majority of children having a positive family history of autoimmune thyroid problems (Huang & LaFranchi, 2016). A congenital form of hyperthyroidism, neonatal thyrotoxicosis, occurs in infants of mothers with Graves disease. This neonatal condition, which can be life threatening, is a self-limiting disorder lasting 2 to 4 months. Less common causes of hyperthyroidism are thyroiditis, thyroid hormone-producing tumors, and pituitary adenomas. Therapeutic management is aimed at decreasing thyroid hormone levels. Current treatment involves antithyroid medication, radioactive iodine therapy, and subtotal thyroidectomy. First-line treatment involves methimazole (MTZ, Tapazole), which blocks the production of T3 and T4 (LaFranchi, 2019). Adjunct therapy with β-adrenergic blockers (such as propranolol or atenolol) may also be used if the child has marked symptoms. Radioactive iodine therapy is restricted to children greater than 10 years as a long-term therapy (LaFranchi, 2019). This therapy is administered orally and results in tissue damage and destruction of the thyroid gland within 6 to 18 weeks, but it can result in hypothyroidism. Subtotal thyroidectomy is used when drug therapy is not possible or other treatments have failed. Risks include hypothyroidism, hypoparathyroidism, or laryngeal nerve damage. Nursing Assessment Initially, symptoms of hyperthyroidism are mild and often overlooked. Many children with hyperthyroidism are first seen in the outpatient setting with a history of a problem with sleep, school performance, and distractibility. They become easily frustrated, overheated, and fatigued during physical education class. The child may complain of diarrhea, excessive perspiration, and muscle weakness. The history may also reveal hyperactivity, heat intolerance, emotional lability, and insomnia. Physical examination of the older child may reveal an increased rate of growth; weight loss despite an excellent appetite; hyperactivity; warm, moist skin; tachycardia; fine tremors; an enlarged thyroid gland or goiter; and ophthalmic changes (exophthalmos, which is less pronounced in children; proptosis; lid lag and retraction; staring expression; periorbital edema; and diplopia) (Fig. 26.4). Elevated pulse and blood pressure may also be noted. Laboratory and diagnostic tests reveal that serum T4 and T3 levels are markedly elevated while TSH levels are suppressed. Nursing Management Once the treatment plan is initiated, educate the family and child about the medication and potential adverse effects, the goals of treatment, and possible complications. Monitor for adverse drug effects such as rash, mild leukopenia, loss of taste, sore throat, GI disturbances, and arthralgia. If the medication is given two or three times a day, teach the family to use a pill dispenser and alarm clock. Inform the family of the need for routine blood tests and follow-up visits with the pediatric endocrinologist every 2 to 4 months until normal levels are reached; then, visits may be decreased to once or twice a year. Instruct the parents to contact the physician or nurse practitioner if the child has tachycardia or extreme fatigue. Help the child and family to cope with symptoms such as heat intolerance, emotional lability, or eye problems. Explain these symptoms to the school or day care personnel and make sure that they understand that the child should take more frequent rest breaks in a cool environment, and should avoid physical education classes until normal hormone levels are attained. Encourage the family to have the child consume a healthy diet with an appropriate level of calories; the child may need to eat five or six meals a day. Provide community referrals such as to the Graves Disease and Thyroid Foundation, a link to which can be found on Encourage the family to obtain a medical ID bracelet or necklace.If surgical intervention is chosen, provide appropriate preoperative teaching and postoperative care. Provide supportive measures such as fluid maintenance, nutritional support, and electrolyte correction. Monitor red blood cell count and liver function tests. Close monitoring for signs and symptoms of hypothyroidism is important.

Acute Myelogenous Leukemia AML

is the second most common type of leukemia in children, with a peak incidence in the adolescent years (Craddock et al., 2018). AML affects the myeloid cell progenitors or precursors in the bone marrow, resulting in malignant (invasive and fast-growing) cells. The French-American-British (FAB) classification system identifies eight subtypes of AML (M0 to M7), depending on myeloid lineage involved and the degree of cell differentiation. These subtypes are useful for determining treatment. The long-term survival rate for childhood AML is about 50% (Craddock et al., 2018). Complications include treatment resistance, infection, hemorrhage, and metastasis. The induction phase of AML requires intense bone marrow suppression and prolonged hospitalization because AML is less responsive to treatment than ALL. Toxicity from treatment is more common in AML and is likely to be more serious than with ALL. Empiric broad-spectrum antibiotics and prophylactic platelet transfusions may be prescribed. After remission is achieved, children require intensive chemotherapy to prolong the duration of remission. HSCT is often required in children with AML, depending on the subtype (Craddock et al., 2018). Nursing Assessment Explore the health history for common signs and symptoms, including recurrent infections, fever, or fatigue. Explore the medical history for risk factors, such as Hispanic race, previous chemotherapy, and genetic abnormalities such as Down syndrome, Fanconi anemia, neurofibromatosis, Wiskott-Aldrich syndrome, and Diamond-Blackfan anemia. Perform a thorough physical examination. Note skin pallor and salmon-colored or blue-gray papular lesions. Palpate the skin for subcutaneous rubbery nodules. Palpate for lymphadenopathy. Note headache, visual disturbance, or signs of increased intracranial pressure, such as vomiting, which may indicate CNS involvement. Upon diagnosis of AML, the child's WBC count is typically extremely elevated (Craddock et al., 2018).

The genotype

is the specific genetic makeup of an individual; it is the internally coded inheritable information and refers to the particular allele (one of two or more alternative versions of a gene at a given position on a chromosome that imparts the same characteristic of that gene).

Medium-chain acyl-CoA dehydrogenase deficiency (MCAD):

lack of an enzyme required to metabolize fatty acids Classic presentation is a child 3 months to 5 years with vomiting and lethargy after a period of not eating (fasting typically associated with a viral illness) Recurrent episodes of metabolic acidosis and hypoglycemia, lethargy, seizures, liver failure, brain damage, coma, and cardiac arrest. Can lead to serious and fatal illness in children not eating well Avoid fasting; have frequent meals. L-carnitine supplementation particularly when ill may be used. Special considerations during illness. If unable to tolerate food, IV dextrose is required www.fodsupport.org: Fatty Oxidation Disorders (FOD) Family Support Group

Biotinidase deficiency:

lack of the enzyme biotinidase results in biotin deficiency Typically no symptoms at birth; in first weeks or months of life, symptoms such as hypotonia, uncoordinated movement, seizures, developmental delay, alopecia, seborrheic dermatitis, hearing loss, optic nerve atrophy, and intellectual disability develop. Metabolic acidosis can lead to death Daily oral free biotin

Substance use

most often begins before age 20. In 2017 in the United States, 60.4% of students reported having had at least one alcoholic drink in their life, with 15.5% reporting they had drunk alcohol before age 13 (CDC, 2018). In addition to alcohol, youths also use marijuana, cocaine, heroin, methamphetamines, inhalants, ecstasy, nonprescribed steroids, and prescription drugs not intended for them (CDC, 2018). Nursing Assessment Note risk factors for substance abuse, such as family history of substance abuse, current parental substance use, dysfunctional family relationships, concurrent mental health disorder, aggressive behaviors, low self-esteem or poor academic performance, negative life events, poor social skills, or peers who use substances. Determine the child's history, noting altered school performance or attendance, changes in peer group participation, frequent mood swings, changes in physical appearance, or an altered relationship with or perception of parents. Document history of insomnia, appetite loss, excessive itching, sleepiness or extreme fatigue, dry mouth, or shakiness. Note violent behavior, drunkenness, stupor, blank expression, drowsiness, lack of coordination, confusion, incoherent speech, extremes in emotions, aggressive behavior, silly behavior, or rapid speech. A screening assessment recommended by the AAP is the CRAFFT Screening Tool (refer to to read more about the screening tool). Perform a complete physical examination. Observe for an odor of alcohol or marijuana smoke. Assess the eyes, noting wateriness or dilated pupils. Inspect the nares, noting rhinorrhea or absence of nasal hair. Inspect the fingers for glue smears or discoloration and the skin for needle marks or tracks. Palpate the hands and feet for coolness. Laboratory and diagnostic tests include toxicology studies, such as urine screening, to determine the presence of stimulants, sedative-hypnotics, barbiturates, hallucinogens, opiates, cocaine, and marijuana. Nursing Management Help the adolescent to acknowledge that he or she has a problem. Explain the negative consequences of substance use and raise the teen's awareness of risks. Remain empathetic yet leave responsibility with the adolescent. Key nursing interventions include promoting participation in treatment programs and preventing substance abuse. Promoting Participation in Treatment Programs Refer the adolescent to a substance abuse program. Outpatient or day treatment programs are useful in most situations. Family-based programs produce the highest level of recovery. Self-help or 12-step groups are an important element in the recovery process. Serious addiction, the presence of one or more comorbid psychiatric conditions, or suicidal ideation requires residential treatment or hospitalization. See Healthy People 2030. Preventing Substance Abuse Establish a trusting relationship with children and adolescents in order to improve acceptance of education about substance use and to provide a safe environment for confiding about their problems. Screen all children and adolescents for risk factors. During routine psychosocial screening, be alert to alterations as noted earlier. Teach all children, beginning at the elementary school level (or earlier in some high-risk communities), that all chemicals have the potential to be harmful to the body, including tobacco, alcohol, and illicit drugs. Educate children and adolescents that no matter which administration route is used, the drug still enters the body and affects it (negatively). Help children to learn problem-solving skills that they can call upon in the future rather than relying on drugs or other substances to avoid their problems. Teach children to "just say no." Reinforce that they are the ones who have control over their body and what they expose it to. Encourage children to participate in the local community DARE program (Drug Abuse Resistance Education) and praise them for completing it. Teach parents that being involved in their child's social life and knowing where they are and with whom when they are outside of the home is an important step toward limiting substance exposure.

SIADH

occurs when ADH (vasopressin) is secreted in the presence of low serum osmolality because the feedback mechanism that regulates ADH does not function properly. ADH continues to be released, and this leads to water retention, decreased serum sodium due to hemodilution, and extracellular fluid volume expansion. SIADH can be caused by CNS infections such as meningitis, head trauma, brain tumors, intracranial surgery, and certain medications such as analgesics, barbiturates, or chemotherapy. SIADH is rare in children; however, when seen, it is often related to excessive administration of vasopressin during the treatment of DI (Breault & Majzoub, 2016b).Therapeutic management of SIADH includes correcting the underlying disorder in addition to fluid restriction and intravenous sodium chloride administration to correct hyponatremia and increase serum osmolality. Nursing Assessment Obtain a health history, noting history of a CNS infection or tumor, intracranial surgery, head trauma, use of the above-mentioned medications, or a history of DI. Note symptoms such as decreased urine output and weight gain, or GI symptoms such as anorexia, nausea, and vomiting. Assess neurologic status, noting lethargy, behavioral changes, headache, altered level of consciousness, seizure, or coma. Neurologic signs develop as the sodium level decreases. Diagnostic tests reveal low serum sodium and osmolality, as well as decreased urea, creatinine, uric acid, and albumin levels. Urine samples demonstrate elevated osmolality, high sodium concentrations, and specific gravity greater than 1.030. Adrenal, thyroid, and renal function studies may be used to rule out other causes of hyponatremia. Nursing Management Nursing goals focus on restoring fluid balance and preventing injury. Institute safety precautions if altered levels of consciousness, confusion, or seizures are present. Notify the physician or nurse practitioner if headache or irritability is present. Monitor fluid intake and output and weigh the child daily. An indwelling urinary catheter may be needed to allow for hourly monitoring of urine volume and specific gravity. Help the child cope with fluid restriction by offering sugarless candy, a wet washcloth, or, perhaps, ice chips. Administer electrolyte replacement as necessary to correct imbalan

The genome

of an organism is its entire hereditary information encoded in the DNA

In X-linked recessive

pattern of inheritance there are more affected males than females because all the genes on a man's X chromosome will be expressed since a male has only one X chromosome (Scott & Lee, 2016c). On the other hand, a female will usually need two abnormal X chromosomes to exhibit the disease and one normal and one abnormal X chromosome to be a carrier of the disease. There is no male-to-male transmission (since no X chromosome from the male is transmitted to male offspring), but any man who is affected with an X-linked recessive disorder will have carrier daughters. If a woman is a carrier, there is a 25% chance she will have an affected son, a 25% chance that her daughter will be a carrier, a 25% chance that she will have an unaffected son, and a 25% chance her daughter will be a noncarrier (Scott & Lee, 2016c) (Fig. 27.3). Common types of genetic disorders that follow X-linked recessive inheritance patterns include hemophilia and Duchenne muscular dystrophy (Genetics Home Reference, 2019e).

Hemoglobin A1c (HbA1c)

provides the physician or nurse practitioner with information regarding the long-term control of glucose levels (refer to Common Laboratory and Diagnostic Tests 26.1). In children, especially infants and children younger than 6 years, hypoglycemia poses some unique risks and can be hard to recognize. Therefore, the HbA1c goals in children need to take into account the risks of severe hypoglycemia and glycemic control goals need to be individualized (American Diabetes Association, 2019) (refer to Table 26.1). However, recent data has shown that lower HbA1c levels lead to reduced long-term complications; therefore, the targets for HbA1c in children have become lower in recent years. Currently, the American Diabetes Association (2019) recommends that children and adolescents have a target HbA1c <7.5%.

Hypogammaglobulinemia

refers to a variety of conditions in which the child does not form antibodies appropriately. It results in low or absent levels of one or more of the immunoglobulin classes or subclasses. Table 25.1 provides an overview of several types of hypogammaglobulinemia. Therapeutic management of most types of hypogammaglobulinemia is periodic administration of intravenous immunoglobulin (IVIG). Nursing Assessment Note history of recurrent respiratory, gastrointestinal, or genitourinary infections. Palpate for enlarged lymph nodes and spleen in the child with X-linked hyper-IgM syndrome. In children presenting for routine administration of IVIG, determine whether any infections have occurred since the previous infusion. Nursing Management Nursing management of hypogammaglobulinemia involves IVIG administration and the provision of education and support to the child and family. Ensure that the child is well hydrated before the infusion to decrease the risk for rate-related reactions and aseptic meningitis after the infusion. Premedication with diphenhydramine or acetaminophen may be indicated in children who have never received IVIG, have not had an infusion in more than 8 weeks, have had a recent bacterial infection, have a history of serious infusion-related adverse reactions, or are diagnosed with agammaglobulinemia or hypogammaglobulinemia (WKCDI, 2019). The rate for infusion of IVIG is generally prescribed as milligrams of IVIG per kilogram of body weight per minute. Carefully calculate the infusion rate. Obtain a baseline physical assessment and set of vital signs. Begin the infusion slowly, increasing to the prescribed rate as tolerated (see Fig. 25.1). Assess vital signs and check for adverse reactions every 15 minutes for the first hour, then every 30 minutes throughout the remainder of the infusion (the frequency of assessments may vary according to institutional protocol). IVIG is a plasma product, so observe closely for signs of anaphylaxis such as headache, facial flushing, urticaria, dyspnea, shortness of breath, wheezing, chest pain, fever, chills, nausea, vomiting, increased anxiety, or hypotension. If these symptoms occur, discontinue the infusion and notify the physician or nurse practitioner. The infusion may be restarted after the symptoms have subsided. Have oxygen and emergency medications such as epinephrine, diphenhydramine, and intravenous corticosteroids available in case of anaphylactic reaction. If the child complains of discomfort at the intravenous site, a cold compress may be helpful.

Genetics

refers to the study of heredity—its transmission, and characteristics variation. Nurses encounter potential or actual alterations in genetics in all types of clients and must detect problems and intervene early to prevent complications. A genetic disorder is a disease caused by an abnormality in an individual's genetic material or genome. Some genetic disorders occur in multiple family members (via inheritance of abnormal genes). Other disorders may occur in only a single family member (via spontaneous mutation).

Non-Hodgkin Lymphoma NHL

results from mutations in the B and T lymphocytes that lead to uncontrolled growth. NHL tends to affect lymph nodes located more deeply within the body. NHL spreads by the bloodstream and in children is a rapidly proliferating, aggressive malignancy that is very responsive to treatment. Prognosis depends on the cell type involved and the extent of the disease at diagnosis. Ninety percent of children with localized NHL have disease-free long-term survival after treatment (Craddock et al., 2018). Complications include metastasis and the development of a secondary malignancy later in life. Remission is induced with chemotherapy and followed with a maintenance phase of chemotherapy lasting about 2 years. NHL tends to spread easily to the CNS, so CNS prophylaxis similar to that used in leukemia is warranted (Craddock et al., 2018). Autologous bone marrow transplantation may be used in some children. Nursing Assessment Children with NHL are usually symptomatic for only a few days or a few weeks before diagnosis because the disease progresses so quickly. Note onset and location of pain or lymph node swelling. Document history of abdominal pain, diarrhea, or constipation. Explore the health history for risk factors such as congenital or acquired immune deficiency. Observe for increased work of breathing, facial edema, or venous engorgement (mediastinal mass). Palpate for the presence of lymphadenopathy and palpate the abdomen for the presence of a mass. Lymph node biopsy and bone marrow aspiration determine the diagnosis. Computed tomography (CT) scan, chest radiography, and bone marrow results may be used to determine the extent of metastasis.

Chromosomal abnormalitie

s of number often result due to nondisjunction (failure of separation of the chromosome pair) during cell division, meiosis, or mitosis. Few chromosomal numerical abnormalities are compatible with full-term development and most result in spontaneous abortion (Bacino & Lee, 2016). Some numerical abnormalities, however, can support development to term because the chromosome on which the abnormality is present carries relatively few genes (e.g., chromosome 13, 18, 21, or X). Two common abnormalities of chromosome number are monosomies or trisomies. In monosomies there is only one copy of a particular chromosome instead of the usual pair; in these cases all fetuses spontaneously abort in early pregnancy. Survival occurs only in mosaic forms of these disorders. In trisomies, there are three of a particular chromosome instead of the usual two. The most common trisomies include trisomy 21 (Down syndrome), trisomy 18, and trisomy 13 (see below for further discussion). Trisomies may be present in every cell or may present in the mosaic form.

precocious puberty,

the child develops sexual characteristics before the usual age of pubertal onset. Puberty, also known as sexual maturation, occurs when the gonads produce increased amounts of sex hormones. Typically, this occurs around 10 to 12 years of age for girls and 11 to 14 years of age for boys. In precocious puberty, secondary sex characteristics develop in girls before the age of 8 years and in boys younger than 9 years (Harrington & Palmert, 2018). The disorder is more common in females and the majority of the time the cause is unknown in females, while in males a structural CNS abnormality is often present (Garibaldi & Chemaitilly, 2016). Other causes include benign hypothalamic tumor, brain injury or radiation, a history of infectious encephalitis, meningitis, congenital adrenal hyperplasia (CAH), and tumors of the ovary, adrenal gland, pituitary gland, or testes. Pathophysiology Central precocious puberty, the most common form, develops as a result of premature activation of the hypothalamic-pituitary-gonadal axis that results in the production of gonadotropin-releasing hormone (GnRH), which stimulates the pituitary to produce luteinizing hormone (LH) and follicle-stimulating hormone (FSH). These hormones in turn stimulate the gonads to secrete the sex hormones (estrogen or testosterone). The child develops sexual characteristics, shows increased growth and skeletal maturation, and has reproductive capability. Peripheral precocious puberty presents with no early secretion of gonadotropin or maturation of gonads but rather early overproduction of sex hormones. The condition results in increased end-organ sensitivity to low levels of circulating sex hormones and leads to premature pubic hair and breast development. If left untreated, the child may become fertile. In addition, the hormones stimulate rapid growth. Therefore, the child may appear taller than peers but will reach skeletal maturity and closure of the epiphyseal plates early, which results in overall short stature. Therapeutic Management The clinical treatment for precocious puberty first involves determining the cause. For example, if the etiology is a tumor of the CNS, the child undergoes surgery, radiation, or chemotherapy. The treatment for central precocious puberty involves administering a GnRH analog. This is available as a subcutaneous injection given daily, an intranasal compound given two or three times each day, a depot injection given every 3 to 4 weeks, a depot injection given quarterly, or a subcutaneous implant yearly. This analog stimulates gonadotropin release initially but when given on a long-term basis will suppress gonadotropin release. With this treatment, the growth rate slows and secondary sexual development stabilizes or regresses. Medroxyprogesterone injections (Depo-Provera) or tablets (Cycrin) reduce secretion of gonadotropins and prevent menstruation. When treatment is discontinued, puberty resumes according to appropriate developmental stages. The overall aim of treatment is to halt or even reverse sexual development and rapid growth as well as promote psychosocial well-being. Health History The health history may reveal complaints of headaches, nausea, vomiting, and visual difficulties due to the circulating hormones. The psychosocial development is typical for the child's age, but the child may show emotional lability, aggressive behavior, and mood swings. Information from the child and family may also reveal risk factors such as exposure to exogenous hormones, history of CNS trauma or infection, or a family history of early puberty. Physical Examination Physical examination may reveal acne and an adult-like body odor. The child will present with an accelerated rate of growth. The Tanner staging of breasts, pubic hair, and genitalia reveals advanced maturation for the child's age, but the child does not typically display sexual behavior. Laboratory and Diagnostic Testing Radiologic examinations and pelvic ultrasound identify advanced bone age, increased uterus size, and development of ovaries consistent with the diagnosis of precocious puberty. Laboratory studies include screening radioimmunoassays for LH, FSH, estradiol, or testosterone. The child's response to GnRH stimulation confirms the diagnosis of central precocious puberty versus gonadotropin-independent puberty. This test involves administering synthetic GnRH intravenously and drawing serial blood levels, about every 2 hours, of LH, FSH, and estrogen or testosterone. A positive result is defined as pubertal or adult levels of these hormones in response to the GnRH administration. CT, MRI, or skull radiography reveals any lesions in the CNS or tumors or cysts present in the abdomen, pelvic area, or testes. Nursing Management In general, nursing management of the child with precocious puberty focuses on educating the child and family about the physical changes the child is experiencing and how to correctly use the prescribed medications and helping the child to deal with self-esteem issues related to the accelerated growth and development of secondary sexual characteristics. Goals of nursing management include appropriate physical development and pubertal progression appropriate for age. Refer to Nursing Process Overview, earlier in the chapter, and individualize care based on the child's and family's response to this disorder. Providing Education Nursing care involves assessing and documenting the physical changes the child is experiencing and administering medications. Demonstrate correct administration of medication and observe for potential adverse effects (teach this information to the family as well). Encourage the family to comply with follow-up appointments, which typically occur every 6 months, and include scheduled stimulation tests. Inform families that pharmacologic intervention stops when the child reaches the age appropriate for pubertal development. Provide appropriate sex education. Dealing With Self-Esteem Issues Due to the body image changes that differ from their peers, these children may develop self-esteem issues. The goal is for the child to exhibit normal psychosocial development and understand the physical and emotional changes that occur with early onset of puberty. Communicate with the child on an age-appropriate level, even when physical characteristics make the child appear older. Maintain a calm, supportive atmosphere and provide for privacy during examinations. Refer the child and family for counseling as needed. Since the child may have issues with self-image and may be self-conscious, encourage him or her to express his or her feelings about the changes, and use role-playing to show the child how to handle teasing from other children. Let the child know that everyone develops sexual characteristics in time.

Myelomeningocele,

the most severe form of neural tube defect, occurs in approximately 1 in 4,000 live births (Kinsman & Johnston, 2016). Myelomeningocele is a type of spina bifida cystica, and clinically the term "spina bifida" is often used to refer to myelomeningocele. It may be diagnosed in utero via ultrasound. Otherwise it is visually obvious at birth. The newborn with myelomeningocele is at increased risk for meningitis, hypoxia, and hemorrhage. In myelomeningocele, the spinal cord often ends at the point of the defect, resulting in absent motor and sensory function beyond that point (see Fig. 22.14). Therefore, the long-term complications of paralysis, orthopedic deformities, and bladder and bowel incontinence are often seen in children with myelomeningocele. The presence of neurogenic bladder and frequent catheterization put the child at an increased risk for urinary tract infections, pyelonephritis, and hydronephrosis, which may result in long-term renal damage if managed inappropriately. Accompanying hydrocephalus associated with type II Chiari defect is seen in 80% of children with myelomeningocele (Kinsman & Johnston, 2016). Due to the improper development and the downward displacement of the brain into the cervical spine, CSF flow is blocked, resulting in hydrocephalus. The lower the deformity is on the spine, the lower the risk of developing hydrocephalus Children with myelomeningocele usually require multiple surgical procedures. In addition, due to frequent catheterizations, these children are at an increased risk of developing a latex allergy (Kinsman & Johnston, 2016). Learning problems and seizures are common in these children, but the majority of those surviving with myelomeningocele have average intelligence (Kinsman & Johnston, 2016). Ambulation is possible for some children, depending on the level of the lesion. Due to advances in medical treatment, research, and improved services, the life expectancy of children with this disorder has increased and the quality of life has been improved (McLone & Bowman, 2018; Wilson & Stewart, 2016). Pathophysiology The cause of myelomeningocele is unknown, but risk factors are consistent with other neural tube defects, such as maternal drug use, malnutrition, and a genetic predisposition (Kinsman & Johnston, 2016). In myelomeningocele, the neural tube fails to close at the end of the fourth week of gestation. As a result, an external sac-like protrusion that encases the meninges, spinal fluid, and in some cases nerves is present on the spine (Fig. 22.13). A myelomeningocele can be located anywhere along the spinal cord, but the highest incidence occurs in the lumbosacral region (Kinsman & Johnston, 2016). The degree of neurologic deficit will depend on the location and size of the lesion (Kinsman & Johnston, 2016). An increase in neurologic deficit is seen with higher lesions as more nerves are affected. Physical Examination Initial assessment after delivery will reveal a visible external sac protruding from the spinal area (Fig. 22.14). Observe the baby's general appearance and assess whether the sac covering is intact. Assess neurologic status and look for associated anomalies. Assess for movement of extremities and anal reflex, which will help determine the level of neurologic involvement. Flaccid paralysis, absence of deep tendon reflexes, lack of response to touch and pain stimuli, skeletal abnormalities such as club feet, constant dribbling of urine, and a relaxed anal sphincter may be found. In the older infant or child, perform a thorough physical examination and focus on the functional assessment. Note the level of paralysis or paresthesia. Inspect skin for breakdown. Determine the child's motor capabilities. Laboratory and Diagnostic Tests A myelomeningocele may be detected prenatally around 16 to 18 weeks' gestation by ultrasound, by a blood test that detects AFP increases, or by analysis of amniotic fluid for AFP increases. Common laboratory and diagnostic studies ordered for the assessment of myelomeningocele include: MRI CT Ultrasound Myelography Preventing Infection Risk for infection related to the presence of the meningeal sac and potential for rupture is a central nursing concern in the newborn with myelomeningocele. Until surgical intervention occurs, the goal is to prevent rupture or leakage of CSF from the sac. Keeping the sac from drying out is important, as is preventing trauma or pressure on the sac. Use sterile saline-soaked nonadhesive gauze or antibiotic-soaked gauze to keep the sac moist. Immediately report any seepage of clear fluid from the lesion, as this could indicate an opening in the sac and provide a portal of entry for microorganisms. Position the infant in the prone position or supported on the side to avoid pressure on the sac. To keep the infant warm, place the infant in a warmer or isolette to avoid the use of blankets, which could exert too much pressure on the sac. Pay special attention while the infant is in a warmer or isolette because the radiant heat can cause drying and cracking of the sac. Keep the lesion free of feces and urine to help avoid infection. Position the infant so that urine and feces flow away from the sac (e.g., prone position, or place a folded towel under the abdomen) to help prevent infection. Placing a piece of plastic wrap below the meningocele is another way of preventing feces from coming into contact with the lesion. After surgery, position the infant in the prone or side-lying position to allow the incision to heal. Continue with precautions to prevent urine or feces from coming into contact with the incision.

Abnormalities of chromosome structure

usually occur when there is a breakage and loss of a portion of one or more chromosomes, and during the repair process the broken ends are rejoined incorrectly. Structural abnormalities usually lead to having too much or too little genetic material. Altered chromosome structure can take on several forms. Deletions occur when a portion of the chromosome is missing, resulting in a loss of that portion of the chromosome. Duplications are seen when a portion of the chromosome is duplicated and an extra chromosomal segment is present. Clinical findings vary depending on how much chromosomal material is involved. Inversions occur when a portion of the chromosome breaks off at two points and is turned upside down and reattached; therefore, the genetic material is inverted. With inversion, there is no loss or gain of chromosomal material and carriers are phenotypically normal, but they do have an increased risk for miscarriage and having chromosomally abnormal offspring (Bacino & Lee, 2016). Ring chromosomes are seen when a portion of a chromosome has broken off in two places and has formed a circle or ring. The most clinically significant structural abnormality is a translocation. This occurs when a portion of one chromosome is transferred to another chromosome and an abnormal rearrangement is present. Structural abnormalities can be balanced or unbalanced. Balanced abnormalities involve the rearrangement of genetic material with neither an overall gain nor loss. Individuals who inherit a balanced structural abnormality are usually phenotypically normal but are at a higher risk for miscarriages and having chromosomally abnormal offspring. Examples of structural rearrangements that can be balanced include inversions, translocations, and ring chromosomes. Unbalanced structural abnormalities are similar to numerical abnormalities because genetic material is either gained or lost. Unbalanced structural abnormalities can encompass several genes and result in severe clinical consequences.

Medical child abuse

was historically termed Munchausen syndrome by proxy. It is a type of child abuse in which the parent creates physical and/or psychological symptoms of illness or impairment in the child. The adult meets his or her own psychological needs by having an ill child. Medical child abuse is difficult to detect and may remain hidden for years. In most cases, the biologic mother is the perpetrator (Roesler & Jenny, 2018). Therapeutic management focuses on ensuring the safety and well-being of the child, as well providing psychotherapy for the perpetrator. Nursing Assessment Take a thorough and detailed health history of the child's illness or illnesses. Use quotations to document the parent's responses. Warning signs of medical child abuse include: Child with one or more illnesses that do not respond to treatment or that follow a puzzling course; a similar history in siblings Symptoms that do not make sense or that disappear when the perpetrator is removed or not present; the symptoms are witnessed only by the caregiver (e.g., cyanosis, apnea, seizure) Physical and laboratory findings that do not fit with the reported history Repeated hospitalizations failing to produce a medical diagnosis, transfers to other hospitals, discharges against medical advice Parent who refuses to accept that the diagnosis is not medical (Roesler & Jenny, 2018) Observe the mother's behavior with the child, spouse or partner, and staff. Use of covert video surveillance may reveal maternal actions causing illness in the child when the nurse, physician, or nurse practitioner is not in the room. Perform a thorough physical examination, noting where the physical examination findings differ from the reported health history. Nursing Management Management of medical child abuse is complex. When abusive activity is identified, notify the social services and risk management departments of the hospital. Ensure that the local child protection team and the caregiver's family or support system is present when the caregiver is confronted. Inform the caregiver of the plan of care for the child and of the availability of psychiatric assistance for the caregiver.

Atopic dermatitis

(AD; also called eczema) is one of the disorders in the atopy family (along with asthma and allergic rhinitis). Atopic dermatitis affects 12.5% to 20% of U.S. children (Kent & Clark, 2018). Onset of symptoms is usually before 2 years of age (Prok & Torres-Zegarra, 2018). Atopic dermatitis is often associated with food allergies, allergic rhinitis, and asthma, though not all children with AD will develop one of those other disorders (Kent & Clark, 2018). The chronic itching associated with atopic dermatitis causes a great deal of psychological distress. The child's self-image may be affected, particularly if the rash is extensive. Difficulty sleeping may occur because of the itching. The child is irritable and has difficulty concentrating, and family life is disrupted. Parents' stress related to the child's condition may increase the child's anxiety and lead to an increase in itching and scratching. The child may outgrow atopic dermatitis, its severity may decrease as the child approaches adulthood, or the child may continue to have difficulties into the adult years. Bacterial superinfection may occur as a complication. Therapeutic management includes good skin hydration, application of topical corticosteroids or immune modulators, oral antihistamines for sedative effects, and antibiotics if secondary infection occurs. Pathophysiology Atopic dermatitis is a chronic disorder characterized by extreme itching and inflamed, reddened, and swollen skin. It has a relapsing and remitting nature. The skin reaction occurs in response to specific allergens, usually food (especially eggs, wheat, milk, and peanuts) or environmental triggers (e.g., molds, dust mites, and cat dander). Other factors, such as high or low ambient temperatures, perspiring, scratching, skin irritants, or stress, may contribute to flare-ups. When the child encounters a triggering antigen, antigen-presenting cells stimulate interleukins to begin the inflammatory process. The skin begins to feel pruritic and the child starts to scratch. The sensation of itchiness comes first, and then the rash becomes apparent. The scratching causes the rash to appear. Sweating causes atopic dermatitis to worsen, as does excessively humid or dry environments. Health History Elicit a description of the present illness and chief complaint. Common signs and symptoms reported during the health history might include: Wiggling or scratching Dry skin Scratch marks noticed by the parents Disrupted sleep Irritability Explore the child's current and past medical history for risk factors such as: Family history of atopic dermatitis, allergic rhinitis, or asthma Child's history of asthma or allergic rhinitis Food or environmental allergies INSPECTION AND OBSERVATION Observe whether the infant is wiggling, or the child is actively scratching. Carefully inspect the skin. Document dry, scaly, or flaky skin, as well as hypertrophy and lichenification (Fig. 23.12). If lesions are present they may be dry lesions or weepy papules or vesicles (fluid-filled lesions). In children younger than 2-years-old, the rash is most likely to occur on the face, scalp, wrists, and extensor surfaces of the arms or legs. In older children it may occur anywhere on the skin but is found more commonly on the flexor areas. Note erythema or warmth, which may indicate associated secondary bacterial infection. Document areas of hyperpigmentation or hypopigmentation, which may have resulted from a prior exacerbation of atopic dermatitis or its treatment. Inspect the eyes, nose, and throat for symptoms of allergic rhinitis. Laboratory and Diagnostic Tests Serum immunoglobulin E (IgE) levels may be elevated in the child with atopic dermatitis. Skin prick allergy testing may determine the food or environmental allergen to which the child is sensitive. Promoting Skin Hydration First and foremost, avoid hot water and any skin or hair product containing perfumes, dyes, or fragrance. Bathe the child twice daily in warm (not hot) water. Use a mild soap to clean only the dirty areas. Recommended mild soaps or cleansing agents include: Unscented Dove or Dove for sensitive skin Tone Caress Oil of Olay Cetaphil Aquanil Slightly pat the child dry after the bath, but do not rub the skin with the towel. Leave the child moist. Apply prescribed topical ointments or creams such as corticosteroids or immune modulators (see Drug Guide 23.1) to the affected area. Apply fragrance-free moisturizer over the prescribed topical medication and all over the child's body. Recommended moisturizers include: Eucerin, Moisturel, Curel (cream or lotion) Aquaphor Vaseline Crisco Apply moisturizer multiple times throughout the day. Avoid clothing made of synthetic fabrics or wool. Avoid triggers known to exacerbate atopic dermatitis. Herbal supplements and oils (such as evening primrose oil) have demonstrated mixed results for reduction in redness and scaling. If not initially recommended by the physician or nurse practitioner, the parent should consult with him or her first before starting these supplements. Headache and nausea are rare adverse effects of these supplements and, if they occur, are usually mild. Chamomile preparations for topical use help some children and are generally considered safe. Maintaining Skin Integrity and Preventing Infection Cut the child's fingernails short and keep them clean. Avoid tight clothing and heat. Use 100% cotton bed sheets and pajamas. In addition to keeping the child's skin well moisturized, it is extremely important to prevent the child from scratching. Scratching causes the rash to appear and further scratching may lead to secondary infection. Antihistamines given at bedtime may sedate the child enough to allow him or her to sleep without awakening because of itching. During the waking hours, behavior modification may help to keep the child from scratching. Have the parent keep a diary for 1 week to determine the pattern of scratching. Help the parent to determine specific strategies that may raise the child's awareness of scratching. A handheld clicker or counter may help to identify the scratching episode for the child, thus raising awareness. The use of diversion, imagination, and play may help to distract the child from scratching. The parent and child may create a game together that results in the child participating in a behavior rather than scratching. Pressing the skin or clenching the fist may replace scratching. It is important for the child to stay active to distract his or her mind from the itching. It is important for the parent to positively reinforce and reward the desired behaviors. See Evidence-Based Practice 23.1.

Bryant traction

Both legs are extended vertically, with child's weight serving as countertraction. Skin traction is applied to both legs. Used to reduce femur fracture in children younger than 2 years or with developmental dysplasia of the hip. Maintain appropriate position. Ensure heels and ankles are free from pressure. Assess condition and position of elastic bandages every shift and rewrap elastic bandages as ordered.

Contact Dermatitis

Direct or indirect contact with the plant's oleoresin found in the leaves, stems, and roots results in an allergic reaction. Even contact with dormant plants or plants perceived to be dead may cause an allergic response. The rash is extremely pruritic and may last for 2 to 4 weeks; lesions continue to appear during the illness. Contact dermatitis is not contagious and does not spread either to other parts of the affected child's skin or to other people. Scratching does not spread the rash, but it may cause skin damage or secondary infection. Complications of contact dermatitis include secondary bacterial skin infections and lichenification or hyperpigmentation, particularly in dark-skinned people. Therapeutic management is directed toward management of itching and the use of topical corticosteroids. Moderate-potency topical glucocorticoid cream or ointment is used for mild to moderate contact dermatitis, and high-potency preparations are used for more severe cases. Some severe cases of contact dermatitis may require the use of systemic steroids. Nursing Management Contact dermatitis may be prevented by avoiding contact with the allergen. When the condition does occur, nursing management focuses on relieving the discomfort associated with the rash. Administer topical or systemic corticosteroids as prescribed and teach the family about use of the medications. Teaching Guidelines 23.2 gives more information about the treatment and prevention of contact dermatitis. TEACHING GUIDELINES 23.2 Prevention and Treatment of Contact Dermatitis Prevention Wear long sleeves and long pants on outings in the woods. Identify and remove offending plants in the yard by using a commercial weed or underbrush killer. Vinyl gloves (not rubber or latex) are an effective barrier. The plant's oil residue may be on clothes, pets, garden and sports equipment, and toys; wash those well with soap and water. If contact occurs, wash vigorously with soap and water within 10 minutes of contact. Zanfel and Tecnu Oak-N-Ivy Outdoor Skin Cleanser (both soap mixtures) may prevent rash if used to wash the skin soon after exposure. Ivy Block (an organoclay) is the only U.S. Food and Drug Administration-approved preventive treatment for contact dermatitis related to poison ivy, oak, or sumac. (Visit for web links that will provide additional information.) It is applied to the skin before possible exposure. Treatment Wash lesions daily with mild soap and water. Mildly debride crusted lesions. Tepid baths (colloidal oatmeal such as Aveeno) are helpful to decrease itching. Avoid hot baths or showers, as they aggravate itching. Apply corticosteroid preparations topically as directed (if using high-potency preparations, do not cover with an occlusive dressing). Weeping lesions may be wrapped lightly; avoid occlusion. Burow or Domeboro solutions with a dressing applied twice daily for 20 minutes may help to dry weepy lesions. Over-the-counter preparations such as calamine lotion or Ivy Rest may reduce itching and help the lesions to dry. Do not use topical antihistamines, benzocaine, or neomycin because of the potential for sensitization.

Lead Poisoning

Due to concerted efforts in the past few decades to screen for lead poisoning in young children, the current prevalence in U.S. children between 1 and 5 years of age is 0.8% (Weitzman, 2017). Lead exerts toxic effects on the bone marrow, erythroid cells, nervous system, and kidneys. The presence of lead in the bloodstream interferes with the enzymatic processes of the biosynthesis of heme. The process results in hypochromic, microcytic anemia, and children may exhibit classic signs of anemia. Risk factors for lead poisoning are related to lead exposure in the home, school, or local environment. Sources of lead include: Paint in homes built before 1978, at which time lead was banned as an additive to paint used in houses Soil where cars that used leaded gas have been in the past (lead was removed from all gasoline in the United States as of 1996) Glazed pottery Stained glass products Lead pipes supplying water to the home On the clothing of parents who work in certain manufacturing jobs (battery makers, cable makers) Certain folk remedies, such as greta or azarcon Old painted toys or furniture (Lowry, 2018) Complications of lead poisoning include behavioral problems and learning difficulties and, with higher lead levels, encephalopathy, seizures, and brain damage. Therapeutic management for high blood levels of lead involves chelation therapy (removal of heavy metals from the body via chelating agents), either orally or intravenously. Drug Guide 24.1 gives further information on chelating agents. Nursing Assessment Explore the health history for subtle signs such as anorexia, fatigue, or abdominal pain. Determine whether behavioral problems, irritability, hyperactivity, or lack of ability to meet developmental milestones have occurred in recent months. Screen children for risk of exposure to lead in the home. Refer to Chapter 9 for a simple screening questionnaire that can be used to determine the need for lead screening in young children. Blood levels of lead greater than 10 μg/dL require conscientious follow-up. Note pallor of the skin. Nursing Management Prevention of elevated lead levels is critical. Screen children for lead exposure risk. The AAP recommends performing a risk assessment at 6, 9, 12, 18, and 24 months, and 3, 4, 5, and 6 years (Hagan, Shaw, & Duncan, 2017). If positive, the decision may be made to evaluate a blood lead level (Weitzman, 2017). Table 24.4 gives recommendations for appropriate follow-up depending on lead levels. Removing old paint is the best way to eliminate the most significant source of lead exposure for a large number of children. If the family rents or lives in public housing, the landlord or owner is responsible for following the guidelines set forth by local and state governmental agencies to correct the problem. Educate families about how to prevent exposure to lead, particularly in young children. Additional resources for families are located on . If the child is undergoing chelation therapy, ensure adequate fluid intake and monitor intake and output closely. Refer children with elevated lead levels and developmental or cognitive deficits to developmental centers. These children may need an early intervention program for further evaluation and treatment of developmental delays. See Healthy People 2030

Obtain a printed or written copy of the child's treatment plan Keep a calendar of all appointment times, blood count lab draw days, and phone numbers of all physicians and nurse practitioners, home care companies, the laboratory, and the hospital Seek medical care IMMEDIATELY if the child's temperature is 38.3°C (101°F) or higher Call the oncologist or seek medical care if any of the following occur: Cough or rapid breathing Increased bruising, bleeding or petechiae, pallor, or increased levels of fatigue Earache, sore throat, nuchal rigidity Blisters, rashes, ulcers Red, irritated skin on the child's buttocks Abdominal pain, difficulty or pain with eating, drinking, or swallowing Constipation or diarrhea For children with central venous catheters: Pus, redness, or swelling at the site Breakage of the catheter Do not give the child aspirin

Education for Families of Children With Cancer

tibial torsion.

In utero, the fetus' hips are usually flexed, abducted, and externally rotated, with the knees also flexed and the lower limbs inwardly rotated. This normal developmental variation is termed internal tibial torsion. The legs straighten with passive motion, and internal tibial torsion should not be confused with "bowlegs" (Fig. 22.2). Internal tibial torsion usually resolves independently within the second or third year of life as the toddler bears weight and the lower extremity muscles and bones mature

Halo traction

Metal halo attached to skull via pins. Used for cervical or high thoracic vertebrae fracture or dislocation and for postoperative immobilization following cervical fusion. Refer to nursing implications for cervical tongs. Tape small wrench to front of brace so that front panel can be quickly removed in an emergency. May become ambulatory in this type of traction; will be top-heavy so may need assistance with balance Assess pin sites and provide pin care as ordered.

Tinea capitis

Patches of scaling in the scalp with central hair loss Risk of kerion development (inflamed, boggy mass that is filled with pustules) (Fig. 23.7) Oral griseofulvin for 4-6 weeks Selenium sulfide shampoo may be used to decrease contagiousness (adjunct only) No school or day care for 1 week after treatment initiated

Tinea pedis (athlete's foot)

Red, scaling rash on soles and between the toes (Fig. 23.9) Topical antifungal cream, powder, or spray Appropriate foot hygiene

Balanced suspension traction

Used for femur, hip, or tibial fracture. Thomas splint suspends the thigh while the Pearson attachment allows knee flexion and supports the leg below the knee. Avoid pressure to popliteal area.

Acne vulgaris

affects about 85% of adolescents, beginning as early as age 7 to 10 years between the ages of 12 and 16 years, and endogenous androgens play a role in its development (Prok & Torres-Zegarra, 2018). It occurs most frequently on the face, chest, and back. Risk factors for the development of acne vulgaris include preadolescent or adolescent age, male gender (due to the presence of androgens), an oily complexion, Cushing syndrome, or another disease process resulting in increased androgen production. Pathophysiology The sebaceous gland produces sebum and is connected by a duct to the follicular canal that opens on the skin's surface. Androgens stimulate sebaceous gland proliferation and production of sebum. These hormones exhibit increased activity during the pubertal years. Abnormal shedding of the outermost layer of the skin (the stratum corneum) occurs at the level of the follicular opening, resulting in a keratin plug that fills the follicle. The sebaceous glands increase sebum production. Bacterial overgrowth of Propionibacterium acnes occurs because the presence of sebum and keratin in the follicular canal creates an excellent environment for growth. Inflammation occurs as the follicular wall perforates, allowing the contents to leak into nearby tissue. Therapeutic Management Therapeutic management focuses on reducing P. acnes, decreasing sebum production, normalizing skin shedding, and eliminating inflammation. Teach the adolescent to cleanse the skin gently twice a day. Medication therapy may include a combination of benzoyl peroxide, salicylic acid, retinoids, and topical or oral antibiotics. Isotretinoin may be used in severe cases. Drug Guide 23.1 gives further information on these medications. In girls, oral contraceptives may help lessen acne by decreasing the effects of androgens on the sebaceous glands. Diode laser or blue ultraviolet light therapy may also be used. CO2 lasers and dermabrasion may be used to treat pitted scarring. Nursing Management Avoid oil-based cosmetics and hair products, as their use may block pores, contributing to noninflammatory lesions. Look for cosmetic products labeled as noncomedogenic. Headbands, helmets, and hats may exacerbate the lesions by causing friction. Dryness and peeling may occur with acne treatment, so encourage the child to use a humectant moisturizer. Mild cleansing with soap and water twice daily is appropriate. Avoid excessive scrubbing and harsh chemical or alcohol-based cleansers. Avoid picking or squeezing the lesions. Using a noncomedogenic sunscreen with an SPF of 30 or higher is recommended (Chen & Williams, 2017). Teach adolescents that the prescribed topical medications must be used daily and that it may take 4 to 6 weeks to see results. Avoid the use of over-the-counter preparations because they are irritating and aggravate the drying effect of prescription acne treatments. Instruct boys to shave gently and avoid using dull razors, so as not to further irritate the condition. Adolescent girls taking isotretinoin who are sexually active must be on a pregnancy prevention program because the drug causes defects in fetal development (Chen & Williams, 2017) (Box 23.2). If the acne is severe, depression may occur as a result of body image disturbances. Provide emotional support to adolescents undergoing acne therapy. Refer teens for counseling if necessary.

Thalassemia

is a genetic disorder that most often affects those of African descent, but it also affects individuals of Caribbean, Middle Eastern, South Asian, and Mediterranean descent (Brandow & Scott, 2019). The genetics of thalassemia are similar to those of SCD in that it is inherited via an autosomal recessive process. Children with thalassemia have reduced production of normal hemoglobin. There are two basic types of thalassemia, α and β. In α-thalassemia, synthesis of the α chain of the hemoglobin protein is affected. Problems with the β chain occur more often, and the condition β-thalassemia can be divided into three subcategories based on severity: Thalassemia minor (also called β-thalassemia trait): leads to mild microcytic anemia; often no treatment is required. Thalassemia intermedia: child requires blood transfusions to maintain adequate quality of life. Thalassemia major: to survive the child requires ongoing medical attention, blood transfusions, and iron removal (chelation therapy). The focus of this discussion will be on β-thalassemia major (Cooley anemia). In β-thalassemia major, the β-globulin chain in hemoglobin synthesis is reduced or entirely absent. A large number of unstable globulin chains accumulate, causing the RBCs to be rigid and hemolyzed easily. The result is severe hemolytic anemia and chronic hypoxia. In response to the increased rate of RBC destruction, erythroid activity is increased. The increased activity causes massive bone marrow expansion and thinning of the bony cortex. Growth retardation, pathologic fractures, and skeletal deformities (frontal and maxillary bossing) result. Hemosiderosis (excessive supply of iron) is an additional complication of significant concern. It occurs as a result of rapid hemolysis of RBCs, the decrease in hemoglobin production, and the increased absorption of dietary iron in response to the severely anemic state. The excess iron is deposited in the body's tissues, causing bronze pigmentation of the skin, bony changes, and altered organ function, particularly in the cardiac system. Additional complications include splenomegaly, endocrine abnormalities, osteoporosis, liver and gallbladder disease, and leg ulcers. Left untreated, β-thalassemia major is fatal usually by age 5 years, but the use of blood transfusions and chelation therapy has increased the life expectancy of these children (Benz, 2018). Therapeutic Management The therapeutic management for children with β-thalassemia includes monitoring hemoglobin and hematocrit and transfusing PRBCs at regular intervals. Blood iron levels are also monitored and iron chelation therapy is provided. Nursing Assessment Infants are usually diagnosed by 1 year of age and have a history of pallor, jaundice, failure to thrive, and hepatosplenomegaly (Benz, 2018). Determine the history of the present illness or whether the child is presenting for a routine blood transfusion. Note medications taken at home and any concerns that have arisen since the last visit. Inspect the skin, oral mucosa, conjunctivae, soles, and/or palms for pallor. Note icteric sclerae or jaundice of the skin. Measure weight and height (or length) and plot on an appropriate growth chart. Observe the child for bony deformities and frontal bossing (prominent forehead) (Fig. 24.12). Measure oxygen saturation via pulse oximetry. Evaluate neurologic status, determining level of consciousness and developmental abilities. Laboratory testing may reveal the following: Hemoglobin and hematocrit are significantly decreased. Peripheral blood smear shows prominence of target cells, hypochromia, microcytosis, and extensive anisocytosis and poikilocytosis (variation in the size and shape of the RBCs, respectively). Bilirubin levels are elevated. Hgb electrophoresis shows the presence of Hgb F and Hgb A2 only. Iron level is elevated. Nursing Management The nursing care of the child with thalassemia is primarily aimed at supporting the family and minimizing the effects of the illness. This includes administering blood transfusions and educating the family. Administering Packed Red Blood Cell Transfusions Administer PRBC transfusions as prescribed to maintain an adequate level of hemoglobin for oxygen delivery to the tissues and to suppress erythrocytosis in the bone marrow. Monitor for reactions to the transfusions. Excess iron is removed by chelation therapy. Administer the chelating agent deferoxamine with the transfusion. Deferoxamine binds to the iron and allows it to be removed through the stool or urine. Oral deferasirox may also be prescribed and is generally well tolerated, with minimal GI side effects. Educating the Family Educate the child and family about the recommended regimen. Ensure that families understand that adhering to the prescribed blood transfusion and chelation therapy schedule is essential to the child's survival. Chelation therapy must be maintained at home to continuously decrease the iron levels in the body. Teach family members to administer deferoxamine subcutaneously with a small battery-powered infusion pump over a several-hour period each night (usually while the child is sleeping). If oral deferasirox is prescribed, instruct the family to dissolve the tablet in juice or water and administer it once daily.

Hemophilia

is a group of X-linked recessive disorders that result in deficiency in one of the coagulation factors in the blood. X-linked recessive disorders are transmitted by carrier mothers to their sons, so usually only males are affected by hemophilia. The coagulation factors in the blood are essential for clot formation either spontaneously or from an injury, and when factors are absent bleeding will be difficult to stop. There are several types of hemophilia, including factor VIII deficiency (hemophilia A), factor IX deficiency or Christmas disease (hemophilia B), and factor XI deficiency (hemophilia C). The most common, hemophilia A, will be the focus of this discussion (Ambruso et al., 2018). Hemophilia A occurs when there is a deficiency of factor VIII in an individual. Factor VIII is essential in the activation of factor X, which is required for the conversion of prothrombin into thrombin, resulting in an inability of the platelets to be used in clot formation. Hemophilia is classified according to the severity of the disease, ranging from mild to severe. The more severe the disease, the more likely it is that there will be bleeding episodes. When bleeding occurs, the vessels constrict and a platelet plug forms, but because of the deficient factor the fibrin will not solidify, and thus bleeding continues. Therapeutic Management The primary goal of managing hemophilia is to prevent bleeding. This is best accomplished by instructing the child to avoid activities with a high potential for injury (e.g., football, riding motorcycles, skateboarding). Instead, encourage the child to participate in activities with the least amount of contact (e.g., swimming, running, tennis). Limiting activities does not mean the child should do nothing; activities that promote health without increased exposure to injury are best. If bleeding or injury occurs, factor administration is prescribed; this practice has been common in outpatient facilities or the child's home for many years. Once the deficient factor is replaced, clotting factors return to fairly normal levels for a period of time. Factor replacement should be given before any surgeries or other procedures that can lead to bleeding, such as intramuscular injections and dental care. Health History Elicit the health history, determining the nature of the bleeding episode or bruise. Include in the history any hemorrhagic episodes in other systems, such as the GI tract (e.g., black tarry stools, hematemesis) or as a result from injury resulting in joint hemorrhage, or hematuria (Fig. 24.15). Inquire about length of bleeding and amount of blood loss. Because hemophilia A results in difficulty with clotting, the child may bleed for a longer period when injury occurs. Preventing Bleeding Episodes All children with hemophilia should attempt to prevent bleeding episodes. Recurrent bleeding into the joints (hemarthroses) may cause joint destruction, thereby limiting range of motion and function over the long term (Ambruso et al., 2018). Teach children and families that regular physical activity or exercise helps to keep the muscles and joints stronger, and children with stronger joints and muscles have fewer bleeding episodes (see Teaching Guidelines 24.4). Refer the child with moderate to severe hemophilia to a pediatric hematologist and/or a comprehensive hemophilia treatment center. TEACHING GUIDELINES 24.4 Preventing Bleeding in the Child With Hemophilia Protect toddlers with soft helmets, padding on the knees, carpets in the home, and softened or covered corners. Children should stay active: swimming, baseball, basketball, and bicycling (wearing a helmet) are good physical activities. Avoid intense contact sports such as football, wrestling, soccer, and high diving. Avoid trampoline use and riding all-terrain vehicles (ATVs). Arrange premedication with Amicar if oral surgery is indicated.

genu varum

The bowlegged appearance is sometimes also referred to as genu varum. As internal tibial torsion or genu varum resolves, physiologic genu valgum occurs. Children usually demonstrate symmetric genu valgum (knock-knees) by the age of 2 to 3 years. In genu valgum, when the knees are touching, the ankles are significantly separated, with the lower portion of the legs angled outward (Fig. 22.3). By age 7 or 8 years, genu valgum gradually resolves in most children.

V

are a common preventable mechanism of injury among children and adolescents. Young children are at highest risk for burns and the mortality rate from burns is highest in children younger than 5 years of age (Joffe, 2019). Most pediatric burn-related injuries do not result in death, but injuries from burns often cause extreme pain and extensive burns can result in serious disfigurement. In young children, 60% to 80% of burns are scald burns (Joffe, 2019). Fires in the home are often related to cooking, cigarette or other smoking materials. Carbon monoxide poisoning often occurs in conjunction with burns as a result of smoke inhalation, and infants and children are at greater risk for carbon monoxide poisoning than adults. Great advances have been made in the care of children with serious burns. As a result of improved burn care, children who in the past would have died as a result of burns over large body surface areas have a much greater chance of survival (Joffe, 2019). Conventional wisdom is that children with severe burns should be transferred to a specialized burn unit. The American Burn Association has developed the following criteria for referral of burned persons to a specialized burn unit: Partial thickness burns greater than 10% of total body surface area Burns that involve the face, the hands and feet, genitalia, perineum, or major joints Full-thickness burns of any size Chemical or electrical burns (including lightning injury) Inhalation injury Burn injury in children who have pre-existing conditions that might affect their care Persons with burns and traumatic injuries Persons who will require special social, emotional, or long-term rehabilitative care Burned children in a hospital without qualified personnel or equipment for the care of children (Joffe, 2019) Pathophysiology Burned tissue begins to coagulate after the injury, and direct coagulation and microvascular reactions in the adjacent dermis may extend the burn. The blood vessels demonstrate increased capillary permeability, resulting in vasodilatation. This leads to increased hydrostatic pressure in the capillaries, causing water, electrolytes, and protein to leak out of the vasculature and result in significant edema. Edema forms very rapidly in the first 18 hours after the burn, peaking at around 48 hours. Capillary permeability then returns to normal between 48 and 72 hours after the burn and the lymphatics can reabsorb the edema fluid. Diuresis occurs, ridding the body of the excess fluid. Fluid loss from burned skin occurs at an amount that is 5 to 10 times greater than that from undamaged skin, and this fluid loss continues until the damaged surface is healed or grafted. Initially, the severely burned child experiences a decrease in cardiac output, with a subsequent hypermetabolic response during which cardiac output increases dramatically. During this heightened metabolic state, the child is at risk for insulin resistance and increased protein catabolism. Children who are burned during an indoor or chemical fire are at an increased risk of respiratory injury. Children who have aspirated hot liquids are particularly at risk for airway-altering edema. Therapeutic Management Therapeutic management of burns focuses on fluid resuscitation, wound care, prevention of infection, and restoration of function. Burn infections are treated with antibiotics specific to the causative organism. If invasive burn damage occurs, surgery may be necessary. Nursing Management Nursing management of the child who has been burned focuses first on stabilizing the child. Place the child on a cardiac/apnea monitor, measure the child with the Broselow tape, monitor pulse oximetry, and apply an end-tidal CO2 monitor if the child is ventilated. Further management focuses on cleansing the burn, pain management, and prevention and treatment of infection. Fluid status and nutrition are important components of burn care, particularly in the early stages. Rehabilitation of the child with severe burns is also an important nursing function. Providing child and family education about the prevention of burns as well as care of burns at home is critical. Nursing Process Overview, on page 809, gives additional interventions related to fluid and nutritional management. Promoting Oxygenation and Ventilation Institute emergency airway management as needed. If the child requires intubation, make sure that the tracheal tube is taped in a very secure manner, as reintubation in these children will become increasingly difficult as the edema spreads. The burned child's respiratory status warrants vigilant evaluation and reevaluation, as airway edema that is secondary to a burn may not become evident until 2 days after the injury. Administer 100% oxygen via nonrebreather mask or bag-valve-mask ventilation to all children with severe burns. Continue to reassess the child's pulmonary status, adjusting the interventions as necessary (refer to Chapter 29 for further information about respiratory emergency care). Restoring and Maintaining Fluid Volume Several formulas are available for the calculation of resuscitative fluids in children. Most experts recommend that pediatric burn therapy include: Fluid calculation based on the body surface area burned (Fig. 23.22) Use of a crystalloid (Ringer lactate) during the first 24 hours; in smaller children, a small amount of dextrose may be added Administration of most of the volume during the first 8 hours (amounts and timing of fluid volume resuscitation will vary from child to child) Reassessment of the child and adjustment of the fluid rate accordingly; fluid requirements greatly decrease after 24 hours and should be adjusted to reflect this. Administration of a colloid fluid later in therapy once capillary permeability is less of a concern Monitoring of the child's urine output as part of ongoing assessment of response to therapy, expecting at least 1 mL/kg/hr Daily weights obtained at the same time each day (the best indicator of fluid volume status) Monitoring of electrolyte levels (particularly sodium and potassium) for their return to normal levels Managing Pain Pain management is of the utmost importance, and several options are available for the treatment of burn-related pain. Local anesthesia, sedatives, and systemic analgesics are commonly used. Children who have less severe burns that are managed at home can be given oral medications such as acetaminophen with codeine 30 to 45 minutes before dressing changes. In burns that result in more severe pain, the child should be hospitalized and given intravenous pain control with medications such as morphine sulfate. Midazolam (a sedative) may be used in conjunction with pain medication for pain reduction during dressing changes. Pain may also occur at any time of the day or night, not just in relation to dressing changes. Assess the child's pain status frequently using an age-appropriate pain assessment scale. Administer pain medications as prescribed and/or use nonpharmacologic techniques to alleviate or decrease the child's perception of pain. Burn Prevention Keep hot water heater temperature lower than 120°F. Test bath water temperature before bathing children. Keep children away from open flames, stoves, and candles. Cook with pots on the inside of the stove with the handles turned in. Keep children away from the stove while cooking. Place hot liquids out of reach of children. Avoid drinking hot beverages while holding a child. Keep curling irons out of reach of children. Teach older children how to safely get out of the house in case of fire. Practice fire drills. Teach children to "stop, drop, and roll" if their clothes catch on fire. Providing Burn Care at Home For First-Degree (Superficial) Burns Run cool water over the burned area until the pain lessens. Do not apply ice to the skin. Do not apply butter, ointment, or cream. Cover the burn lightly with a clean, nonadhesive bandage. Administer acetaminophen or ibuprofen for pain. Have the child seen by the physician or nurse practitioner within 24 hours. Ongoing care: clean in tub or shower with fragrance-free mild soap; pat or air dry. Apply a thin layer of antibiotic ointment. Cover with a nonadherent dressing such as Adaptic, and then cover with dry gauze. For More Extensive Burns Remove clothing only if it comes off easily or if it is still smoldering. Check the child's ABCs (airway, breathing, and circulation) and perform cardiopulmonary resuscitation (CPR) if necessary. Do not apply butter, ointment, or any other type of cream. Cover the burn with a clean, lint-free bandage or sheet. Avoid applying large, wet sheets, as this can cause the child to become too cold. Do not attempt to break any blisters. If the child appears to be in shock, elevate the legs while protecting the burn and call 911.

Pectus excavatum and pectus carinatum

are anterior chest wall deformities. Pectus excavatum, a funnel-shaped chest, accounts for greater than 90% of all congenital chest wall deformities (Boas, 2016). A depression that sinks inward is apparent at the xiphoid process (Fig. 22.15). Pectus carinatum, a protuberance of the chest wall, accounts for only 5% to 15% of anterior chest wall deformities (Boas, 2016). The remainder are mixed deformities. Male predominance is evident in both types (Boas, 2016). Pectus excavatum does not resolve as the child grows; rather, it progresses with growth. The chest depression may be minimal or marked. When the pectus is more pronounced, cardiac and pulmonary compression occurs. Symptoms of this compression most often present during puberty, when the pectus quickly worsens. Children may complain of shortness of breath, withdraw from physical activities, and have a poor body image. Therapeutic Management Therapeutic management of pectus excavatum is based on the severity and physiologic compromise. Options include observation, use of physical therapy to work on musculoskeletal compromise, and surgical correction, preferably before puberty, when the skeleton is more pliable. Various surgical techniques may be used and generally involve either the placement of a surgical steel bar or using a piece of bone in the rib cage to lift the depression. This discussion will focus on care of the child who undergoes surgical steel bar placement for pectus correction. Nursing Assessment Elicit the health history, noting progression of the defect and effects on the child's cardiopulmonary function. Note shortness of breath, exercise intolerance, or chest pain. Observe the child's chest for anterior wall deformity, noting depth and severity. Auscultate the lungs to determine the adequacy of aeration. Radiographs, CT, or MRI may be used to determine the extent of the anomaly and compression of inner structures. Nursing Management Prepare the child preoperatively by allowing a tour of the surgical area and the pediatric intensive care unit. Introduce the child to the pain scale that will be used in the postoperative period. Postoperatively, nursing management focuses on assessment, protection of the surgical site, and pain management. Auscultate lung sounds frequently to determine the adequacy of aeration and to monitor for development of the complication of pneumothorax. Assess for signs of wound infection that would necessitate removal of the curved bar. During the first few postoperative days positioning is challenging; do not allow the child to roll in bed, lie on either side, or rotate or flex the spine (these positions may disrupt the bar's position). Administer analgesics as needed either intravenously or via the epidural catheter. Teach families that the child will not be allowed to lie on his or her side at home for 4 weeks after the surgery to ensure that the band does not shift. Encourage aerobic activity at home after being cleared by the surgeon (this will increase the child's vital capacity, previously hindered by the pectus). The bar will be removed 2 to 4 years after the initial placement.

Disseminated Intravascular Coagulation DIC

is a complex condition that leads to activation of coagulation; it usually occurs in critically ill children. Common triggers of DIC include septic shock, presence of endotoxins and viruses, tissue necrosis or injury, and cancer treatment (Ambruso et al., 2018). In DIC, thrombin is generated, fibrin is deposited in the circulation, and platelets are consumed. Deficiencies of coagulation and anticoagulation pathways occur. Hemorrhage and organ tissue damage result and can be irreversible if not recognized and treated immediately. Therapeutic management of children with DIC requires careful consideration of the etiology. Initial treatment focuses on treating the underlying cause. For example, if DIC occurs secondary to an infection, appropriate antibiotics would be used to treat the infection. Heparin is also used at lower doses to counteract the deficiency in the coagulation/anticoagulation pathway. Heparin reduces consumption of the platelets, resulting in improved platelet counts. Since heparin is an anticoagulant, there is an increased risk of bleeding. Nursing Assessment Because DIC occurs as a secondary condition, it may occur in a child hospitalized for any reason. DIC may affect any body system, so a thorough physical examination is warranted. Inspect for signs of bleeding such as petechiae or purpura, blood in the urine or stool, or persistent oozing from venipuncture or from the umbilical cord in the newborn. Evaluate respiratory status and determine the level of tissue oxygenation via pulse oximetry. Perform a complete circulatory assessment and note signs of circulatory collapse such as poor perfusion, tachycardia, prolonged capillary refill, and weak distal pulses. Note altered level of consciousness and decreased urine output. Careful abdominal palpation may reveal hepatomegaly or splenomegaly. Laboratory testing may reveal prolonged prothrombin time (PT), partial thromboplastin time (PTT), activated partial thromboplastin time (aPTT), bleeding time, and thrombin time and decreased levels of fibrinogen; platelets; clotting factors II, V, VIII, and X; and antithrombin III. Increases will be noted in levels of fibrinolysin, fibrinopeptide A, positive fibrin split products, and D-dimers. Nursing Management Continue to provide nursing care related to the triggering event. Assess the child's status frequently. If bleeding is observed, apply pressure to the area along with cold compresses. Elevate the affected body part if this does not affect the child's overall stability. If neurologic deficits are assessed, report the findings immediately so that treatment to prevent permanent damage can be started. Administer anticoagulation therapy (even though hemorrhage is a concern) to interrupt the coagulation process that is present in this condition. Provide ventilatory support as needed and provide continuous cardiac monitoring. Administer clotting factors, platelets, and cryoprecipitate as prescribed to prevent severe hemorrhage. Report changes in laboratory values to the physician or nurse practitioner. Changes can occur rapidly, and vigilance is necessary to prevent further tissue damage to the affected syste

Fractures

occur frequently in children and adolescents and common sites include the forearm and wrist (Baldwin, Wells, & Dormans, 2016). Most pediatric fractures heal well with minimal treatment (Baldwin et al., 2016). Midclavicular, humerus, or femur fractures can occur as a result of birth trauma. They typically heal well but may require limiting mobility or splinting. Fractures in children result most frequently from accidental trauma (Baldwin et al., 2016). Nonaccidental trauma (child abuse) and other disease processes are the other causes of fractures. Pathophysiology The growth plate is the most vulnerable portion of the child's bone and is frequently the site of injury. The Salter-Harris classification system is used to describe fractures involving the growth plate (Table 22.8). The thicker, more elastic periosteum in children yields to the force encountered with trauma, resulting more frequently in nondisplaced fractures in children. The increased vascularity and decreased mineral content make the child's bones more flexible. Plastic or bowing deformities and buckle and greenstick fractures are the result. Complete fractures do occur in children, but they tend to be more stable than in the adult, resulting in improved healing and function. Spiral, pelvic, and hip fractures are rare in children. Table 22.9 explains common types of fractures in children. Fractures in children heal more rapidly and result in less disability and deformity than adults. The younger the child, the more quickly the bone heals. However, plastic deformity and Salter-Harris type IV fractures may result in an angular deformity. Though healing of fractures is usually quick and without incident in children, delayed union, nonunion, or malunion can occur. Additional complications include infection, avascular necrosis, bone shortening from epiphyseal arrest, vascular or nerve injuries, fat embolism, reflex sympathetic dystrophy, and compartment syndrome, which is an orthopedic emergency (Baldwin et al., 2016). Therapeutic Management The vast majority of childhood fractures would heal well with splinting only, but casting of these fractures is performed to provide further comfort to the child and to allow for increased activity while the fracture is healing. Displaced fractures require manual traction to align the bones, followed by casting. More severe fractures may require traction for a period of time, usually followed by casting. Severe or complicated fractures may alternatively require open reduction and internal fixation for healing to occur. Complex fractures are often treated with external fixation Health History Elicit a description of the present illness and chief complaint. Common signs and symptoms reported during the health history might include recent injury, trauma, or fall; complaint of pain; difficulty bearing weight; limp; or refusal to use an extremity. Young children often demonstrate sudden onset of irritability and refusal to bear weight. Ask about the mechanism of injury and obtain a description of the traumatic event. Be alert to inconsistencies between the history and the clinical picture or mechanism of injury; inconsistency may be an indicator of child abuse. Explore the child's current and past medical history for risk factors such as: Rickets Renal osteodystrophy Osteogenesis imperfecta Participation in sports, particularly contact sports Failure to use protective equipment as recommended for various physical activities and sports (e.g., wrist guards while rollerblading) PALPATION Carefully palpate the joint or injured part. Distract the young child with a toy or activity while palpating. Note point tenderness, which is a reliable indicator of fracture in children. Assess neurovascular status, noting distal extremity temperature, spontaneous movement, sensation, numbness, capillary refill time, and quality of pulses. The neurovascular assessment is critical to providing a baseline so that any changes associated with compartment syndrome can be identified quickly. Laboratory and Diagnostic Tests Usually plain x-ray films are all that is required to identify a simple fracture. Complicated fractures that require surgical intervention may require further evaluation with CT or MRI. Nursing Management Immediately after the injury, immobilize the limb above and below the site of injury in the most comfortable position with a splint. Use cold therapy to reduce swelling in the first 48 hours after injury. Elevate the injured extremity above the level of the heart. Perform frequent neurovascular checks. Assess pain level and administer pain medications as needed. Utilize nonpharmacologic methods of pain relief as needed. Administer tetanus vaccine in the child with an open fracture if he or she has not received a tetanus booster within the past 5 years. Additional nursing interventions include providing family education and teaching on fracture prevention.

Idiopathic Thrombocytopenia Purpura

ITP is thought to be an immune response following a viral infection that produces antiplatelet antibodies. These antibodies destroy platelets, which then lead to the development of petechiae, purpura, and excessive bruising. Petechiae are pinpoint hemorrhages that occur anywhere on the body and do not blanch to pressure (Fig. 24.13). Purpura are larger areas of hemorrhage in which blood collects under the tissues; they are purplish (see Fig. 24.13). ITP usually develops a few weeks after a viral infection, is most common in young children, and within a few months, most children will recover spontaneously (Ambruso et al., 2018). Complications include severe hemorrhage and bleeding into vital organs and intracranial hemorrhage, although these rarely occur. For children with platelet counts below 10,000/mm3, corticosteroids may be administered for 2 to 3 weeks. A single dose of intravenous immunoglobulin (IVIG) may be used as an adjunct (Ambruso et al., 2018). Platelet transfusions are not indicated unless life-threatening bleeding is present. Refer the child for follow-up care with a pediatric hematologist. ITP is usually self-limiting, but if it persists for a year or longer, splenectomy may be indicated. Nursing Assessment Elicit the child's health history (usually a previously healthy child who recently has developed increased bruising, epistaxis, or bleeding of the gums). Note history of blood in the stool. Note risk factors such as recent viral illness, recent MMR immunization, or ingestion of medications that can cause thrombocytopenia. Inspect for petechiae, purpura, and bruising, which may progress rapidly within the first 24 to 48 hours of the illness. Document the size and location of each lesion. Inspect the lips and buccal mucosa for petechiae. The remainder of the physical examination is usually within normal limits. Usual laboratory findings include an extremely low platelet count (less than 50,000), normal WBC count and differential, and normal hemoglobin and hematocrit unless hemorrhage has occurred (this is rare). Bone marrow aspiration may be performed to rule out leukemia. Nursing Management Many children require no medical treatment except observation and reevaluation of laboratory values. Educate the family about avoiding aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), and antihistamines because these medications may precipitate the development of anemia in these children. The use of acetaminophen for pain control is more appropriate when necessary. Teach the family to prevent trauma by avoiding activities that may cause injury, such as contact sports. Instead, encourage activities, such as swimming, that provide physical activity with less risk of trauma. Explain to parents the signs and symptoms of serious bleeding and whom to call if it is suspected.

Slipped Capital Femoral Epiphysis

SCFE is a condition in which the femoral head dislocates from the neck and shaft of the femur at the level of the epiphyseal plate. The epiphysis slips downward and backward. The left hip is more often affected (Kienstra & Macias, 2019). The exact cause is unknown, but it is thought that during the teenage growth spurt the femoral growth plate weakens and becomes less resistant to stressors. Hormonal alterations during this period may also play a role. SCFE is classified based on its severity and whether the slip is acute or chronic. Chronic SCFE may lead to shortening of the affected leg and thigh atrophy. Therapeutic Management Promptly refer the child with SCFE to an orthopedic surgeon, as early surgical intervention will decrease the risk of long-term deformity. The goals of therapeutic management are to prevent further slippage, minimize deformity, and avoid the complications of cartilage necrosis (chondrolysis) and avascular necrosis of the femoral head. Surgical intervention may include in situ pinning, in which a pin or screw is inserted percutaneously into the femoral head to hold it in place. Osteotomy may be used for more severe cases. Osteoarthritis may be a long-term complication of SCFE. Nursing Assessment Elicit a health history, determining the onset and extent of pain. In acute SCFE, the pain is usually sudden in onset and results in inability to bear weight. Chronic SCFE may present with an insidious onset of pain and limp. Note risk factors for SCFE, including obesity (significant risk factor), age 9 to 16 years, African-American or Polynesian race, sedentary lifestyle, rapid growth spurt, and male gender (slightly higher incidence seen in males) (Kienstra & Macias, 2019a; Sankar et al., 2016). Observe ambulation, noting Trendelenburg gait. Assess for pain that is in the hip or that is referred to the groin, medial thigh, or knee. Note decreased range of motion in the affected hip with external rotation. Radiographs will be obtained to confirm the diagnosis (anteroposterior and lateral frog-leg views of hips). Bone scan can rule out avascular necrosis, and CT scan helps define the extent of slippage. Nursing Management Enforce bed rest and activity restriction. If traction is used for a period before surgery, perform routine traction care and neurovascular assessments. If surgery is performed, provide routine pre- and postoperative care. Assess pain and administer analgesics as needed. After in situ pinning, assist the child with crutch walking. Teach the family that weight bearing is usually resumed about a week after the surgery and that the pin will be removed later. Prolonged immobility may isolate the adolescent from usual peer interactions, so encourage phone calls and visits with friends. Provide books, games, electronic devices, and magazines for distraction during the period of immobility. Provide education and support to the child and family.

Diaper candidiasis

(also called monilial diaper rash) Fiery red lesions, scaling in the skin folds, and satellite lesions (located further out from the main rash) (Fig. 23.10) Topical nystatin with diaper changes for several days See section on diaper dermatitis for additional information

Congenital clubfoot

(also termed congenital talipes equinovarus) is a congenital anomaly that occurs in about 1 of 1,000 live births (Winell & Davidson, 2016). Clubfoot consists of: Talipes varus (inversion of the heel) Talipes equinus (plantarflexion of the foot; the heel is raised and would not strike the ground in a standing position) Cavus (plantarflexion of the forefoot on the hindfoot) Forefoot adduction with supination (the forefoot is inverted and turned slightly upward) The foot resembles the head of a golf club (Fig. 22.18). Half of all cases occur bilaterally and males are affected more frequently than females (Winell & Davidson, 2016). The exact etiology of clubfoot is unknown. Clubfoot may be classified into four categories: postural, neurogenic, syndromic, and idiopathic. Postural clubfoot often resolves with a short series of manipulative casting. Neurogenic clubfoot occurs in infants with myelomeningocele. Clubfoot in association with other syndromes (syndromic) is often resistant to treatment. Idiopathic clubfoot occurs in otherwise normal healthy infants. The approach to treatment is similar regardless of the classification. Therapeutic Management The goal of therapeutic management of clubfoot is achievement of a functional foot; treatment starts as soon after birth as possible. Weekly manipulation with serial cast changes is performed; later, cast changes occur every 2 weeks. Other infants require corrective shoes or bracing. In some infants surgical release of soft tissue may be necessary. Following surgery, the foot is immobilized with a cast for up to 12 weeks, and then ankle-foot orthoses (AFOs) or corrective shoes are used for several years. Complications of clubfoot and its treatment include residual deformity, rocker-bottom foot, awkward gait, weight bearing on the lateral portion of the foot if uncorrected, and disturbance to the epiphysis. Nursing Assessment Note family history of foot deformities and obstetric history of breech position. Inspect the foot for position at rest. Perform active range of motion, noting inability to move foot into normal positioning at midline. X-rays are obtained to determine bony abnormality and note progress during treatment. Nursing Management Perform neurovascular assessment and cast care for infants requiring casting. Provide emotional support, as treatment often begins in the newborn period and families may have a difficult time adjusting to the diagnosis and treatment required for their new baby. Teach families cast care and about the use of orthotics or braces as prescribed.

Erythema multiforme

, though uncommon in children, is an acute, self-limiting hypersensitivity reaction. It may occur in response to viral infections, such as adenovirus or Epstein-Barr virus; Mycoplasma pneumoniae infection; or a drug (especially sulfa drugs, penicillins, or immunizations) or food reaction. Stevens-Johnson syndrome and toxic epidermal necrolysis are the most severe forms of erythema multiforme and most often occur in response to certain medications or to Mycoplasma infection (Box 23.1). Therapeutic management of erythema multiforme is generally supportive because it resolves on its own. Nursing Assessment Note history of fever, malaise, and achiness (myalgia). Determine onset and progression of rash, and presence of pruritus and burning. Document the child's temperature upon assessment. Inspect the skin for lesions, which most commonly occur over the hands and feet and extensor surfaces of the extremities, with spread to the trunk. Lesions progress from erythematous macules (flat reddened areas) to papules, plaques, vesicles, and target lesions over a period of days (hence the name multiforme) (Fig. 23.14). Nursing Management Discontinue the medication or food if it is identified as the cause. Ensure that treatment for Mycoplasma is instituted if present. Encourage oral hydration. Administer analgesics and antihistamines as needed to promote comfort. If oral lesions are present, encourage soothing mouthwashes or use of topic oral anesthetics in the older child or teen. Oral lesions may be debrided with hydrogen peroxide.

Aplastic anemia

(failure of the bone marrow to produce cells) is characterized by bone marrow aplasia and pancytopenia (decreased numbers of all blood cells). Most cases are acquired, but there are a few rare types of inherited aplastic anemias (Brandow & Scott, 2019). The inherited types present as congenital bone marrow failure; the best known is Fanconi anemia, an autosomal recessive disorder. Acquired aplastic anemia is thought to be an immune-mediated response. Most cases are idiopathic, meaning the trigger remains unidentified. Other causes include exposure to environmental toxins, viruses, myelosuppressive drugs, or radiation. Complications of aplastic anemia include severe overwhelming infection, hemorrhage, and death. Therapeutic management of aplastic anemia in children involves HSCT from a human leukocyte antigen (HLA)-matched sibling donor; if one is not available, immunosuppressive therapy or high-dose cyclophosphamide can be given. Nursing Assessment Determine history of exposure to myelosuppressive medications or radiation therapy. Obtain a detailed family, environmental, and infectious disease history. Note history of epistaxis, gingival oozing, or increased bleeding with menstruation. Anemia may lead to headache and fatigue. On physical examination, note ecchymoses, petechiae or purpura, oral ulcerations, tachycardia, or tachypnea. In addition to suppression of all blood cells, laboratory and diagnostic testing may reveal: Guaiac-positive stool Blood in the urine Severe decrease in or the absence of hematopoietic cells on bone marrow aspiration Nursing Management Safety is of the utmost concern in children with aplastic anemia. It is important to prevent injury in order to avoid hemorrhage. Stool softeners may be used to prevent anal fissures associated with constipation. Administer only irradiated and leukocyte-depleted PRBCs or platelet transfusions as necessary. This limits exposure to HLA antigens should the child require bone marrow transplantation in the future. If the child requires HSCT, refer to the section earlier in this chapter for additional nursing management information. Refer families whose child has only mild or moderate disease to the Aplastic Anemia and Myelodysplastic Syndrome International Foundation, a link to which can be found on

contractures

(the shortening and hardening of muscles, tendons or tissues leading to fixated and stiff joints) and atrophy that may result from disuse of muscles, ensure that unaffected extremities are exercised. Assist the child to exercise the unaffected joints and to use the unaffected extremity if this does not disrupt traction alignment. Promote use of a trapeze if not contraindicated to involve the child in repositioning and assist with movement. Encourage deep-breathing exercises to prevent the pulmonary complications of long-term immobilization.

Orthotics, braces

Adaptive positioning devices specially fitted for each child by the physical or occupational therapist or orthotist. Used to maintain proper body or extremity alignment, improve mobility, and prevent contractures Cerebral palsy, spinal cord injury, spina bifida, muscular dystrophy, spinal muscular atrophy; used to immobilize a body part or prevent deformity through positioning. Used to treat developmental dysplasia of the hip and scoliosis; also may be used for a period of time after cast removal Provide frequent assessments of skin covered by the device to avoid skin breakdown. Cotton undergarment worn under the brace helps to maintain skin integrity. Follow the therapist's schedule of recommended "on" and "off" times. Encourage families to comply with use.

Casts

Casts are used to immobilize a bone that has been injured or a diseased joint. When a fracture has occurred, a cast serves to hold the bone in reduction, thus preventing deformity as the fracture heals. Casts are constructed of a hard material, traditionally plaster but now more commonly fiberglass. The hard nature of the cast keeps the bone aligned so that healing may occur more quickly. In a fracture that would heal on its own without specific immobilization, a cast may be used to reduce pain and to allow the child increased mobility. The choice of cast material and type of cast will be determined by the physician or nurse practitioner or orthopedic surgeon. Figure 22.4 shows selected casts used in children.

External Fixation

External fixation may be used for complicated fractures, especially open fractures with soft tissue damage. A series of pins or wires are inserted into bone and then attached to an external frame. The fixator apparatus may be adjusted as needed by the physician or nurse practitioner. Once the desired level of correction is achieved, no further adjustment occurs and the bone is allowed to heal. Advantages of external fixation include increased comfort for the injured child and improved function of muscles and joints when complicated fracture occurs.

Knee 90-90 traction

For femur fracture reduction when skin traction is inadequate. Skeletal traction with force applied through pin in distal femur. A foam boot may be used for suspension of the lower leg. Force of traction applied to femur via the pin. The amount of weight used is just enough to hold lower limb suspended.

Insect Stings and Spider Bites

Members of the Hymenoptera class of insects sting. This class includes bees, wasps, ants, yellow jackets, and hornets. Spiders inject their venom when they bite. Stings and bites usually result in a local reaction. A systemic or anaphylactic reaction to a Hymenoptera sting may also occur, possibly resulting in airway compromise (refer to Chapter 25 for additional information on anaphylaxis). Serious reactions may occur with brown recluse or black widow spider bites. This discussion will focus on local reactions. Local reactions to insect stings and spider bites include pruritus, pain, and edema. A hypersensitivity reaction thought to be mediated by IgE occurs in response to the venom. This may be a physiologic response to the antigens present in the insect's or spider's saliva and other fluids that are transmitted during stinging or biting. Bacterial superinfection may occur as a complication and as a result of scratching. Therapeutic management includes antihistamines to decrease itching and in some cases corticosteroids to decrease inflammation and swelling. Nursing Management Remove jewelry or constrictive clothing if the sting is on an extremity. Cleanse the wound with mild soap and water. If the stinger is present, scrape it away with your fingernail or a credit card. Apply ice intermittently to decrease pain and edema. Administer diphenhydramine as soon as possible after the sting in an attempt to minimize the reaction. Prevent insect stings and spider bites by wearing protective clothing and shoes when outdoors. Use insect repellants (with a maximum concentration of 30% n,n-diethyl-meta-toluamide [DEET] in infants and children older than 2 months) (Stein & Barnett, 2017). Teach children never to disturb a bee or wasp nest or an ant hill.

Practice meticulous hygiene (oral, body, perianal). Avoid known ill contacts, especially persons with chickenpox. Immediately notify the physician or nurse practitioner if exposed to chickenpox. Avoid crowded areas. Do not let the child receive live vaccines. Do not take the child's temperature rectally or give medications by the rectal route. Administer twice-daily trimethoprim-sulfamethoxazole for 3 consecutive days each week as ordered for prevention of Pneumocystis pneumonia.

Preventing Infection in Children Receiving Chemotherapy for Cancer

Physical therapy, occupational therapy, or speech therapy

Physical therapy focuses on attainment or improvement of gross motor skills. Occupational therapy focuses on refinement of fine motor skills, feeding, and activities of daily living. Speech therapy is warranted for the child with a speech impairment or feeding difficulty related to oral muscular issues Cerebral palsy, spina bifida, spinal cord injury, muscular dystrophy, spinal muscular atrophy; restore function after injury or surgery; promote developmental activities when limb use is compromised, as in limb deficiency Provide follow-through with prescribed exercises or supportive equipment. Success of therapy is dependent upon continued compliance with the prescribed regimen. Ensure that adequate communication exists within the interdisciplinary team.

PREVENTING ANEMIA

To maintain blood volume, limit blood draws to the minimum volume required. Encourage the child to eat an appropriate diet that includes adequate iron. Administer EPO injections as ordered. Teach families to give the injections at home if prescribed.

Thus far, there is insufficient evidence that supports a particular strategy of pin care (Iobst, 2017). More randomized trials are needed. The National Association of Orthopaedic Nurses has published minimal guidelines, which include: Perform pin care weekly after the first 48 to 72 hours. Perform earlier if large amount of drainage is present, dressing becomes wet, or infection is suspected. The most effective solution for pin site care may be chlorhexidine 2 mg/mL in alcohol. If child has sensitivity to this, use normal saline. Use a nonshedding material for cleaning. Cover pin sites with a nonshedding dressing. Teach children and their families pin site care along with instructions on the signs and symptoms of infection before discharge (Holmes, Brown, & Pin Site Care Expert Panel, 2005; Walker, 2018).

Providing Pin Care

Dunlop side-arm 00-90

Skeletal traction through an olecranon screw or pin in distal humerus. Lower arm is held in balanced suspension. See side-arm 90-90. In addition, provide appropriate pin site care.

Cervical skeletal tongs

Tongs attached to skull via pins. Used with fractures or dislocations of the cervical or high thoracic vertebrae. Assess frequently for increased pain, respiratory distress, and spinal cord, cranial nerve, or brachial plexus injury. Place on Stryker frame or specially equipped bed to ease positioning without disruption of alignment.

Impetigo

is a readily recognizable skin rash (Fig. 23.2). Nonbullous impetigo generally follows some type of skin trauma or may arise as a secondary bacterial infection of another skin disorder, such as atopic dermatitis. Bullous impetigo demonstrates a sporadic occurrence pattern and develops on intact skin, resulting from toxin production by S. aureus

Spinal Muscular Atrophy

SMA is a genetic motor neuron disease that affects the spinal nerves' ability to communicate with the muscles. It is inherited via an autosomal recessive mechanism. The motor neuron protein survival of motor neurons (SMNs) is deficient as a result of a faulty gene on chromosome 5. The motor neurons are located mostly in the spinal cord. Without adequate SMN, the signals from the neurons to the muscles instructing them to contract are ineffective, so the muscles lose function and over time atrophy. The proximal muscles, those closer to the body's center, are usually more affected than the distal muscles. Cognition is unaffected by this disease (Bodamer, 2019). There are several types of SMA, classified as type 0 to type 4, based on age of onset, severity of weakness, and clinical course. Their usual progression and prognosis are compared in Table 22.4. Respiratory muscle weakness may occur with all types of SMA and is usually the cause of death in type 1 SMA. Upper respiratory tract infections and aspiration related to dysphagia or gastroesophageal reflux often develop into pneumonia and eventual respiratory failure, as the affected child cannot effectively cough independently in order to clear the airway. Many children with severe type 1 SMA are ventilator dependent. Pectus excavatum develops in children with type 1 and type 2 SMA who exhibit paradoxical breathing (use of the diaphragm without intercostal muscle support). The chest becomes funnel shaped and the xiphoid process is retracted (pectus excavatum), further restricting respiratory development. Inability to appropriately suck and swallow leads to difficulty feeding in the child with type 1 SMA. Weak back muscles affect the developing spine, resulting in the complication of scoliosis, kyphosis, or both. Therapeutic management of SMA is supportive, aimed at promoting mobility, maintaining adequate nutrition and pulmonary function, and preventing complications. Spinal fusion may be performed in older children with significant scoliosis. Since the discovery of the disease-causing gene for SMA, further research and improved diagnostic techniques have occurred. Therapies, such as nusinersen (which is an intrathecally injected medication) and gene replacement have both shown promising results (Bodamer, 2019). Nursing Assessment Note history of attainment of developmental milestones, as well as loss of milestones. SMA should be suspected in a child showing symmetric, unexplained weakness that is more proximal than distal and greater in the legs than arms, diminished or absent tendon reflexes, history of difficulty with motor skills, or loss of motor skills (Bodamer, 2019). In the infant or child with known SMA, assess for recent hospitalizations or respiratory illness. Determine the respiratory support regimen used at home (if any). Note level of motor ability and identify the orthoses or adaptive equipment used. Elicit history related to feeding patterns at home. Assess for floppy appearance in the infant with SMA. Note decreased ability to initiate spontaneous muscle movement. In the infant or young child with SMA, note narrow chest with decreased excursion, relatively protuberant abdomen, and paradoxical breathing pattern (Fig. 22.25). Observe the chest for formation of pectus excavatum. Auscultate the lungs for diminished or adventitious breath sounds. Monitor laboratory testing, which may include: Creatine kinase (CK): elevated when muscular damage is occurring Genetic testing: identifies presence of gene for SMA Muscle biopsy: shows the muscle abnormality Nerve conduction velocity test and electromyelogram: to determine extent of involvement Nursing Management Nursing management of type 2 and type 3 SMA focuses on promoting mobility, maintaining pulmonary function, and preventing complications. Children with type 1 SMA need additional interventions related to prevention of complications from immobility and assistance with nutrition. Refer to the Nursing Process Overview earlier in the chapter for interventions related to these areas. Individualize the nursing plan of care based on the individual child's responses to the disorder. Promote mobility through the use of range-of-motion exercises, lightweight orthotics, standing frames, and wheelchair use as appropriate. Support parents in their efforts to comply with physical and occupational therapy regimens. Older children may exercise with assistance in a warm pool. Position the child in a fashion that maintains appropriate body alignment. Provide airway clearance techniques such as manual or mechanical cough assistance, chest percussion, and postural drainage to assist with clearance of secretions. In collaboration with respiratory therapy, teach families the use of noninvasive ventilation support, in which positive pressure is delivered to the lungs through a mask or mouthpiece (Fig. 22.26). Provide routine tracheostomy care if the child has a tracheostomy (refer to tracheostomy section of Chapter 18). Administer gastrostomy tube feedings if ordered, and teach families gastrostomy tube care. Use bracing as prescribed to prevent spinal curvature. Make frequent inspections for skin breakdown in areas affected by bracing.

Legg-Calvé-Perthes disease

is a self-limiting condition that involves avascular necrosis of the femoral head. It most often affects boys between 4 and 8 years of age, but it can occur as early as 18 months and up until skeletal maturity (Sankar et al., 2016). The etiology is unknown, but interruption of the blood supply to the femoral head results in bone death, and the spherical shape of the femoral head may be lost. Swelling of the soft tissues around the hip may occur. As new blood vessels develop, the area is supplied with circulation, allowing bone resorption and deposition to take place. During this period of revascularization, which takes 18 to 24 months, the bone is soft and more likely to fracture. Over time, the femoral head reforms. Therapeutic Management The goal of therapeutic management is to maintain normal femoral head shape and to restore appropriate motion. Treatment of Legg-Calvé-Perthes disease includes anti-inflammatory medication to decrease muscle spasms around the hip joint and to relieve pain. Activity limitation may be prescribed, and sometimes bracing, casting, or traction is recommended to contain the femoral head. Serial x-ray follow-up determines progress of the disease. If surgery becomes warranted, which is rarely done, then osteotomy may be performed. Complications include joint deformity, early degenerative joint disease, persistent pain, loss of hip motion or function, and gait disturbance. Nursing Assessment Explore the health history for short stature, delayed bone maturation, related trauma, or a family history of Legg-Calvé-Perthes disease. Note painless limp, which may be intermittent over a period of months. Mild hip pain may result and may be referred to the knee or the thigh. Pain may be aggravated by exercise. Observe the child walking and note Trendelenburg gait. Perform range of motion, noting internal rotation of the hip and limited abduction. Muscle spasm may result with hip extension and rotation. Hip radiographs are obtained to evaluate the extent of epiphyseal involvement. MRI or bone scan may also be used to differentiate Legg-Calvé-Perthes disease from other disorders. Ultrasound and arthrograms may also be useful. Nursing Management Nursing care of Legg-Calvé-Perthes disease is highly variable and depends on the stage of the disease and its severity. Administer anti-inflammatory medications, noting their effect on pain. If activities are restricted, exercise the unaffected body parts. Assist families with use of the brace if prescribed. The brace may be wiped with a damp cloth if it becomes dirty. Some children will be prescribed no treatment other than avoidance of contact or high-impact sports. Swimming and bicycle riding help to maintain range of motion with little risk. If mobility equipment is needed, educate the child and family on its use. If osteotomy is performed, provide routine postoperative care, including education and support of the child and family.

Skeletal or cervical traction

Traction is an application of a pulling force on an extremity or body part To minimize or prevent trauma to the spinal cord; fracture reduction, dislocations, correction of deformities To maintain even, constant traction: Ensure weights hang free at all times and ropes remain in the pulley grooves. Keep weights out of child's reach. Maintain prescribed weight. Elevate head or foot of bed only with physician order. Monitor for complications: Perform neurovascular checks at least every 4 hours. Monitor neurologic status closely. Assess for signs and symptoms of infection or impaired skin integrity. Provide appropriate pin site care. Caring for the Child in Traction Nursing care of the child in any type of traction focuses not only on appropriate application and maintenance of traction but also on promoting normal growth and development and preventing complications (see Table 22.1). Apply skin traction over intact skin only so that the pull of the traction is effective. Prepare the skin with an appropriate adhesive before applying the traction tapes to ensure that the tapes adhere well, preventing skin friction. After application of the traction tapes, apply the elastic bandage or use the foam boot. Attach the traction spreader block and then apply the prescribed amount of weight via a rope attached to the spreader block. Ensure that the rope moves without obstruction and that the weights hang freely without touching the floor. In skeletal traction, apply weight via ropes attached to the skeletal pins. Treat the pin sites as surgical wounds (see section on pin site care). Protect the exposed ends of the pins to avoid injury. Whether skin or skeletal traction is used, be sure that constant and even traction is maintained.

Traction

Traction, another common method of immobilization, may be used to reduce and/or immobilize a fracture, to align an injured extremity, and to allow the extremity to be restored to its normal length. Traction may also reduce pain by decreasing the incidence of muscle spasm. In running traction, the weight pulls directly on the extremity in only one plane. This may be achieved with either skin or skeletal traction. In balanced suspension traction, additional weights are used to provide a counterbalance to the force of traction. This allows for constant pull on the extremity even if the child changes position somewhat. Comparison Chart 22.1 discusses skin versus skeletal traction.

Neural tube defects

account for the majority of congenital anomalies of the central nervous system. The neural tube closes between the third and fourth week of gestation. The cause of neural tube defects is not known, but many factors, such as drugs, malnutrition, chemicals, and genetics, can hinder normal central nervous system development. Strong evidence exists that maternal preconception supplementation of folic acid can decrease the incidence of neural tube defects in pregnancies by 50% or more (American Academy of Pediatrics [AAP], Committee on Genetics, 1999, reaffirmed 2016). Beginning in 1992 and continuing until today, the U.S. Public Health Service along with the Centers for Disease Control and Prevention (CDC) recommends that all women of childbearing age who are capable of becoming pregnant take 0.4 mg (400 mcg) of folic acid daily (CDC, 2018a). Pregnant women who had a previous child with a neural tube defect are recommended to take a higher dosage and should consult with their physician or nurse practitioner (AAP, Committee on Genetics, 1999, reaffirmed 2016). Prenatal screening of maternal serum for α-fetoprotein (AFP) and ultrasound examination can help identify fetuses at risk. Neural tube defects primarily affecting spinal cord development include spina bifida occulta, meningocele, and myelomeningocele (Fig. 22.13). Neural tube defects primarily affecting brain development are discussed in Chapter 16.

Human and Animal Bites

Yearly, significant emergency room visits occur as a result of bites from mammals. Dog bites account for the majority of injuries, but in children human and cats bites account for the most infected bites (Carney & Roswell, 2018). The hand and face are common locations for animal bites. A dog is most often provoked to bite a child when the child is playing with the dog or when the child hits, kicks, hugs, grabs, or chases the dog. Therapeutic management involves cleansing and irrigating the wound, wound suturing or stapling if necessary, and administering topical and/or systemic antibiotic therapy. Rabies prophylaxis is indicated if the rabies status of the dog is unknown. Secondary bacterial infection of the bite wound with streptococci, staphylococci, or Pasteurella multocida may occur. Nursing Assessment Nursing Management Provide rabies immunoprophylaxis and a tetanus booster vaccination if indicated. Thoroughly cleanse the wound with soap and water or a povidone-iodine solution. Irrigate the wound well with normal saline after cleansing. If the animal may be rabid, cleanse the wound for at least 10 minutes with a virucidal agent such as povidone-iodine solution. Administer antibiotics as prescribed. Prevention of animal bites is important. Teach children the following: Never provoke a dog with teasing or roughhousing. Get adult permission before interacting with a dog, cat, or other animal that is not your pet. Do not bother an eating, sleeping, or nursing dog. Avoid high-pitched talking or screaming around dogs. Display a closed fist first for the dog to sniff. Keep ferrets away from the face. If a cat hisses or lashes out with the paw, leave it alone.

Hematopoietic Stem Cell Transplantation HSCT,

also called bone marrow transplantation, is a procedure in which hematopoietic stem cells are infused intravenously into the child. This follows a period of purging of abnormal cells in the child that is accomplished through high-dose chemotherapy or irradiation. The use of high-dose chemotherapy and total body irradiation kills the tumor cells but also destroys the child's bone marrow. The transplanted cells migrate to the empty spaces in the child's bone marrow and reestablish normal hematopoiesis in the child. HSCT is used for a variety of childhood cancers, including leukemia, lymphoma, brain tumors, neuroblastoma, and other solid tumors. For most pediatric cancers, it is not the first line of treatment but is used for refractory or advanced disease. Autologous HSCT is achieved through harvest and treatment of the child's own bone marrow, followed by infusion of the treated stem cells. Risk for relapse of the original disease is highest in autologous HSCT. Allogenic HSCT refers to transplantation using stem cells from another individual that are harvested from the bone marrow, peripheral blood, or umbilical cord blood. Allogenic HSCT requires human leukocyte antibody (HLA) matching for antigen-specific sites on the leukocytes. Closely matched HLA donors may be difficult to find from a donor listing, and sibling donors are often the closest match. The degree of match is inversely related to the risk for graft rejection and the development of graft-versus-host disease (GVHD). In other words, the lesser the degree of HLA matching in the donor, the higher the risk for graft rejection and GVHD (Ibanez et al., 2019). In addition to graft rejection and GVHD, additional initial complications of HSCT are infection, electrolyte imbalance, bleeding, and organ, skin, and mucous membrane toxicities. Long-term complications include impaired growth and fertility related to endocrine dysfunction, developmental delay, cataracts, pulmonary and cardiac disease, avascular necrosis of the bone, and development of secondary cancers. NURSING MANAGEMENT OF THE CHILD DURING THE HSCT PRETRANSPLANT PHASE In the pretransplant phase, the child is being prepared to receive the transplant. The child's own bone marrow cells are eradicated through high-dose chemotherapy and total body irradiation. This phase usually occurs over 7 to 10 days. The child will be hospitalized because he or she is at extreme risk for serious infection. Maintain protective isolation in a positive-pressure room and limit visitors. Administer gamma globulin, acyclovir, or antibiotics as ordered to prevent or treat infection. Lymphohematopoietic rescue occurs with infusion of the donor or autologous cells (Kline, 2014). NURSING MANAGEMENT OF THE CHILD DURING THE HSCT POSTTRANSPLANT PHASE The posttransplant phase is also a time of high risk for the child. Monitor closely for symptoms of GVHD such as severe diarrhea and maculopapular rash progressing to redness or desquamation of the skin (especially palms or soles) (Fig. 24.5). If GVHD occurs, administer immunosuppressive drugs such as cyclosporine, tacrolimus, or mycophenolate (which place the child at further risk for infection) (Kline, 2014). PROVIDING SUPPORTIVE CARE FOLLOWING THE HSCT During the supportive care phase, which lasts several months after the transplant, continue to monitor for and prevent infection. Administer PRBCs or platelets and GCSF as needed. Families and children who undergo HSCT need prolonged and extensive emotional and psychosocial support. A medical social worker and psychologist or counselor are usually members of the transplant team and are excellent resources for these families' needs (Kline, 2014)

Acne neonatorum

occurs as a response to the presence of maternal androgens or to transient androgen production in the newborn. It may be present immediately after birth but often occurs between 2 and 4 weeks of age (Prok & Torres-Zegarra, 2018). Usually no treatment is necessary, but in severe cases there is a risk of scarring, so a topical preparation may be prescribed. Nursing Assessment Note oily face or scalp. Examine the face (especially the cheeks), upper chest, and back for inflammatory papules and pustules. Document absence of fever. Nursing Management Instruct parents to avoid picking or squeezing the pimples; to do so places the infant at risk for secondary bacterial infection and cellulitis. Teach parents to wash the affected areas daily with clear water. Avoid using fragranced soaps or lotions on the area with acne. Inform the parents that as the newborn's hormones stabilize over time, the acne usually resolves without additional intervention.

DDH (developmental dysplasia of the hip)

refers to abnormalities of the developing hip that include dislocation, subluxation, and dysplasia of the hip joint. In DDH, the femoral head has an abnormal relationship to the acetabulum. Frank dislocation of the hip may occur, in which there is no contact between the femoral head and acetabulum. Subluxation is a partial dislocation, meaning that the acetabulum is not fully seated within the hip joint. Dysplasia refers to an acetabulum that is shallow or sloping instead of cup shaped. DDH may affect just one or both hips. The dysplastic hip may be provoked to subluxation or dislocated and then reduced again (Fig. 22.19). Pathophysiology While dislocation may occur during a growth period in utero, the laxity of the newborn's hip allows dislocation and relocation of the hip to occur. The hip can develop normally only if the femoral head is appropriately and deeply seated within the acetabulum. If subluxation and periodic or continued dislocation occur, then structural changes in the hip's anatomy occur. Continued dysplasia of the hip leads to limited abduction of the hip and contracture of muscles. DDH is more common in females, probably due to the greater susceptibility of the female newborn to maternal hormones that contribute to laxity of the ligaments (Sankar et al., 2016). Mechanical factors such as breech positioning or the presence of oligohydramnios also contribute to the development of DDH. Genetic factors also play a role. There is an increased incidence of DDH among persons of Native American and Eastern Europe descent, with very low rates among people of African or Chinese heritage (Sankar et al., 2016). Complications of DDH include avascular necrosis of the femoral head, loss of range of motion, recurrently unstable hip, femoral nerve palsy, leg-length discrepancy, and early osteoarthritis. Therapeutic Management The goal of therapeutic management is to maintain the hip joint in reduction so that the femoral head and acetabulum can develop properly. Treatment varies based on the child's age and the severity of DDH. Infants younger than 6 months of age may be treated with a Pavlik harness, which reduces and stabilizes the hip by preventing hip extension and adduction and maintaining the hip in flexion and abduction (Sankar et al., 2016). The Pavlik harness is successful in the treatment of DDH in the majority of infants younger than 6 months of age if it is used on a full-time basis and applied properly (Sankar et al., 2016). Children from 6 months to 2 years of age often require closed reduction (Sankar et al., 2016). Skin or skeletal traction may be used first to gradually stretch the associated soft tissue structures. Closed reduction occurs under general anesthesia, with the hip being gently maneuvered back into the acetabulum. A spica cast worn for 12 weeks maintains reduction of the hip. After the cast is removed, the child may wear an abduction brace full time (except for baths) (Sankar et al., 2016). Then the brace is worn at night and during naps until development of the acetabulum is normal. Children older than 2 years of age or those who have failed to respond to prior treatment require an open surgical reduction followed by a period of casting (Sankar et al., 2016). Follow-up continues until the age of skeletal maturity. INSPECTION AND OBSERVATION Ensure that the infant is on a flat surface and is relaxed. Note asymmetry of thigh or gluteal folds with the infant in a prone position. Document shortening of the affected femur observed as limb-length discrepancy. Older children may exhibit Trendelenburg gait, due to the weakness of the hip abductors, the child's trunk is shifted over the affected hip during ambulation. Figure 22.20 illustrates these assessments. PALPATION Note limited hip abduction while performing passive range of motion. Abduction should ordinarily occur to 75 degrees and adduction to within 30 degrees with the infant's pelvis stabilized. Perform Barlow and Ortolani tests, feeling for, or noting, a "clunk" as the femoral head dislocates (positive Barlow) or reduces (positive Ortolani) back into the acetabulum. Force is not necessary when performing the Barlow and Ortolani maneuvers (Fig. 22.20 and Nursing Procedure 2 Nursing Management Earlier recognition of hip dysplasia with earlier harness use results in better correction of the anomaly. Excellent assessment skills and reporting of any abnormal findings are critical. Initially, the infant will need to wear the Pavlik harness continuously (Fig. 22.21). The physician or nurse practitioner makes all appropriate adjustments to the harness when applied so that the hips are held in the optimal position for appropriate development. Teach parents use of the harness and assessment of the baby's skin. If started early, harness use usually continues for about 3 months (Teaching Guidelines 22.4). Breastfeeding can continue throughout the harness treatment period, but creative positioning of the infant may be needed. For infants or children diagnosed later than 6 months of age or those who do not improve with harness use, surgical reduction may be performed (Sankar et al., 2016). Postoperative casting followed by bracing or orthotic use is common. Caring for the child in the postoperative period is similar to care of any child in a cast. Pain management and monitoring for bleeding are priority activities. Teach families care of the cast at home. Caring for a Child in a Pavlik Harness Do not adjust the straps without checking with the physician or nurse practitioner first. Until your physician or nurse practitioner instructs you to take the harness off for a period of time each day, it must be used continuously (for the first week or sometimes longer). Change your baby's diaper while he or she is in the harness. Place your baby to sleep on his or her back. Check skin folds, especially behind the knees and diaper area, for redness, irritation, or breakdown. Keep these areas clean and dry. Once the baby is permitted to be out of the harness for a short period, you may bathe your baby while the harness is off. Long knee socks and an undershirt are recommended to prevent rubbing of the skin against the brace. Note location of the markings on the straps for appropriate placement of the harness. Wash the harness with mild detergent by hand and air dry. If using the dryer, use only the air fluffing setting (no heat). Call the doctor if: Your baby's feet are swollen or bluish. The harness appears too small. Skin is raw or a rash develops. Your baby is unable to actively kick his or her legs.

Diaper Dermatitis

refers to an inflammatory reaction of the skin.refers to an inflammatory reaction of the skin in the area covered by a diaper. It is a nonimmunologic response to a skin irritant that results in skin cell hydration disturbance. Prolonged exposure to urine and feces may lead to skin breakdown (Fig. 23.11). Diaper wearing increases the skin's pH, activating fecal enzymes that further contribute to skin maceration. Nursing Assessment Determine from the history whether the infant or child wears diapers. Ask about the onset and progression of the rash, as well as any treatments and response. Inspect the skin in the diaper area for erythema and maceration (see Fig. 23.11). Ordinary diaper dermatitis does not usually result in a bumpy rash but starts as a flat red rash in the convex skin creases. It may appear red and shiny and may or may not also have papules. Untreated, it may become more widespread or severe. Some cases of diaper dermatitis are caused by overgrowth of C. albicans (see Fig. 23.9 and the section on fungal infections). Nursing Management Prevention is the best management of diaper dermatitis. Topical products such as ointments or creams containing vitamins A, D, and E; zinc oxide; or petrolatum are helpful to provide a barrier to the skin. Teaching Guidelines 23.1 gives further information on prevention and management of diaper dermatitis. See above for treatment of diaper rash caused by C. albicans infection. Prevention and Management of Diaper Dermatitis Change diapers frequently. Change stool-soiled diapers as soon as possible. Avoid rubber pants. Gently wash the diaper area with a soft cloth, avoiding harsh soaps. Use baby wipes in most children but avoid wipes that contain fragrance or preservatives. Once a rash has occurred, follow all the prevention tips above and add the following: Allow the infant or child to go diaperless for a period of time each day to allow the rash to heal. Blow-dry the diaper area/rash area with the dryer set on the warm (not hot) setting for 3 to 5 minutes.

Folliculitis,

infection of the hair follicle, most often results from occlusion of the hair follicle. It may occur as a result of poor hygiene, prolonged contact with contaminated water, maceration, a moist environment, or use of occlusive emollient products.

Transient synovitis of the hip

(also termed toxic synovitis) is a common cause of hip pain and limping in children in the United States, typically occurring in children between 3 and 8 years of age (Sankar et al., 2016). The exact cause is unclear, but it is thought to be associated with recent or active infection, trauma, or allergic hypersensitivity (Sankar et al., 2016). It is a self-limiting disease and most cases resolve within a week, but it may last as long as 3 to 6 weeks. Therapeutic management involves nonsteroidal anti-inflammatory medications, analgesics, and bed rest to relieve weight bearing on the affected hip joint. Nursing Assessment Explore the health history for risk factors such as antecedent trauma, concurrent or recent upper respiratory tract infection, pharyngitis, or otitis media. Note sudden acute onset of moderate to severe pain of one hip. Sometimes pain is referred to the anterior thigh or knee. Pain is usually the worst upon arising in the morning, and the child refuses to walk; pain then decreases throughout the day. Temperature usually will be normal or low grade (less than 38°C). Observe for a limp or for refusal to bear weight. Observe position of the affected hip: it will be held in a flexed and externally rotated position. Note restricted range of motion for abduction and internal rotation. Nursing Management Nursing care focuses on educating the family including instructions on administering nonsteroidal anti-inflammatory medications, analgesics, and bed rest. Parents are very concerned when their child refuses to walk; therefore, provide significant support and reassure the child and family of the self-limiting nature of the disease.

Tinea cruris

Erythema, scaling, maceration in the inguinal creases and inner thighs (penis/scrotum spared) Topical antifungal preparation for 4-6 weeks

Tinea corporis (ringworm)

Annular lesion with raised peripheral scaling and central clearing (looks like a ring) (Fig. 23.6) Topical antifungal cream is required for at least 4 weeks fungal infection on the arms or legs

immunosuppression, infection, myelosuppression, nausea, vomiting, constipation, oral mucositis, alopecia, pain. Long-term complications include microdontia and missing teeth as a result of damage to developing permanent teeth; hearing and vision changes; hematopoietic, immunologic, or gonadal dysfunction; endocrine dysfunction, including altered growth and precocious or delayed puberty; various alterations of the cardiorespiratory, gastrointestinal (GI), and genitourinary systems; development of a second cancer as an adolescent or adult (ACS, 2019b).

Adverse effects common to chemotherapeutic drugs include

Crutches

Ambulatory devices that transfer body weight from lower to upper extremities Used whenever weight bearing is contraindicated Top of crutch should reach 2-3 fingerbreadths below the axillae to prevent nerve palsy. Teach child appropriate ambulation with crutches or reinforce teaching if performed by physical therapist.

Cold therapy

Application of ice bags, commercial cold packs, or cold compresses Most often used in acute injuries to cause vasoconstriction, thereby decreasing pain and swelling Apply for 20-30 minutes, then remove for 1 hour, and then reapply for 20-30 minutes. Discontinue when numbness occurs. Place a towel between the cold pack and the skin to prevent thermal injury.

PREVENTING HEMORRHAGE

Assess for petechiae, purpura, bruising, or bleeding. Determine changes from baseline that warrant intervention. Encourage quiet activities or play to avoid trauma. Avoid rectal temperatures and examinations to avoid rectal mucosal damage that results in bleeding. Post a sign at the head of the bed stating "no rectal temperatures or medications." Avoid intramuscular injections and lumbar puncture if possible to decrease the risk of bleeding from a puncture site. If bone marrow aspiration must be performed, apply a pressure dressing to the site to prevent bleeding. For active or uncontrolled bleeding, transfuse platelets as ordered to control bleeding.

Iron-Deficiency Anemia

Iron-deficiency anemia occurs when the body does not have enough iron to produce Hgb. In the United States, iron-deficiency anemia has a peak prevalence in children between the ages of 12 and 24 months, and again at the age during adolescence (McFarren & Levy, 2017). Cow's milk consumption contributes to iron-deficiency anemia in older infants and young children due to its poor iron availability (Powers & Mahoney, 2019). The heme portion of Hgb consists of iron surrounded by protoporphyrin. When not enough iron is available to the bone marrow, Hgb production is reduced. Adequate dietary intake of iron is required for the body to make enough Hgb. As Hgb levels decrease, the oxygen-carrying capacity of the blood is decreased, resulting in weakness and fatigue. In addition to delayed growth, iron-deficiency anemia has been associated with cognitive delays and behavioral changes. Therapeutic Management Iron supplements are usually provided in the form of ferrous sulfate or ferrous fumarate and are available over the counter. The recommended dose is 4 to 6 mg of elemental iron daily (Brandow & Scott, 2019). In more severe cases, blood transfusions may be indicated. Transfusion of PRBCs is reserved for uncompensated anemia (McFarren & Levy, 2017). When PRBC administration is warranted, follow specific blood bank guidelines for administration. Monitor subsequent laboratory results for improvement. Health History Elicit a description of the current illness and chief complaint. Common signs and symptoms reported during the health history may include irritability, headache, dizziness, weakness, shortness of breath, pallor, and fatigue. Other symptoms may be subtle and difficult for the clinician to identify; these include difficulty feeding, pica, muscle weakness, or unsteady gait. Explore the health history for risk factors such as: Maternal anemia during pregnancy Poorly controlled diabetes during pregnancy Prematurity, low birth weight, or multiple birth Cow's milk consumption before 12 months of age Excessive cow's milk consumption (greater than 24 oz a day) Infant consumption of low-iron formula Lack of iron supplementation after age 6 months in breast-fed infants Excessive weight gain Chronic infection or inflammation Chronic or acute blood loss Restricted diets Use of medication interfering with iron absorption, such as antacids Low socioeconomic status Recent immigration from a developing country (Powers & Mahoney, 2019) Physical Examination Observe the child for fatigue and lethargy. Inspect the skin, conjunctivae, oral mucosa, palms, and soles for pallor. Note spooning of the nails (concave shape) (Fig. 24.6). Obtain a pulse oximeter reading. Evaluate the heart rate for tachycardia. Auscultate the heart for a flow murmur. Palpate the abdomen for splenomegaly. Laboratory and Diagnostic Tests Laboratory evaluation will reveal decreased Hgb and Hct, decreased reticulocyte count, microcytosis, hypochromia, decreased serum iron and ferritin levels, and an increased free erythrocyte protoporphyrin (FEP) level. Providing Dietary Interventions Ensure that iron-deficient infants are fed only formulas fortified with iron. Interventions for breast-fed infants include beginning iron supplementation around the age of 4 or 5 months. Iron supplementation may range from adding iron-fortified cereals to the child's diet to giving iron-containing drops. Encourage breastfeeding mothers to increase their dietary intake of iron or take iron supplements when breastfeeding so that the iron may be passed on to the infant. For children over 1 year of age, limit cow's milk intake to 24 oz per day to decrease risk of microscopic GI bleeding and increase appetite for other foods. Limit fast-food consumption and encourage intake of iron-rich foods such as red meats (iron from red meat is the easiest for the body to absorb), tuna, salmon, eggs, tofu, enriched grains, dried beans and peas, dried fruits, leafy green vegetables, and iron-fortified breakfast cereals. Teach the parents about dietary intake of iron. Encourage parents to provide a variety of foods for iron support and vitamins and other minerals necessary for growth. A big problem for toddlers is their picky eating. This often becomes a means of control for the child, and parents should guard against getting involved in a power struggle with their child. Referring parents to a developmental specialist who can assist them in their approach to diet may prove beneficial. Refer families who meet the financial limits and who have children aged 5 and younger to the Women, Infants, and Children (WIC) program, which provides for supplementation of infants' and children's diets.

MANAGING NAUSEA, VOMITING, AND ANOREXIA

Many chemotherapeutic drugs produce the adverse effect of nausea and vomiting, which often leads to anorexia. The cycle of nausea, vomiting, and anorexia is difficult to break once it begins. In addition, taste alterations are common in children who have received chemotherapy. During or after chemotherapy, children may develop an aversion to a food that was previously their favorite. Provide foods the child desires or asks for in order to increase the likelihood of eating. Prevent nausea by administering antiemetic medications prior to the administration of chemotherapy and on a routine schedule around the clock for the first 1 to 2 days rather than on an as-needed (PRN) basis. Herbal or complementary therapies may provide another option for management of nausea. Bright lights and noise may worsen nausea. Therefore, keep the child's environment dimly lit and calm. Relaxation therapy and guided imagery may also be helpful in preventing or treating nausea and vomiting. Refer to the Nursing Process Overview section for additional interventions.

Pressure Ulcers

Skin breakdown involves changes in intact skin, which may range from blanchable erythema to deep pressure ulcers. The term pressure ulcer refers to damage to the skin resulting in skin loss and development of a crater that may range from mild to deep. Pressure ulcers develop from a combination of factors, including immobility or decreased activity, decreased sensory perception, increased moisture, impaired nutritional status, inadequate tissue perfusion, and the forces of friction and shear. Common sites of pressure ulcers in hospitalized children include the occiput and toes, while children who require wheelchairs for mobility have pressure ulcers in the sacral or hip area more frequently. Nursing Assessment Note history of immobility (chronic, related to a condition such as paralysis) or lengthy hospitalization, particularly in intensive care. Inspect the skin for areas of erythema or warmth. Note ulceration of the skin. Use the facility's wound assessment scale to document the extent of the ulcer. Take a photo of the ulcer if possible. Nursing Management Position the child to alleviate pressure on the area of the ulcer. Use specialized beds or mattresses to prevent further pressure areas from developing. Perform prescribed wound care meticulously, noting the formation of granulation tissue as the ulcer begins to heal. Prevent pressure ulcers in the child who is hospitalized for long periods of time by turning the child frequently, assessing the entire surface of the child's skin at least every shift, using pressure-alleviating beds and mattresses, and maintaining the child's nutritional status.

Cervical skin traction

Skin traction applied with a skin strap (head halter). Used for neck sprains/strains, torticollis, or nerve trauma. Ensure that head halter or skin strap does not place pressure on ears or throat. Limit of 5 to 7 lb of weight.

Russell traction

Skin traction for femur fracture, hip, and specific types of knee injuries or contractures. Uses a knee sling. In split Russell traction, a portion of the traction weight may be redistributed via a pulley from the sling to the head of the bed (used for femur fracture, Legg-Calvé-Perthes disease, slipped capital femoral epiphysis). Wrap bandages from ankle to thigh on children younger than age 2 years, from ankle to knee on children older than 2 years. Use a foot support to prevent foot drop. Ensure heel is free from bed. Assess popliteal region for skin breakdown from the sling. Mark leg to ensure proper replacement of sling.

Buck traction

Skin traction for hip and knee contractures, Legg-Calvé-Perthes disease, slipped capital femoral epiphysis. Used to rest an injured limb or to prevent spasms of injured muscles or joints. Traction force delivered in straight line. Remove traction boot every 8 hours to assess skin. Leg may be slightly abducted.

Side-arm 90-90

Skin traction used to treat fractures of the humerus and injuries in or around the shoulder girdle. Maintain elbow flexed at 90 degrees. Fingers and hand may feel cool because of elevation. Child may turn to affected side only.

Radial Head Subluxation

Subluxation of the radial head ("nursemaid's elbow") occurs when a pulling motion on the arm causes the annular ligament surrounding the radial head to stretch or tear, therefore displacing the radial head. The ligament becomes entrapped within the joint, preventing spontaneous reduction. It usually occurs in children younger than 5 years of age (Carrigan, 2016). In most cases a parent, sibling, or caregiver inadvertently injures the child while holding or pulling on a pronated upper extremity. Radiologic examination may be done, especially if the mechanism of injury is not clear, to rule out fracture or dislocation. To reduce the injury, the elbow is flexed to 90 degrees and then the forearm is fully and firmly supinated, causing the ligament to snap back into place. With appropriate reduction of the radial head, no complications result. Nursing Assessment Elicit a health history to help determine the mechanism of injury. Common precipitators of this injury include pulling on the child's arm while leading him or her in one direction, helping the child up the stairs, a child dropping or falling to the ground while an adult is holding the hand, or swinging or lifting the child by the hands. Assess neurovascular status and examine the extremity. The child will hold the arm slightly flexed at the side or across the abdomen and refuse to move it. When the arm is still, the child apparently has no discomfort. Neurovascular status is normally intact with no bruising or swelling present. Nursing Management After treatment, usually hyperpronation to reduce the dislocation, assess the child's ability to use the arm without pain. Typically after reduction the child will demonstrate less pain almost immediately. Educate parents that once a radial head subluxation occurs it may recur. Teach parents to avoid excessive pulling or pulling up on the child's arm, particularly in an abrupt jerking fashion, to prevent recurrence. Encourage parents and caregivers to always lift the child under the arms.

Tinea versicolor

Superficial tan or hypopigmented oval scaly lesions, especially on upper back and chest and proximal arms More noticeable in the summer with tanning of unaffected areas (Fig. 23.8) Apply selenium sulfide shampoo all over body (from face to knees) and allow to stay on skin overnight, rinsing in the morning, once a week for 4 weeks (this may cause skin irritation) Topical antifungals in the imidazole family may be used instead fungal infection on the trunk and extremities

Fixation

Surgical reduction of a fracture or skeletal deformity with an internal or external pin or fixation device Fractures, skeletal deformities No additional care for internal fixation. External fixation: perform pin care as prescribed by the surgeon. Assess for excess drainage or pin slippage, notifying physician or nurse practitioner if this occurs. Velcro or snaps on sleeves and pant legs help with dressing.

Splinting

Temporary stiff support of injured area Temporary fracture reduction, immobilization and support of sprains Similar to cast care. Some splints are removable and are replaced when the child is up out of bed. Teach family appropriate use of splints.

RBC count: the actual number of counted RBCs in a certain volume of blood Hemoglobin (Hgb): measure of the protein made up of heme (iron surrounded by protoporphyrin) and globin, α- and β-polypeptide chains, primarily responsible for the transport of nutrients and oxygen to the tissues Hematocrit (Hct): an indirect measure of RBCs (number and volume) RBC indices Mean corpuscular volume (MCV): average size of the RBC Mean corpuscular hemoglobin (MCH): a calculated value of the oxygen-carrying capacity of the Hgb in the RBCs Mean corpuscular hemoglobin concentration (MCHC): a calculated value that reflects the concentration of Hgb inside the RBC Red cell distribution width (RDW): a calculated value that is a measure of the width of RBCs WBC count: actual count of the number of WBCs in a volume of blood Platelet count: number of platelets per blood volume Mean platelet volume (MPV): a measurement of the size of the platelets

The components of the CBC are:

Cold Injury

The term "frostbite" implies freezing of the tissues. It is described on a continuum from first to fourth degree. When a child is exposed to an extremely cold environment, changes in cutaneous circulation help to maintain the core body temperature. Because circulation is shunted to the core, the most peripheral body parts are those at highest risk for frostbite. Local damage occurs when the tissue temperature drops to 32°F (0°C). Initially skin sensation is lost, the vasculature constricts, and plasma leakage occurs. Ice crystals develop in the extracellular fluid, and eventually vascular stasis leads to endothelial cell damage, necrosis, and sloughing of dead tissue. Nursing Assessment Note history of cold exposure. Inquire about pain or numbness. Examine the skin for indications of frostbite. First-degree frostbite results in superficial white plaques with surrounding erythema. Second-degree frostbite demonstrates blistering with erythema and edema. In third-degree frostbite, hemorrhagic blisters occur, progressing to tissue necrosis and sloughing in fourth-degree frostbite. Nursing Management Remove wet or tight clothing. Avoid vigorous massage to decrease the chance of damaging the skin further. Immerse the affected part in 104°F water for 15 to 30 minutes. Thawing may cause significant pain, so administer analgesics. Keep the thawed part loosely covered, warm, and dry. Splinting may be used to help decrease associated edema. Consult the wound care specialist or plastic surgeon for further management. Prevent frostbite by: Dressing warmly in layers, and keeping warm and dry Avoiding exertion Not playing outside when wind chill advisories are in effect, and locking doors with high locks to prevent toddlers from going outside

Overuse Syndromes

The term "overuse syndrome" refers to a group of disorders that result from repeated force applied to normal tissue. The connective tissues fail in response to repetitive stress, leading to a small amount of tissue breakdown. They develop over the course of weeks to months. There is usually no identifiable injury associated with overuse syndromes. Pain is usually associated with the activity and worsens with continued participation in the activity. The incidence of overuse injuries in the young athlete has increased as participation of youths in organized sports has grown along with children today participating in sports year-round and sometimes in multiple sports simultaneously, the increased competitive nature of youth sports, and the early specialization in a particular sport with inadequate periods of rest (Brenner & The Council on Sports Medicine and Fitness, 2007, reaffirmed 2014; Magrini & Dahab, 2016). The young athlete is at risk for more serious overuse injuries due to the following: The growing bones of the young athlete cannot handle as much stress as mature bones in adults. The child is just learning the proper mechanisms for skills, such as throwing a baseball. The child is unable to recognize vague signs of injury such as fatigue and poor performance (Brenner & The Council on Sports Medicine and Fitness, 2007, reaffirmed 2014; Magrini & Dahab, 2016). Nursing Management Initially, apply ice when pain is severe. Anti-inflammatory medications such as ibuprofen may be helpful. Encourage the child to limit exercise and participate in a different activity. After a few weeks, most overuse syndromes resolve; at that point, the athlete may resume the prior activity. Osgood-Schlatter disease is the exception and may require 6 to 18 months to resolve (Kienstra & Macias, 2019b). Using pads or braces that are appropriate to the painful body part is also helpful. Supporting the arm with a sling may relieve stress on the proximal humerus when epiphysiolysis occurs. Heel cups used in athletic shoes help relieve stress on the heels associated with Sever disease. To prevent overuse syndromes, encourage athletes to perform appropriate stretching exercises during a 20- to 30-minute warm-up period before each practice or game. Also encourage several weeks of conditioning training before the season begins. There is currently limited research pertaining to overuse injuries in the young athlete. The American Academy of Pediatrics has developed some guidelines to help prevent these injuries such as the following: encourage 1 to 2 days off per week of competitive athletics, sports training, and competitive practice; encourage 2 to 3 months away from a specific sport during the year; and educate to increase weekly training time, number of repetitions, or total distance by no more than 10% a week; participation in sports should be about fun, skill acquisition, sportsmanship, and safety

Metatarsus adductus,

a medial deviation of the forefoot, is one of the most common foot deformities of childhood (Fig. 22.17). It occurs most commonly as a result of in utero positioning (Winell & Davidson, 2016). Half of all cases occur bilaterally (Winell & Davidson, 2016). The degree of flexibility is important and determines treatment. If the forefoot is flexible past neutral manipulation passively, observation is often sufficient. If the forefoot is flexible only to neutral manipulation, stretching exercises may be beneficial. If the forefoot is rigid and is not flexible to neutral manipulation, serial casting, preferably before the age of 8 months, may be required (Winell & Davidson, 2016). Surgical intervention is rarely needed. Nursing Assessment The deformity is usually noted at birth. Note inward deviation of the forefoot with the hindfoot remaining in normal position. The great and second toes might be separated. Determine forefoot flexibility. Range of motion of the ankle, hindfoot, and midfoot is normal. Nursing Management Most cases will resolve without treatment and nursing care is aimed at education and reassurance of the parents. Nursing care for the child with severe metatarsus adductus is similar to that of the child with clubfoot (see below).

Urticaria,

commonly called hives, is a type I hypersensitivity reaction caused by an immunologically mediated antigen-antibody response of histamine release from mast cells. Vasodilation and increased vascular permeability result, and erythema and wheals then occur. Urticaria usually begins rapidly and may disappear in a few days or may take up to 6 weeks to resolve. The most common causes of this reaction are foods, drugs, animal stings, infections, environmental stimuli (e.g., heat, cold, sun, tight clothes), and stress. Therapeutic management focuses on identifying and removing the cause as well as providing antihistamines or steroids. Nursing Assessment Obtain a detailed history of new foods, medications, symptoms of a recent infection, changes in environment, or unusual stress. Inspect the skin, noting raised, edematous hives anywhere on the body or mucous membranes (Fig. 23.15). The hives are pruritic, blanch when pressed, and may migrate. Angioedema may also be present and is identifiable as subcutaneous edema and warmth, occurring most frequently on the extremities, face, or genitalia. Carefully assess airway and breathing, as hypersensitivity reactions may affect respiratory status. Nursing Management Identify and remove the offending trigger. Discontinue antibiotics. Administer antihistamines, corticosteroids, and topical antipruritics as prescribed. Inform the child and family that the episode should resolve within a few days. If it lasts up to 6 weeks, the child should be reevaluated (Covar, Fleisher, Cho, & Boguniewicz, 2018). Advise the family to obtain a medical alert bracelet for the child if the reaction is severe.

Limb deficiencies,

either complete absence of a limb or a portion of it or deformity, occur as the fetus is developing. The limb either fails to form normally or does not form at all. The cause is unknown. Certain behaviors and exposures can increase the risk of limb deficiencies such as exposure to certain chemicals, viruses, medications, and possible maternal exposure to tobacco smoke (CDC, 2018b). These defects can be attributed to an amniotic band constricting the limb, resulting in either incomplete development or amputation of the limb. Many children born with limb deformities also have congenital anomalies such as craniofacial abnormalities, cardiac and abdominal wall defects (CDC, 2018b). Therapeutic management is aimed at improving the child's functional ability. Physical therapy and occupational therapy may be helpful. Adaptive equipment such as a prosthesis also may be prescribed. Nursing Assessment Note the extent of limb deformity, providing an accurate description of the presence or absence of a portion of the arm or leg, or missing fingers or toes. Assess the child's ability to use the extremity as a helper (arms) or in ambulation (legs). Determine status of acquisition of developmental skills. Nursing Management Reinforce prescribed activities that are meant to improve the child's function. Provide activities in which the child is capable of participating. If the limb deficiency is significant, refer the infant to the local early intervention office as soon as possible after birth. Early intervention, available in all 50 states, is designed to promote development from birth to age 3 years. Absence of a limb or a significant portion of a limb will have a considerable impact on the child's ability to meet developmental milestones as expected.

(metatarsus adductus)

in-toeing as a result of in utero positioning (see Fig. 22.2). The feet remain flexible and may be passively moved to midline and in a straight position. This also resolves as the infant's musculoskeletal system matures. Pes planus (flat feet) is noted in infants when they begin to walk. The long arch of the foot is not yet developed and makes contact with the floor, resulting in a medial bulge. As the child grows and the muscles become less lax, the arch generally develops. Some children may continue with flexible flat feet, and this is considered a normal variation.

Seborrhea

is a chronic inflammatory dermatitis that may occur on the skin or scalp. In infants it occurs most often on the scalp and is commonly referred to as cradle cap. Infants may also manifest seborrhea on the nose or eyebrows, behind the ears, or in the diaper area. It usually resolves over a period of weeks to months (Sasseville, 2019). Adolescents manifest seborrhea on the scalp (dandruff) and on the eyebrows and eyelashes, behind the ears, and between the shoulder blades. It is thought that seborrhea is an inflammatory reaction to the fungus Pityrosporum ovale and is worsened by sebaceous involvement related to maternal hormones in the infant and androgens in the adolescent. Nursing Management Wash or shampoo the affected areas with a mild soap. Apply anti-inflammatory cream to skin lesions if prescribed. In the infant, apply mineral oil to the scalp, massage it well with a washcloth, and then shampoo 10 to 15 minutes later, using a brush to gently lift the crusts; do not forcibly remove the crusts. If needed, selenium sulfide shampoo may safely be used on the infant, following the aforementioned procedure. The adolescent may require daily shampooing with an antidandruff shampoo.

Acne,

is a common skin condition in childhood (Prok & Torres-Zegarra, 2018), is a disorder that affects the pilosebaceous unit. It affects males and females, as well as all ethnic groups. Acne that persists past the usual course of time for infantile or adolescent acne may be caused by endocrine abnormalities. It may also occur in response to the use of certain types of drugs such as corticosteroids, androgens, phenytoin, and others. The usual presentation and nursing management of acne neonatorum and acne vulgaris is presented below.

Hemoglobinopathy

is a condition in which abnormal hemoglobin is present. A large percentage of the newborn's hemoglobin is fetal hemoglobin (Hgb F). Hgb F can exchange oxygen molecules at lower oxygen tensions compared to adult hemoglobin. Over the first several months of life, Hgb F levels fall as it is replaced with Hgb A (adult hemoglobin). The healthy older infant then displays Hgb AA. In hemoglobinopathies, this normal hemoglobin configuration is disturbed. Causes of hemoglobinopathies are genetic and include sickle cell disease (SCD), hemoglobin SC disease, α-thalassemia, and β-thalassemia. This discussion will focus on SCD and β-thalassemia (Cooley anemia).

Anemia

is a condition in which the level of RBCs is lower than the age-appropriate normal value. Anemia may develop as a result of decreased production of RBCs or loss and destruction of RBCs. The loss of production can be related to lack of dietary intake of the nutrients needed to produce the cells, alterations in the cell structure, or malfunctioning tissues (e.g., bone marrow). Anemia related to nutritional deficiency includes iron deficiency, folic acid deficiency, and pernicious anemia. Anemia may also result from toxin exposure (lead poisoning) or as an adverse reaction to a medication (aplastic anemia). Blood loss may result from surgery or trauma. Alteration or destruction of cells occurs in certain genetic and cellular development disorders (McFarren & Levy, 2017). Anemia caused by the alteration or destruction of the RBCs is termed hemolytic anemia. There are several types of hemolytic anemia, such as sickle cell disease (SCD) and thalassemia; these two disorders are discussed under the section on hemoglobinopathies. Anemia related to insufficient intake of specific nutrients is the most common type of anemia in children. Nutrient intake may be reduced in children due to food dislikes or conditions that produce malabsorption.

Rickets

is a condition in which there is softening or weakening of the bones. Childhood rickets may occur as a result of nutritional deficiencies such as inadequate consumption of calcium or vitamin D or limited exposure to sunlight (required for adequate production of vitamin D). Rickets caused by vitamin D deficiency is a preventable condition but cases continue to be reported in infants, children, and adolescents (Misra, 2018). Rickets may also occur if the body cannot regulate calcium and phosphorus in the appropriate balance, such as in chronic renal disease. Gastrointestinal disorders in which fat absorption is altered (e.g., Crohn disease, celiac disease, and cystic fibrosis) may lead to rickets, as vitamin D is a fat-soluble vitamin. Calcium is primarily laid down in the bones of the fetus during the third trimester. Premature infants miss this period of calcium accumulation and also suffer from inadequate calcium intake in the neonatal period. Thus, premature infants often demonstrate rickets of prematurity. Regardless of the underlying cause, rickets is most likely to occur during periods of rapid growth. Vitamin D regulates calcium absorption from the small intestine and levels of calcium and phosphate in the bones. When calcium and phosphate levels in the blood are imbalanced, then calcium is released from the bones into the blood, resulting in loss of the supportive bony matrix. Therapeutic Management Treatment of rickets is aimed at correcting the calcium imbalance so that the skeleton may develop properly and without deformity. Calcium and phosphorus supplements are given, and some children also require vitamin D supplements. If rickets is not corrected while the child is still growing, permanent skeletal deformities and short stature may result. Nursing Assessment Obtain a health history, determining risk factors such as: Limited exposure to sunlight Strict vegetarian diet or lactose intolerance (either one without milk product ingestion) Exclusive breastfeeding by a mother who has a vitamin D deficiency Dark-pigmented skin Prematurity Malabsorptive gastrointestinal disorder Chronic renal disease Nursing Management Administer calcium and phosphorus supplements at alternate times to promote proper absorption of both of these supplements. Encourage exposure to moderate amounts of sunlight and administer vitamin D supplements as prescribed. Teach families that good dietary sources of vitamin D are fish, liver, and processed milk.

Henoch-Schönlein purpura

is a condition that affects mostly male young children and develops in association with a viral or bacterial infection (most often respiratory) (Ambruso et al., 2018). The classic presentation is vasculitis with immunoglobulin A (IgA)-dominant immune deposits affecting small vessels. These small vessels are generally in the skin, gut, and kidney. In most children the course of the disease is benign and the prognosis is good. In a few children, however, ongoing nephrotic syndrome may occur as a result of renal injury, and those children may have hypertension. Pulmonary, cardiac, and neurologic complications can also occur. No specific treatment exists for Henoch-Schönlein purpura, since most of the cases resolve without treatment. Treatment with corticosteroids, such as prednisone, may be helpful in children with severe joint or GI manifestations (Ambruso et al., 2018). If renal injury occurs, children may require renal function testing, and evaluation for hypertension and treatment when present. Nursing Assessment Note history of viral or bacterial infection. Determine the onset of the complaint and how it has progressed or changed. Note history of joint or abdominal pain. Measure blood pressure. Inspect the skin for a purpuric palpable rash and document the size and location of lesions. Palpate the rash to determine its extent (Fig. 24.14). Gently palpate the joints for tenderness. Palpate the abdomen for tenderness. Note visible or occult blood in the stool. Note cherry- or tea-colored urine, indicating the presence of blood in the urine; urinalysis can verify the amount of blood present in the urine. Serum IgA levels may be elevated. Nursing Management Treatment of the symptoms is the focus. In children with severe joint or abdominal pain, administer analgesics as prescribed and note the response to pain medications. If the child has normal renal function, maintaining hydration is the most important intervention. Monitor intake and output. Note the color of urine. Administer corticosteroids and anticoagulants, alone or together, if ordered to reduce renal impairment. Teach the child and family about the therapy, such as management of hypertension with medications, and sodium restriction. Teach them about signs of renal injury, such as blood in the urine and changes in weight, as well as frequency and volume of urine output.

Tibia vara (Blount disease)

is a developmental disorder affecting young children. There are three types: infantile (1 to 3 years), juvenile (4 to 10 years), and adolescent (11 years or older) (Baldwin & Wells, 2016). Infantile is the most common and is discussed here (Baldwin & Wells, 2016). The normal physiologic bowing or genu varum becomes more pronounced in the child with tibia vara. The cause of tibia vara is unknown, but it is considered to be a developmental disorder because it occurs most frequently in children who are early walkers. Most cases occur in African-American females and both extremities are affected (Baldwin & Wells, 2016). In addition to early walking, obesity is also a risk factor. If left untreated, the growth plate of the upper tibia ceases bone production. Asymmetric growth at the knee then occurs and the bowing progresses. Severe degenerative arthritis of the knee is an additional long-term complication. Therapeutic management is aimed at stopping the progression of the disease through bracing or surgical treatment. Medical or surgical treatment should begin early, before 4 years of age. Nursing Management Bracing may include a modified knee-ankle-foot orthosis that relieves the compression forces on the growth plate, allowing bone growth resumption and correction of bowlegs. To be successful, bracing must be continued for months to years and the brace must be worn 23 hours per day. Compliance is the most significant barrier to successful treatment. Parents have a difficult time forcing their toddler to stay in a brace that inhibits mobility for the bulk of the day (particularly a bilateral brace). Support parents by encouraging and praising their compliance with bracing. Teach parents to assess for potential skin impairment from brace rubbing. When surgical treatment is required, the leg(s) will be immobilized in a long-leg bent knee or spica cast after the osteotomy is performed. Perform routine cast care. Refer to the Nursing Process Overview earlier in the chapter for additional interventions related to care of the immobilized child.

Sprains

result from a twisting or turning motion of the affected body part. The tendons and ligaments stretch excessively and may tear slightly. They are uncommon in young children as their growth plates are weaker than their muscles and tendons, making them more prone to fracture. They may occur at any joint, but the most common are ankle and knee sprains. Therapeutic management of sprains includes rest, ice, compression, and elevation (RICE). Other treatment options may include activity restrictions, splints or casts, crutches or wheelchair, and physical therapy. On initial evaluation, sprains need to be differentiated from torn ligaments and meniscal tears, as those conditions are more serious and may require surgical intervention. Nursing Management Instruct the child and family in appropriate treatment of sprains, which includes: Rest: limit activity. Ice: apply cold packs for 20 to 30 minutes, remove for 1 hour, and repeat (for the first 24 to 48 hours). Compression: apply an Ace wrap or other elastic bandage or brace; check skin for alterations when rewrapping. Elevation: elevate the injured extremity above the level of the heart to decrease swelling (Fig. 22.33).

Von Willebrand Disease vWD

is a genetically transmitted bleeding disorder that may affect both genders and all races. The disorder is a deficiency in von Willebrand factor (vWF). Under ordinary circumstances vWF serves two functions: to bind with factor VIII, protecting it from breakdown, and to serve as the "glue" that attaches platelets to the site of injury. Deficiency in this factor results in a mild bleeding disorder. Children with vWD bruise easily, have frequent nosebleeds (epistaxis), and tend to bleed after oral surgery. Pubescent girls often have menorrhagia. Therapeutic management of vWD is similar to that of hemophilia. Prevention of injury is important. When bleeding or injury does occur, vWF is administered. Desmopressin may also be used to release the factors necessary for clotting. Desmopressin raises the plasma level from stores in the endothelium of blood vessels; this releases factor VIII and vWF from these stores into the bloodstream. These may also be administered before dental work or surgery. Nursing Management Nursing management is also similar to the management of the child with hemophilia. The major difference is the administration of desmopressin. Administer desmopressin nasal spray as prescribed when a bleeding episode occurs. Desmopressin may also be given via an intravenous infusion or subcutaneously (less common). Stimate is the only brand of desmopressin nasal spray that is used for controlling bleeding; the other brands are used for homeostasis and enuresis. Desmopressin is an antidiuretic hormone, so closely monitor fluid balance. Twenty-four hours should lapse between doses, as lessening of the response (tachyphylaxis) occurs with more frequent use (Rick, 2019). vWD may also be treated with intravenous infusion of vWF, similar to factor VIII infusion for hemophilia A. Teach children and their families how to avoid or minimize bleeding episodes (see Teaching Guidelines 24.4).

Scoliosis

is a lateral curvature of the spine that exceeds 10 degrees. It may be congenital, associated with other disorders, or idiopathic. Table 22.6 explains the types of scoliosis. Idiopathic scoliosis, with the majority of cases occurring during adolescence, is the most common scoliosis (Mistovich & Spiegel, 2016). Hence, this discussion will focus on adolescent idiopathic scoliosis. The etiology of idiopathic scoliosis is not known, but genetic factors, growth abnormalities, and bone, muscle, disc, or central nervous system disorders may contribute to its development. Early screening and detection of scoliosis result in improved outcomes. Pathophysiology In the rapidly growing adolescent, the involved vertebrae rotate around a vertical axis, resulting in lateral curvature, and asymmetry of the shoulder and waistline is evident. The vertebrae rotate to the convex side of the curve, with the spinous processes rotating toward the concave side, resulting in displacement of the ribs and rib asymmetry (Mistovich & Spiegel, 2016). As the curve progresses, the shape of the thoracic cage continues to change and respiratory and cardiovascular compromise may occur (the main complications of severe scoliosis). Therapeutic Management Treatment of scoliosis is aimed at preventing progression of the curve and decreasing the impact on pulmonary and cardiac function. Treatment is based on the age of the child, expected future growth, and severity of the curve. Observation with serial examinations and spine radiographs is used to monitor curve progression. For curves of 25 to 45 degrees, bracing may be sufficient to decrease progression of the curve (Mistovich & Spiegel, 2016). Box 22.2 describes types of scoliosis braces and Figure 22.28 shows examples of braces. The choice of brace will depend on the location and severity of the curve. Some curves will progress despite appropriate bracing and compliance. Surgical correction is often required for curves greater than 45 degrees; it is achieved with rod placement and bone grafting (Mistovich & Spiegel, 2016). Partial spinal fusion accompanies many of the corrective surgeries. Multiple surgical approaches and techniques with various instrumentation methods exist for fusion and rod placement. The surgical approach may be anterior, posterior, or both. Traditional rod placement (Harrington rod) involves a single rod fused to the vertebrae, resulting in curve correction but also a flat-backed appearance. Newer rod instrumentations allow for scoliosis curve correction with maintenance of normal back curvature. The rods are shorter, and several are wired or grafted to the appropriate vertebrae to achieve correction. Figure 22.29 shows one example of surgical rod instrumentation. Physical Examination The physical assessment of a child with possible or actual scoliosis involves mainly inspection and observation. Auscultate the heart and lungs to determine compromise related to severe curvature. Observe the child at rest, sitting, and standing for evidence of poor posture. Inspect the child's back in a standing position. Note asymmetries such as shoulder elevation, prominence of one scapula, uneven curve at the waistline, or a rib hump on one side. Measure shoulder levels from the floor to the acromioclavicular joints. Note the difference between the height of the high and low shoulder in centimeters. Measure heights of anterior and posterior iliac spines and note the difference in centimeters. View the child from the side, noting abnormalities in the spinal curve. With the child bending forward, arms hanging freely, note asymmetry of the back (pronounced hump on one side). Figure 22.30 shows scoliosis noted upon visual inspection. Note leg-length discrepancy if present. During the neurologic examination, balance, motor strength, sensation, and reflexes should all be normal. Encouraging Compliance With Bracing Bracing is intended to prevent progression of the curve but does not correct the current curve. Although modern braces display an improved appearance, with no visible neckpiece, and can be worn under clothes, many adolescents are not compliant with brace wear. The brace is recommended to be worn 18 hours per day to prevent curve progression, although recent studies have found 13 hours to be sufficient in some cases (Scherl, 2019). Many factors may contribute to noncompliance, including the discomfort associated with brace wear such as pain, heat, and poor fit. The family environment may not be conducive to compliance with brace wear, and teenagers are very concerned about body image. Inspect the skin for evidence of rubbing by the brace that may impair skin integrity. Teach families appropriate skin care and recommend they check the brace daily for fit and breakage. Encourage the teen to shower during the time of day that the brace is off and to ensure that the skin is clean and dry before putting the brace back on. Wearing a cotton T-shirt under the brace may decrease some of the discomfort associated with brace wear. Exercises to strengthen back muscles may prevent muscle atrophy from prolonged bracing and maintain spine flexibility. Providing Postoperative Care The goal of nursing management in the postoperative period after spinal fusion with or without instrumentation is to avoid complications. Perform neurovascular checks with each set of vital signs. When turning the child, use the log-roll technique to avoid flexion of the back (Fig. 22.31). Provide proper pain management and medicate for pain before repositioning and ambulation. Administer prophylactic intravenous antibiotics if ordered. Assess for drainage from the operative site and for excess blood loss via the Hemovac or other drainage tube. Maintain Foley patency, as the child will be confined to bed for the first couple of days. Maintain strict recording of fluid intake and output. Administer transfusions of packed red blood cells if ordered. Ambulation, once ordered, should be done slowly to avoid orthostatic hypotension. Assist the family with arrangements to continue the teen's schoolwork while hospitalized and/or arrange for home tutoring during the several-week recovery period.

Cellulitis

is a localized infection and inflammation of the skin and subcutaneous tissues and is usually preceded by skin trauma of some sort (Fig. 23.3). Periorbital cellulitis is a bacterial infection of the eyelids and tissue surrounding the eye. The bacteria may gain entry to the skin via an abrasion, laceration, insect bite, foreign body, or impetiginous lesion. Periorbital cellulitis may also result from a nearby bacterial infection, such as sinusitis. Staphylococcus aureus, Streptococcus pyogenes, and Streptococcus pneumoniae are the most commonly implicated bacteria. The bacteria produce either an enzyme or endotoxins that initiate the inflammatory response. Redness, swelling, and infiltration of the skin by the inflammatory mediators occur

Torticollis

is a painless muscular condition presenting in infants or in children with certain syndromes. Congenital muscular torticollis may result from in utero positioning or difficult birth. Preferential turning of the head to one side while in the supine position after birth may also lead to torticollis. Torticollis results from tightness of the sternocleidomastoid muscle, resulting in the infant's head being tilted to one side. Therapeutic management involves passive stretching exercises. These exercises should be effective in 90% of cases of congenital torticollis, especially if treatment is started within the first 3 months of life (O'Toole & Spiegel, 2016). Physical therapy may be prescribed and a tubular orthosis for torticollis (TOT) collar may also be used. Surgery is not common but may be done in the preschool years if other methods have been unsuccessful. Plagiocephaly may result from the continued pressure on the side of the skull to which the neck is turned. Nursing Assessment Note history of head tilt and infant's lack of desire to turn the head in the opposite direction. Observe the infant for wryneck (tilting of the head to one side; Fig. 22.22). Note limited movement of the neck while performing passive range of motion. Palpate the neck, noting a mass in the sternocleidomastoid muscle on the affected side. Examine the head for evidence of plagiocephaly. Accompanying hip dysplasia is seen in 8% to 20% of cases. Therefore, careful examination of the hips is warranted Nursing Management Teach parents gentle neck-stretching exercises to be performed several times a day. While immobilizing the shoulder on the affected side, gently sustain a side-to-side stretch toward the unaffected side, holding the stretch for 10 to 30 seconds. Repeat 10 to 15 times per session. Perform an ear-to-shoulder stretch in a similar fashion. To prevent the development of torticollis in the unaffected infant, prevent positional plagiocephaly. Prevent flatness of one side of the head by varying the infant's head position, and do not always turn the infant's head to one side while he or she is in the infant seat, in the swing, or lying supine.

Spina bifida

is a term that is often used to refer to all neural tube disorders that affect the spinal cord. This can be confusing and a cause of concern for parents. There are well-defined degrees of spinal cord involvement, and it is important for healthcare professionals to use the correct terminology. Spina bifida occulta is a defect of the vertebral bodies without protrusion of the spinal cord or meninges. This defect is not visible externally and in most cases has no adverse effects (see Fig. 22.13). Spina bifida occulta is a common anomaly. It is estimated that it affects 10% to 20% of otherwise healthy people (National Institute of Neurological Disorders and Stroke [NINDS], 2013). Children with spina bifida occulta need no immediate medical intervention. Complications are rare but may include more significant abnormalities of the spinal cord such as tethered cord, syringomyelia, or diastematomyelia. Nursing Assessment In most cases, spina bifida occulta is benign and asymptomatic and produces no neurologic signs. The defect, which is usually present in the lumbosacral area, often goes undetected. However, there may be noticeable dimpling, abnormal patches of hair, or discoloration of skin at the defect site. If so, further investigation, including magnetic resonance imaging (MRI), may be warranted. Nursing Management Nursing care will focus on educating the family. Inform parents of its presence and what the diagnosis means. Many times parents will confuse this diagnosis with spina bifida cystica, a much more serious defect. Occasionally, children with spina bifida occulta eventually need surgical intervention due to degenerative changes or involvement of the spine and nerve roots resulting in complications such as tethered cord, syringomyelia, or diastematomyelia. When these associated problems occur, the condition is often termed "occult spinal dysraphism" to avoid confusion.

Staphylococcal scalded skin syndrome

results from infection with S. aureus that produces a toxin, which then causes exfoliation. It has an abrupt onset and results in diffuse erythema (reddening of the skin) and skin tenderness (Fig. 23.4). Scalded skin syndrome is most common in infancy and rare beyond 5 years of age (Prok & Torres-Zegarra, 2018). Of particular concern are community-acquired bacterial skin infections caused by methicillin-resistant S. aureus (CA-MRSA) (Baddour, 2019). CA-MRSA most commonly occurs as a skin or soft tissue infection, such as cellulitis or an abscess. Risk factors for CA-MRSA are turf burns, towel sharing, participation in team sports, or attendance at day care or outdoor camps. If the child presents with a moderate to severe skin infection or with an infection that is not responding as expected to therapy, it is important to culture the infected area for MRSA. Therapeutic management of most bacterial skin infections includes topical or systemic antibiotics and appropriate hygiene (see Table 23.1). Treatment of periorbital cellulitis focuses on intravenous antibiotic administration during the acute phase followed by completion of the course with oral antibiotics. Complications of periorbital cellulitis include bacteremia and progression to orbital cellulitis, which is a more extensive infection involving the orbit of the eye.

Glucose-6-phosphate dehydrogenase (G6PD)

is an enzyme that is responsible for maintaining the integrity of RBCs by protecting them from oxidative substances. G6PD deficiency is an X-linked recessive disorder that occurs when the RBCs have insufficient G6PD, or the enzyme is abnormal and does not function properly. The RBCs are then affected by oxidative stress more easily. Triggers that may result in oxidative stress and hemolysis include bacterial or viral illness or exposure to certain substances such as medications (e.g., sulfonamides, sulfones, malaria-fighting drugs [such as quinine], or methylene blue [for treating urinary tract infections]), naphthalene (an agent in mothballs), or fava beans. G6PD deficiency occurs most commonly in children of African, Mediterranean, or Asian descent (Ambruso et al., 2018). Complications include prolonged neonatal jaundice and life-threatening acute episodes of hemolysis. Therapeutic management is primarily aimed at avoiding triggers that cause oxidative stress. Nursing Assessment Note health history, including fatigue. Determine the parents' understanding of the disorder and the medications and foods to avoid. Inspect the skin for pallor or jaundice. Evaluate neurologic status, which may also be affected. Measure heart rate and respiratory rate, noting elevations. Determine oxygen saturation via pulse oximeter or blood gas analysis. Note tea-colored urine. Palpate the abdomen for splenomegaly. Laboratory studies will reveal anemia. Nursing Management Administer oxygen and treat the symptoms. Once the triggering agent is removed or the child recovers from an illness, the child will improve. Provide further education to the child and family about triggers and advise them that the child should avoid contact with these agents.

Spinal cord injury

is damage to the spinal cord that results in loss of function. Frequent causes are trauma, such as car accidents, falls, diving into shallow water, gunshot or stab wounds, sports injuries, child abuse, or birth injuries. Spinal cord injuries are relatively uncommon in children, but when they do occur they have a devastating impact on the child's physical and functional status, social and emotional development, and family functioning. Spinal cord injury is a medical emergency and immediate medical attention is required. Cervical traction is often used initially and surgical intervention is sometimes necessary. Ongoing medical treatment will be based on the child's age and overall health and the extent and location of the injury. Therapeutic management focuses on rehabilitation and prevention of complications. Spinal cord injury in children is managed similarly to that in adults. Nursing Assessment Symptoms vary based on the location and severity of the injury. Common signs and symptoms associated with spinal cord injury include: Inability to move or feel extremities Numbness Tingling Weakness Nursing Management Any child who requires hospitalization due to trauma should be considered at risk for a spinal cord injury. Immobilization of the spine is essential until full evaluation of the injury is complete and spinal cord damage is ruled out. Nursing management will be similar to management of the adult with a spinal cord injury and will focus on optimizing mobility, promoting bladder and bowel management, promoting adequate nutritional status, preventing complications associated with extreme immobility such as contractures and muscle atrophy, managing pain, and providing support and education to the child and family. Refer to the myelomeningocele section of this chapter for information related to urinary and bowel elimination. The nurse plays an important role not only in the acute care of children with spinal cord injury but also during rehabilitation. Recovery from a spinal cord injury requires long-term hospitalization and rehabilitation. An interdisciplinary team of physicians, nurses, therapists, social workers, and case managers will work to manage the child's complex and long-term needs. Promoting communication among the interdisciplinary team is essential and will be a key nursing function. Rehabilitation will need to focus on the ever-changing developmental needs of the child as he or she grows. Prevention of spinal cord injuries is an important nursing consideration. Educate the public on vehicular safety, including seat belt use and the proper use of age-appropriate safety seats. Additional education topics include bicycle, sports, and recreation safety; prevention of falls; violence prevention including gun safety; and water safety, including the risk of diving. This education can help decrease the incidence of spinal cord injury in children.

Polydactyly

is the presence of extra digits on the hand or foot (Fig. 22.16). One third of the time, polydactyly occurs in both the hand and foot (Winell & Davidson, 2016). It usually involves digits at the border of the hand or foot, but can also occur by a central digit (Winell & Davidson, 2016). Syndactyly is webbing of the fingers and toes. Both polydactyly and syndactyly can be normal variants in the newborn and can also be inherited and associated with other genetic syndromes (Winell & Davidson, 2016). Therapeutic management includes surgical removal of the digit. No treatment is usually required for syndactyly, though surgical repair is sometimes performed for cosmetic reasons. Nursing Assessment Inspect the hands and feet for the presence of extra digits. Note whether the additional digits are soft (without bone) or are full or partial digits with bone present. Note location of webbing. Nursing Management When surgical removal is necessary, provide routine pre- and postoperative care as appropriate.

Nadir

is the time after administration of the drug when bone marrow suppression is expected to be at its greatest and the neutrophil count is expected to be at its lowest (neutropenia). Nadir is individual for each drug and ranges from 7 to 28 days after dosing. An absolute neutrophil count (ANC) below 500 places the child at greatest risk, although an ANC below 1,500 usually warrants evaluation (Ahmed & Flynn, 2019). Refer to Box 24.2 for information related to calculating the ANC. Depending on institutional policy, precautions for neutropenia will be followed if the ANC is depressed. Precautions related to neutropenia generally include: Place the child in a private room. Perform hand hygiene before and after contact with each child. Monitor vital signs every 4 hours. Assess for signs and symptoms of infection at least every 8 hours. Avoid rectal suppositories, enemas, or examinations; urinary catheterization; and invasive procedures. Restrict visitors with fever, cough, or other signs/symptoms of infection. Do not permit raw fruits or vegetables or fresh flowers or live plants in the room. Place a mask on the child when he or she is being transported outside of the room. Perform dental care with a soft toothbrush if the platelet count is adequate.

Meningocele,

the less serious form of spina bifida cystica, occurs when the meninges herniate through a defect in the vertebrae. The spinal cord is usually normal and there are typically minor or no associated neurologic deficits. Treatment for meningocele involves surgical correction of the lesion (see Fig. 22.13). Nursing Assessment Initial assessment after delivery will reveal a visible external sac protruding from the spinal area. It is most often seen in the lumbar region but can be anywhere along the spinal canal. Most are covered with skin and pose no threat to the child. However, assessment to ensure that the sac covering is intact remains important. Assess neurologic status carefully. Before surgical correction the infant will be thoroughly examined to determine whether there is any neural involvement or associated anomalies. Diagnostic procedures such as computed tomography (CT), MRI, and ultrasound may be performed. Nursing Management Surgical correction may be delayed if the skin covering the sac is intact and the child has normal neurologic functioning (Kinsman & Johnston, 2016). However, as in a child with myelomeningocele, immediately report any evidence of leaking cerebrospinal fluid (CSF) to ensure prompt intervention to prevent infection. Nursing management will be supportive. Provide pre- and postoperative care similar to the child with myelomeningocele to prevent rupture of the sac, to prevent infection, and to provide adequate nutrition and hydration. Monitor for symptoms of constipation or bladder dysfunction that may result due to increasing size of the lesion. Resulting hydrocephalus has been associated with some cases of meningocele (Kinsman & Johnston, 2016). Therefore, monitor head circumference and watch for signs and symptoms of increased intracranial pressure (ICP).

Radiation therapy

uses high-energy radiation to damage or kill cancer cells. Radiant energy in either a gamma or particle form is emitted during the treatment. Radiation affects not only cancer cells but also any rapidly growing cells with which they are in contact. It may be used as a curative, adjuvant, or palliative treatment, either alone or in combination with chemotherapy. Radiation therapy is also used to shrink a tumor prior to surgical resection. The area to be treated is marked carefully to minimize damage to normal cells. Adverse effects of radiation therapy include fatigue, nausea, vomiting, oral mucositis, myelosuppression, and alterations in skin integrity at the site of irradiation. Long-term complications are related to the area of the body that was irradiated and include alterations in growth; hormone dysfunction; hearing and vision alterations; learning problems; cardiac dysfunction; pulmonary fibrosis; hepatic, sexual, or renal dysfunction; osteoporosis; and development of secondary cancer (particularly at the site of irradiation) (Mitin, 2017). Monitoring the Child Receiving Radiation Therapy Assess the child's skin daily (particularly at the treatment site), as radiation causes damage to the cells in a localized area, which may include normal cells in addition to the cancerous cells. Teach parents not to scrub ink off of the marked radiation field and avoid adhesive tape in that area. Cleanse gently, using a mild soap and pat dry rather than rubbing, so as to avoid skin irritation. Moisturize the skin with aloe vera lotion or other aqueous cream. Administer diphenhydramine or apply hydrocortisone 1% cream to reduce itching and urge to scratch. Apply Silvadene cream once or twice a day to areas of desquamation related to radiation. Avoid perfumed lotions or soaps, deodorants, heat, cold, or sun, as these will further irritate the skin in the irradiated area. Instruct the child and family that clothing should fit loosely so as not to irritate the site (ACS, 2019c). During, and for 8 weeks after, the radiation treatment, the skin will be more photosensitive. Explain the importance of protecting the skin with a high-SPF (30 or higher) sunscreen.