NSG-434 Final Exam

the childhood cancer survivor

-5 year survival rate 80% -Complications common with combined treatment modalities -Late effects are of concern: •Changes related to treatment •Interactions between treatment modes •Effects may occur months to years later -Radiotherapy can decrease growth in bones or reproductive organs -Treatments may cause hormonal dysfunction -Growth and Tanner stages are important to document -Assess all body areas for abnormalities based on treatment used

acute leukemia

-A broad group of malignant diseases of bone marrow and lymphatic system -Leukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body -Acute lymphoid leukemia (ALL): •ALL is the most common form of childhood cancer •Occurs more frequently in boys and in Caucasians. •Peak onset is between 2 and 5 years of age •Before the use of antileukemic agents (in the 40's) a child with ALL lived only 2-3 months. •The 5-year survival rate for children with ALL has greatly increased over time and is now more than 85% overall. -Acute myelogenous leukemia (AML): •The overall 5-year survival rate for children with AML has also increased over time, and is now in the range of 60% to 70% •20% of childhood cases of leukemia •Highest rates seen in the first year of life -Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count -Greatly elevated immature cells or blasts -Immature cells do not attack and destroy normal blood cells -Cellular destruction takes place by infiltration and subsequent competition for metabolic elements -Proliferating cells decrease bone marrow production due to the competition for essential nutrients -Three primary consequences: •Anemia •Infection and neutropenia •Bleeding -Bones weaken and are prone to fractures; bones hurt a lot -Bone pain from increased pressure -Signs and symptoms: fever, pallor, fatigue, anorexia, hemorrhage, bone, joint pain -Kids with leukemia bruise very easily, and they have large bruises Diagnostics: -Based on history and physical manifestations: •Minor infection does not disappear •Pale, listless, irritable, febrile, anorexia -Peripheral blood smear: •Immature leukocytes •Frequently low blood cell counts -Lumbar puncture to evaluate central nervous system (CNS) involvement •Lumbar puncture can be disconcerting because the patient cannot see what is going on with them •Always tell the kids what is going to happen, be honest: can tell them that it might hurt -Bone marrow aspiration or biopsy Therapeutic Management: -IV and chemotherapy -Three phases of therapy: 1. Induction •4 to 5 weeks, begins almost immediately 2. Intensification (consolidation) therapy: to eradicate residual leukemic cells •Chemotherapy given periodically over 6 months 3. Maintenance therapy: To preserve remission •Weekly or monthly CBCs •Treatment usually stops after 2 to 3 years •Monitor for relapse -Reinduction after relapse: •Each relapse means poorer prognosis •Testes are resistant to chemotherapy and may lead to relapse •Relapse in testes leads to intensive chemotherapy and irradiation -Bone marrow transplantation: used in both ALL and AML -When kids are on chemo, watch their labs -Also watch for infection

clubfoot

-A complex deformity of the ankle and foot that includes forefoot adduction, midfoot supination, hindfoot varus, and ankle equinus. -The foot is pointed downward and inward in varying degrees of severity. -May occur as an isolated deformity or in association with other disorders or syndromes, such as chromosomal abnormalities, arthrogryposis, or spina bifida. -50% of the cases are bilateral. -Talipes varus: inversion or bending inward -Talipes valgus: eversion or bending out -Talipes equinus: plantar flexion with toes lower than the heel -Talipes calcaneus: dorsiflexion with toes higher than the heel Therapeutic Management: -The goal of treatment is to achieve a painless, plantigrade, functional foot. -Three stages: •Correction of the deformity •Maintenance of the correction until normal muscle balance is regained •Follow-up to avert possible recurrence -Ponseti method (serial casting): begun soon after birth with weekly gentle manipulation and stretching of the foot and then serial casting for 6-10 weeks -Usually require a tenotomy (surgical cutting of the heel-cord) -After tenotomy, another long-leg cast is applied for 3 weeks. -Ponseti sandals are used after casting to maintain the correction and prevent recurrence. -If normal foot alignment is unable to be achieved, other surgical interventions will be used.

Reye's syndrome

-A disorder defined as toxic encephalopathy associated with other characteristic organ involvement -Characterized by fever, profoundly impaired consciousness, and disordered hepatic function -Cause is not well understood -Most cases follow common viral illness: influenza, varicella, etc. -Potential association exists between aspirin therapy for fever and development of Reye's syndrome -Characterized pathologically by cerebral edema and fatty changes of the liver. -The onset is notable for profuse effortless vomiting and lethargy that quickly progresses to neurological impairment, including delirium, seizures, and coma, that ultimately leads to increased ICP, herniation, and death. Diagnostics: -Definitive diagnosis is established by liver biopsy. -The staging criteria for Reye's syndrome are based on liver dysfunction and neurologic signs that range from lethargy to coma. -If it does not involve the liver, then the disorder is a lot harder to diagnose Therapeutic Management: -The most important aspect of successful management of a child with Reye's syndrome is early diagnosis and aggressive supportive therapy. -Rapid progression to coma and high peak ammonia concentrations are associated with a more serious prognosis. -Cerebral edema with increased ICP represents the most immediate threat to life. Prognosis: -Good, in view of the gravity of the disease -Kids often recover if we can keep their ICP down -If we can't keep ICP down, their brains will herniate and they will die (most common cause of death with this syndrome)

hemophilia

-A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors -In 80% of cases, the inheritable pattern is X-linked recessive -Identification of a specific factor deficiency allows for definitive treatment -More frequently seen in boys Diagnostic Evaluation: -History of bleeding episodes: •Overt prolonged bleeding •Hemarthrosis: bleeding into the joint cavities •Ecchymosis: bruising -X-linked inheritance -Laboratory findings: •Low levels of factor VIII or IX; prolonged partial thromboplastin time •Normal: platelet count, parathormone level, and fibrinogen level Therapeutic Management: -Replace missing clotting factors: •Aggressive replacement therapy with factor concentrate •Home infusion -Desmopressin (DDAVP): •Synthetic vasopressin •IV administration or nasal spray •Causes two to four times increase in factor VIII activity •Used for mild hemophilia Prognosis: -No cure -Symptoms can be controlled -Potentially crippling deformities can be reduced or avoided -Mild-to-moderate hemophilia: patients live near-normal lives -Control of symptoms and limiting joint damage improve quality of life -Treatment options: gene therapy; a working copy of the factor VIII gene is introduced Care Management: -Prevent bleeding: •Safe environment: sports (swimming, walking, jogging, tennis, golf, fishing, bowling) •Dental hygiene: water irrigating device; soften toothbrush in warm water; sponge-tipped toothbrush -Recognize and control bleeding: RICE -Prevent the crippling effects of bleeding: repeated episodes of hemarthrosis, incompletely absorbed blood in the joints, and limitations of motion, bone, and muscle changes occur that result in flexion contractures and joint fixation -Support the family and home care: •Genetic counseling •Mother is usually the carrier and therefore may have a feeling of responsibility for this condition •Technology can now ID carriers using DNA testing

osteogenesis imperfecta

-A group of heterogeneous inherited disorders of connective tissue -Characterized by excessive fragility and defects of bones -Defective periosteal bone formation and reduced cortical thickness of bones -Hyperextensibility of ligaments -Various types of OI that range from mild to lethal before birth Clinical Manifestations: -Varying degree of bone fragility and deformity -Short stature -Blue sclera: discoloration of the sclera is due to the underlying choroidal veins which show through. This is due to the sclera being thinner than normal because of the defective type I collagen has not formed correctly -Hearing loss (occurs between the ages of 20 and 30 years of age) -Hypoplastic discolored teeth -Alkaline phosphate is high all the time -Life expectancy is very low Therapeutic Management: -Primarily supportive care -Intravenous bisphosphonate therapy: promotes increased bone density and prevents fractures -May rule out osteogenesis imperfecta if multiple fractures occur -Rehabilitative approach for prevention of further complications" •Positional contractures and deformities •Muscle weakness and osteoporosis •Malalignment of lower-extremity joints -No cure: terminal; may live to be a teenager or not -Parents must understand that there will be many doctor's appointments with PT and OT. -Teach parents to carefully handle their infant -Do not hold up by the ankles when changing the infant's diaper: lift by the buttocks -Braces and splints can help support limbs, prevent fractures, and aid in ambulation

hodgkin disease

-A lymphoma more prevalent among children 15 to 19 years of age -Neoplastic disease originating in lymph system -Primarily involves lymph nodes -Often metastasizes to spleen, liver, bone marrow, lungs, or other tissues Clinical Manifestations: -Painless enlarged cervical or supraclavicular lymphadenopathy -Cough, abdominal discomfort, anorexia -Fever, weight loss, night sweats -Can cause difficulty swallowing or breathing: pushes on epiglottis and trachea Diagnostics: -History and physical, CBC, uric acid, liver function, CRP or ESR, alkaline phosphatase, urinalysis, radiological tests -Lymph node biopsy, presence of Sternberg-Reed cells -Alkaline phosphatase tells how strong the bones are -CRP: C-Reactive Protein measured in your blood, increases when there's inflammation in your body -ESR = erythrocyte sedimentation rate. It is often referred to as the sedimentation rate or the sed rate. -An ESR blood test is usually done to check and understand the degree of inflammation in the body such as cancers, various kinds of infections as well as autoimmune disorders and diseases. Treatment: -Radiation -Chemotherapy (alone or with radiation) -Prognosis: long-term survival is excellent -Aggressive therapy increases chances of complications

SCID (severe combined immunodeficiency disease)

-Absence of immunity, both humoral and cell-mediated -Susceptible to infection early in life: most common manifestation; most often occurs in the first months -Chronic infections, failure to completely recover from infections, frequent reinfections, and infection with unusual agents -Failure to thrive is a consequence of the persistent illness Treatment: -Treated with donor bone marrow transplant if diagnosed within the first 3 months of life -IVIG provides passive immunity until transplant performed -Prophylaxis -Key is to prevent infections and support child and family -Prognosis is very poor if bone marrow transplant not available

Guillain-Barre syndrome

-Also called "infectious polyneuritis" -Acute demyelinating polyneuropathy with progressive paralysis -An uncommon syndrome -Children less often affected than adults -Occurrence in children most often between ages 4 and 10 years -Immune-mediated disease -Often associated with viral or bacterial infection or administration of vaccines -Inflammation and edema in spinal and cranial nerves: •Progresses to impaired nerve conduction and then partial or complete paralysis of involved muscles -Hallmark of GBS is acute peripheral motor weakness. The paralysis occurs approximately 10 days after a nonspecific viral infection. -It has also been reported after certain vaccines (rabies, influenza, polio, and meningococcal). Clinical Manifestations: -Initially: muscle tenderness, paresthesia, muscle weakness -Paralysis: rapidly ascends from lower extremities; may involve trunk, arms, face -Flaccid paralysis, loss of reflexes -Intercostal and phrenic nerve involvement -In many patients: urinary incontinency or retention and constipation -Kids with this disorder often end up paralyzed -May die due to phrenic nerve obstruction (diaphragm no longer works) Therapeutic Management: -Treatment: symptomatic; primarily supportive -Possibly steroids, intravenous immune globulin, and plasmapheresis -Respiratory support -Medications to prevent complications: •IVIVG is now recommended as the primary treatment of GBS when administered within 2 weeks of disease onset •Corticosteroids alone do not decease the symptoms or shorten the duration of the disease -Phases: •Acute: patients are hospitalized because of respiratory and pharyngeal involvement and may require assisted ventilation and sometimes a temporary tracheostomy; starts when symptoms begin and continues until new symptoms stop appearing or deterioration ceases; may take as long as 4 weeks. •Plateau: symptoms remain constant without further deterioration; may last from days to weeks •Recovery: patient begins to improve and progress to optimal recovery; it usually lasts a few weeks to months depending on the deficits incurred by the illness -Have equipment for intubation at bedside for emergency use Course and Prognosis: -Better outcomes are associated with younger ages; most patients have complete recovery -In most patients, muscle function begins to return 2 days to 2 weeks after onset of symptoms, but period to complete recovery is prolonged -Most deaths caused by respiratory failure

Duchenne muscular dystrophy

-Also called "pseudohypertrophic muscular dystrophy" -Most severe and most common of muscular dystrophies in childhood -X-linked inheritance pattern; one third of cases result from fresh mutations -Incidence: 1 per 3600 male births -Dystrophin, a protein product in skeletal muscle, is absent from the muscles of children with DMD Clinical Manifestations: -Onset between ages 3 and 7 years -Progressive muscle weakness, wasting, and contractures; muscle weakness starts at their feet and works its way up -Hypertrophic calf muscles in most patients -Progressive generalized weakness in adolescence -Death from respiratory or cardiac failure -Difficulties in running, riding a bicycle, and climbing stairs are usually the first symptoms noted. -Lordosis occurs as a result of weakened pelvic muscles, and the waddling gait is a result of weakness in the gluteus medius and maximus muscles -Cardiomyopathy is seen in approximately 50% to 80% of patients with DMD -Waddling gait, frequent falls, Gower sign -Lordosis -Enlarged muscles, especially in thighs and upper arms -Profound muscular atrophy in later stages -Mild to moderate mental impairment -Obesity: often on the obese side because of inflammation and not being active -Usually come to doctor because they are falling so much -If progresses to diaphragm or heart, can lead to respiratory failure or cardiac failure Diagnostics: -Prenatal diagnosis as early as 12 weeks of gestation -Established primarily by blood polymerase chain reaction for dystrophic chain mutation -Confirmation by electromyelography, muscle biopsy, and serum enzyme measurement -Positive family history and display of the usual characteristics of the disease -Gower sign: a child with DMD attains standing posture by kneeling and then gradually pushing his torso upright (with knees straight) by "walking" his hands up his legs. Therapeutic Management: -No effective treatment established -Primary goal: maintain function in unaffected muscles as long as possible -Keep child as active as possible -Range-of-motion exercises, bracing, performance of activities of daily living, surgical release of contractures as needed -Genetic counseling for family Care Management: -Multidisciplinary team helps child and family cope with chronic, progressive, debilitating disease -Design program to foster independence and activity as long as possible -Teach child self-help skills -Provide appropriate health care assistance as child's needs intensify (e.g., home health, skilled nursing facility, respite care for family) -Survival in individuals with DMD may be prolonged for several years with the use of non-invasive ventilation and mechanically-assisted coughing as alternatives to tracheostomy and airway suctioning. -These kids and their families need a lot of support

lymphocytic thyroiditis

-Also known as "Hashimoto's disease" or "autoimmune thyroiditis" -Most common cause of thyroid disease in children and adolescents and accounts for the largest percentage of juvenile hypothyroidism -Can occur during the first 3 years of life, it occurs more frequently after 6 years of age, with peak incidence occurring during adolescence. -More common in children and adults -Almost always the cause of a thyroid problem Clinical Manifestations: -Enlarged thyroid gland: •Noted by parents when child swallows •Usually symmetric •Firm, nontender •Freely moveable •Doesn't hurt -Tracheal compression: •Sense of fullness •Hoarseness, dysphagia •Makes it hard to breathe or swallow -Hyperthyroidism (possible) Therapeutic Management: -Goiter may be transient, asymptomatic -Goiter may resolve spontaneously within 1 to 2 years -Oral thyroid hormone administration often decreases the goiter significantly -Surgery is contraindicated in this disorder

contusion

-Also known as a bruise -Damage to soft tissue, subcutaneous tissue, and muscle -Escape of blood into tissues causes ecchymosis (black-and-blue discoloration) -The tearing of these tissues and small blood vessels and the inflammatory response lead to hemorrhage, edema, and associated pain when the child attempts to move the injured part. -Swelling, pain, disability -Crush injuries occur when children's extremities or digits are crushed (finger slammed in the car door). -A severe crush injury involves the bone, with swelling and bleeding beneath the nail and sometimes laceration of the pulp of the nail. -These hematomas may require a release of blood by creating a hole at the proximal end of the main with a special cautery device or a heated sterile 18-gauge needle. -Treatment for contusions: application of cold

Ewing sarcoma

-Arise in marrow spaces -Originates in shaft of long and trunk bones: pelvis, femur, tibia, fibula, humerus, ulna, vertebrae, scapula, ribs, skull -Tumor is on inside of leg Therapeutic Management: -Limb salvage or amputation depending on situation: •Limb salvage procedures might be feasible in extremity lesions •Amputation may be considered if the results of radiation render the extremity useless or deformed -Radiation -Chemotherapy

type 2 diabetes mellitus

-Arises because of insulin resistance -Onset usually after age 45 -Occurs more in people who are overweight, sedentary, or have a family history -Native American, Hispanic, and African-American children at increased risk for type 2 diabetes mellitus -Affected people may require insulin injections -Recurrent vaginal and urinary tract infections, especially candida albicans, are often an early sign of type 2 DM, especially in adolescents

retinoblastoma

-Arises from retina -May be caused by genetic alterations -Retinal cancer that shows up in child's pupil -Average age of diagnosis: 2 years of age -60% are unilateral and nonhereditary; 25% are bilateral and hereditary; and 15% are unilateral and hereditary -When a tumor arises in the macular region (the area directly at the back of the retina when the eye is focused straight ahead), a white reflex may be visible when the tumor is small. Strabismus is also a common sign. -Long-term concern is secondary tumors Clinical Manifestations: -Few obvious signs -Whitish "glow" in the pupil -Strabismus -Blindness (late) Diagnostic Evaluation: -Detailed family history -Ophthalmoscopy -Ultrasound -CT and MRI Therapeutic Management: -Radiation -Chemotherapy -Surgical implantation of iodine-125 for radiation -Chemo has been used to decrease the size of the tumor and then plaque brachytherapy (surgical implantation of an iodine-125 applicator on the sclera until the maximum radiation dose has been delivered to the tumor) -Photocoagulation: use of a laser beam to destroy retinal blood vessels that supply nutrition to the tumor -Cryotherapy: freezing of the tumor -Enucleation may be used to treat advanced disease with optic nerve invasion in which there is no hope for salvage of vision. -Eye is usually taken out

Legg-Calve-Perthes disease

-Aseptic avascular necrosis of femoral head -Self-limiting, idiopathic; occurs in children 2 to 12 years of age -Most common in white males 4 to 8 years of age -10% of cases: bilateral hip involvement -Most cases: delayed bone age -Cause related to temporary disturbance of circulation or vascular supply to the femoral head with ischemic avascular necrosis -The s/s can last from 18 months to several years. -The reformed femoral head may be severely altered or minimally impacted. -Bones don't grow the way they should Clinical Manifestations: -Insidious onset; possible history of limp, soreness, or stiffness; limited range of motion; vague history of trauma -Pain and limp most evident on arising and at end of activity -Diagnosed from history and findings on radiographs and magnetic resonance images (MRI) -After resolution: femoral head may be normal or have severe alteration -Femoral head will ache and won't have normal range of motion -First sign is limping Therapeutic Management: -Treatment goal: keep head of femur in acetabulum, restore and maintain adequate hip range of motion, prevent femoral head collapse, extrusion or subluxation; and preserve as well-rounded femoral head as possible at the time of healing. -Containment with various appliances and devices -Initial therapy is rest or activity restrictions and limited weight bearing, which helps reduce inflammation and irritability of the hip. -Nonsteroidal antiinflammatory drugs (NSAIDs) for pain relief -Surgery in some cases -Home traction in some cases -PT or range-of-motion exercises help restore hip motion. -Children older than 8 years old have a significant risk for degenerative arthritis

technologic management of hematologic and immunologic disorders

-Blood transfusion: •PRBCs, platelets •FFP and cryoprecipitate are other blood products •Be alert to transfusion reactions •Standard guidelines for all transfusion monitoring •Use infusion pump -Apheresis: •Removal of blood, separation of components, reinfuse portion •Used to remove and save platelets from healthy donors

hydrocephalus

-Caused by an imbalance in the production and absorption of CSF -Kids with hydrocephalus will basically be vegetables: won't ever ride a bike, go to school, tell their parents they love them, get married and have kids of their own -About 20% of kids recover and do fine Pathophysiology: -Impaired absorption of CSF within the subarachnoid space -Obstruction through the ventricular system -When it occurs: before fusion of the cranial sutures, it causes enlargement of the skull and dilation of the ventricles. -In children younger than age 12, previously closed suture lines, especially the sagittal suture, may become opened. -After 12 years of age, sutures are fused and will not open. Causes: -Result of developmental defects -Usually apparent in early infancy -Other causes include neoplasms, infection, and trauma -Often associated with myelomeningocele Therapeutic Management: -Relief of hydrocephalus -Treatment of complications -Assessment of problems related to the effects of motor development -Treatment: most often surgical •Ventriculoperitoneal shunt •Drain excess fluid •Brain ventricles are sterile •This fluid goes under the diaphragm and into the peritoneal cavity (because the peritoneal cavity is sterile) Shunt Infection: -Period of greatest risk is 1 to 2 months after shunt placement -Infections: •Septicemia •Bacterial endocarditis •Wound infection •Shunt nephritis •Meningitis -Treatment: Massive-dose antibiotics or shunt removal -Give a lot of antibiotics so infection does not reach the blood brain barrier -Shunt obstruction: headache cardinal sign of obstruction

seizure disorders

-Caused by excessive and disorderly neuronal discharges in the brain -Determined by site of origin -Most common neurologic dysfunction in children -Symptom of an underlying disease process -A seizure is defined as a "transient occurrence of signs and/or symptoms due to abnormal excessive and synchronous neuronal activity in the brain". -Seizures are the most common pediatric neurologic disorder. -About 4-10% of children will have at east one seizure by the age of 16 years old. -The manifestations of seizures depends on the region of the brain in which they originate and may include unconsciousness or altered consciousness, involuntary movements, and changes in perception, behaviors, sensations, and/or posture. -Have kids keep a journal of when they have seizures to help figure out triggers Epilepsy: -Two or more unprovoked (unexplainable) seizures -Caused by a variety of pathologic processes in the brain -Optimal treatment and prognosis: require an accurate diagnosis and determination of cause Causes of Seizures: -Acute symptomatic: •Head trauma or meningitis -Remote symptomatic: •Prior brain injury such as encephalitis or stroke -Cryptogenic: •No clear cause -Idiopathic: •Genetic in origin Seizure Classification: -Partial: •Local onset and involves a relatively small location of the brain -Generalized: •Involves both hemispheres without local onset -Unclassified Therapeutic Management of Seizure Disorders: -Goal: to control seizures or reduce the frequency and severity -Discovery and correction of the cause -Management: drug therapy -A detailed description of the seizure should be obtained from the caregiver(s) or person witnessing the seizure. -Ask questions about the child's behavior during the event, especially at the onset, and the time at which the seizure occurred (eg., early morning, while awake, while sleeping). -Any factors that may have precipitated the seizure are important including fever, infection, head trauma, anxiety, fatigue, sleep deprivation, menstrual cycle, alcohol, and activity. -Record any sensory phenomena (aura) that the child can describe and if the child was able to hear during the seizure. -The duration and progression of the seizure (if any) and the postictal feelings and behavior should be noted. -Start on one medication at the lowest dose possible and gradually increase until the seizures are controlled to the maximum recommended dosage has been achieved and seizures are still not controlled. -A second drug can be added in gradually increasing doses. -Phenytoin is often used for seizures (no drug questions about seizures on final) -Sometimes kids will outgrow their seizures, and sometimes they will not Febrile Seizures: -Transient disorder of childhood -Affect approximately 2% to 5% of children -Usually occur between ages 6 months and 3 years -Rare after age 5 years -Twice as frequent in boys -Cause: uncertain; viral infection

iron deficiency anemia

-Caused by inadequate supply of dietary iron -Generally preventable: •Iron-fortified cereals and formulas for infants (WIC programs) •Special needs of premature infants: reduced fetal iron supply •Adolescents at risk because of rapid growth and poor eating habits -Can get iron from red meat, beans, kale, raisins, some fruits Pathophysiology: -Caused by any number of factors: during last trimester, iron is transferred from mother to fetus •Adequate 5 to 6 months in full term •Only 2 to 3 months in preemies -"Milk babies": •Overweight infant because of excessive milk ingestion •2 reasons become anemic: milk (poor source of iron) and increased fecal loss -Therapeutic management: increase in the amount of iron the child receives -Prognosis: very good -Nursing Care Management: -Diet -Iron supplementation: •Adherence to oral iron supplement with appropriate administration •Liquid preparation: may temporarily stain teeth; brush teeth after admin to lessen staining If Your Child Has Iron Deficiency Anemia: -Make sure your child takes the iron supplements exactly as prescribed. -Include iron-rich foods in the family's diet. -Good sources of iron include: iron-fortified cereals, lean meat, poultry, fish, tofu, egg yolks, beans, and raisins -Serve fruits and vegetables high in vitamin C or a glass of orange juice at mealtimes. This helps the iron get absorbed. -Talk to a dietitian or your doctor if your child is a vegetarian. They can recommend foods to help your child get enough iron. -To help prevent iron-deficiency anemia in young children: •Don't give cow's milk to babies under 1 year old. •Limit cow's milk in kids over 1 year old to less than 2 cups of milk a day. •Giving them more can make them feel full and lower the amount of iron-rich foods they eat.

diabetes mellitus

-Characterized by a total or partial deficiency of the hormone insulin -The most common endocrine disorder of childhood -Peak incidence between 10 and 15 years of age -Increased risk for African-American and Hispanic children Pathophysiology: -With a deficiency of insulin, glucose is unable to enter the cell and remains in blood, causing hyperglycemia -When serum glucose exceeds renal threshold, glucose spills into urine (glycosuria) -Cells break down protein for conversion to glucose by the liver (glucogenesis) Ketoacidosis: -When glucose is unavailable for cellular metabolism, the body breaks down alternative sources of energy; ketones are released, and excess ketones are eliminated in urine (ketonuria) or by the lungs (acetone breath) -Ketones in the blood are strong acids that lower serum pH and produce ketoacidosis -Blood sugars may be 700 to 800: emergency Diabetic Ketoacidosis: -Pediatric emergency -Results from progressive deterioration with dehydration, electrolyte imbalance, acidosis, coma; may cause death -Therapy: should be instituted in an intensive care unit setting -Lower blood sugar quickly -Monitor electrolytes, give fluids Long Term Complications of Diabetes Mellitus: -Microvascular complications, especially nephropathy and retinopathy -Macrovascular disease, neuropathy -With poor control, vascular changes as early as 2.5 to 3 years after diagnosis -With excellent control, can be delayed 20 years Diagnostics: -Check risk factors -Glycosuria with or without ketonuria -Four different tests to confirm DM : •8-hour fasting blood glucose of 126 mg/dL •Random blood glucose 200 mg/dL or more •Oral glucose tolerance test of 200 mg/dL or more in 2-hour sample •Hemoglobin A1C of 6.5% or more -Three Groups of Children: •Have glycosuria, polyuria, and a history of weight loss or failure to gain despite a voracious appetite •Transient or persistent glycosuria •Metabolic acidosis with or without stupor or coma -Glycosuria, with or without ketonuria, and unexplained hyperglycemia -Normal A1C for nondiabetics: below 6 -A1C of 6.5 and above: prediabetes or diabetes Therapeutic Management: -Goal of therapy: maintain near-normal blood glucose values while avoiding too frequent episodes of hypoglycemia -Multidisciplinary approach: •Insulin therapy: cornerstone of management of type 1 DM •Multiple daily injection program reduces microvascular complications of diabetes in young, healthy patients with type 1 DM •Insulin requirements change continuously during growth and development; need varies according to child's activity and pubertal status. •Still give kids insulin even if they are vomiting •Dosage: twice-daily insulin regimen of combination of rapid-acting and intermediate-acting insulin drawn up in the same syringe and injected before breakfast and before evening meal •Continuous SQ insulin infusion: portable insulin pump •Numerous blood glucose measurements (4x/day): to adjust the insulin delivery rate •Complications: infection from the insertion site •Methods of administration -Monitoring: •Blood glucose: self monitoring is successful by children from the onset of their diabetes •Glycosylated hemoglobin •Urine: no longer used for diabetes management due to poor correlation between simultaneous glycosuria and blood glucose concentrations -Nutrition: need sufficient calories to balance daily expenditure for energy and to satisfy the requirements for growth and development •Dietary fiber increasing important - aids digestion, absorption, and metabolism; diminishes the rise in blood glucose after meals •Total caloric intake to appetite and activity -Exercise: children's activities are unplanned; therefore snack during the prolonged activity •Activity often results in reduction of insulin requirements -Hypoglycemia: early recognition and prompt relief will result in child's activity being interrupted for no more than a few minutes •Monitor for morning hypoglycemia -Goals of illness management: restore euglycemia, treat urinary ketones, a maintain hydration -Therapeutic management of diabetic ketoacidosis: fluid and electrolyte therapy Interprofessional Care Management: -Communication with team and other individuals is essential: coach, school nurse, teachers -Hospital management -Child and family education: •If hypoglycemic, can give kids milk (with a snack), cheese and crackers, PB and apples •If blood sugar below 60, need to give them sugar and follow that with protein to stable them out -Medical identification -Nature of diabetes -Meal planning -Traveling: teach parents they need to take insulin and snacks with them when they travel -Insulin: •Injection procedure •Continued subcutaneous insulin infusion •Dosage needs changes continuously during growth and development -Monitoring: urine testing -Signs of hyperglycemia -Signs of hypoglycemia -Hygiene -Exercise -Record keeping -Self-management -Child or adolescent and family support

type 1 diabetes mellitus

-Characterized by destruction of beta cells, usually leading to absolute insulin deficiency -Onset typically in childhood and adolescence but can occur at any age -Most childhood cases of diabetes mellitus are type 1 -More prominent in non-Hispanic whites, followed by African-Americans, then Hispanics; lowest percentage among Native Americans -No insulin secreted by the pancreas at all -Can occur at any age: can be born with type 1 -Nonreversible, uncurable

fractures

-Common injury in children -Methods of treatment are different in children than in adults -Rare in infants, warrants investigation -Distal forearm (radius, ulna, or both): the most frequently broken bone in childhood -Clavicle fractures are also common in children younger than 10 years of age -School age: bike-related, sports injuries Types of Fractures: -Simple or closed: does not produce a break in the skin -Open or compound: fractured bone protrudes through the skin -Complicated: bone fragments have damaged other organs or tissues -Comminuted: small fragments of bone are broken from fractured shaft and lie in surrounding tissue -Plastic deformation -Buckle -Greenstick -Complete -Complete with periosteal hinge Bone Healing and Remodeling: -Typically rapid healing in children -Neonatal period: 2 to 3 weeks -Early childhood: 4 weeks -Later childhood: 6 to 8 weeks -Adolescence: 8 to 12 weeks -Bone healing is rapid in growing children because of the thickened periosteum and generous blood supply. -New bone cells are formed in immense numbers almost immediately after the injury. -Fractures heal in less time in children than in adults. Diagnosing Fractures: -Radiographs -History taking -Suspicion of fracture in a young child who refuses to walk or bear weight Goals of Fracture Management: -To regain alignment and length of the bony fragments (reduction) -To retain alignment and length (immobilization) -To restore function to the injured parts -To prevent further injury and deformity Treatment: -Fractures are splinted or casted to immobilize and protect the injured extremity. -Children with displaced fractures may have immediate surgical reduction and fixation (internal or external) rather than immobilization by traction. -This practice is more common and holds true for all types of fractures, including femur fractures Assessment of Fractures (The Six Ps): -Assess for possible compartment syndrome -Pain: severe pain that is not relieved by analgesics or elevation of the limb, movement that increases pain -Pulselessness: inability to palpate a pulse distal to the fracture or compartment -Pallor: pale appearing skin, poor perfusion, capillary refill greater than 3 seconds -Paresthesia: tingling or burning sensation; sensation distal to the fracture site -Paralysis: inability to move extremity or digits; distal to the fracture site -Pressure: involved limb or digit may feel tense and warm, skin is tight and shiny; pressure within the compartment is elevated Cast Care: -Elevate casted extremity for first day -Observe the extremities (fingers/toes) for swelling, discoloration -Check movement and sensation of fingers/toes -Do not allow child to put anything inside the cast -Cool hair dryer can help with itching

hematologic and immunologic dysfunction assessment

-Complete blood cell count -History and physical examination -Comments by the parent regarding child's lack of energy -Food diary of poor iron sources -Frequent infections -Bleeding that is difficult to control -Examine skin for pallor, petechiae, and bruising

juvenile hypothyroidism

-Congenital: •Congenital hypoplastic thyroid gland -Acquired: •Partial or complete thyroidectomy for cancer or thyrotoxicosis •After radiation therapy for Hodgkin lymphoma or other malignancy •Rarely occurs from dietary insufficiency in the United States Clinical Manifestations: -Mental decline: if left untreated -Constipation -Sleepiness -Myxedematous skin changes: •Dry skin •Sparse hair •Puffiness around eyes -Because brain growth is nearly complete by 2 to 3 years of age, intellectual disability and neurologic impairment does not occur. -Children treated early continue to have mild delays in reading, comprehension, and arithmetic but catch up. -However, adolescents may have problems with memory, attention, and visuospatial processing. -Mental decline: can't think properly, start to not make sense, basically go backwards developmentally Therapeutic Management: -Oral thyroid hormone replacement -Prompt treatment needed for brain growth in infant -May administer in increasing amounts over 4 to 8 weeks to avoid symptoms of hyperthyroidism -Compliance with medication regimen: crucial -Children to take responsibility for medication regimen as soon as they are able

precocious puberty

-Defined as sexual development before age 9 years in boys or before age 8 years in girls -Mean onset of puberty 10.2 years Caucasian girls and 9.6 years African American girls -Evaluation for pathologic cause: required in white girls younger than 7 years and African-American girls younger than 6 years -Age limit for defining when puberty is precocious in girls is occurring earlier than previous studies have documented -Almost always occurs in girls rather than in boys -Secondary sex characteristics develop younger, as do the emotions and mindset -Look at epiphyseal plates to diagnose Therapeutic Management: -Treatment of specific cause if known -May be treated with leuprolide (Lupron): •Give once every 4 to 12 weeks depending on the preparation •Slows prepubertal growth to normal rates •Treatment is discontinued at age when normal pubertal changes are expected to resume -Psychologic support for child and family -Dress and activities need to be appropriate for child's chronological age

cerebral palsy

-Definition: a group of permanent disorders of the development of movement and postures, causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain -Characterized by abnormal muscle tone and coordination -Incidence: 2.4 to 3.6 per 1000 live births -Most common permanent physical disability in childhood -Affected children: 15% to 60% also have epilepsy Causes: -Prenatal brain abnormalities -Gross abnormalities of the brain -Vascular occlusion -Laminar degeneration -Effects of low birth weight -Anoxia (most common cause) -Hypoxic infarction or hemorrhage Diagnostics: -Infants at risk: require careful assessment during early infancy -Neurologic examination and history: primary means of diagnoses -Neuroimaging -Metabolic and genetic testing -Very often, cerebral palsy won't show up until 6 months or older because kids are not reaching their developmental milestones Clinical Manifestations: -Delayed gross motor development -Abnormal motor performance -Alterations in muscle tone: can be either very flaccid or hypertonic -Abnormal postures -Reflex abnormalities: may not have reflexes at all -Associated disabilities -A lot of these kids have movements they cannot control -Many of these kids are cognitively normal -In the most severe cases, kids are wheelchair bound Goals of Therapy: -To establish locomotion, communication, and self-help skills -To achieve optimal appearance and integration of motor functions -To correct associated defects as effectively as possible -To provide educational opportunities adapted to the child's capabilities -To promote socialization experiences Therapeutic Management: -Ankle-foot braces: may be worn -Orthopedic surgery: to correct spastic deformities •Tendon-lengthening procedures, release of spastic muscles, and correction of hip and adductor muscle spasticity or contracture to improve locomotion -Pharmacologic agents: to treat pain related to spasms and seizures -Botulinum toxin A injections (Botox): used to reduce spasticity in targeted muscles. The drug inhibits the release of acetylcholine thus reducing spasticity -Dental hygiene: especially important for those on phenytoin due to possible gingival hyperplasia -Physical/ occupational therapy -CBD may be a treatment Prognosis: -Depends on type and severity of the condition -Moderate impairment: 85% of patients can achieve ambulation -Cognitive impairments: in 30% to 50% -Growth affected -Survival dependent on comorbid conditions

disorders of pituitary function

-Disorders may be due to organic defects or have an idiopathic cause -Problem may involve a single hormone or a combination of several hormonal deficiencies -Clinical manifestations depend upon the hormone involved -Disorder may result in an overproduction or hormone deficiency -Can be a defect, obstruction, or mass

levels of consciousness

-Earliest indicator of changes in neurological status -LOC is determined by observations of the child's responses to the environment. -Parents may be helpful during assessment as children may not respond in an unfamiliar environment or to unfamiliar voices -Motor activity, reflexes, and vital signs are not parallel to the depth of comatose state -In descending order: •Full consciousness •Confusion: impaired decision-making •Disorientation: to time and place •Lethargy: sluggish speech

pituitary hyperfunction

-Excess GH before closure of epiphyseal shafts results in overgrowth of long bones -Patients can reach heights of 8 feet or more before closure of epiphyseal shafts -Vertical growth is accompanied by increased muscle -Weight is generally in proportion to height -Delayed closure of fontanels -Acromegaly: very tall -Kids with this disorder don't get overweight or underweight: generally proportioned -Excess GH after epiphyseal closure is called "acromegaly"; more common in boys -Typically, several facial features undergo overgrowth: •Head •Lips, tongue, jaw, nose •Paranasal, mastoid sinuses •Separation and malocclusion of the teeth -Kids with this disorder are often clumsy and not coordinated -After closure of epiphyseal shafts, growth is in transverse direction -Increased risk for DM -Develops slowly leading to delay of dx and tx Diagnostics: -History of excessive growth during childhood -Evidence of increased levels of GH -Radiographic studies: may reveal a tumor -Endocrine studies -Epiphyseal plates, precocious puberty, and pituitary tumor can help to diagnose Therapeutic Management: -Surgical treatment to remove tumor -Radiation and radioactive implants usually done before surgery to make tumor smaller and easier to remove -Hormone replacement therapy after surgery in some cases: •Thyroid extract •Cortisone •Sex hormones Care Management: -Early identification of children with excessive growth rates (a headache may be a sign of a tumor) -Early treatment for improved outcomes -Observation for signs of tumor -Emotional support -Addressing body image concerns (support group)

developmental dysplasia of the hip

-Formerly called "congenital hip dysplasia" or "congenital dislocation of the hip" -A spectrum of disorders related to abnormal development of the hip that may occur at any time during fetal life, infancy or childhood -Infant issue, often caused by a breached delivery or not enough amniotic fluid -Incidence: 1.5 per 1000 live births -Girls: more commonly affected -Strong family history Types of DDH: -Acetabular dysplasia: the mildest form of DDH, in which there is a delay in acetabular development evidenced by osseous hypoplasia of the acetabular roof that is oblique and shallow. The femoral head remains in the acetabulum. -Sublaxation: the largest percentage of DDH, incomplete dislocation of the hip. The femoral head remains in contact with the acetabulum, but a stretched capsule and ligamentun teres causes the head of the femur to be partially displaced. Pressure on the cartilaginous roof inhibits ossification and produces a flattening of the socket. -Dislocation: the femoral had loses contact with the acetabulum and is displaced posteriorly and superiorly over the fibrocartilaginous rim. The ligamentum teres is elongated and taut. Clinical Manifestations (in infants): -Hip joint laxity -Shortened limb on affected side -Restricted abduction of hip on affected side -Unequal gluteal folds when infant prone -Positive Ortolani test result: hip is reduced by abduction -Positive Barlow test result: hip is dislocated by adduction -Both tests are most reliable during the first 4 weeks of life. Therapeutic Management: -Importance of early intervention -Birth to age 6 months: Pavlik harness for abduction of hip -Ages 6 to 24 months: dislocation unrecognized until child begins to stand and walk; closed reduction and spica cast -Older child: operative reduction, tenotomy (surgical cutting of a tendon), osteotomy; correction is very difficult after age 4 years Pavlik Harness: -Skin care is an important aspect of care of an infant in a harness. -Check frequently (2-3x/day) for red areas or skin irritation in skin folds and under the straps -Gently massage healthy skin under the straps once per day to stimulate circulation -Always place diapers under the straps -Remove harness only to quickly bathe the infant if allowed by HCP -Do not adjust the harness -Always make sure kids are fully clothed to help prevent skin breakdown -Usually in harness 4-9 months depending on severity of condition

juvenile idiopathic arthritis

-Formerly known as "juvenile rheumatoid arthritis" -Also called "idiopathic arthritis of childhood" -Autoimmune disease: inflammation in the joint synovium and surrounding tissue with eventual erosion, destruction, and fibrosis of the articular cartilage. -Starts before age 16 years -Can affect one or multiple joints -Incredibly painful -Possible causes: •Immunogenic susceptibility •Environmental trigger -Peak onset: 1 to 3 years of age -Often undiagnosed -Systemic onset (high fever, rash, hepatosplenomegaly, pericarditis, pleuritis, lymphadenopathy): •Oligoarthritic (involves ≤4 joints) •Polyarthritic (involves ≥5 joints) •Psoriatic (enthesitis and undifferentiated, worst kind) -Affected children: 90% have negative rheumatic factor -Symptoms: may "burn out" and become inactive -Chronic inflammation of synovium with joint effusion, destruction of cartilage, and ankylosis of joints as disease progresses -May go away Diagnostics: -No definitive diagnostic tests -X-rays are the best initial imaging studies and may show soft-tissue swelling and joint space widening from increased synovial fluid in the joint -Elevated sedimentation rate in some cases (important, tells how much inflammation there is) -Antinuclear antibodies common, but not specific for juvenile idiopathic arthritis -Leukocytosis during exacerbations -Diagnosis based on criteria of American College of Rheumatology Therapeutic Management: -No specific cure -Goals of therapy: •Preserve function •Prevent deformities •Relieve symptoms and pain -Primarily outpatient care -Nonsteroidal antiinflammatory drugs: ibuprofen and naproxen -Disease-modifying antirheumatic drugs: methotrexate -Biologic disease-modifying antirheumatic drugs: tumor necrosis factor inhibitors such as Humira and Remicade -Glucocorticoids: during acutely active symptoms; have significant adverse effects -Physical therapy, occupational therapy -Promotion of general health -Facilitating compliance -Encouraging the use of heat and exercise -Support child and family -Goal is to prevent joints from becoming deformed and locked

coma assessment

-Glasgow Coma Scale (GCS): •Eye, verbal, and motor response -Decrease in GCS score indicates deterioration -Brain death requires: •Complete cessation of brain function •Irreversibility of condition -A score of 8 or below is generally accepted as a definition of coma. -A 3 indicates deep coma.

hyperthyroidism (Graves' disease)

-Graves disease constitutes largest percentage of cases of childhood hyperthyroidism -It is believed to be caused by autoimmune response to TSH receptors -Signs include enlarged thyroid gland and exophthalmos -Incidence peaks between ages 12 to 14 years, but condition may be present at birth -Familial association exists Therapeutic Management: -Therapy: controversial •Goal of therapy: to retard rate of hormone secretion -Treatments: •Antithyroid drugs •Subtotal thyroidectomy •Ablation with radioiodine: radioactive iodine ablation (the surgical removal of body tissue) usually effective but slower; concern about link to thyroid cancer Care Management: -Identify children with hyperthyroidism -Be alert for signs and symptoms -Child needs quiet environment, rest periods -Help family cope with emotional lability -Dietary requirements are higher to meet child's increased metabolic rate -Medications have side effects

hypopituitarism

-Growth hormone deficiency -Deficient secretion of pituitary hormones -Inhibits somatic growth and development of secondary sex characteristics -Consequences: •Depend upon the degree of dysfunction •Gonadotropin deficiency •Growth hormone (GH) deficiency •Thyroid-stimulating hormone (TSH) deficiency •Corticotropin deficiency Clinical Manifestations: -Normal growth during first year -Slowed growth curve after first year -Appear overweight due to stunted height -Delayed sexual development Diagnostics: -Family history: a lot of endocrine problems are familial or genetic in some form -Growth patterns and previous health status -Physical examination -Psychosocial evaluations -Radiographic survey -Endocrine studies -Only a small number of children with delayed growth or short stature have hypopituitary dysfunction -Accurate height and weight; comparison with standard growth charts are essential. -Definitive dx is based on absent or subnormal reserves of pituitary GH. -Poor linear growth, delayed bone age, and abnormal GH stimulation tests are considered GH deficient. Therapeutic Management: -Treatment is directed toward correction of the underlying disease process -Replacement with GH is successful in 80% of affected children -Therapy is ended when growth rates are less than 1 inch/year: •Girls: at 14 years of age •Boys: at 16 years of age Care Management: -Identifying and assisting with the diagnosis -Child and family support -Emotional adjustment of the child -Preparation for testing and medication administration -GH administered SQ at bedtime: mirrors physiological release of GH during first 45 to 90 minutes after onset of sleep -Providing increased attention to child during testing

osteomyelitis

-Infectious process in the bone -The limbs most commonly affected include the foot, femur, tibia, and pelvis -Can occur at any age but most frequently is seen in children 10 years of age or younger. -Boys are more commonly affected than girls and the median age is 5 to 6 years of age -Sometimes dismissed as "growing pains" -May be caused by exogenous or hematogenous sources: •Staphylococcus aureus: the most common causative organism -Types of osteomyelitis: •Acute hematogenous: results when a blood borne bacterium causes an infection in the bone. Common foci include infected lesions, URI, otitis media, tonsillitis, abscessed teeth, pyelonephritis, and infected burns. •Exogenous: is acquired from direct inoculation of the bone from a puncture wound, open fracture, surgical contamination, or adjacent tissue infection. •Subacute: has a longer course and may be caused by less virulent microbes with a walled off abscess; typically seen in the tibia •Chronic: a progression of acute osteomyelitis and is characterized by dead bone, bone loss, and drainage and sinus tracts Clinical Manifestations: -Signs/symptoms begin abruptly, resemble symptoms of arthritis and leukemia -Marked leukocytosis -Bacteria adhere to bone, causing a supportive infection with inflammatory cells, edema, vascular congestion, and small-vessel thrombosis; the result is bone destruction, abscess formation, and dead bone. -Can cause bone necrosis -Bone cultures obtained from biopsy or aspirate -Radiographs: may appear normal at first -Bone scans for diagnosis Therapeutic and Care Management: -Prompt, vigorous intravenous antibiotics for extended period (3 to 4 weeks or up to several months): •Typically nafcillin or clindamycin -Monitor hematologic, renal, hepatic responses to treatment -Complete bed rest and immobility of limb -Pain management concerns -Long-term intravenous access (for antibiotic administration) -Long-term hospitalization/ therapy

soft tissue injury

-Injuries to the muscles, ligaments, and tendons are common in children. -In young children: they are usually a result from a mishap while paying. -In older children and adolescents: participation in sports is a common cause of these injuries. Therapeutic Management: RICE and ICES: •Rest the injured part •Ice immediately (max. 30 minutes at a time) •Compression with wet elastic bandage •Elevation of the extremity •Immobilization and support (casts or splints as appropriate to injury)

neural tube defects

-Largest group of congenital anomalies -Normally: the spinal cord and cauda are encased in a protective sheath of bone and meninges -Failure of neural tube closure: produces defects of varying degrees -Incidence: •0.3 per 1000 births •Occurs more often in white and Hispanic children Spina Bifida: -Failure of the neural tube to close during the embryo's early development (approximately 3-4 weeks after conception) -Multifactorial causes -Genetic mutation -Additional factors: •Maternal obesity •Maternal diabetes mellitus •Low vitamin B12 level •Can also be caused by low folic acid -Types: •Spina bifida occulta •Meningocele •Myelomeningocele: can be with intact sac or ruptured sac, ruptured sac more serious •Cover sac with saline and gauze: keep it sterile, wet, and warm; bags often used as well Therapeutic Management of Neural Tube Defects: -Postnatal care -Orthopedic considerations: DDH, clubfeet, scoliosis -Management of genitourinary function: •Latex allergy -Bowel control -Prevent trauma to membranous cyst -Assess for level of neuro involvement: hydrocephalus common -Monitor urinary output; neuropathic (neurogenic) bladder dysfunction -Use of incubator or warmer to maintain temp -Observe for early signs of infection, temp instability, irritability, and lethargy. -Prevent drying of the sac -Change dressings frequently -Favorable outcomes if closed within the first 24 to 72 hours after birth -Position kids very specifically to keep them comfortable and keep them from moving around too much both before and after surgery -These kids don't have bowel and bladder control, so they need to be cathed every 4 hours

non-Hodgkin lymphoma

-More prevalent among children less than 14 years of age -Clinical appearance: •Disease usually diffuse rather than nodular •Classification is based on the cell type •Mediastinal involvement and invasion of meninges -Clinical manifestations: •Depends on anatomic site involved •Metastasis to bone marrow or CNS may appear like leukemia -Diagnostic evaluation: •Surgical biopsy for confirmation of disease •Bone marrow aspiration •Radiologic studies, CT scans of the lungs and GI -Prognosis improved with young age, low mediastinal involvement, low tumor burden, and good response to initial therapy. -The use of aggressive combination of radiation and chemotherapy has a major impact on the survival rate. -The most effective treatment regimens result in cure in 85-95% of children with limited disease involvement, and 70-90% of children with extensive disease are cured -Conscious sedation: patient is awake but they don't feel what you are doing; have to do neuro assessments often

down syndrome

-Most common chromosome abnormality: 1 per 691 to 1000 live births -Causes unknown; probably multiple -Occurs in populations of all races Etiology: -Nonfamilial trisomy 21: •Extra chromosome 21 in 95% of cases •Translocation of chromosomes 15 and either 21 or 22 in 3% to 4% of cases •Mosaicism, a mixture of abnormal and normal cells, in 1% to 2% of cases -Maternal age: •Age 35: Risk is 1 per 350 births •Age 40: Risk is 1 per 100 births Clinical Manifestations: -Square head with upward slant to eyes -Flat nasal bridge, protruding tongue -Hypotonia (decreased muscle tone) -Congenital heart disease (CHD): ASD, VSD are most common -Ears are often slanted -Simian creases common on both palms -Hypothyroidism -Leukemia -Decreased muscle tone compromises respiratory expansion -Nasal bone is underdeveloped therefore inadequate drainage of mucus -Protruding tongue interferes with feeding -Decreased muscle tone affects gastric motility, predisposed to constipation Diagnostics: -Chromosome analysis Therapeutic Management: -Available therapies: •Surgery to correct congenital anomalies •Evaluation of hearing and sight •Periodic testing of thyroid function -Prognosis: lower than the general population; survive to 60 years and beyond -Care management: •Supporting child's family at time of diagnosis •Preventing of physical problems •Assist in prenatal diagnosis and genetic counseling •Provide for long term health care and social and leisure needs

neuroblastoma

-Most common extracranial solid tumor -Tumor is on the outside, so you can see it -Most common cancer diagnosed in infancy -Primary site is in abdomen -Median age of diagnosis is 19 months -Originates from the embryonic neural crest cells that normally give rise to the adrenal medulla and the sympathetic nervous system. -Therefore, most tumors arise from the adrenal gland or from the retroperitoneal sympathetic chain -The primary site is within the abdomen; other sites include the head and neck region, chest and pelvis -Neuroblastoma is a "silent" tumor. In more than 70% of cases, diagnosis is made after metastasis occurs, with the first signs cause by involvement in the nonprimary site, usually the lymph nodes, bone marrow, skeletal system, or liver Clinical Manifestations: -Depend on location and stage -Firm, non-tender, irregular mass in the abdomen -Neurological impairment -Respiratory obstruction -Paralysis -Periorbital edema -Exophthalmos -Supraorbital ecchymosis Diagnostics: -CT scan of abdomen, pelvis or chest to located primary tumor -Bone scan to evaluate for skeletal metastasis -Bone marrow biopsies -Staging and prognosis: •Diagnosis often made after metastasis occurs •Age and stage of disease impacts prognosis •Younger age = more likely survival •Because of the frequency of invasiveness, the prognosis is generally poor: 80% for children younger than 1 year of age, less than 50% for children older than 1 year of age -Therapeutic management: •Surgery to remove as much as possible •Irradiation postoperatively •Chemotherapy

Wilms' tumor

-Most common kidney tumor of childhood -AKA nephroblastoma. -75% of cases are diagnosed when the child is younger than 5 years old. -Peak incidence between the ages of 2 and 3. Clinical Manifestations: -Painless swelling or mass in abdomen -Firm, nontender, confined to one side, deep within flank area -Often discovered during routine bathing/dressing -If the tumor ruptures, it can send cancer cells throughout the abdomen, lymph channel, and bloodstream Diagnostic Evaluation: -History and physical exam: •Looking for the presence of congenital anomalies; a family hx of cancer; signs of malignancy (weight loss, enlarged liver and spleen, indications of anemia, and enlarged lymph nodes) -Signs of malignancy -Ultrasound, CT (to look for metastases in the lungs), MRI, CBC, urinalysis Therapeutic Management: -Surgery and chemotherapy -May or may not have radiation -In surgery, the tumor, affected kidney, and adjacent adrenal gland are removed -Radiation and chemo usually started right after surgery Preoperative Care: -Surgery usually occurs within 24 to 48 hours of diagnosis -Do not palpate tumor— careful bathing and handling of child important -Tell parents what to expect; keep terms simple -Teach parents to never press on the tummy

rhabdomyosarcoma

-Most common soft-tissue sarcoma in children -Most common in children 9 years and under -Most often around the eye, visible skin tissue -Not seen in adults and usually not seen in adolescents -Found in striated (rhabdo) muscle throughout the body: the most common for this cancer is head and neck, especially the eye orbit (thus the myo, meaning eye, in the name) -The tumor tends to compress the adjacent organs -Multimodal therapy demonstrates high survival in non-metastatic disease (more than 60%) -Complete removal of primary tumor whenever possible -Chemotherapy effective and radiation may also be used Clinical Manifestations: -Signs and symptoms related to site of tumor and compression of organs -Earache or runny nose -May cause personality changes -If tumor bursts open, may cause death Diagnostic Evaluation: -History and physical examination -PET/CT or MRI scans -Biopsies -LP

brain tumors

-Most common solid tumor in children -Specific cells may be involved -Benign or malignant -Signs and symptoms are related to anatomic location and size: •Hydrocephalus •Headache •Vomiting •Nystagmus •Ataxia •Dysarthria •Diabetes insipidus •Growth failure Therapeutic Management: -Surgery, radiotherapy, chemotherapy -Treatment of choice is removal of tumor without neurological damage -Radiation to shrink tumor before removal -Chemotherapy alone or in combination

idiopathic scoliosis

-Most common spinal deformity -Complex spinal deformity in three planes: •Lateral curvature •Spinal rotation, causing rib asymmetry •Thoracic hypokyphosis -May be congenital or develop during childhood -Multiple potential causes; most cases idiopathic -Generally becomes noticeable after preadolescent growth spurt -Clothes: may be "ill-fitting" -School screening: controversial Diagnostics: -Standing radiographs to determine degree of curvature -Asymmetry of shoulder height, scapular or flank shape, or hip height -In addition to primary curve, compensatory curve often present to align head with gluteal cleft -X-rays may also be done Therapeutic Management: -Team approach to treatment -Bracing: has been found to be ineffective if curvature is greater than 45 degrees. -Exercise -Surgical intervention for severe curvature (instrumentation and fusion): •Harrington rods •Luque rods •When rods are implanted, spinal fusion also takes place. •Very painful surgery: usually discharged after 5-7 days . •Will require PT and may also require a brace. -Concerns of body image -Concerns of prolonged treatment of condition -Preoperative care -Postoperative care: log-rolling when changing positions -Family issues

dislocation

-Occurs when the force of stress on a ligament, that is holding two bones or a bone and socket together, is so great as to displace the normal position of opposing bone ends or of bone ends to socket -Occurs when force of stress on ligament is sufficient to cause displacement -Pain (predominant symptom): increases with active or passive movement of affected extremity -The reason may be, but not always, an obvious deformity and inability to move the joint. -More common in children with Down syndrome (naturally lax joints) -Dislocation of the phalanges is the most common type seen in children followed by elbow dislocation. -In adolescents, shoulder dislocations are more common and dislocation unaccompanied by fracture is rare. -Depending on the location of the dislocation and the age of the child, immobilization may be required with a sling (especially for shoulder dislocation). -In children younger than 5 years of age, the hip can be dislocated by a fall. The greatest risk after this injury is the potential loss of blood supply to the head of the femur. -Relocation of the hip within 60 minutes after the injury provides the best chance for prevention of damage to femoral head. -Simple dislocations should be reduced as soon as possible -An unreduced dislocation may be complicated by increased swelling, making reduction difficult and increasing the risk for neurovascular problems.

effects of immobilization

-One of the most difficult aspects of illness in a child is immobility -Frequent reasons for immobility: •Congenital defects •Degenerative disorders •Infections or injuries that impair integumentary, neurologic, or musculoskeletal systems •Therapies that prolong immobilization -Children's tendency to be active influences all aspects of their G & D. -Impaired mobility presents a challenge to children, their families, and their caregivers. -Muscular system: decreased muscle strength, tone and endurance; muscle atrophy -Respiratory system: decreased need for oxygen and decreased chest expansion and diminished vital capacity -Immobilization also effects the child psychologically. -When children are immobilized by disease or as part of a treatment regimen, they experience diminished environmental stimuli with a loss of tactile input and altered perception of themselves and their environment. -The quest of mastery for every stage of development is related to mobility. The monotony of immobility can lead to sluggish intellectual and psychomotor responses. -Children are likely to become depressed over their loss of ability to function. -It is very difficult for kids to understand why they can't move

bone tumors (osteosarcoma)

-Osteosarcoma is most common type of bone tumor -Very painful -Not always caught right away because it's thought to be "growing pains" in their bones -More common in legs, ankles, knees -Likely arises from bone-forming mesenchyme -Found mostly in lower extremities near growth plate Clinical Manifestations: -Localized pain at affected site (severe/dull sometimes attributed to growing pains) -Limping, decreased physical activity, unable to hold heavy objects -Palpable mass -Fever -Respiratory distress Diagnosis: -Thorough history and physical -Rule out trauma or infection -Functional status of affected area -Signs of inflammation -Size of mass -Anemia -Weight loss -Infections -X-ray, CT, MRI -Biopsy Therapeutic Management: -Surgery and chemotherapy -Surgery for biopsy and to save or amputate limb -Limb salvage includes prosthetic bone placement -Chemotherapy to decrease tumor size Care Management: -Depends on surgical approach (amputation vs. salvage) -Prepare family for surgical intervention and outcome -Allow child to ask questions; expect grief response Prognosis: -Most can be cured

nursing care of the unconscious child

-Outcome and recovery of unconscious child may depend on level of nursing care and observational skills -Emergency management: •Airway •Reduction of ICP •Treatment of shock -Osmotic diuretics for cerebral edema: Mannitol -Be very careful with these kids: how you move them, what you do with them -Passive range of motion can help

disorders of parathyroid function

-Parathyroid glands secrete parathormone (PTH) -Function of PTH is to maintain serum calcium level by: •Increasing release of calcium and phosphate from bone demineralization •Increasing absorption of calcium and excretion of phosphate by the kidneys •Promote calcium absorption in GI tract

sprain vs. strain

Sprain: -Trauma to a joint from a ligament partially or completely torn or stretched by force -May be associated with damage to blood vessels, muscles, tendons, and nerves -Presence of joint laxity as indicator of severity -Rapid onset of swelling with disability Strain: -A microscopic tear to musculotendinous unit -Similar to sprain -Swollen, painful to touch -Generally incurred over time

epistaxis (nosebleed)

-Isolated and transient epistaxis is common in childhood -Recurrent or severe episodes may indicate underlying disease -Vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (von Willebrand disease and hemophilia) Care Management: -Remain calm; keep child calm -Bleeding usually stops within 10 minutes after nasal pressure -Have child sit up and lean forward -Apply pressure to the soft lower part of the nose -Evaluate further if bleeding continues

HIV/AIDS

-Life-threatening change in immune system -HIV/AIDS constitute one the world's most costly and serious medical, public health, and social challenges of our time. -Improved diagnosis and treatments increase life expectancy, making HIV/AIDS a chronic condition rather than deadly -Although kids with HIV positive moms are usually born HIV positive, they often become HIV negative because the babies make their own blood -HIV moms are able to breastfeed: fix their milk so that it does not have HIV in it -Moms can't feed straight from the breast, but it is still their milk -Epidemiology: principle modes of transmission in pediatric population are mother to child and adolescent risky behavior (sexual activity and IV drug use) -HAART therapy (Highly Active AntiRetroviral Therapy) prevents perinatal transmission; given to mothers during prenatal period -Etiology: found in blood, semen, vaginal secretions, anal secretions, breast milk; transmitted by lymphocytes and monocytes with an incubation or latency period of months to years -Pathophysiology: •Virus takes over CD4+T lymphocytes •CD4+T cell count drops, increasing risk of infections -Manifestations: •Malnutrition, short stature, cardiomyopathy •Enlarged lymph nodes and spleen, oral candidiasis -Diagnosis: •Infants born to HIV + mothers will test positive because of the presence of maternal antibodies derived transplacentally •Maternal antibodies may persist for up to 18 months in the infant; therefore, other diagnostic tests are used - most commonly polymerase chain reaction (PCR) •Early testing using recombinase polymerase amplification (RPA); a faster test that only takes 20 minutes for results •CDC classification system: system indicates severity of S/S and the degree of immunosuppression. Prognosis: -New combination medications decrease death rate -Early recognition decreases complications -NO CURE Care Management: -Education: transmission and control, standard precautions -Prevention counseling for adolescents -Pain management: due to infections; adverse effects of meds; encephalopathy; deep musculoskeletal pain; various procedures (venipunctures, lumbar punctures, biopsies, endoscopies) -Look at psychosocial aspects: stigma -Confidentiality

sickle cell anemia

-Partial or complete replacement of normal Hgb with abnormal HbS -Defect is inherited -Newborns with SCA are generally asymptomatic because of the protective effect from the mother -Rapidly decreases during the first year, then child manifests symptoms Causes: -Autosomal recessive disorder: •9% of African-Americans are carriers (have sickle cell trait) •40% of native Africans are carriers •If both parents have the trait, each of their children has a 25% chance of having the disease -In areas of the world where malaria is common, individuals with sickle cell trait tend to have a survival advantage over those without the trait Clinical Manifestations: -Obstruction caused by sickled RBCs -Vascular inflammation -Increased RBC destruction -Abnormal adhesion, entanglement, and meshing of rigid sickle-shaped cells -Local hypoxia -Cellular death -Dactylitis is inflammation of a digit (either finger or toe) and is derived from the Greek word dactylos meaning finger. The affected fingers and toes swell up into a sausage shape and can become painful. -Priapism is a prolonged erection of the penis. The persistent erection continues hours beyond or isn't caused by sexual stimulation. -Priapism is usually painful. Although priapism is an uncommon condition overall, it occurs commonly in certain groups, such as people who have sickle cell anemia. -Diagnosis: universal screening of newborns in the United States Management: -Prevent sickling -Rest and minimize energy expenditure -Hydration -Electrolyte replacement: look at sodium, potassium, and magnesium (muscle spasms) values -Analgesia -Blood replacement -Antibiotics -Oxygenation -Pain management Prognosis: -No cure (except possibly bone marrow transplantation) -Supportive care/prevention of sickling episodes -Frequent bacterial infections because of immunocompromise -Bacterial infection: leading cause of death in young children with sickle cell disease -Usual life span: into the fifth decade Nursing Management: -Seek early intervention for problems -Given penicillin as ordered: penicillin prophylaxis significantly reduces the risk for pneumococcal infection in children with SCD; started at 2 months of age -Recognize signs of stroke and respiratory problems -Treat child normally -Prevent sickling Sickle Cell Crisis: -Anything that increases body's need for oxygen or alters transport of oxygen -Trauma -Infection, fever -Physical and emotional stress -Increased blood viscosity caused by dehydration -Hypoxia Vaso-Occlusive Crisis (VOC): -Preferably called a "painful episode" -Characterized by ischemia causing mild to severe pain -Lasts from minutes to days or longer Sequestration Crisis: -Pooling of a large amount of blood -Usually in the spleen and infrequently in the liver -Causes decreased blood volume and ultimately shock -The spleen or liver can explode, leading to internal bleeding -Emergency: kids will go to surgery quickly Therapeutic Management of Sickle Cell Crisis: -Treat the medical emergencies of sickle cell crisis -Rest to minimize energy loss -Hydration through oral or IV therapy -Electrolyte replacement: due to dehydration -Analgesia for pain (children tend to be undermedicated): •VOC initially mild to moderate pain: nonsteroidal antiinflamatory (ibuprofen), acetaminophem •Severe pain: opioids such as morphine and oxycodone; administration either IV or PO and given around the clock; PCA has been used successfully, too -Blood replacement for anemia -Antibiotics for infection: possible prophylactic antibiotics for 2 months to 5 years -Monitoring of reticulocyte count regularly to evaluate bone marrow function: reticulocyte count tells you how well the body is making new red blood cells -Blood transfusion: if given early in crisis, may reduce ischemia

fragile X syndrome

-Second most common genetic cause of cognitive impairment after Down syndrome -1 per 3600 boys -1 per 4000 to 6000 girls -Caused by abnormal gene on the lower end of the long arm of the X chromosome Clinical Manifestations: -Large head circumference -Long face with a prominent jaw (prognathism) -Large, protruding ears -Large testes (postpubertal) -Mild-to-severe cognitive impairment -Delayed speech and language -Hyperactivity -Hypersensitivity to taste, sounds, and touch -Autistic-like behaviors -Aggressive behaviors -Don't like bright lights -Don't like to be touched or hugged -Not nice kids: hit, bite, etc. -Hard to handle Therapeutic Management: -Tegretol/Prozac for behavioral control -Stimulants for hyperactivity (similar to management of attention deficit/ hyperactivity disorder) -Referral to early intervention program -Prognosis: normal life span expected -Care management: genetic counseling

diabetes insipidus

-The principal disorder of the posterior pituitary gland -Results from hyposecretion of antidiuretic hormone (ADH) -Produces uncontrolled diuresis -Primary causes: familial or idiopathic -Secondary causes: trauma, tumors, central nervous system infection, aneurysm -Kind of idiopathic Clinical Manifestations: -Cardinal signs: polyuria and polydipsia -Infants: irritability relieved with feedings of water but not milk; dehydration often occurs -Small children require close observation during fluid deprivation to prevent them from drinking, even toilet bowls, flower vases, and other unlikely sources of fluid -Thirst never goes away -Diagnosed by reducing fluid intake with little or no effect on urine output Therapeutic Management: -Instruct parents in difference between diabetes insipidus and diabetes mellitus -Daily hormone replacement -Drug of choice: Vasopressin (DDAVP) •Nasal spray or intravenous administration •Subcutaneous or intramuscular injection •Treatment required for life •Alleviates polyuria and polydipsia

thyrotoxicosis

-Thyroid "crisis" or "storm" -May occur from sudden release of thyroid hormone -Unusual in children but can be life-threatening -May be precipitated by infection, surgery, or discontinuation of antithyroid therapy

disorders of thyroid function

-Thyroid hormone regulates basal metabolic rate -Thyroid secretes two types of hormones: •Thyroid hormone, which is made up of Thyroxin (T4) and Triiodothyronine (T3) •Calcitonin -Patients may have hypothyroidism or hyperthyroidism -Patients may have disturbance in secretion of TSH -Most common endocrine problems of childhood

growth plate injuries

-Weakest point of long bones: the cartilage growth plate (epiphyseal plate) -Frequent site of damage during trauma -May affect future bone growth -Treatment: may include open reduction and internal fixation to prevent growth disturbances

increased intracranial pressure (ICP)

-The brain is well protected but vulnerable to pressure that may accumulate within the cranium -As pressure increases, signs and symptoms become more pronounced, and level of consciousness (LOC) deteriorates -Early signs and symptoms may be subtle -The total volume of the cranium: brain (80%), cerebrospinal fluid (10%), and blood (10%); must remain approximately the same at all times. -A change in the proportional volume of one of these components must be accompanied by a compensatory change in another. -Children with open fontanels compensate for increased volume by skull expansion and widening sutures. -An increase in ICP may be caused by tumors or other space-occupying lesions, accumulation of fluid within the ventricular system, bleeding, or edema of cerebral tissues. Assessment Parameters for Increased ICP: -LOC -Pupillary reaction, pupil size (may be pinpoint or large depending on situation, focus more on reactivity) -Vital signs -Frequency of assessment: temp is often elevated; HR can be rapid, slow and bounding, or feeble; BP may be normal, elevated or very low; RR is often slow, deep, and irregular •Depends on condition; •Ranges from every 5-15 minutes to every 2 hours Clinical Manifestations: -Irritability, poor feeding -High-pitched cry, difficult to soothe -Fontanels: tense, bulging -Cranial sutures: separated -Eyes: setting-sun sign -Scalp veins: distended -Headache -Forceful vomiting: painful -Seizures (one of the last signs) -Drowsiness, lethargy -Diminished physical activity -Inability to follow simple commands -Less interest in the things they used to like Late Signs of Increasing ICP: -Bradycardia -Decreased motor response to command -Decreased sensory response to painful stimuli -Alterations in pupil size and reactivity -Extension or flexion posturing (decorticate and decerebrate) -Decreased consciousness -Coma Setting-sun Sign (also called sundowning, indicative of ICP): -What? The setting-sun phenomenon is an ophthalmologic sign in young children resulting from upward-gaze paresis. -In this condition, the eyes appear driven downward, the sclera may be seen between the upper eyelid and the iris, and part of the lower pupil may be covered by the lower eyelid. -Why? As the hydrocephalus progresses, the eyes are displaced downward by pressure on thinned orbital roof. -This displacement of eyes causes the sclera to be visible above the iris, or the 'sunset eye' sign which is a valuable early sign of hydrocephalus requiring prompt neuroimaging and urgent surgical intervention. Diagnostics: -Laboratory tests: blood glucose, BUN, electrolytes; clotting studies; CBC; liver function tests; blood culture if fever is present; toxicology screen if clinically indicated -Electroencephalography (EEG) -Assessment of evoked potentials (auditory and visual) -Imaging: •Radiography (rule out skull fractures, dislocations; evaluate degenerative changes, suture lines) •Computed tomographic (CT) scan •Magnetic resonance imaging (MRI) -Lumbar Puncture: •Diagnostic purpose to measure spinal fluid pressure and/or to obtain CSF for lab analysis •Therapeutic purpose to inject medication •Contraindicated in patients with increased ICP or infected skin over puncture site •Lumbar puncture ordinarily is delayed if intracranial hemorrhage is suspected and in contraindicated in the presence of ICP because of the potential for brainstem herniation. Indications for ICP Monitoring: -Glasgow Coma Scale score of less than 8 -Traumatic brain injury with abnormal CT scan -Deteriorating neurologic condition -Subjective judgment regarding clinical appearance and response

anemia

-The most common hematologic disorder of childhood -Decrease in number of RBCs or hemoglobin (Hgb) concentration below normal, or both -Decreased oxygen-carrying capacity of blood -Causes and physiology: depletion of RBCs or Hgb, or both; may be caused by a dietary depletion of iron -Morphology: characteristic changes in RBC size, shape, or color, or a combination of these -Signs and symptoms: lack of energy, easy fatigability, and pallor; cyanosis is typically not evident -Diagnostic evaluation: sometimes defined as Hgb <10 or 11 g/dL; however, this cutoff may be inappropriate for children Aplastic Anemia: -A condition that occurs when your body stops producing enough new blood cells. -Treatment for aplastic anemia may include medications, blood transfusions, or a stem cell transplant, also known as a bone marrow transplant. -Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. -In PRCA, the bone marrow ceases to produce red blood cells. -Immune thrombocytopenic purpura (ITP) is a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. -People with the disease have too few platelets in the blood. -Disseminated intravascular coagulation (DIC) is a rare, life-threatening condition. -In the early stages of the condition, DIC causes your blood to clot excessively. -As a result, blood clots may reduce blood flow and block blood from reaching bodily organs. Therapeutic Management of Anemia: -Diagnosis: history and physical, CBC -Treatment of underlying cause: transfusion after hemorrhage if needed; nutritional intervention for deficiency anemias -Supportive care: intravenous (IV) fluids to replace intravascular volume; oxygen therapy; bed rest Care Management of Anemia: -Assessment: •Age of the child: iron deficiency anemia more common in toddlers between 12 to 36 months and during the growth spurt in adolescence •Racial or ethnic background •Careful history •Stool for occult blood: chronic intestinal bleeding •Abnormal Hgb levels: Southeast Asians, Africans , and Mediterranean ancestry -Prepare child and family for laboratory tests: •Explain significance of each test and why tests are not all done at one time •Encourage parents to be with child •Allow play with equipment on doll or participate by holding Band Aid -Decrease tissue oxygen needs: •Signs of exertions include tachycardia, palpitations, tachypnea, dyspnea, shortness of breath, hyperpnea (increased depth and rate of breathing), dizziness, lightheadedness, diaphoresis, and change in skin color. •The child looks fatigued (e.g., sagging, limp posture; slow, strained movements; inability to tolerate additional activity; difficulty sucking in infants.) -Prevent complications: prone to infection due to cellular dysfunction that weakens the body's defense against infectious agents Explaining Blood Components to Children: -Red blood cells: carry the oxygen you breathe from your lungs to all parts of your body. -White blood cells: help keep germs from causing infection. -Platelets: small parts of cells that help make bleeding stop by forming a clot (or scab) over the hurt area. -Plasma: the liquid portion of blood, which has clotting factors that help make bleeding stop.

care of children at the end-of-life

Causes of Death: Infants: -Congenital anomalies, respiratory distress syndrome -Disorders related to prematurity and low birth weight -Sudden Infant Death syndrome (SIDS) Children Ages 5 - 9: -Injuries (accidents), malignant neoplasms -Congenital anomalies, assault (homicide) -Heart disease Children Ages 10 - 14: -Injuries (accidents), malignant neoplasm -Suicide (3rd leading cause of death) Youth Ages 15 - 19: -Assault (homicide), suicide -Malignant neoplasms, heart disease Principles of Palliative Care: -Involves a multidisciplinary approach including the family -"Active total care of patients whose disease is not responsive to curative treatment. Control of pain, of other symptoms, and of psychologic, social, and spiritual problems is paramount. The goal of palliative care is the achievement of the best possible quality of life for patients and their families." - World Health Organization -Can be provided in hospitals, community health centers and in the child's home -Parents don't often accept hospice care: they usually choose palliative care -When cure is no longer possible and life-prolonging measures result in pain, suffering, and distress to the child, parents need information about care options that are available to assist them in deciding how they want the remaining time with their child to be managed by the health care team. -It is important that families are reassured that although their child cannot be cured, active care will continue to be provided to maintain the child's comfort. Support is provided to assist the child and family during the dying process. Decision Making at the End of Life: -Ethical Considerations: when the prognosis for a patient is poor and death is the expected outcome, it is ethically acceptable to: •Withhold or withdraw treatments that may cause pain and suffering •Provide interventions that promote comfort and quality of life -Parents may ask what you would do, but as the nurse tell them you cannot answer that question: it is not your decision to make Treatment Options for Terminally Ill Children: -Hospital: •If home care is not an option •Make hospital room as home-like as possible -Home Care -Hospice Care: •Family members are usually the principal caregivers with support from professionals •The family needs are considered as important as the patient's •Concerned with the family's post-death adjustment and care may be continued for 1 year or longer Pain & Symptom Management: -Must be given the highest priority -Provide continuous pain medication -Symptoms: from the disease or side effects from medications; treat aggressively -Symptoms include fatigue, n/v, constipation, agitation, dyspnea, congestion, seizures, anxiety, depression, restlessness, and confusion Parents' Fears: -Fear of child dying alone or of not being present when the child dies -Parents' and siblings' need for education and support -Parents' greatest fear is that their child will be in pain -Parents and siblings need you to be honest with them: don't sugarcoat things Grief and Mourning: -Grief is a process, not an event, of experiencing physiologic, psychologic, behavioral, social, and spiritual reactions to the loss of a child. -Grief is highly individualized. It is neither orderly or predictable. -Parental grief after a death of a child has been found to be the most intense, complex, long-lasting, and fluctuating grief experience. -Different grieving styles between the parents can hinder communication and support for each other and can put a strain on their relationship. -Sibling grief: depends on their understanding (based on their age and developmental level). -Children express their grief through play and behavior. Nurses' Reactions to Caring for Dying Children: -Most stressful aspect of nursing -Response similar to that of family members -Self-care measures: maintain good general health, develop well-rounded interests, use distancing techniques such as taking time off when needed, developing and using professional and personal support systems, focus on the positive aspects of caregiving. -Attend funeral service: •Attendance at shared remembrance rituals can assist some nurses in dealing with their grief. •Attending the funeral services can be a supportive act for both the family and the nurse: does not detract from the professionalism of care.

cerebral trauma

Cerebral Trauma: -Head injury -Three major causes of brain damage in childhood: •Falls •Motor vehicle injuries •Bicycle- or sports-related injuries Pathophysiology of Head Injury: -Force of intracranial contents cannot be absorbed by the skull and musculoligamentous support of the head -The head is especially vulnerable to acceleration-deceleration injuries -A child's response is different because of larger head size in relation to body and because of insufficient musculoskeletal support Concussion: -An alteration in neurologic or cognitive function with or without loss of consciousness -Transient and reversible -Results from trauma to the head -Generally followed by amnesia and confusion -Usually resolves in 1 to 3 weeks without complications, but the child should rest until symptoms resolve, and then resume activities gradually -Not reversible if happens repeatedly Complications of Head Trauma: -Epidural hemorrhage: •Bleeding between the skull and the dura •Forms a hematoma •As the hematoma enlarges, the dura is stripped from the skull, forcing the underlying brain contents downward and inward as the brain expands. •Classic clinical picture of epidural hemorrhage consists of momentary unconsciousness, followed by a normal period, then followed with lethargy and coma due to blood accumulation in the epidural space and compression of the brain. •Kids with an epidural hemorrhage slip into a coma more easily -Subdural hemorrhage: •Bleeding between the dura and the arachnoid membrane •Usually as a result of rupture of cortical veins that bridge the subdural space. •Tends to develop more slowly and spreads thinly and widely, crossing cranial sutures, until it is limited by the dural barriers. •Presenting signs include irritability, vomiting, increased head circumference, bulging fontanels in infants, lethargy, coma, or seizures. -Cerebral edema: •Associated with traumatic brain injury •Increased ICP with herniation Cerebral edema peaks at 24 to 72 hours after injury and may account for changes in a child's neuro status. •Cerebral edema peaks at 24 to 72 hours after injury and may account for changes in a child's neuro status. •Most serious complication of head trauma Diagnostic Evaluation of Head Trauma: -Initial assessment: •Detailed history •Assessment of airway, breathing, and circulation •Evaluation for shock •Neurologic examination, LOC assessment (most important assessment) •Assessment of vital signs -Special tests: •CT scan •MRI •Behavioral assessment Therapeutic Management: -Care in hospital if severe injuries, LOC for several minutes, prolonged or continued seizures -Nothing administered orally at first until it is determined that vomiting will not occur -Sedating drugs are commonly held in the acute phase. -Acetaminophen can be given for headache. -Surgical therapy: •Scalp lacerations are sutured •Depressed fractures require surgical reduction and removal of bone fragments. •Torn dura is sutured. -Prognosis: outcome of craniocerebral trauma depends on the extent of the injury and complications; in general, the prognosis is more favorable for children than for adults.

hypoparathyroidism

Clinical Manifestations: -Dry, scaly skin with eruptions -Brittle hair, thin nails with transverse grooves -Tetany, paresthesias, tingling, laryngeal stridor, spasms, or a combination of these -Headache, seizures, emotional lability, depression, confusion, memory loss -Diagnosis based on clinical symptoms associated with decreased serum calcium and increased serum phosphorus -Low plasma PTH -Seizures can be problematic, but it is usually a late sign Therapeutic Management: -Goal is to maintain calcium and phosphate levels -Recognize hypocalcemia: convulsions, irritability (esp to external stimuli) , GI symptoms (diarrhea, vomiting, cramping) , and positive signs of tetany -Tetany immediately corrected -Vitamin D therapy -Vitamin toxicity: weakness, fatigue, lassitude, headache, nausea, vomiting, and diarrhea -Monitor renal function, blood pressure, serum vitamin D levels -Maintain seizure and safety precautions -Monitor for laryngospasm: stridor, hoarseness, and feeling of tightness in throat -Monitor BP, which means we also have to monitor the kidneys

cancer

Epidemiology and Etiology of Childhood Cancer: -Childhood cancer is rare: 16,400 cases/year -Cancer deaths: 1300 children younger than 15 each year -Incidence of cancer caused by direct inheritance is low -Chromosome abnormalities have been identified in many childhood malignancies: •Can be confined to the tumor •Can all be present in all cells in the body •Can cause translocation (rearrangement of information between 2 chromosomes) •Can cause abnormal numbers of chromosomes -The incidence of specific types of cancer can vary according to age, sex, and race. -Boys have an overall higher incidence of cancer than females -Caucasian children have higher incidences of cancer than African American children -The incidence of cancer is more pronounced in children ages 0 to 4 years of age and then again in adolescents 15 to 19 years of age Risk Factors: -Radiation exposure -Carcinogenic drugs -Immunosuppressive therapy -Infections -Race -Genetic conditions Diagnostics: -History and physical -Laboratory tests: CBC, LFTs, coagulation studies, urinalysis -Diagnostic procedures: lumbar puncture, bone marrow aspiration -Diagnostic imaging: CT, MRI, PET, MIBG -Surgery -Pathologic evaluation: biopsy Treatment Modalities: -Multimodal therapy: combination surgery, chemotherapy, and radiotherapy -Surgery: remove traces of tumor, restore normal function -Chemotherapy: •Primary form of treatment •Combination drug regimens •IV infusion through venous access devices •Precautions: experienced and trained nurses -Radiotherapy: •In conjunction with chemotherapy •Side effects related to lethal damage to tissue -Biologic response modifiers: •Alter the host's immunologic mechanisms •Direct antitumor effect -Blood or marrow transplantation: •Used with high doses of chemotherapy or to replace dysfunctional marrow •Cells previously stored are given IV after conditioning regimen •Bone marrow transplant patients are very susceptible to infection •They are almost always in reverse isolation - ungown and remove other PPE after you leave the room Signs and Symptoms of Cancer in Children: -Fever and pain, common in childhood, leading to easily missed problem -Unexplained loss of energy -Sudden vision changes -Excessive, rapid weight loss -Malignant tumors cause kids to lose weight, while benign tumors do not -Nutrients go towards the growth of the cancer rather than to the rest of the body Managing Side Effects of Cancer Treatment: -Damage occurs to normal tissues as well as malignant tissues -Infection: •Monitor for septic shock •Fever with absolute neutrophil count (ANC) lower than 500/mm3 •Prevention of infection is a priority -Hemorrhage: •Prevented or controlled with platelet infusions •Teach to avoid activities that may cause injury: riding a bike or skateboards; contact sports -Anemia: •Common during induction therapy •Limits tolerance of physical activity -Usual signs of infection and inflammatory response limited -Collect specimens for evaluation -Start broad spectrum antibiotic therapy -Reduction of infection risk using Colony Stimulating Factors (CSFs) -Platelet transfusions generally reserved for active bleeding not responding to basic treatment measures -Repeated transfusions increase risk of sensitization and antigen formation -Nausea and vomiting: •Antiemetics used to manage side effect; administer before chemo •Synthetic cannabinoids are being used in children •Administer antiemetic 30 minutes before chemotherapy begins and routinely for 24 hours after chemotherapy -Altered nutrition: •Monitor intake and nutritional status •Monitor height and weight routinely •High protein, high calorie, full-fat rather than reduced fat options -Mucosal ulceration: •Red, eroded, painful in mouth; may extend to rectal area •Increase anorexia due to pain •Use bland, moist, soft food, and soft sponge toothbrush mouthwashes can be helpful but avoid those with alcohol. •Mouthwashes can be helpful but avoid those with alcohol •Also avoid viscous lidocaine •One popular topical agent that appears to get recommended by treatment staffs is a so-called "magic mouthwash". •Some patients report good results with a combination of Lidocaine (a numbing agent), Benadryl, Maalox, and Nystatin (an antifungal) •Sitz baths can be helpful with rectal lesions -Neurological problems: •Severe constipation: monitor stool habits; request orders for stool softeners •Foot drop, weakness and numbness lead to difficulty walking •Severe jaw pain: kids often clench their jaws a lot because of pain or nausea •Manage severe jaw pain with analgesics •Children may have a decrease in oral intake due to jaw pain -Alopecia: •Use anticipatory guidance •Warn the child and the family of possible side effects early in the treatment to allow them time for preparation •Explain that hair falls out in clumps, leads to patchy baldness •The child may choose to cut hair short or shave. •Explain that hair regrows in 3 to 6 months but may be a different texture (now curly when it was straight before treatment) or a different color -Steroid effects: •Cushingoid appearance •Can be distressful for older children due to weight gain; moon face appearance •Avoid salt intake to reduce fluid retention