Q1 (10%) Prematurity/Special Needs

What is a hernia? Most common in infants? children?

-A protrusion of an organ or organs through ana bnormal opening -Infants= UMBILICAL hernia -Children= INGUINAL hernia -incarcerated/ strangulated inguinal hernia-> progress to NECROTIC BOWEL s/sx: obstruction, constpiation/ obsipation, abdominal distension, colicky abdominal pain, bowel sounds gradually dminishead/cease

Poor Thermoregulation (4) mgmt?

-immature vasculature tone (vessels don't constrict) -disproportional body surface to body weight (large BSA) -immature, thin skin with dec. subcutaneous fat (30-31 wks formed) -decreased fat leads to poor insulation and heat conversation Mgmt: -Swaddle -Radiant warmers/ Ohio Stables w/ thermostats that on and off

What dietary information should the nurse include in the teaching plan for parents of an infant with galactosemia? Select all that apply. 1 Eliminate milk. 2 Substitute meat for eggs. 3 Provide soybean-based formulas. 4 Avoid baby cereals containing wheat flour. 5 Give prescribed pancreatic enzyme capsules with meals.

1 Eliminate milk. 3 Provide soybean-based formulas. Milk and dairy products are high in lactose content and should be eliminated from the diet. Soybean-based formulas are permissible because they do not contain lactose. Both meat and eggs are permitted because neither contains lactose. Cereals containing wheat products are eliminated from the diet of children with celiac disease. Pancreatic enzymes are prescribed for children with cystic fibrosis, not galactosemia.

1. The most common complication that should be anticipated and observed for in an infant with myelomeningocele after surgical repair of the defect is: a. Urinary stress b. Chiari malformation c. Hydrocephalus d. Latex allergy 2. A 14-year-old male with a spinal cord injury (SCI) is placed on a standing table and suddenly begins to sweat profusely and complain of a headache. The nurse takes a set of vital signs and notes a significant increase in systolic blood pressure and a heart rate of 50 bpm. The most helpful intervention in this situation would be for the nurse to: a. Place the adolescent back in his wheelchair and take him to his room b. Palpate the bladder for distention c. Administer a routine analgesic for his headache and discontinue the therapy d. Place the standing table in a horizontal position and allow the adolescent to rest for a few minutes 3. The primary risk factor for the development of cerebral palsy (CP) is: a. Maternal chorioamnionitis b. Premature birth c. Birth asphyxia d. Intraventricular hemorrhage 4. Urinary system distress (neurogenic bladder) in children with spina bifida (SB) is managed by: a. DDAVP (1-deamino-8-D-arginine vasopressin) b. Clean intermittent catheterization (CIC) c. Continuous urinary catheterization d. Mitrofanoff procedure 5. Which of these statements accurately describes Duchenne muscular dystrophy (DMD)? Select all that apply. a. The absence of dystrophin leads to muscle fiber degeneration. b. DMD is inherited as an X-linked recessive trait. c. Cognitive and intellectual impairment are rare in children with DMD. d. Affected children have a waddling gait and lordosis and fall frequently. e. Ambulation usually becomes impossible by 12 years old, and affected children are confined to a wheelchair. f. Affected children must be hospitalized when ambulation becomes impossible.

1= C hydrocephalus 2= D - palpate the bladder! 3= B (premature birth or low birthweight) 4= B (clean intermittent cathetirization) 5= A, B, D, E

A nurse is caring for an infant with Down syndrome. What does the nurse recall as the most common serious anomaly associated with this disorder? 1 Renal disease 2 Hepatic defects 3 Congenital heart disease 4 Endocrine gland malfunction

3 Congenital heart disease Many children with Down syndrome have cardiac anomalies, most often ventricular septal defects, which can be life threatening

Esophageal Atresia and Tracheoesophageal fISTULA S/SX?? What confirms? What is anticipated/ nursing mgmt?

ATRESIA (esophagus terminates in a blind pouch) FISTULA (distal segment of esophagus attached to trachea) 3 C'S= COUGHING, CHOKING, CYANOSIS Confirmation= chest x-ray *NO ORAL INTAKE! (IVF/ TPN) *NEED SURGERY to do end-to-end anastomosis -MAY NEED GT FEEDS until repair is done (PEG), STOMA IN NECK TO BREATHE

What are some associated problems of prematurity?

"P I I I I I G J" -Poor thermal regulation -Immature CNS (apnea + bradycardia) -Immature immunity (infection) -Intraventricular hemorrhage (trauma, asphyxia) -Immature cardiopulmonary system ->risk for acidosis -Immature GI system (NEC) (late developing!) -Glucose instability (hypoglycemia r/t immature pancreas) -Jaundice (can't breakdown bilirubin in RBC)

Which is an example of a malformation? Select all that apply. (a) cleft palate (b) clubbed foot (c) teeth growing in wrong positions (d) congenital heart defects (e) down syndrome

(a) cleft palate (d) congenital heart defects def: malformation is when as structure is formed incorrectly initially ---- note teeth growing in wrong positions is a dysplasia, clubbed foot is an example of a deformation

Newborn screening (Guthrie Test) is done on an infant. Explain the procedure, where is it done? What do we look for? Why is this important?

-Nurse does a HEEL STICK at sides of heel when the baby is bornl, uses a heal warmer. 4 drops of blood in each circle 1 Congenital hypothyroidism 2 Galactosemia 3 PKU 4 Sickle cell anemia If we don't identify the damage early, it happens right away!

MAINTENANCE FLUIDS (daily) <10 kg? 10-20kg? >20kg? max per day?

<10kg = 100mL/kg example 7 kg baby gets 700 mL fluid 10-20kg= 1,000 mL+ 50ml per kg above 10 kg example 15 kg baby gets 1,000 mL+ 50(5)= 1250 mL >20 kg= 1,500 mL + 20mL/kg above 20 23kg baby gets 1,500 + 3(20)= 1560 mL Max= 2,500 mL per day!!!

Amniotic bands cutting off blood supply and causing an infant to be born with less than ten fingers is an example of a (a) disruption (b) dysplasia (c) malformation (d) deformation

(a) disruption

Clubbed foot is an example of a (a) disruption (b) dysplasia (c) malformation (d) deformation

(d) deformation (growing correctly then wrong, multipara (twins/triplets)= risk factor for clubbed foot disruption= amniotic bands and fingers

Hydrocephalus (CSF in brain) associated with which previous condition? Etiology? S/SX? BAD SIGNS??! NURSING MGMT? DX?? POST-OP CARE/ POSITIONING??

-Associated with myelomeningocele -Etio: infection, IVH, tumor, prematurity S/SX: ***BULGING/ TENSE ANTERIOR FONTANEL ** high pitched cry ( may further increase ICP) Bad Sx: HEAD LAG! -MONITOR FOR INCREASE IN ICP! (PULSE DOWN, BP UP, opposite of shock) -Measure head circumference= dx -Prepare for removal of obstruction -Prepare for insertion of ventricular peritoneal shunt (to create fluid pathway), craniotomy/ surgery -DO NOT pump shunt unless prescribed -DO NOT USE scalp vein for fluids *HOB FLAT TO DECREASE RAPID EXCESS DRAINAGE OF CSF***, LIE ON UNAFFECTED SIDE

Which symptoms present in a child indicate Turner syndrome? Select all that apply. 1 Webbed neck 2 Impaired language 3 Tall stature with long legs 4 Low position of posterior hairline 5 Shield-shaped chest with wide space between the nipple

1 Webbed neck 4 Low position of posterior hairline 5 Shield-shaped chest with wide space between the nipple Impaired language skills are seen in clients with triple X or superfemale syndrome. The client with Turner syndrome has short stature. Tall stature with long legs is a finding in Klinefelter syndrome.

A 7 year old child has a bad headache and has vomited for three times. He has a history of insertion of a VP shunt. What should the nurse do? (a) increase lighting in the room to assess the child's pupils (b) medicate the child for pain and nausea (c) provide the child with fluids immediately (d) notify the health care provider

(d) notify the health care provider -> shunt is failing! and NOT draining, these are sx of increased ICP, a repair is anticipated (a)-> increase ICP (b)-> failure of recognition (c) fluid restriction not fluid resuscitation is anticipated

Intraventricular hemorrhage Strong risk factors? (5) mgmt?

1 extreme prematurity 2 presence of labor (pressure to fragile vessels) 3 Birth asphyxia (not breathing) and need for vigorous resuscitation (bagging) -dilation of vessels calls rupture 4 Mechanical ventilation 5 Sudden change in BPs as a result of rapid hypertonic volume expansion (as with fluid bolus) Identify and reduce risk factors

A nurse is assessing a 3-week-old infant who has been admitted to the pediatric unit with hydrocephalus. What finding denotes a complication requiring immediate attention? 1 Tense anterior fontanel 2 Uncoordinated eye/muscle movement 3 Larger head circumference than chest circumference 4 Inability to support the head while in the prone position 1-month-old infant with hydrocephalus is scheduled to have surgery for the insertion of a ventriculoperitoneal shunt. What is the primary focus of nursing interventions for this infant? 1 Maintaining a satisfactory comfort level to limit crying 2 Applying bandages to the infant's head to protect it from injury 3 Establishing a fixed feeding schedule to ensure appropriate hydration 4 Providing play objects to maintain age-appropriate stimulation for the child Which observation during a developmental appraisal of a 6-month-old infant is most important to the nurse in light of a diagnosis of hydrocephalus? 1 Head lag 2 Babinski reflex 3 Inability to sit unsupported 4 Absence of the grasp reflex An infant with hydrocephalus has a ventriculoperitoneal shunt surgically inserted. What nursing care is essential during the first 24 hours after this procedure? 1 Medicating the infant for pain 2 Placing the infant in a high Fowler position 3 Positioning the infant on the side that has the shunt 4 Monitoring the infant for increasing intracranial pressure

1 Tense anterior fontanel 1 Maintaining a satisfactory comfort level to limit crying 1 Head lag 4 Monitoring the infant for increasing intracranial pressure The shunt may become obstructed, leading to an accumulation of cerebrospinal fluid and increased intracranial pressure. Although providing pain relief for the infant is an important part of postsurgical care, monitoring for potentially severe complications such as increased intracranial pressure takes precedence. Positioning the infant flat helps prevent complications that may result from a too-rapid reduction of intracranial fluid. The infant is positioned off the shunt to prevent pressure on the valve and incision area.

An infant with a cleft lip is fed with a special nipple. What should the nurse teach the parents about feeding their infant to minimize regurgitation? 1 Offer a thickened formula. 2 Burp frequently during a feeding. 3 Place the child in an infant seat during feedings. 4 Position the child on the side with the bottle propped The nurse knows that additional discharge instructions are needed for parents whose infant has just undergone corrective surgery for cleft palate when the parent makes what statement? 1 "We need to schedule regular hearing tests, even at this young age." 2 "Lying on the abdomen is prohibited, so we'll keep him in an infant seat." 3 "We know that some difficulty breathing is expected, so we'll position him upright." 4 "We'll use the elbow restraints you provided to keep him from putting his hands in his mouth." A nurse is teaching the parents of an infant with a cleft lip and palate how to prevent infection. What information should the nurse include about why the infant is predisposed to infection? 1 Waste products accumulate along the defect. 2 Circulation to the defective area is insufficient. 3 Inefficient feeding behaviors result in inadequate nutrition. 4 Mouth breathing dries the oropharyngeal mucous membranes The mother of an infant who just underwent cleft lip repair tells the nurse, "He seems restless. May I hold him?" What information influences the nurse's response? 1 Holding may meet needs and reduce tension on the suture line. 2 Sedation limits activity and decreases tension on the suture line. 3 Handling may increase irritability, causing tension on the suture line. 4 Arm movements cannot be controlled, placing tension on the suture line.

2 Burp frequently during a feeding. Because of the cleft (opening) in the lip, infants with this condition tend to suck in excessive air; burping helps prevent regurgitation of formula. Thickened formula is given to infants with reflux problems, such as vomiting after each feeding. The semi-Fowler position may be used for infants with reflux problems; this infant should be held during feedings. The bottle should never be propped, because aspiration may occur. 2 "Lying on the abdomen is prohibited, so we'll keep him in an infant seat." After cleft palate repair the child is allowed to lie on the abdomen, especially immediately after surgery; this will allow drainage of secretions from the mouth. Children with cleft palate have an increased risk of middle ear infections, which can result in hearing loss, so hearing tests are scheduled early and repeated periodically throughout childhood. Until the infant adjusts to breathing through the mouth, he may exhibit difficulty breathing after surgery; this seldom requires more than positioning and support. Elbow restraints may be prescribed to keep the child's hands out of his mouth. 4 Mouth breathing dries the oropharyngeal mucous membranes Infants with cleft lip and palate breathe through their mouths, bypassing the natural humidification and filtration provided by the nose; as a result, the mucous membranes become dry and cracked and are at risk for infection. Although some waste products may accumulate along the defect, it is not difficult to keep the area clean by cleansing it 1 Holding may meet needs and reduce tension on the suture line.

A nurse is caring for an infant born with a myelomeningocele who is scheduled for surgery. What is the priority preoperative goal for this infant? 1 Keeping the infant sedated 2 Keeping the infant infection free 3 Ensuring maintenance of leg movement 4 Ensuring development of a strong sucking reflex An infant who has undergone surgical correction of a myelomeningocele is to be discharged. What information should the nurse include when preparing the parents to care for their infant at home? 1 The need to limit the infant's fluid intake to formula 2 The need to provide a quiet environment to limit external stimuli Incorrect 3 The positions to be avoided to help prevent the infant from turning 4 How to perform range-of-motion exercises for the lower extremities An infant with a myelomeningocele is scheduled for surgery to close the defect. Which nursing action best facilitates the parent-child relationship in the preoperative period? 1 Encouraging the parents to stroke their infant 2 Allowing the parents to hold their infant in their arms 3 Referring the parents to the Spina Bifida Association of America (Canada: Spina Bifida and Hydrocephalus Association of Canada) 4 Teaching the parents to use special techniques when feeding the infant

2 Keeping the infant infection free Prevention of infection is the priority both before and after the repair of the sac. Sedatives are not indicated; analgesics are administered as needed. Leg movement may be a postoperative goal, although it may be unrealistic because these infants' lower bodies are usually paralyzed. The sucking reflex is not associated with myelomeningocele. 4 How to perform range-of-motion exercises for the lower extremities Passive range of motion, positioning, and stretching may help decrease the risk of muscle contractures in the lower extremities. Fluid intake should be unrestricted to provide adequate kidney function and prevent constipation. The infant needs stimulation to develop mentally and socially. Development of mobility should be encouraged; the infant's movements should not be restricted. 1 Encouraging the parents to stroke their infant Because the infant cannot be held, tactile stimulation helps meet the infant's needs and fosters bonding with the parents. An infant with an unrepaired myelomeningocele cannot be held in the arms. Referrals will be more appropriate at a later time. Although special feeding techniques are important in the postoperative period, they may not improve the parent-infant relationship

A parent brings a 2-month-old infant with Down syndrome to the pediatric clinic for a physical and administration of immunizations. Which clinical finding should prompt the nurse to perform further assessment? 1 Flat occiput 2 Small, low-set ears 3 Circumoral cyanosis 4 Protruding furrowed tongue

3 Circumoral cyanosis Circumoral cyanosis is not a specific characteristic of Down syndrome. It is a clinical finding associated with congenital heart disease, which these infants may have as a concurrent problem. A flat occiput (head) and a broad nose with a depressed bridge (saddle nose) are features of children with Down syndrome. Small, misshapen, low-set ears are also a clinical manifestation of Down syndrome. Children with Down syndrome often keep their mouths open, with their tongues protruding; the surface of the tongue is often wrinkled.

A nurse is teaching the parents of an infant with cerebral palsy how to provide optimal care. What should the nurse include in the teaching? 1 Focusing on cognitive rather than motor skills 2 Maintaining immobility of the limbs with splints 3 Preserving muscle tone to prevent joint contractures 4 Continuing to offer a special formula to limit gagging

3 Preserving muscle tone to prevent joint contractures Children with cerebral palsy are especially prone to muscle tone disorders, including spasticity, which can lead to joint contractures. The therapy program must be balanced to promote progress in all areas of growth and development. Splinting of limbs is contraindicated because immobility promotes the development of joint contractures. Although these infants tend to gag and choke during feedings, a special formula is not necessary unless the child is allergic to dairy products.

Define a per-term infant. Define a full term infant. Preterm birth occurs in about ___ pregnancies. Accounts for largest number of ___ admission. Leads to ___% of early nenonatal mortality and morbidity.

A pre-term infant is one born before 37 weeks gestation regardless of birth weight. A full term infant is one born at 40 weeks gestation. -Preterm birth occurs in about 1 in 8 pregnancies. -Accounts for largest number of NICU admission -Leads to 75% of early neonatal mortality and morbidity

Congenital hypothyroidism S/sx: Nursing implications? Inadequate tx s/sx? Tx?

-A common preventable cause of cognitive impairment S/sx: Low T4, Increased TSH; "QUIT GOOD" BABY, prolonged jaundice, lethargy, poor appetite/constipation, VS changes (ALL VITALS DOWN, hypothermia, bradycardia, bradypnea, hypotension, dec. O2 sat), MACROGLOSSIA (large tongue), decreased activity, poor weight gain CRISIS= MYXEDEMA COMA (ALTERED LOC + RESPIRATORY ACIDOSIS/ FAILURE) Nursing Implications: -Start treatment IMMEDIATELY (prevent cognitive impairment) -Inadequate tx:= FATIGUE, SLEEPY, DEC. APPETITE, CONSTIPATION, COGNITIVE IMPAIRMENT Tx: levothyroxine every day for life! (WATCH FOR Overdose s/sx HYPERTHYROIDISM, heat intolerance, tremors, thyroid storm, elevated vitals,d yspnea, sweating, anxiety, insomnia)

Clinical Presentation of Necrotizing Enterocolitis (NEC)? Management of NEC? KNOW THIS!

-Abdominal distension dt gas producing bacteria -Visible bowel loops -Feeding intolerance/ INCREASED RESIDUAL -Bilious vomiting (green/ olive color)-> notify HCP! -LETHARGY** -BLOOD GLUCOSE INSTABILITY -TEMPERATURE INSTABILITY -apnea, bradycardia, oxygen desaturation *STOP ALL FEEDINGS*!! -Ventilation by ETT if in resp. failure -Agressive IV fluid/ electroltye replacement -NG DECOMPRESSION and early TPN -Broad spectrum antibiotics (ampicillin, gent, & clindaymycin) -Pain control + minimal handling -Serial abdominal x-rays, frequent labs -SURGERY!!!

Phenylketouria (PKU) T/F Babies with PKU can drink breast milk. What is it? S/sx? Refer to?? Special Nursing implications? (2) Drugs that treat it? (2)

-An autosomal recessive metabolic genetic disorder caused by the mutation of the gene require to metabolize the amino acid phenylalanine. (error of metabolism) -Accumulation of phenylalanine is TOXIC to the brain, demyelinates it, and accumulation leads to cognitive impairment. F. Babies with PKU cannot drink breast milk due to the phenylalanine content in it S/sx: -fair skin/ hair, -eczema -intellectual disability -behavior problems - convulsions/ seizures - musty body odor Refer to DIETITIAN 1 Assess patients nutritional history. Teach pt PKU diet (NO meat, fish, dairy products, nuts, legumes/ beans, eggs, AVOID ASPARTAME, CEREALS in moderation, FRUITS + VEGETABLES can be eaten with freedom) Drugs: 1. sapropterin 2. pegvaliase (enzyme therapy)

Down syndrome s/sx?

-Cognitive impairment -CONGENITAL HEART DEFECTS -HYPOTONICITY (lack of muscle strength, can't cough well) -RISK FOR LUNG INFXNS -RISK OF LEUKEMIA (10X) -Flattened nose and face, upward slanting eyes, flat occiput, single palmer crease, short fifth finger that curves inward, widely separated first and second toes and increased skin creases

Galactosemia What is it? Tx/ nursing mgmt? T/F Is honey okay?

-Deficiency in hepatic enzymes involved in the conversion of galactose to glucose TX: USE SOY formulas! -NO MILK OR DAIRY PRODUCTS! -BABY CANNOT BE BREAST FED! NO BREAST MILK! -NO HONEY FOR NEONATES BC BOTULISM!!

What are some examples of teratogens? Their effects on the baby?

-ETOH-> fetal alcohol syndrome -Tobacco-> low birth weight -> CP -Marijuana -> attention deficits -Isotretinoin/ retinoids (Accutane)-> Liver damage -TORCH (Toxoplasmosis, Other= HIV, Syphilis, Gonorrhea, Chlamydia, HPV, Rubella, Cytomegalovirus, Herpes Simplex Virus -Rubella/ CMV-> Hearing loss/ visual disturbances -RADIATION-> May increase cancer risk Meds: -Phenytoin (Dilantin) -Warfarin (Coumadin) -lithium -diethylstilbestrol

Cleft lip and palate Feeding challenges management? T/F Can a mother breast feeed? How do we know if a baby is getting adequately fed???*** KNOW THIS CLINICAL CONSIDERATIONS?? post-op challenges??? positioning???

-LONGER, SOFTER nipples, X or Y nipples, squeezable bottle -NG or rarely GT for insufficient PO intake -T. Breast tissue must be positioned to close off the entire cleft, another option is to have the breast milk pumped into a bottle, and then bottle feed the baby -If their urine output= 2mL urine/ kg/ hr ** -Weigh dry diaper, then weigh wet diaper in g, difference in g= urine ml *Multidisciplinary care/ PSYCHOSOCIAL IMPLICATIONS (BONDING IS IMPORTANT) *AIRWAY is priority, KEEP SUTURE LINE CLEAN, FEEDING ISSUES *PAIN MANAGEMENT TO LIMIT CRYING, ELBOW RESTRAINTS *At risk for hearing loss, EARLY screening with AABR (automated auditory brainstem response) position= SUPINE AND SIDELYING POSITIONS **REMOVE ELBOW RESTRAINTS PERIODICALLY TO CUDDLE INFANT

Necrotizing Enterocolitis (NEC) Why does it happen? T/F This is not an emergency.

-MOST COMMON GI disorder in premature infants -Immature blood supply to GI bc during wk 30 does that GI tract start to develop, -Interrelationship among: immaturity, ischemia, infection, immunity and nutrition -GI mucus protection is decreased an causes enzyme to breakdown tissue, Pneumonitis, a GI bacteria, creates air -Infant is at risk for nutritional deficiencies due to short bowel syndrome F. This is the most common surgical emergency in the neonatal period!

Cleft lip and palate How can it be diagnosed? Causes? Tx?--> WHEN? Associated problems? Main problems???

-May be diagnosed prenatally or not at the 20th week gestation via ultrasound -Causes: teratogens, FOLATE DEFICIENCY, genetics -Tx= surgery CLEFT LIP REPAIR-> usually 2 to 3 months when RULE OF TENS! Child is usually 10 weeks old, 10 lbs, 10 hgb CLEFT PALATE-> completed before 12 months 1 ORTHODONTIC (teeth alignment) , SPEECH, HEARING/ OTITIS MEDIA (milk enters eustachian of tube, causes infection, scars tympanic membrane) -FEEDING CHALLENGES!

Which hemoglobin defect should we look for in newborn screening? Which demographic is at risk? What type of screening confirms? What do we give them if they have it? What can cause a sickle cell crisis? (4) Immediate Tx?

-Sickle cell anemia -African Americans -Hgb electropheresis -Give them prophylactic antibiotics such as pencillin, to prevent infection, because infection can actually cause the Hgb to turn sickle!!! 1 High elevations 2 Dehydration 3 Infection/ increased temperature 4 Stress Tx: "HOP" hydrate, oxygen, pain meds

Latex allergy common in? Identifying Latex Allergy- QUESTIONS?

-Spinal Bifida -report linked intraoperative anaphylaxis with latex in children with SB. Latex, a natural product derived from the rubber tree, is used in combination with other chemicals to give elasticity, strength, and durability to many products. - Children with SB are at high risk for developing latex allergy because of repeated exposure to latex products during surgery and procedures. Therefore, such children should not be exposed to latex products from birth onward to minimize the occurrence of latex hypersensitivity. Allergic reactions range from urticaria, wheezing, watery eyes, and rashes to anaphylactic shock. More severe reactions tend to occur when latex comes in contact with mucous membranes, wet skin, the bloodstream, or an airway. -There also can be cross-reactions to a number of foods (e.g., BANANA, AVOCADO, KIWI) , • Does your child have any symptoms, such as sneezing, coughing, rashes, or wheezing, when handling rubber products (e.g., balloons, tennis or Koosh balls, adhesive bandage strips) or when in contact with rubber hospital products (e.g., gloves, catheters)? • Has your child ever had an allergic reaction during surgery? • Does your child have a history of rashes; asthma; or allergic reactions to medication or foods, especially milk, kiwi, bananas, or chestnuts? • How would you identify or recognize an allergic reaction in your child? • What would you do if an allergic reaction occurred? • Has anyone ever discussed latex or rubber allergy or sensitivity with you? • Has your child had any allergy testing? • When did your child last come in contact with any type of rubber product? Were you present?

What is duchenne muscular dystrophy? (DMD) common in males or females? S/sx? DX?

-X-linked recessive disease, progresses to lung/heart defects/ death by 25 -Males -WADDLING gait, lordosis, increase clumsiness, muscle weakness -GOWERS SIGN= difficulty rising to standing position, has to "walk up" legs using hands -ELEVATED CPK/ALT/AST -Eventual wheelchair dependency/ bed confinement/ heart/lung failure *genetic counseling! Characteristics of Duchenne Muscular Dystrophy • Early onset, usually between 3 and 7 years old • Progressive muscular weakness, wasting, and contractures • Calf muscle pseudohypertrophy in most patients • Loss of independent ambulation by 9 to 12 years old • Slowly progressive, generalized weakness during teenage years Typically, affected boys have a waddling gait and lordosis, fall frequently, and develop a characteristic manner of rising from a squatting or sitting position on the floor (Gower sign) Mental deficiency (common) • Mild (≈20 IQ points below normal) • Mental deficit present in 25% to 30% of patients DX: Diagnostic Evaluation The diagnosis of DMD is primarily established by blood polymerase chain reaction (PCR) for the dystrophin gene mutation Tx: -Therapeutic Management No curative treatment exists for childhood MD. The use of the corticosteroids prednisone and deflazacort has been evaluated as a treatment for DMD. Several clinical trials demonstrated increased muscle strength and improved performance and pulmonary function, with significant decrease in the progression of weakness, when prednisone was administered for 6 months to 2 years, AVOID CONTRACTURES

Myelomeningocele (spina bifida) Risk factor? Cause? Prevention? Consequences? WHAT POSITION SHOULD THIS BABY BE IN? S/SX: Which is more effective in preventing ortho abnormalities in kids with this? Prenatal or postnatal surgery?! TELL TALE LAB THAT INDICATES PROBABLE SPINA BIFIDA?

-defined as a deformity related to FOLATE DEFICIENCY, a defect of the SPINE (NEURAL TUBE DEFECTS) -0.4 mg or 400 mcg of folate supplementation daily prenetally prevents NEURAL TUBE DEFECTS CONSEQUENCES 1 INFECTION 2 hydrocephalus 3 NEUROGENIC BOWEL/ BLADDER 4 PARALYSIS/ ORTHO ABNORMALITIES position= PRONE! -LOOK FOR DIMPLE IN BACK, can be picked up ULTRASOUND ~20 weeks ***PRENATAL SURGERY *Elevated AFP levels in amniotic fluid/ serum may indicate neural tube defects

Immature Respiratory System why? management? (3)

-surfactant deficiency elads to alveoli collapse, respiratory destress syndrome (RDS), immature neurological control center= secondary apnea 1 intubation with endotracheal tube and CPAP, oxygen 2 surfactant replacement therapy sprayed down E-tube 3 serial chest x-rays, continous monitoring: cardiorespiratory status, pulse oximetry, BP, ABG...

The neonate has a protruding tongue and a crease that transverses the entire width of each palm. The nurse recognizes that these findings are characteristic of what congenital condition? 1 Hypothyroidism 2 Down syndrome 3 Turner syndrome 4 Fetal alcohol syndrome

2 Down syndrome Dysmorphic features that are characteristic of Down syndrome include a protruding tongue and simian creases across the palms. A protruding tongue but not the transverse palmar creases may also occur with hypothyroidism. Turner syndrome is characterized by a webbed neck and peripheral edema. Children with fetal alcohol syndrome have dysmorphic features, but these are different from the ones that occur with Down syndrome. The chromosomal complement 45,XO is seen in children with Turner syndrome. A child with Turner syndrome will have short stature and a webbed neck.

A nurse is caring for a 6-day-old preterm infant in the neonatal intensive care unit. What complications should the nurse be alert for in this infant? 1 Meconium ileus 2 Duodenal atresia 3 Imperforate anus 4 Necrotizing enterocolitis A nurse is caring for preterm infants in the neonatal intensive care unit. The nurse knows that these infants are at risk for intestinal obstruction associated with what? 1 Meconium ileus 2 Imperforate anus 3 Duodenal atresia 4 Necrotizing enterocolitis

4 Necrotizing enterocolitis Necrotizing enterocolitis (NEC) is an inflammatory disease of the gastrointestinal mucosa that is related to several factors (e.g., prematurity, hypoxemia, high-solute feedings); it involves shunting of blood from the gastrointestinal tract, decreased secretion of mucus, greater permeability of the mucosa, and increased growth of gas-forming bacteria, eventually resulting in obstruction. NEC usually manifests 4 to 10 days after birth. Meconium ileus occurs within the first 24 hours when the newborn cannot pass any stool. It is not related to the development of NEC; it is a complication of cystic fibrosis. Duodenal atresia is a congenital defect that occurs early in gestation and is present at birth. Imperforate anus is an anorectal malformation that results in the absence of an external anal opening; it is present at birth.

CP Cerebral Palsy *** T/F Cerebral Palsy is a progressive disorder of the developing brain KEY S/SX?? other s/sx? RISK FACTORS?? MOST IMPORTANT? CP diagnosis? FEEDING IMPLICATIONS??? MANAGEMENT?

F. Cerebral palsy is a NON-PROGRESSIVE (PERMANENT) disorder of the developing brain Key S/SX= HYPERTONICITY (diminished ability to inhibit muscle movement, ALL muscles are engaged, STIFF BABY!), abnormal SCISSOR GAIT (QUADS SO ENGAGED), TOES POINTED INWARD, ON TIPPY TOES (calf engaged) -Seizures, attention/ behavioral, communication speech/ visual + hearing distrbances, cognitive (MORE MOTOR) 1 MOST IMPORTANT= VERY LOW BIRTH WEIGHT (tobacco causes this) 2 Birth asphyxia (deprivation of O2 due to meconium aspiration) 3 Malformation of brain/ intrauterine infection/ preterm infant CP Diagnosis: 1 Neuro exam (hypertonicity) 2 History (smoking/ birth asphyxia) 3 Posture/Tone 4 PERSISTENCE OF PRIMITIVE REFLEXES (E.G. MORO AFTER 4 MONTHS) 5 MRI checks if structural cause -FEEDING: UPRIGHT, SUPPORT THE JAW (sometimes G tube d/t swalloing risk/ aspiration PNA) MGMT= TEAM APPROACH (PT/OT/RN/MD) -AFOS (Ankle Foot Orthosis)-> straightening (PT/OT) -Enhance communication (Speech language therapist) -Bracing, casting, surgery -MEDICATIONS to dec spasticity 1. botulinin toxin (inhibits nerve conduction) 2. baclofen PO/ intraeTHECAL PUMP (enhances ability of nerves to inhibit muscles) - Nutrition assistance (dietation/ safety) -Family support

Nursing management of myelomeningocele: PRE-OP + POST-OP Meds? LONG TERM CARE???!

PRE-OP -Cover sac with MOIST STERILE DRESSING to prevent infection -Be mindful of thermoregulation (put under radiant incubator) -NO PRESSURE over affected area (lie baby prone) -ANTIBIOTICS, IV FLUID ***LATIX PRECAUTIONS (NO LATEX GLOVES, TORUNIQUET, NO LATEX CATHETER IF NEUROGENIC BLADDER, child may have lifetime LATEX allergy) POSTOP: -PRONE for 7 days, then on sides, NEVER supine -Foley to intermittent catheritizing -Proect incision from stool and urine -observe for leaks (red=blood, halo sign= CSF) -ADMINISTER ABX -Daily head circumferance and weekly head ultrasounds MEDS: urecholine to reduce urinary retention and colace to soften the stool LONG TERM CARE= PHYSICAL THERAPY

QA A nurse is caring for an infant after a cleft lip repair. Which item should the nurse use to feed the infant for several days after the surgery? 1 Preemie nipple 2 Nasogastric tube 3 Gravity-flow nipple 4 Rubber-tipped syringe QB At the age of 3 weeks an infant undergoes surgery to repair a cleft lip. What should postoperative nursing care include? 1 Using a spoon to administer oral feedings 2 Cleansing the suture line to prevent infection 3 Offering a pacifier for sucking to prevent crying 4 Using wrist restraints to keep the infant's hands away from the face QC An infant is born with a cleft lip. What nursing intervention is unique to infants with cleft lip? 1 Changing the infant's position often 2 Using modified techniques for feeding 3 Monitoring the infant's daily intake and output 4 Keeping the infant's head elevated during feeding QD A nurse is feeding an infant who recently underwent surgical repair of a cleft lip. What does the nurse plan to do for the infant just after each feeding? 1 Burp several times. 2 Rinse the suture line. 3 Place on the abdomen. 4 Hold for several minutes.

QA 4 Rubber-tipped syringe A rubber-tipped syringe minimizes sucking and is not irritating to the suture line. Using a preemie nipple is one method of feeding before surgery. A nasogastric tube is unnecessary; the infant is hungry enough to feed even if deprived of sucking. Using a gravity-flow nipple is one method of feeding before surgery. QB 2 Cleansing the suture line to prevent infection Meticulous care of the suture line is necessary to prevent infection and to help ensure the best cosmetic effect. Using a spoon is contraindicated, because it could disrupt the suture line; the infant may be fed with a device that is designed especially for this purpose. Offering a pacifier is contraindicated, because sucking will put tension on the suture line and may result in disruption of the sutures. Elbow restraints are used; this allows the infant to move the arms without bending the elbows and thus prevents the infant from touching the face. QC 2 Using modified techniques for feeding QD 2 Rinse the suture line. Meticulous care of the suture line is necessary, because inflammation and sloughing of tissue disrupt healing. Burping should be done throughout the feeding. Placing the infant on the abdomen is contraindicated, not only because the infant may rub the face on the sheet and irritate the suture line but also because this position has been linked to sudden infant death syndrome. The infant may be held at any time.

What does VATER(L) syndrome stand for?

Vertebral defects Anus= imperforate (no anus upon birth) Tracheo-Esophageal Atresia Renal defects Limb defects

You are caring for a child with hydrocephalus who is postoperative from a shunt revision. Which assessment finding is your priority for increased intracranial pressure (ICP)? a. Nausea and refusal to eat postoperatively b. Complaint of a headache c. Irritability and wanting to sleep d. Decrease in heart rate over the last hour

d. Decrease in heart rate over the last hour