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CONGENITAL HEART DISEASE 1. Acyanotic diseases Pulmonary circulation and systemic circulation are not connected If there is a connection, the pressure is higher in the left side than in the right side PATENT DUCTUS ARTERIOSUS Most common congenital heart defect Symptoms depend on size of the vessel and age of the child May have no symptoms; indication may be a murmur Increasing dyspnea, full bounding pulse, wide pulse pressure Spontaneous closure after infancy rarely occurs Without treatment – life expectancy short ATRIAL SEPTAL DEFECTS 10% of all forms of congenital heart disease Allows oxygenated blood returning from the lungs to pass into the right atrium VENTRICULAR SEPTAL DEFECT constitute 20% of all forms of CHD allows systemic venous and oxygenated arterial blood to mix may produce no symptoms at all require no specific treatment and often close spontaneously COARCTATION OF THE AORTA narrowing of the lumen of the aorta may be an isolated defect or associated with other cardiac malformation Assessment: measure BP in both arms and a leg and to assess the pulse in both upper and lower extremities surgical repair – only permanent treatment; usually deferred until 3 years of age PULMONIC VALVE STENOSIS usually do not produce symptoms; typical murmur

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CONGENITAL HEART DISEASE

1. Acyanotic diseases• Pulmonary circulation and systemic circulation are not connected• If there is a connection, the pressure is higher in the left side than in the

right side

PATENT DUCTUS ARTERIOSUS• Most common congenital heart defect• Symptoms depend on size of the vessel and age of the child• May have no symptoms; indication may be a murmur• Increasing dyspnea, full bounding pulse, wide pulse pressure• Spontaneous closure after infancy rarely occurs• Without treatment – life expectancy short

ATRIAL SEPTAL DEFECTS• 10% of all forms of congenital heart disease• Allows oxygenated blood returning from the lungs to pass into the right

atriumVENTRICULAR SEPTAL DEFECT• constitute 20% of all forms of CHD• allows systemic venous and oxygenated arterial blood to mix• may produce no symptoms at all• require no specific treatment and often close spontaneously

COARCTATION OF THE AORTA• narrowing of the lumen of the aorta• may be an isolated defect or associated with other cardiac malformation• Assessment: measure BP in both arms and a leg and to assess the

pulsein both upper and lower extremities

• surgical repair – only permanent treatment; usually deferred until 3 yearsof age

PULMONIC VALVE STENOSIS• usually do not produce symptoms; typical murmur

AORTIC VALVE STENOSIS• mild to moderate asymptomatic; typical murmur

CYANOTIC DISEASES

TETRALOGY OF FALLOT1. VSD2. Overriding of the aorta3. Pulmonary valve stenosis4. Enlarged right ventricular wall

Assessment:• Primary sign – cyanosis• Hypoxic spells – usually initiated by crying• Fainting – due to cerebral hypoxia• Stunted growth, clubbed fingers and toes• Squatting position – characteristic position to relieve dyspnea

Implementation

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• Decrease hypoxic spells – do not permit child to cry• Place in knee chest position• O2 as needed

TRANSPOSITION OF THE GREAT VESSELS• aorta arises from the right ventricle• degree of cyanosis depends on the abnormal connections

Assessment:• cyanotic at birth• develop polycythemia à risk for emboli and thromboses• may develop CHF or pulmonary vascular obstructive

Implementation:• require emergency medical treatment• cardiac catheterization• ballool atrial septostomy

TRICUSPID ATRESIA• condition in which tricuspid valve did not form• no opening between the right atrium and right ventricle• 2% of congenital heart defects• profound cyanosis and dyspnea at birth• emergency catheterization with balloon atrial septotomy

TOTAL ANOMALOUS VENOUS RETURN• condition in which all the pulmonary venous blood returns to the heart• cyanosis and severe respiratory distress• emergency surgical intervention

TRUNCUS ARTERIOSUS• single vessel arising from the ventricles just above a large VSD• retarded growth; enlarged liver and heart• usually infants die within the first year

CARDIOVASCULAR DEFECTS• Monitor vital signs closely• Monitor respiratory status• Auscultate breath sounds for crackles, ronchi or rales• if respiratory effort is increased, place child in reverse Trendelenburg

position• administer humidified oxygen• Monitor for hypercyanotic spells:1. Place infant in a knee chest position2. Administer 100% oxygen by mask3. Administer morphine as ordered4. IVF as prescribed

• Assess for signs of CHF• Assess peripheral pulses• Keep child stress free as possible; allow maximal rest

CARDIAC SURGERYPostoperatively:• monitor for signs of discomfort• monitor for signs of sepsis (fever, lethargy, diaphoresis, altered LOC)

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• Monitor lines, tubes or catheters ; remove promptly• administer pain medications; note effectiveness• encourage rest periods• facilitate parent-child contact as soon as possible

Home Care• omit activities in which child could fall for 2-4 weeks• avoid crowds for 2 weeks after discharge• no added salt diet• do not put creams, lotions or powders on the incision site• child may return to school 3rd week after discharge• no physical education for 2 months• follow up after 2 weeks• avoid immunizations, invasive procedure and dental visits for 2 months• advise parents regarding importance of dental visit every 6 months• inform dentist of cardiac problem• instruct parents to call MD if with coughing, tachypnea, cyanosis,

diarrhea

CONGESTIVE HEART FAILURE• inability of the heart to pump sufficiently to meet the metabolic demands

of the body• infants – most commonly caused by congenital heart defects• combination of both left sided and right sided heart failure• goal of treatment: to improve cardiac function, remove accumulated fluid

and sodium, decrease cardiac demands, improve tissue oxygenation

Assessment:• tachycardia• tachypnea• profuse scalp sweating especially in infants• fatigue and irritability• sudden weight gain• respiratory distress

Implementation:• monitor vital signs closely and for early signs of CHF• monitor for respiratory distress• monitor I and O; weigh diapers• Monitor daily weight to assess for fluid retention; weight gain of 0.5 kg

( 1 lb /day)• monitor for facial or peripheral edema , auscultate lung sounds• elevate HOB• maintain neutral thermal environment to prevent cold stress in infants• administer cool, humidified oxygen • organize nursing activities to allow uninterrupted sleep• maintain adequate nutritional status• provide rest; decrease environmental stimuli• feed when hungry and soon after awakening• infant should be well rested before feeding• provide small, frequent feedings• administer sedation• administer digoxin• check with physician parameters for witholding digoxin• Note that infants rarely receive more than 1 mL (50 ug or 0.05 mg) of

digoxin• Administer diuretics; monitor for hypokalemia• administer potassium supplements

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• monitor serum electrolytes• restrict fluid in acute stages• check with physician regarding sodium restriction; infant formulas have

slightly more sodium than does breast milk

Home care Instructions for Administering Digoxin• administer 1 hr before or 2 hrs after feeding• do not mix medication with food or drink• if the child vomits, do not administer a second dose• if more than 2 consecutive doses have been missed, notify MD; do not

increase or double the dose for missed doses• keep the medication in a locked cabinet• if the child becomes ill, notify MD

RHEUMATIC FEVER• an inflammatory autoimmune disease• affects connective tissue of the heart, joints, subcutaneous tissues and

blood vessels of the CNS• most serious complication – RHD affecting the cardiac valves• presents 2-6 weeks following an untreated Group A beta hemolytic strep• Jones criteria

Asssessment:• signs of carditis: SOB, edema of the face, abdomen or ankles, precordial

pain• signs of polyarthritis: edema, inflammation of the large joints, joint pain• erythema marginatum: macular rash on trunk and extremities• subcutaneous nodules• fever• elevated ASO• elevated ESR• elevated CRP

Implementation:• Assess vital signs• Control joint pain and inflammation with massage• Febrile phase – provide bed rest• limit physical exercise in child with carditis• Administer antibiotics (penicillin) as prescribed• administer salicylates and anti-inflammatory agents• Instruct parents about the importance of follow up and need for

antibiotic prophylaxis for dental work, infection, invasive procedures• advise child to inform the parents if anyone in school develops a

strep throat infection

KAWASAKI DISEASE• known as mucocutaneous lymph node syndrome• acute systemic inflammatory illness• unknown cause• cardiac involvement – most serious complication

Assessment:• fever• conjunctival injection• red throat• Swollen hands, rash, enlargement of the cervical lymph nodes

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Implementation:• monitor temperature frequently• assess heart sounds and rhythm• assess extremities for edema, redness, desquamation• monitor mucus membrane for inflammation• weigh daily• administer IV immune globulin• instruct parents in the administration of prescribed meds

aspirin – need to monitor bleeding

GASTROINTESTINAL SYSTEM

Physiology of DIGESTIVE SYSTEM• Mechanical functions of digestion immature at birth

No voluntary control over swallowing until 6 weeksStomach capacity decreasedRelaxed sphincter contributes to tendency to regurgitate

• Liver functions immature throughout infancy• Gastric acidity low in infants à rises until age 10• Digestive processes are mature by toddlerhood

Assessment:• Presenting problem

a. Vomitingb. Abnormal bowel habits: diarrhea, constipation, bleedingc. Weight loss, failure to thrived. Pain

• Nutritional history

Physical Examination:• General appearance:

a. Height and weightb. Measure mid arm circumferencec. Observe color: jaundice

• Mouth: dentition• Abdomen

a. Skin integrityb. Abdominal distension; visible peristaltic wavesc. Inspect for herniasd. Auscultate bowel sounds ( every 10-30 seconds)e. Palpate for tendernessf. Liver span (inferior edge palpated 1-2 cm below RCM)g. Spleen (felt on inspiration 1-2 cm below left costal margin)

DISORDERS OF THE TEETH

DENTAL CARIES• erosion of the enamel and dentine of teeth• results from combination of fermenting sugars and starched

and acid forming organisms

Prevention:• dental hygiene• regular dental hygiene• good nutrition

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• fluoridation

CLEFT LIP AND CLEFT PALATE

• 1 in 1000 births• multifactorial• common among children with chromosomal abnormalities• results from failure of the embryonic structures of the face to unite• may occur separately or may combine to produce a single unilateral

or bilateral cleft from the lip through the soft palate• interfere with the child’s capacity to meet oxygenation and

nutritional needs • may seriously hamper normal bonding process of children with parents

Assessment:• facial abnormality visible at birth

- cleft lip or palate or both, unilateral or bilateral• difficulty sucking• inability to form airtight seal around nipple• formula/milk escapes through nose in infants with cleft palate• predispose to infection – communication between mouth and nose• difficulty swallowing• abdominal distension – air swallowing

Management:• team approach• otolaryngologist, audiologist, pediatrician, dentist, orthodontist, speech

therapist• Surgical correction:• early correction – prevent speech defects

Cheiloplasty • correction of cleft lip• unite edges to allow lips to be both functional; aesthetic reasons• performed usually at age 2 months

Cleft palate repair• not done until age 18 months – anticipation for speech development• child should be weaned and able to take liquids from a cup before

palate repair

NURSING INTERVENTION:

Cleft Lip (PreOP)• feed in upright position• burp frequently• press cleft lip together with fingers – to encourage sucking and to

strengthen muscles• if unable to suck, use a rubber tipped syringe; drip onto side

of mouth• finish feeding with water to wash away formula in palate area• provide small, frequent feedings

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• provide emotional support for parents

Post OP: Cleft Lip repair• maintain patent airway• monitor amount of swallowing to detect hemorrhage• do not place in prone position or with pressure on cheeks• avoid any tension on suture line• avoiding straining on suture line

• prevent crying• keep child comfortable and contented• use elbow restraint• keep suture line clean – clean after each feeding with saline,

peroxide or water to remove crusts• pain relief

Nursing Intervention: Preop cleft palate repair• prepare parents to care fro child after surgery• instruct concerning feeding methods and positioning

Post Op cleft palate repair• position on side for drainage of blood• have suction available • prevent injury or trauma on suture line1. Use cups only for liquids; no bottles2. avoid straws, utensils, popsicle sticks, chewing gum3. provide soft toys4. Use elbow and wrist restraints5. Provide liquid diet initially à soft à normal diet6. Give water after each feeding to clean suture line7. Hold and cuddle

ESOPHAGEAL ATRESIA and TRAACHEOESOPHAGEAL FISTULA

Esophageal atresia• congenital defect; upper segment of the esophagus ends in a blind

pouch

TEF• defect in which embryonic structures fail to divide into a separate

esophagus and trachea• opening between the two structures• usually occur together

Assessment:• copious oral and nasal secretions –first sign of a defect• choke or cough• when suctioning or gavage is attempted – catheter cannot pass

into stomach

Medical and Surgical intervention• prevention of aspiration• drainage tube may be placed in the blind pouch – to suction secretions• esophageal atresia – medical emergency

- end to end anastomoses• feeding tube maybe inserted into the stomach through gastrostomy until

repair heals

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Nursing intervention:• provide nutrition1. Provide gastrostomy tube feeding until anastomosis site has healed2. start oral feedings when infant can swallow well• promote respiratory function

1. position properly

PYLORIC STENOSIS• narrowing of the outlet of the stomach• caused by excessive growth of circular muscles that surrounds the

pylorus• hypertrophy develops over 4-6 weeks of life when symptoms begin to

appear• more common in Caucasian; first born, full term boys

Assessment:• olive size bulge under right rib cage• vomiting – projectile; non bilous• peristaltic waves during and after feeding• failure to thrive• dehydration• diagnostic tests:

a. UGIS – narrowing of diameter of pylorusb. Decreased serum Na, K, Clc. Increased Hctd. Metabolic alkalosis

Nursing intervention: Preop• replace fluids and electrolytes• prevent vomiting1. Give thickened feedings2. High Fowler’s3. Place on right side after feeding4. Minimize handling5. Strict I and O, daily weights, urine sp gravity

Nursing Intervention: post Op• Advance diet as tolerated• Place on right side after feeding• Observe incision for signs of infection• Provide client teaching and discharge planning

INTUSSUSCEPTION• telescoping of bowel into itself • ileocecal region• edema, necrosis of bowel, obstruction• most common at age 6 months• more in boys than in girls• associated with cystic fibrosis

Assessment:• piercing cry• severe abdominal pain (pulls leg up)• vomiting of bile stained fluid• bloody mucus in stool• “currant jelly” stool

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Management:1. barium enema2. surgery

Nursing Intervention:1. provide routine pre and post op care2. monitor for peritonitis

HIRSCHSPRUNG’S DISEASE• absence of autonomic parasympathetic ganglion cells in large intestines• results in decreased motility in that portion of the colon and signs of

functional obstruction

Assessment:• failure or delay in passing meconium• abdominal distension• failure to pass stool• temporary relief following rectal exam• loose stools – only liquid can pass thru• ribbon like stools• nausea, anorexia, fecal vomiting• weight loss, failure to grow• volvulus – bowel twists upon itself• diagnostic tests: rectal biopsy – confirms presence of aganglionic cells•

Nursing intervention:• enema as ordered

A. Mineral oil or isotonic salineB. Do not use water or soap suds – water intoxicationC. Use volume appropriate to weight of child

infants – 150-200mlchildren – 250-500 ml

• administer TPN as ordered• Provide low residue diet• provide client teaching and discharge teaching• Colostomy care• Low residue diet

IMPERFORATE ANUS• during embryonic life – membrane that separates the rectum and anus is

absorbed• absorption fails to occur -> imperforate anus• anus appear as a dimple• fistula may be present – rectovaginal, rectourethral

Assessment:• inspection of anus, check rectum for patency• insert rectal thermometer• absence of stool during 1st 24 hours• development of a distended abdomen

Intervention:• surgery

anoplasty – if possibletemporary colostomy

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• if with distension – nasogastric suction

Nursing intervention:• keep operative site clean• observe infant for signs of hypovolemic shock and infection• if anoplasty heals – regular dilatation of anus to prevent stricture• colostomy care

a. Kept cleanb. zinc oxide

• after surgery – side lying or prone with hips elevated

CELIAC DISEASE• malabsorption syndrome characterized by intolerance of gluten,

found in rye, oats, wheat and barley• familial disease• more common in Caucasians• cause unknown• flat mucosal surface and atrophy of villi of the intestine• reduced absorptive surface -> marked malabsorption of fats

Assessment:• steatorrhea• chronic diarrhea• failure to thrive• distended abdomen• abdominal pain, irritability, listlessness, vomiting• symptoms of ADEK deficiency• diagnostic tests:

• 1. pancreatic enzymes• 2. jejunal or duodenal biopsies

Management:• diet therapy• gluten free diet• TPN in children

Nursing Intervention:• gluten free diet (BROW – Barley, Rye, Oatmeal, Wheat)• supplemental fat soluble vitamins• client teaching• 1. gluten free diet• 2. importance of reading the label• 3. avoidance of infection• 4. adhering to diet even if symptoms are controlled• 5. importance of long term follow up

APPENDICITIS• inflammation of the appendix• ischemia, gangrene, rupture, peritonitis – if untreated• school age children – most common• due to mechanical obstruction or anatomic defects

Assessment:• diffuse pain à RLQ• nausea, vomiting• guarding of abdomen• rebound tenderness

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• decreased bowel sounds• fever• diagnostic tests:

1. CBC2. elevated acetone in urine

Nursing Intervention:• antibiotics/ antipyretics as ordered• no enemas• no heating pads• routine preop care• post op care:1. semi-Fowler’s position2. Monitor NGT3. Monitor penrose drains

PARASITISM• roundworms, pinworms

Assessment:• pinworms – anal itching, disturbed sleep• roundworms – colic , abdominal pain, lack of appetite, weight loss

Nursing intervention:• obtain stool culture• observe all excreta for worms• Scoth tape swab • instruct parents to change clothing, bed linens, towels and launder in hot

water• instruct all family members to scrub hands and fingernails prior to eating

and after using toilet

DIABETES MELLITUS• most common endocrine disease of children• onset maybe any age• type 1 IDDM• risk of complication is high – retinopathy, neuropathy, nephropathy,

skin changes, predisposition to infection

Assessment:• rapid onset• polyuria, polydipsia, polyphagia, fatigue• weight loss• ketoacidosis

-Dry flushed skin

Management:• insulin• diet therapy• exercise• prevention of complications

Nursing intervention:• administer insulin as ordered• force fluids without sugar• monitor blood glucose levels daily

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• observe for hypoglycemia: behavior changes, sweating• provide client teaching

a. Daily regimen for home careb. Urine and blood glucose monitoringc. Nutrition managementd. Effects of infection and exercise on CHO metabolisme. Prevention of acute and chronic complications

RESPIRATORY SYSTEM

EPIGLOTTITIS• inflammation of the epiglottis• H. influenzae Type b ; Strep pneumoniae• age 2-5• abrupt onset; winter• considered an emergency situation

Assessment:• fever• sore, red and inflamed throat• drooling, difficulty of swallowing• inspiratory stridor• muffled voice• nasal flaring

Implementation• maintain patent airway• assess respiratory status and breath sounds• use of accessory muscles; presence of stridor• assess temperature• do not attempt to visualize the posterior pharynx or obtain throat culture• lateral neck film• do not force child to lie down• do not restrain• administer antibiotics; IV fluids • cool mist oxygen• high humidification • have resuscitation equipment available• immunization

LARYNGOTRACHEOBRONCHITIS (LTB)• inflammation of the larynx, trachea, bronchi• most common type of croup; maybe viral or bacterial• parainfluenza virus• gradual onset ; preceded by URI

Assessment:• fever• irritability and restlessness• hoarse voice• inspiratory stridor and suprasternal retractions• crackles and wheezing• cyanosis

Implementation:• patent airway• vital signs• elevate HOB

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• humidified oxygen; IVF• nebulized epinephrine• resuscitation equipments available

BRONCHITIS• infection of the major bronchi• assessment: cough, worse at night; becomes productive in 2-3 days• Implementation:1. Monitor for respiratory distress2. Increased fluid intake3. Acetaminophen

BRONCHIOLITIS/RESPIRATORY SYNCYTIAL VIRUS (RSV)• Inflammation of the bronchioles; production of mucus that occludes

bronchiole tubes and small bronchi• RSV – highly communicable, usually transferred by hands

Assessment:• URI• Lethargy, poor feeding, tachypnea• Expiratory wheezes and grunt• Diminished breath sounds

The child with RSV:• isolate in a single room or place with same patient• good handwashing• nurses caring for these children do not care for other high risk children• wear gowns when soiling may occur• ribavirin – may be aerosol• nurses wearing contact lenses must wear goggles – ribavirin may

dissolvecontact lenses

PNEUMONIA• inflammation of the alveoli• inhalation of causative agent à bloodstream• Primary atypical pneumonia – most common cause of pneumonia

between 5-12 years

Implementation:• antimicrobial therapy• oxygen• cool humidification• encourage child to lie on the affected side• isolation procedures as needed• antipyretics• IVF; liberal fluid intake

ASTHMA• chronic inflammatory disease of airways• commonly caused by physical and chemical irritants common symptoms

– coughing in the absence of respiratory infection,especially at night

Assessment:• episodes of wheezing, breathlessness, dyspnea, chest tightness

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• SOB, cough, wheezing• child speaks in short, broken phrases• retractions• exercise induced bronchospasm• severe spasm or obstruction

Implementation• continuously monitor respiratory status• administer quick-relief (rescue) medication• initiate an IV line; prepare to correct dehydration, acidosis or electrolyte

imbalance

Medications1. Quick relief• To treat symptoms and exacerbations• Short acting b2 agonists• Anticholinergics – for relief of acute bronchospasm (ipratropium bromide)

2. Long term control• Corticosteroids• Long acting b2 agonist• Long acting bronchodilator

3. Nebulizer, MDI• Used to deliver many medications• Non-CFC (chlorofluorocarbon) – albuterol

4. Chest physiotherapy• Includes breathing exercises

Home care measures:• instruct in measures to eliminate allergens• avoid extremes of environmental temperature• avoid exposure to individuals with a viral infection• instruct the child in how to recognize early symptoms • instruct the child in the cleaning of devices used for inhaled medications• keep immunization up to date

OTITIS MEDIA• Infection of the middle ear occuring as a result of a blocked eustachian

tube, preventing normal drainage• common complication of ARI• infants and children more prone – ET shorter, wider, straighter

Assessment:• fever• irritability and restlessness• rolling of head from side to side• pulling or rubbing the ear• earache; signs of hearing loss• purulent ear discharge• otoscopic exam

Intervention• Encourage fluids• upright position when feeding• avoid chewing – increases pain

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• have the child lie with the affected ear down• instruct on appropriate technique to clean drainage from the ear with

sterile cotton swabs• administer analgesics and antibiotics (10-14 days)• screening for hearing loss• otic medications 1. If younger than age 3 – auditory canal is straightened by pulling the pinna

down and back2. If older than 3 years – pull pinna up and back

Myringotomy• Insertion of tympanoplasty tubes into the middle ear to equalize pressure

and keep ear aerated• Keep ears dry• Earplugs should be worn during bathing, shampooing, swimming

TONSILLECTOMY AND ADENOIDECTOMYAssessment:• persistent or recurrent sore throat• enlarged bright red tonsils ; with exudates at times• dysphagia• mouth breathing; unpleasant mouth odor• fever; cough

Preop:• assess for presence of infection• assess bleeding and clotting studies• assess for loose teeth• prepare the child for a sore throat

Postoperatively:

• Position – prone or side lying to facilitate drainage• have suction equipment available• monitor for signs of hemorrhage• may give apply ice collar• discourage coughing or clearing the throat• provide clear, cool, noncitrus and non carbonated drinks• avoid milk products intially – will coat the throat• avoid red liquids• do not give child any straw, forks, or sharp objects• administer paracetamol as ordered• instruct parents to notify MD if bleeding, persistent earache or fever

occurs• instruct parents to keep child away from crowds until healing occurred

HEMATOLOGIC SYSTEM

LEUKEMIA – the most frequent type of childhood cancerBrain tumors – 2nd

Etiology: 1. Environmental

2. Viruses3. Familial/genetic4. Host factors

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STAGES OF TREATMENT1. INDUCTION• Goal: to remove bulk of tumor• Methods: surgery, radiotherapy, chemotherapy, BM transplant• Effects: often the most intensive phase• Side effects are potentially life threatening

2. CONSOLIDATION• Goal: to eliminated any remaining malignant cells• Methods: chemo/radio• Side effects will still be evident

3. MAINTENANCE• Goal: to keep the child disease free• Chemotherapy • This phase may last several years

4. OBSERVATION• Goal: to monitor the child at intervals for evidence of recurrent

disease and complications of treatment• Method: treatment is complete• Child may continue in this stage indefinitely

5. LATE EFFECTS OF TREATMENT• Impaired growth and development• CNS damage• Impaired pubertal development• Development of secondary malignancy• Psychologic problems related to living with a life threatening disease and

complex treatment regimen•

NURSING INTERVENTION1. Help child cope with intrusive procedures• Provide information geared to developmental level and emotional

readiness• Explain what is going to happen, why it is necessary, how it will feel• Allow child to handle to handle and manipulate equipments• Allow child some control in situations

- positioning, selecting injection site

2. Support child and parents• Maintain frequent clinical conferences to keep all informed• Always tell the truth• Acknowledge feelings and encourage child/family to express them• Provide contact with another parent or support group

3. Minimize side effects of treatmenta. Skin breakdown• Keep clean and dry; wash with warm water; no soaps or creams• Do not wash off radiation marks• Avoid all topical agents with alcohol• Do not use heating pads or hot water bottle

b. Bone marrow suppression• Provide frequent rest periods

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• Avoid crowds• Evaluate any potential site of infection• Monitor temperature• Avoid use of aspirin• Select activities that are physically safe

c. Nausea and vomiting• Administer antiemetic at least half an hour before chemotherapy• Eat light meal prior to administration of therapy• Administer IVF if needed

d. Alopecia• Reduce trauma of hair loss• Buy wig before hair falls out• Discuss various head coverings • Avoid exposing head to sunlight

4. Nutrition deficits• Establish baseline• Provide high calorie, high protein

5. Developmental delay• Facilitate return to school as soon as possible• Discuss limit setting, discipline

LEUKEMIA• most common form of childhood cancer• peak 3-5 years• proliferation of abnormal wbc that do not mature beyond the blast phase• blast cells – infiltrate other organs – liver, spleen, lymph tissue

Types:1. Acute Lymphocytic leukemia (ALL)• 80-85% • Acute• 75% chance of surviving

2. Acute nonlymphocytic leukemia• Includes granulocytic and monocytic types• 60-80% will obtain remission• 30-40% cure rate

Assessment:1. anemia, weakness, pallor, dyspnea2. Bleeding, petecchiae, spontaneous bleeding, ecchymoses3. Infection, fever, malaise4. Enlarged lymph node5. Enlarged spleen and liver6. Bone pain

Management:1. diagnosis: blood studies, BMA2. Treatment stagesa. Inductionb. CNS prophylaxis

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c. Maintenance

Nursing Intervention:1. Provide care for the child receiving chemo and radiotherapy2. Provide support for the family/child3. Support child during painful proceduresa. Use distraction, guided imageryb. Allow the child to retain as much control as possiblec. Administer sedation prior to procedure as ordered

Sickle Cell Disease• hemoglobin A is partly or completely replaced by abnormal sickle

hemoglobin (HgbS)• HgbS sensitive to changes in the oxygen content of the red blood cell• Risk factors: African American• Insufficient oxygen causes the cells to assume a sickle shape and the

cells become rigid and clumped together, obstructing capillary blood flow• The sickling response – reversible under adequate oxygenation• Sickle cell crises – vaso-occlusive crisis, splenic sequestration, aplastic

crisis

Assessment:1. Vaso-occlusive crisis• Most common type of crisis• Caused by stasis of blood with clumping of the

cells in microcirculation, ischemia and infraction• Fever, pain and tissue engorgement

2. Splenic sequestration• Pooling of blood in the spleen• Profound anemia, hypovolemia, shock

3. Aplastic crisis• Caused by the diminished production and increased destruction of RBCs,

triggered by viral infection or the depletion of folic acid • Profound anemia and pallor

Implementation:• Administer oxygen and blood transfusions• administer analgesics• maintain adequate hydration and blood flow with IV normal saline as

prescribed and with oralfluids

• Assist the child to assume a comfortable position so that the child keeps the extremities extendedto bed no more than 30 degrees

• avoid putting strain on painful joints• encourage consumption of a high calorie, high protein diet with folic acid

supplementation• administer antibiotics as prescribed• Monitor for signs of increasing anemia and shock (pallor, vital sign

changes) IRON DEFICIENCY ANEMIA

• Iron stores are depleted, resulting in a decreased supply of iron for the manufacture for the

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manufacture of hemoglobin in RBCs• Results from blood loss, increased metabolic demands, syndromes of GI

malabsorption, dietaryinadequacy

Assessment:• pallor• weakness and fatigue• irritability

Implementation:• Increase the oral intake of iron• Instruct the child and parents in food choices that are high in iron• Administer iron supplements as prescribed• Liquid iron prep stains – stains teeth; with straw• side effects of iron therapy

APLASTIC ANEMIA• a deficiency of circulating erythrocytes resulting from the arrested

development of RBCs within the bone marrow• causes: exposure to toxic agents, viruses, infection, autoimmune

disorders, allergic states• definitive diagnosis: BMA• Therapeutic management: blood transfusions, splenectomy,

corticosteroids, immunosuppressive therapy, bone marrow transplantation

Assessment• Pancytopenia • Petecchiae, purpura, bleeding, pallor, weakness, tachycardia and

fatigue

Implementation:• Blood transfusion• corticosteroids and immunosuppresives• splenectomy• bone marrow transplant• Medic Alert bracelet

HEMOPHILIA• x linked recessive trait• Hemophilia A – deficiency of Factor VIII• Hemophilia B – deficiency of factor IX• Males inherit hemophilia from their mothers and females inherit the

carrier status from theirfathers

Assessment:• prolonged bleeding after minor injury1. At birth after cutting cord2. Following circumcision3. Following IM immunization4. Increase bruising as child learns to crawl and walk• abnormal bleeding in response to trauma • joint bleeding – pain, tenderness, swelling limited range of motion• tendency to bruise easily• prolonged PTT

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• normal BT, PT, platelet count

Implementation:• prepare to administer Factor VIII concentrate /cryoprecipitatea. Thaw slowlyb. Gently rotate bottlec. Infuse immediately; deteriorates at room temperature• monitor for bleeding• monitor for joint pain; immobilize the affected extremity if joint pain

occurs• assess neurological status ( child at risk for IC bleed)• monitor urine for hematuria• Control bleeding by immobilization, elevation, application of ice; apply

pressure (15 mins)for superficial bleeding

• instruct parents regarding activities of the child• avoidance of contact sports

BETA THALASSEMIA MAJOR• Cooley’s anemia• autosomal recessive disorder• characterized by reduced production of one of the globin chains in the

synthesis of hemoglobin• incidence highest in Mediterranean descent

Assessment:• severe anemia• pallor• failure to thrive• hepatosplenomegaly• microcytic, hypochromic RBCs

Implementation• Instruct the administration of folic acid (Vit B9)• administer blood transfusion as prescribed• Monitor for iron overload• iron overload – chelation therapy with deferoxamine• genetic counseling

IDIOPATHIC THROMBOCYTOPENIC PURPURA• increased destruction of platelets with resultant platelet count less than

100,000/mm3• characterized by petechiae and ecchymoses of skin• exact mechanism unknown• often preceded by a viral illness• spleen not enlarge

Assessment:• petechiae: spider web appearance of bleeding under the skin due to

small size of platelets• ecchymosis• blood in any body secretions, bleeding from mucus membranes,

nosebleeds• diagnostic test: platelet count decreases, anemia

Management:

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• steroids and immunosuppressives• platelet transfusion• surgery; splenectomy

Nursing Intervention• control bleeding1. Administer platelet transfusion as ordered2. Apply pressure to bleeding sites as needed3. Position bleeding part above heart level if possible

• Prevent bruising• Protect from infection• Measure normal circumference of extremities for baseline• Administer meds orally, rectally or IV rather than IM; hold pressure on site

for 5 mins• Avoid aspirin• Provide client teaching and discharge planning1. Pad crib and playpen2. Provide soft toys3. Provide protective headgear during toddlerhood4. Use soft toothbrush5. Avoid contact sports

INTEGUMENTARY SYSTEM

ECZEMA• atopic dermatitis - often the first sign of an allergic predisposition in a

child• usually manifests during infancy

Asssessment:• erythema, weeping vesicles that rupture and crusts• severe pruritus; scratching causes thickening and darkening• dry skin, sometimes urticaria

Intervention:• topical steroids• antihistamines• coal tar preparation• colloid baths• diet therapy: elimination of offending food

Nursing Intervention:• avoid heat and prevent sweating• check materials in contact with child’s skin (sheets, lotions, soap)• avoid frequent baths• avoid use of soap• provide lubricant immediately after bath• administer topical steroids as ordered• use cotton instead of wool• keep child’s nails short; use elbow or glove restraints if needed• apply wet saline or Burrow’s solution compresses

DIAPER RASH• contact dermatitis• plastic/rubber pants and linings of disposable diapers

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exacerbate the condition by prolonging contact withmoist, warm environment

• skin further irritated by acidic urine

Assessment:• erythema/excoriation in the perineal area• irritability

Nursing intervention:• keep area clean and dry; clean with mild soap and water after each stool

and soon as child urinates• take off diaper and expose area to air during the day• client teaching:1. Proper hygiene2. Avoid use of plastic pants or disposable diapers with a plastic lining3. Avoid commercially prepared diaper wipes containing alcohols4. Avoid cornstarch; good medium for bacteria

MUSCULOSKELETAL SYSTEM

Care of the child with a Cast

• if cast is of plaster – will remain wet for at least 24 hrs• use only the flats of their hands to move children• casts must remain open to the air until dry• casted extremities are elevated to help blood return and reduce swelling• Initial chemical hardening reaction may cause a change in an infant’s

body• Choose toys too big to fit down cast• do not use baby powder near cast – medium for bacteria• prepare for anticipated casting by having child help apply cast in a doll

Diagnoses and interventions:

1. Potential for alteration in tissue perfusion related to constriction of cast• Check color, sensation and motion distal to the cast every half hour• Check pedal or radial pulse• Check for tightness by slipping finger under edge; if impossible – cast is

too tight• Ask child to move toes or fingers• Elevate casted extremity

2. Potential for alteration in skin integrity• Remove plaster flakes from skin• Handle wet cast carefully so as not to cause indentations• Expose wet cast to air to hasten drying• Support heavy cast with sling or pillow to decrease pressure of cast edges• Check cast for foul or musty odors

3. Potential for fear and loneliness• Encourage expression of feelings• Provide diversional play• Encourage friends and family to visit children as often • Provide educational opportunity for children confined for long periods

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4. Potential for knowledge deficit of family• Encourage discussion of feelings and fears• Provide information and reassurance as appropriate• Involve family in child’s care in hospital• Prepare family for some emotional regression

CONGENITAL HIP DISLOCATION• displacement of the head of the femur from the acetabulum• present at birth although not always diagnosed• familial disorder• unknown cause; may be fetal position in utero• acetabulum is shallow and the head of femur is cartilaginous at birth

Assessment:• maybe unilateral or bilateral• limitation of abduction (cannot spread legs to change diaper)• Ortolani’s clicka. With an infant supine, bend knees and place thumb on bent knees,

fingers at hip jointb. Bring femur 90degrees to hip, then abductc. Palpable click – dislocation• Barlow’s testa. With infant on back, bend kneesb. Affected knee will be lower because the head of the femur dislocates

towards the bed of gravity• additional skin folds with knees bent• when lying on abdomen, buttocks of affected side will be flatter• Trendelenburg test – if child can walka. Have child stand on affected leg onlyb. Pelvis will dip on normal side as child attempts to stay erect

Management:• Goal : to enlarge and deepen the socket• Early treatment: positioning the hip in abduction with the head of the

femur in the acetabulum and maintaining it in position for several months• Traction and casting (hip spica)• Surgery

Nursing intervention:• Maintain proper positioning: keep legs abducted1. Use triple diapering2. Use Frejka pillow splint (jumperlike suit to keep legs abducted)3. Place infant on abdomen with legs in “frog” position4. Use immobilization devices• Provide adequate nutrition• Provide sensory stimulation• Client teaching and discharge planning:

CLUBFOOT (Talipes)• abnormal rotation of foot at ankle

Varus – inward rotation; bottom of feet face each otherValgus – outward rotationCalcaneous – upward rotation; would walk on heelsEquinas – downward rotation; would walk on toes

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Most common – talipes equinovarus

Assessment: • foot cannot be manipulated by passive exercises into correct position

Management:• exercises• casting• Denis Browne splint (bar shoe)• surgery and casting

Nursing Intervention:• perform exercises as ordered• provide cast care• child who is learning to walk must be prevented from trying to stand;

apply restraints if necessary• provide diversional activities• provide skin care• client teaching

SCOLIOSIS• lateral curvature of the spine• most commonly in adolescent girls• familial pattern; associated with other nueromuscular condition• idiopathic majority

Assessment:• failure of curve to straighten when child bends forward with knees

straight and arms hanging down feet• uneven bra strap marks• uneven hips• uneven shoulders• asymmetry of rib cage• xray: reveals curvature

Management:• stretching exercises• Milwaukee brace – worn 23 hours/day for 3 years• plaster jacket vest• spinal fusion•

Nursing Intervention:• teach/encourage exercise• provide care for the child with Milwaukee bracea. Child wears brace 23 hours/dayb. Monitor pressure pointsc. Promote positive body image with brace• Provide cast care• Assist with modifying clothing for immobilization devices• Adjust diet with decreased activity• Provide client teaching and discharge instructionsa. Exerciseb. Cast carec. Correct body mechanicsd. Alternative education for long term hospitalizatione. Availability of community agencies

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JUVENILE RHEUMATOID ARTHRITIS• systemic, chronic disorder of connective tissue• autoimmune reaction• results from eventual joint destruction• affected by stress, climate and genetics

Types:1. Monoarticular JRA• Fewer than 4 joints involved (usually legs)• Asymmetric• Good prognosis• Mild signs of arthritis

2. Polyarticular JRA• Multiple joints affected• Symmetrical• Involvement of TMJ• Remissions and exacerbations• poor prognosis3. Systemic disease with polyarthritis (Still’s disease)• Fever, rash, LADP, anorexia, weight loss• Exacerbations and remissions

Assessment:• No specific diagnostic tests• ESR, ASO, RF- not specific

Intervention• Drugs: ASA, corticosteroids• NSAIDS• Physical therapy – strengthening muscles, preventing deformities• Splints – used for knees, wrists and hands – to reduce pain and prevent

or reduce flexion deformities

Nursing intervention:

• Assess joints for pain, swelling, tenderness• promote maintenance of joint mobility

a. ROM exercisesb. Isometric exercises

• change position frequently; alternate sitting, standing, lying• promote comfort and relief• provide firm mattress• maintain proper body alignment• keep joints mainly in extension, not flexion• cold treatments: in acute episodes• focus on child’s strength

GENITOURINARY SYSTEM

• Nephrons continue to develop after birth• GFR is 30% below adult level at birth• reaches normal level by age 2 years• tubular function immature at birth; reach adult level

by age 2 years• urethra shorter in children and more prone to ascending

infection

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UNDESCENDED TESTES (Cryptorchidism)• unilateral or bilateral absence of testes in scrotal sac• testes normally descend at 8 months of gestation• increased incidence in those with genetic disorder• 75% will descend spontaneously by age 1 year

Assessment:• unable to palpate testes in scrotal sac

Management:• if testes remains in abdomen, damage to testes is possible because of

increased• chorionic gonadotropin• orchipexy: surgical procedure to retrieve and secure testes placement;

performed between ages 1-3 yrs

Nursing Management:• provide treatment options• support parents if surgery will be done• post op: avoid disturbing the tension mechanism• avoid contamination of incision

HYPOSPADIAS• urethral opening located anywhere along the ventral surface of penis

Assessment:• urinary meatus misplaced• inability to make straight stream of urine

Management:• minimal defects – no intervention• neonatal circumcision delayed, tissue maybe needed for corrective repair• surgery at age 3-9 months

Nursing Intervention:• diaper normally• provide support for parents• post op: check pressure pressure dressing • monitor catheter drainage

PHIMOSIS• an abnormal narrowing of the foreskin so that it cannot be retracted over

the glans penis• may be present at birth or may develop as a result of poor hygiene with

accumulation of smegma

Management:• Prevention – regular pulling the foreskin back and cleaning• circumcision

Nursing intervention:• circumcision care:1. Close observation for bleeding2. Ice application3. Administration of analgesics

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ENURESIS• involuntary passage of urine after the age of control is expected (4 years)• types:1. Primary – in children who have never achieved control2. Secondary: in children who have developed complete

control• May occur anytime but most frequent at night• More common in boys• No organic cause

• Etiologic possibilities:1. Sleep disturbances2. Delayed neurologic development3. Immature development of bladder4. Psychologic problems

Assessment:• PE normal• History of repeated voluntary urination management:• bladder retention exercises• drug therapy1. TCA – imipramine2. Anticholinergics

Nursing Intervention:• Provide information/counseling to family:1. Confirm that this not conscious behavior and that the child is not

purposely misbehaving2. Assure parents that they are not responsible and that this is a relatively

common problem• Involve child in care; give praises and support with small accomplishments1. Age 5-6 years – can strip wet beddings2. Age 10-12 years – can do laundry and change bed• Avoid scolding and belittling the child

EXSTROPHY OF THE BLADDER• congenital malformation ; nonfusion of abdominal

and anterior walls of the bladder during embryologicdevelopment

• anterior surface of bladder lie open on abdominal wall

Assessment:• Associated structural changes1. Prolapsed rectum2. Inguinal hernia3. Widely split symphysis4. Rotated hips

• Associated anomalies1. Epispadias2. Cleft scrotum or clitoris3. Undescended testes4. Chordee (downward deflection of the penis)

Management:• reconstructive surgery

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• urinary diversion• delayed until 3-6 months

Nursing intervention: Preop:• Provide bladder care; prevent infectiona. Keep area as clean as possibleb. Change diaper frequently; keep loose fittingc. Wash with mild soap and waterd. Cover exposed bladder with vaseline gauze

Postop:• Design play activities to foster toddler’s need for autonomy

- child will be immobilized for extended period of time• Prevent trauma; as child gets older and more mobile,

trauma is more likely

NEPHROTIC SYNDROME• autoimmune process • structural alteration of glomerular membrane • results in increased permeabilityb to plasma proteins• course: exacerbations and remissions over a period of months to years• commonly affects preschoolers; boys>girls

Assessment:1. Proteinuria2. Hypoproteinemia3. Hyperlipidemia4. Dependent edema• Puffiness around the eyes in morning• Ascites• Scrotal edema• Ankle edema5. anorexia, vomiting, diarrhea6. Pallor, lethargy7. Hepatomegaly

Management:• drug therapy1. Corticosteroids2. Antibiotics3. Thiazide diuretics

Nursing Intervention:• Provide bed rest1. Conserve energy2. Find activities for quiet play

• Provide high protein, low sodium diet – during edema phase• Maintain skin integrity• Avoid IM injections – meds not absorbed in edematous tissues• Obtain morning urine for protein studies• Provide scrotal support• Monitor I and O, vital signs • Daily weight• Administer as ordered• Protect from sources of infection

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ACUTE GLOMERULONEPHRITIS• immune complex disease• beta hemolytic strep• More frequently in boys; 6-7 years• resolves in 14 days• self limiting

Assessment:1. History of strep infection (URTI or impetigo)2. Edema, anorexia, lethargy3. Hematuria or dark colored urine4. Fever5. Hypertension6. Diagnostic tests:• Urinalysis – rbc,wbc,protein, cellular casts• Urine specific gravity increased• BUN, crea increased• ESR elevated• Hgb, Hct decreased

Management:• antibiotics• Antihypertensives• digitalis – if with CHF• fluid restriction • peritoneal dialysis – if severe renal complication occurs

Nursing Management:1. Monitor I and O, BP

2. Weigh daily3. Provide diversional activity4. Provide client teaching and discharge planning• Medication administration• Prevention of infection• Signs of renal complications• Importance of long term follow up

WILM’S TUMOR (NEPHROBLASTOMA)• large, encapsulated tumor that develops in the renal parenchyma,• more frequently in left kidney• originates during fetal life• peak age : 1-3 years

Assessment:• non tender mass, usually midline near liver• hypertension• hematuria• test: IVP

Nursing intervention:1. Do not palpate abdomen – to avoid dissemination of CA cells2. Handle child carefully when bathing and giving care3. Provide care for the client with a nephrectomy4. Provide care for the child receiving chemotherapy

MALFORMATIONS OF THE NERVOUS SYSTEM

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Cranial Defects:1. Craniosynostosis• one or more of the sutures close too soon• ICP increases; interfeeres with normal brain growth à MR

Assessment: • suture lines of the skull manually palpated • Radiographs are made to confirm

2. Microcephaly• The brain fails to grow• May be due to a chromosomal defect or from drugs, toxins or radiation• MR 3. Hydrocephalus• Imbalance of CSF absorption or production• caused by malformations, tumors, hemorrhage, infection, trauma

Types:1. Communicating – impaired absorption within arachnoid space2. Non-communicating – obstruction of CSF flow within the ventricular

system

Assessment:• Infant – increased HC• Macewen’s sign – cracked-pot sound on percussion of bones of head• Anterior fontanel tense, bulging• Scalp veins dilated• Frontal bossing, sunsetting eyes• Child – behavior changes• Headache, nausea and vomiting• Ataxia, nystagmus

Surgical Implementation: Hydrocephalus

Goal: to prevent further CSF accumulation by bypassing the blockage anddraining the fluid from the ventricles to a location where it may be reabsorbed

1. VP Shunt – CSF drains into the peritoneal cavity from the lateral ventricle2. AV shunt – CSF drains into the right atrium

PostOP Care:1. Keep child flat as prescribed – to avoid rapid reduction of intracranial fluid2. Observe increase ICP – if present, elevate HOB 15-30 deg3. Monitor for infection4. Measure HC5. Monitor I and O6. Provide comfort measures; administer medications (diuretics, antibiotics,

or anticonvulsants)7. Toddler – headache and anorexia à earliest common signs of shunt

malfunction

SPINA BIFIDA• CNS defect that occurs as a result of neural tube failure to

close during embryonic development

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• defect closure usually done during infancy

Types:1. Spina bifida occulta• Posterior vertebral arches fail to close in the lumbosacral area• Spinal cord intact; not visible• Meninges not exposed on the skin surfaces2. Meningocoele• Protrusion involves meninges and a sac-like cyst• Lumbosacral area3. Myelomeningocoele• Protrusion of meninges, CSF, nerve roots, portion of spinal cord• Sac covered by a thin membrane à may rupture or leak• Neuro deficit evidence

Assessment:• Depends on spinal cord involvement• Visible spinal defect• Flaccid paralysis of legs• Altered bladder and bowel function

Implementation:• Evaluate sac; measure lesion• neuro check• monitor for increase ICP• measure HC; assess fontanelles• Protect the sac

1. Cover with sterile, moist (normal saline) non-adherentdressing

2. Change dressing every 2-4 hours • Place prone position• head is turned to one side for feeding• diapering may be C/I until defect repaired• Aseptic technique• Watch for early signs of infection• Administer antibiotics• Administer anticholinergics – improve urinary continence• Administer laxatives , antispasmodics

MENINGITIS• infectious process of the CNS caused by bacteria and viruses• acquired as a primary or as a result of complications• diagnosis – CSF analysis (increase pressure, cloudy CSF, high protein,

low glucose• bacterial or viral

Assessment:• signs and symptoms vary depending of age group• fever, chills• vomiting, diarrhea• poor feeding or anorexia• altered LOC• bulging anterior fontanel• nuchal rigidity

Implementation:• isolation; maintain for at least 24 hours after antibiotics are initiated

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• administer antibiotics as prescribed• monitor VS and neuro status• Monitor I and O• assess nutritional status• determine close contacts of the child with meningitis

SEIZURE DISORDERS• Sudden transient alterations in brain function resulting from excessive

levels of electrical activity in the brain

Assessment:• obtain information from parents about the time of onset, precipitating

events and behavior before and after the seizure• seizure precautions:1. Raise side rails2. Pad side rails3. Place waterproof mattress on bed4. Instruct child to swim with companion5. Alert caregivers to the need for special precautions

Emergency Treatment for Seizures:• Ensure patency of airways• If the child is standing or sitting, ease the child down to the floor• place pillow or folded blanket under the child’s head• loosen restrictive clothing• clear area of any hazards• if vomiting occurs, turn child to one side• do not restrain child; do not place anything in the child’smouth• Remain with the child until fully recovers• Prepare to administer medications

CEREBRAL PALSY• disorder characterized by impaired movement and posture resulting from

an abnormality in the extrapyramidal motor system• spastic type- most common

Assessment:• extreme irritability and crying• feeding difficulties• stiff and rigid arms and legs• delayed gross development• abnormal motor performance• alterations of muscle tone• abnormal posturing• persistence of primitive reflexes

Implementattion:• early recognition• PT, OT, speech therapy, eduaction and recreation• assess the child’s developmental level and intelligence• early intervention• encourage communication and interaction with the child on a functional

level• provide safe environment• position upright after meals

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• provide safe, appropriate toys for age and developmental level