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  • 8/3/2019 Systemic Bullets




    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


    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


    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


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


    aorta arises from the right ventricle degree of cyanosis depends on the abnormal connections

    Assessment: cyanotic at birth

    develop polycythemia 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

    slightlymore 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


    an inflammatory autoimmune disease affects connective tissue of the heart, joints, subcutaneous tissues andblood 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


    Physiology of DIGESTIVE SYSTEM

    Mechanical functions of digestion immature at birth

    No voluntary control over swallowing until 6 weeks

    Stomach capacity decreasedRelaxed sphincter contributes to tendency to regurgitate

    Liver functions immature throughout infancy

    Gastric acidity low in infants rises until age 10

    Digestive processes are mature by toddlerhood


    Presenting problem

    a. Vomitingb. Abnormal bowel habits: diarrhea, constipation, bleeding

    c. 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


    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)



    erosion of the enamel and dentine of teeth

    results from combination of fermenting sugars and starched

    and acid forming organisms


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    dental hygiene

    regular dental hygiene

    good nutrition



    1 in 1000 births


    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 childs capacity to meet oxygenation and

    nutritional needs

    may seriously hamper normal bonding process of children with parents


    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


    team approach

    otolaryngologist, audiologist, pediatrician, dentist, orthodontist, speech

    therapist Surgical correction:

    early correction prevent speech defects


    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


    Cleft Lip (PreOP)

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    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

    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 line

    1. Use cups only for liquids; no bottles2. avoid straws, utensils, popsicle sticks, chewing gum3. provide soft toys

    4. Use elbow and wrist restraints5. Provide liquid diet initially soft normal diet6. Give water after each feeding to clean suture line7. Hold and cuddle


    Esophageal atresia

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



    defect in which embryonic structures fail to divide into a separate

    esophagus and trachea

    opening between the two structures

    usually occur together

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    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

    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


    narrowing of the outlet of the stomach

    caused by excessive growth of circular muscles that surrounds the


    hypertrophy develops over 4-6 weeks of life when symptoms begin toappear

    more common in Caucasian; first born, full term boys


    olive size bulge under right rib cage

    vomiting projectile; non bilous

    peristaltic waves during and after feeding

    failure to thrive


    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 vomiting

    1. Give thickened feedings2. High Fowlers

    3. 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

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    Observe incision for signs of infection

    Provide client teaching and discharge planning


    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


    piercing cry

    severe abdominal pain (pulls leg up)

    vomiting of bile stained fluid

    bloody mucus in stool

    currant jelly stool

    Management:1. barium enema2. surgery

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


    absence of autonomic parasympathetic ganglion cells in large intestines

    results in decreased motility in that portion of the colon and signs of

    functional obstruction


    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 orderedA. Mineral oil or isotonic saline

    B. 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

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    Provide low residue diet

    provide client teaching and discharge teaching

    Colostomy care

    Low residue diet


    during embryonic life membrane that separates the rectum and anus is


    absorption fails to occur -> imperforate anus

    anus appear as a dimple

    fistula may be present rectovaginal, rectourethral


    inspection of anus, check rectum for patency

    insert rectal thermometer absence of stool during 1st 24 hours

    development of a distended abdomen



    anoplasty if possibletemporary colostomy

    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


    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



    chronic diarrhea failure to thrive

    distended abdomen

    abdominal pain, irritability, listlessness, vomiting

    symptoms of ADEK deficiency

    diagnostic tests:

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    1. pancreatic enzymes

    2. jejunal or duodenal biopsies


    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


    inflammation of the appendix

    ischemia, gangrene, rupture, peritonitis if untreated

    school age children most common

    due to mechanical obstruction or anatomic defects


    diffuse pain RLQ

    nausea, vomiting

    guarding of abdomen

    rebound tenderness

    decreased bowel sounds


    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-Fowlers position2. Monitor NGT3. Monitor penrose drains


    roundworms, pinworms


    pinworms anal itching, disturbed sleep

    roundworms colic , abdominal pain, lack of appetite, weight loss

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    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


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

    and after using toilet


    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


    rapid onset

    polyuria, polydipsia, polyphagia, fatigue

    weight loss


    -Dry flushed skin



    diet therapy


    prevention of complications

    Nursing intervention:

    administer insulin as ordered

    force fluids without sugar

    monitor blood glucose levels daily

    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



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

    Assessment: fever

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    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 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

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    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 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


    episodes of wheezing, breathlessness, dyspnea, chest tightness 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


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

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    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


    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 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 medications1. 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


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    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


    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


    instruct parents to keep child away from crowds until healing occurred


    LEUKEMIA the most frequent type of childhood cancerBrain tumors 2nd

    Etiology:1. Environmental

    2. Viruses3. Familial/genetic

    4. Host factors


    Goal: to remove bulk of tumor

    Methods: surgery, radiotherapy, chemotherapy, BM transplant

    Effects: often the most intensive phase

    Side effects are potentially life threatening


    Goal: to eliminated any remaining malignant cells

    Methods: chemo/radio

    Side effects will still be evident


    Goal: to keep the child disease free


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    This phase may last several years


    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


    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


    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

    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

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    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


    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)



    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, dyspnea

    2. 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

    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 imagery

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    b. 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 thecells become rigid and clumped together, obstructing capillary blood flow

    The sickling response reversible under adequate oxygenation

    Sickle cell crises vaso-occlusive crisis, splenic sequestration, aplasticcrisis

    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


    Administer oxygen and blood transfusions

    administer analgesics

    maintain adequate hydration and blood flow with IV normal saline asprescribed and with oralfluids

    Assist the child to assume a comfortable position so that the child keepsthe 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 acidsupplementation

    administer antibiotics as prescribed

    Monitor for signs of increasing anemia and shock (pallor, vital signchanges)


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

    manufacture for themanufacture of hemoglobin in RBCs

    Results from blood loss, increased metabolic demands, syndromes of GImalabsorption, dietaryinadequacy



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    weakness and fatigue



    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


    a deficiency of circulating erythrocytes resulting from the arresteddevelopment of RBCs within the bone marrow

    causes: exposure to toxic agents, viruses, infection, autoimmunedisorders, allergic states

    definitive diagnosis: BMA

    Therapeutic management: blood transfusions, splenectomy,corticosteroids, immunosuppressive therapy, bone marrow transplantation



    Petecchiae, purpura, bleeding, pallor, weakness, tachycardia andfatigue


    Blood transfusion

    corticosteroids and immunosuppresives


    bone marrow transplant

    Medic Alert bracelet


    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 thecarrier status from their



    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 normal BT, PT, platelet count


    prepare to administer Factor VIII concentrate /cryoprecipitatea. Thaw slowlyb. Gently rotate bottle

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    c. Infuse immediately; deteriorates at room temperature

    monitor for bleeding

    monitor for joint pain; immobilize the affected extremity if joint painoccurs

    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


    Cooleys anemia

    autosomal recessive disorder

    characterized by reduced production of one of the globin chains in the

    synthesis of hemoglobin incidence highest in Mediterranean descent


    severe anemia


    failure to thrive


    microcytic, hypochromic RBCs


    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


    increased destruction of platelets with resultant platelet count less than100,000/mm3

    characterized by petechiae and ecchymoses of skin

    exact mechanism unknown often preceded by a viral illness

    spleen not enlarge


    petechiae: spider web appearance of bleeding under the skin due tosmall size of platelets


    blood in any body secretions, bleeding from mucus membranes,nosebleeds

    diagnostic test: platelet count decreases, anemia


    steroids and immunosuppressives

    platelet transfusion

    surgery; splenectomy

    Nursing Intervention

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    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 sitefor 5 mins

    Avoid aspirin

    Provide client teaching and discharge planning1. Pad crib and playpen2. Provide soft toys3. Provide protective headgear during toddlerhood4. Use soft toothbrush

    5. Avoid contact sports



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


    usually manifests during infancy


    erythema, weeping vesicles that rupture and crusts

    severe pruritus; scratching causes thickening and darkening

    dry skin, sometimes urticaria


    topical steroids


    coal tar preparation

    colloid baths

    diet therapy: elimination of offending food

    Nursing Intervention:

    avoid heat and prevent sweating

    check materials in contact with childs 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 childs nails short; use elbow or glove restraints if needed apply wet saline or Burrows solution compresses


    contact dermatitis

    plastic/rubber pants and linings of disposable diapers

    exacerbate the condition by prolonging contact with

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    moist, warm environment

    skin further irritated by acidic urine


    erythema/excoriation in the perineal area


    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 alcohols

    4. Avoid cornstarch; good medium for bacteria


    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 infants

    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 childs 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)

    Ortolanis clicka. With an infant supine, bend knees and place thumb on bent knees,fingers at hip joint

    b. Bring femur 90degrees to hip, then abductc. Palpable click dislocation Barlows 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


    failure of curve to straighten when child bends forward with kneesstraight 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 (Stills 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 childs strength


    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 levelby age 2 years

    urethra shorter in children and more prone to ascending

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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


    unable to palpate testes in scrotal sac


    if testes remains in abdomen, damage to testes is possible because ofincreased

    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


    urethral opening located anywhere along the ventral surface of penis

    Assessment: urinary meatus misplaced

    inability to make straight stream of urine


    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


    an abnormal narrowing of the foreskin so that it cannot be retracted overthe glans penis

    may be present at birth or may develop as a result of poor hygiene withaccumulation of smegma

    Management: Prevention regular pulling the foreskin back and cleaning


    Nursing intervention:

    circumcision care:1. Close observation for bleeding

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    2. Ice application3. Administration of analgesics


    involuntary passage of urine after the age of control is expected (4 years)


    1. Primary in children who have never achieved control2. Secondary: in children who have developed complete


    May occur anytime but most frequent at night

    More common in boys

    No organic cause

    Etiologic possibilities:1. Sleep disturbances2. Delayed neurologic development

    3. Immature development of bladder4. Psychologic problems


    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


    Associated structural changes1. Prolapsed rectum2. Inguinal hernia3. Widely split symphysis

    4. Rotated hips

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

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    reconstructive surgery

    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


    Design play activities to foster toddlers 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


    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


    1. Proteinuria2. Hypoproteinemia3. Hyperlipidemia4. Dependent edema

    Puffiness around the eyes in morning


    Scrotal edema

    Ankle edema5. anorexia, vomiting, diarrhea6. Pallor, lethargy7. Hepatomegaly


    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

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    Daily weight

    Administer as ordered

    Protect from sources of infection


    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. Fever

    5. Hypertension6. Diagnostic tests:

    Urinalysis rbc,wbc,protein, cellular casts

    Urine specific gravity increased

    BUN, crea increased

    ESR elevated

    Hgb, Hct decreased




    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


    large, encapsulated tumor that develops in the renal parenchyma,

    more frequently in left kidney

    originates during fetal life

    peak age : 1-3 years


    non tender mass, usually midline near liver hypertension


    test: IVP

    Nursing intervention:1. Do not palpate abdomen to avoid dissemination of CA cells2. Handle child carefully when bathing and giving care

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    7. Toddler headache and anorexia earliest common signs of shuntmalfunction

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

    close during embryonic development 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 area

    3. Myelomeningocoele Protrusion of meninges, CSF, nerve roots, portion of spinal cord

    Sac covered by a thin membrane 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

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    altered LOC bulging anterior fontanel nuchal rigidity


    isolation; maintain for at least 24 hours after antibiotics are initiated 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, precipitatingevents 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 childs 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 childsmouth 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

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    assess the childs developmental level and intelligence early intervention encourage communication and interaction with the child on a functional

    level provide safe environment

    position upright after meals provide safe, appropriate toys for age and developmental level