Systemic Bullets

8/3/2019 Systemic Bullets

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CARDIO

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 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

RHEUMATIC FEVER

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

GASTROINTESTINAL SYSTEM

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

Assessment:

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

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:


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dental hygiene

regular dental hygiene

good nutrition

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 childs 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)


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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 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


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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

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 toappear

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 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

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

Management:1. barium enema2. surgery

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

HIRSCHSPRUNGS 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


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

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

if with distension nasogastric suction


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:


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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 RLQ

nausea, vomiting

guarding of abdomen

rebound tenderness

decreased bowel sounds

fever

diagnostic tests:

1. CBC2. elevated acetone in urine


antibiotics/ antipyretics as ordered

no enemas

no heating pads

routine preop care

post op care:

1. semi-Fowlers position2. Monitor NGT3. Monitor penrose drains

PARASITISM

roundworms, pinworms

Assessment:

pinworms anal itching, disturbed sleep

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


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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


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

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


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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

Assessment:

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

imbalance

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

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 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

TONSILLECTOMY AND ADENOIDECTOMYAssessment:


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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

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/genetic

4. Host factors

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


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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

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

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, 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

Implementation:

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 DEFICIENCY ANEMIA

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

Assessment:

pallor


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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 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

Assessment

Pancytopenia

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

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 thecarrier status from their

fathers

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 normal BT, PT, platelet count

Implementation:

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

BETA THALASSEMIA MAJOR

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

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 than100,000/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 tosmall size of platelets

ecchymosis

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

diagnostic test: platelet count decreases, anemia

Management:

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

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


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

DIAPER RASH

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

Assessment:

erythema/excoriation in the perineal area

irritability


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

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 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

Assessment:

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


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

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 levelby age 2 years

urethra shorter in children and more prone to ascending


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infection

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 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

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


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 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

circumcision


circumcision care:1. Close observation for bleeding


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2. Ice application3. Administration of analgesics

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 development

3. 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 symphysis

4. Rotated hips

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


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Management:

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

Postop:

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

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


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

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. Fever

5. 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

WILMS 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 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

Implementation:

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

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