•DDH•Cleft Lip and Palate•Club foot•Tay Sachs Disease•Down Syndrome

HEALTH PROBLEMS During Infancy I

•Feeding Difficulties•GERD•Omphalocele & Gastroschisis•Failure to Thrive •Skin abnormalities•SIDS

Developmental Dysplasia of the Hip (DDH)

Spectrum of disorders of the hip that may develop any time: fetal life, infant, child

Predisposing factors: Physiologic: maternal hormones &

intrauterine posture Mechanical factors: breech and C sections Genetic: positive family hx

Tests: Ortolani and Barlow (birth – 3m) Shortening of limb on that side Asymmetric thigh and gluteal folds Broadening of perineum: √ when diapering Problems when begins to walk

Management of DDHTx: Pavlik harness, hip spica cast, surgeryLink with great pictures and tx with Pavlik

harnessNursing care focuses on safe and careful

assessment of circulation and skin care both with spica cast and the harness.Color~ capillary refill <3 sec.Mobility~ able to move toes easilyTemperature~ warm to touchSensation~ no numbness or tingling

Cleft Lip and Cleft PalateMaxillary & nasal tissue fail to fuse

during embryonic development (6-12 wks)Result: abn opening in lip, palate, and/or

noseCL with or w/o CP most common craniofacial

malformationCleft lip more common in malesCleft palate more common in femalesEtiology unknown – may occur as part of

genetic or environmental factors

Cleft Lip and Palate at birth

Clinical ManifestationsCL: may be unilateral or bilateral

Simple notching of lip to deep cleft extending thru lip or into nose

CP: midline fissue or opening in hard &/or soft palate areas (may have absence of nasal septum)

Difficulty forming seal for suckingCoughing & choking w/feedingNasal distortion & congestionFailure to thrive if unrepairedExcellent sites:

http://www.pedisurg.com/PtEduc/Cleft_Lip-Palate.htm

http://www.cleftline.org/

ManagementFeeding b/4 surgery: enc. parents

immediately; special bottles neededBreastfeeding possible and encouragedBaby’s head must be uprightFrequent burping neededPatience and support needed

Surgical correction: CL before CPZ-plasty for CL as soon as stableSurgery for CP usually later but b/4 speech

develops

Post Operative CareCL repair (Cheiloplasty); CP (Palatoplasty)

Meticulous care of suture line(s)Restraints frequently neededPain managementMonitor for signs of infection or bleedingAvoid placing anything hard in mouthPosition in semi-Fowler or partial side-lying to

prevent aspiration and maintain open airway

Read her story at this link

Congenital ClubfootTalipes Equinovarus (TEV)Deformity of ankle & foot (pp 424-425, Wong et al,

9 ed.)Bone deformity and malposition w/soft tissue

contracture3 categories: positional, syndromic, congenital or

idiopathic (most common 95%)Readily apparent at birth; ↑risk DDHTx~ Correction, maintenance, follow-upCasting soon after birth; serial casting common;

possible surgeryNursing care ~ Assess distal extremities for Color,

Mobility, Temperature, and Sensitivity (CMTS). Teach parents. See cast care instructions p. 1643-1645.

Tay-Sachs DiseaseAutosomal recessive genetic disorderLeads to progressive destruction of the CNS

Fatty substance accumulates in brain nerve cells Normal development at first (~ 6 mos) til nerve

cells become distended w/fatty materialBlindness, deaf, unable to swallow, atrophy of

muscles leading to paralysisMost die by age 5

Occurence higher in persons of eastern European (Ashkenazi) Jewish descent

National Tay-Sachs & allied Diseases Assoc.:www.ntsad.org

Down SyndromeMost common chromosome abnormality

1 in 800 to 1000 birthsAlso known as trisomy 21

Extra chromosome 21 in 92% to 95%Mosaicism in 1% to 3%Translocation of chromosome 21 in 3% to 6%

Not related to maternal ageCould be a carrier so higher risk w/another PG

Characteristics of Down Syndrome

Wide variations physically and mentallyEffects entire body with variations in the

head,eyes, nose, ears, mouth, teeth, chest, neck, abdomen, genitalia, hands, feet, skin, and musculoskeletal functions

40% to 45% heart defectsRenal, Hirschsprung, TE fistulaAltered immune functionSkeletal defects

Atlantoaxial instability

Nursing ConsiderationsLOTS of parental support, esp. at birth

Encourage bonding; promote acceptanceEncourage expressions of grief and loss

Teach prevention of physical problemsSafe handling d/t hypotonicityPrevention of respiratory problems

Bulb syringe use, humidification, position changesGood handwashingSkin careHelp w/feeding difficulties

Parental SupportReferrals to support groups and appropriate

web siteswww.ndss.org National Down Syndrome www.nads.org Nat. Assoc. for Down Syndrome

click on FAQPhotos of siblings—one with and one without

Information on early intervention programsRefer for genetic counseling

FEEDING DIFFICULTIESRegurgitation-return of undigested food

with burpingSpitting up-dribbling of unswallowed

formula from mouth after feedingEnc. frequent burping, minimum

handling during and after, position child on rt. side with head up after eating

Avoid skin breakdown – keep dry; apply ointment

If regurgitation is persistent – eval. for GERD

Food SensitivityFood allergy = immunologicMost common: eggs, cow’s milk, peanuts,

soy, wheat, corn, tree nuts, shell fish, fishAllergy w/hereditary tendency – atopy1 parent 50% greater risk; 2 parents up

to 100% risk of developing allergyMilk allergy: no definitive test, best is to

eliminate followed by challenge testChange to casein hydrolysate formula

where protein is broken down(Nutramigen) – NOT soy

Food IntoleranceFood Intolerance: non immunologic

Lactose deficiencyCongenital lactase deficiency: rareLate-onset/primary-most common, 3-7 yrs.Asians, southern Europeans, Arabs,

Israelis, and African-AmericansSecondary – from damage to intestinal

lumen, cystic fibrosis, sprue, infections, etc.

S/S: abd. pain, bloating, gas, diarrhea within 30 min. to hrs. after ingestion

Enzyme tablets, pretreated milk, yogurt, soy

Vegetarian DietsLacto-ovovegetarian – fewest problemsLactovegetarians – no meat or eggsVegans (purists) – no milk or eggs or meatZen macrobiotics – fruits, vegies, legumesSemi-vegetarian – some fish & poultryEncourage Fe supplementation – educate

on factors that affect iron absorption

ColicParoxysmal abd. pain or cramping with loud

crying and drawing legs up to the abdomenMore common < 3 mosSeveral theories – Carb malabsorption most

common and acceptedAssess for cow’s milk allergyTx: medication, variety of approaches (p.

532-534), assess daily routine & home environment

Provide emotional support for parents

PICA

Disorder characterized by the compulsive and excessive ingestion of both food and NONFOOD substances

Differing theories on cause: psychologic, nutritional deficiency (lead or zinc)

Complications: parasites, lead poisoning, intestinal obstruction, inflammation

Gastroesophageal Reflux (GER)

Def: regurgitation of gastric contents into the esophagus; results of relaxation or incompetence of the lower esophageal (cardiac) sphincter.

Etiology:Inappropriate LES relaxation.Delayed emptying.Pathophysiology:Repeated reflux damages the esophageal

mucosaSevere cases require surgery: Nissen

fundoplication; the gastric fundus is wrapped around the distal esophagus.

Gastroesophageal RefluxSymptoms:Effortless spitting up 1-2 mouthfuls (under age one)IrritabilityFailure to thrive, weight-lossAspiration - recurrent respiratory infectionsAnemiaApnea, worsening asthmaEsophagitis

Gastroesophageal Reflux

Diagnosis:Barium swallow or ultrasound: √ for: Hiatal

hernia Pyloric Stenosis Malrotation

Esophageal Manometry: √ control of lower esophageal sphincter

Endoscopy & pH probe - 24 hours/Gold Standard;pH is placed down to the distal esophagus - if pH is below 4.0 suggests reflux.

Endoscopy is the most sensitive test for esophagitis & also gastric outlet syndrome; biopsies obtained at same time.

Gastroesophageal RefluxManagement Maintained child in the upright position during feedings &

30 min. pcProvide frequent small feedings; frequent burping

w/infantsAdd rice cereal to formula to thicken feedingsAvoid fatty foods, citrus juices, chocolate, carbonated

drinksAvoid vigorous play after feedings & feeding b/4 bedtimeProne vs supine sleeping position for infants; ↑ SIDS riskAssess respiratory status before and after feedingsMonitor hydration status, I & O, & frequency of emesisMeds:AntacidsHistamine blockers-, Cimetidine, ranitidineReglan

Omphalocele~link for photoHerniation of abdominal contents thru

umbilical ring; intact peritoneal sacAssociated with other anomolies:

imperforate anus; ileal atresia; bladder exstrophy; trisomies 13,18, 21

50% have CV, GU, MS, CNS, and alimentary tract defects

Prognosis: 71% or lower d/t serious associated anomalies

http://www.caringbridge.org/cb/inputSiteName.do?method=search&siteName=boporter

GastroschisisCongenital defect in ant. abdominal wall

lateral to umbilical cord, usually to the rtBowel herniates thru defect in wall &

rectus muscle leaving bowel exposed with no membrane to cover it

40% SGA or premature; often seen on ultrasound

Not associated w/other congenital anomalies

Better prognosis than omphalocele (88% - 100%) depending on risk factors

Nursing ManagementImmediately cover contents w/warm, moist sterile

saline-soaked dressing to keep moist and maintain heat

Sterile technique imperativeNG tube for decompressionIV fluid d/t high fluid lossesPositioning & handling to prevent ruptureCardiac & apnea monitor with oximetry; monitor VSThermoregulation critical w/fluid lossesAntibiotics started ASAPAssess fluid/electrolyte statusMay need TPNSurgery always

Failure to ThriveInadequate growth from inability to obtain

and/or use calories neededWt. falls below the 5th percentilePersistent deviation from growth curve

most importantOrganic – physical, i.e. heart defectNonorganic (NFTT)– unrelated to disease;

poor care; psychosocial factors; attachment problems, child may not make eye contact

Idiopathic – Unexplained by the above but often classified as NFTT

Factors of NFTTPoverty – uninsured, homeless, insuf.foodHealth beliefs – fad diets; obsess abt. wtInadequate nutrition knowledgeFamily Stress Feeding Resistance – non oral nutritional

therapy early d/t med problemInsufficient breast milk Most Difficult – parent/child disturbance

Failure to ThriveMultidisciplinary team approach neededMay be multiple psychosocial problemsPrognosis related to the causePrognosis poor with: severe feeding

resistance, lack of cooperation or awareness from parents, low income, low ed. level, teen Mom, early onset

Takes time and patience –see guidelines p. 534-538 for feeding the infant with NFTT

Diaper DermatitisPeak age: 9-12 monthsProlonged contact with urine, feces, friction

of wet diaper; soap, detergent, wipesChange diaper ASAP, expose skin to air,

use oint./zinc oxide, corn starch not talcCandida infection: bright red, confluent

lesions w/raised borders; satellite lesions; painful; need antifungal cream

Seborrheic Dermatitis“Cradle Cap” – chronic, recurrent,

inflammatory reaction of the skinEyelids, ears, nose, inguinal areaOver growth of Pityrosporum yeastThick, yellow, adherent, scaly, oily

patchesNOT associated w/family hx for allergyInfants and also after pubertyDaily shampoo, let soak to soften crusts,

rinse & use fine tooth comb to remove; reassure no harm to fontanel

Atopic DermatitisPruritic eczema associated with allergy Hereditary tendency – atopyUsually begins in infancy (2-6 m); Infantile

eczema w/remission by 3 yrs.Childhood – may follow infantile or begin at

2-3 yrs; 90% by 5 yrsPreadolescent – begins abt. 12 yrs and

cont. indefinitely

Major Goals of ManagementHydrate the skin: tepid bath w/no soap or mild

(Dove, Neutrogena) w/immediate application of emollient (Eucerin)

Relieve pruritus: antihistamine drugs, colloid baths, topical steroids

Prevent/control secondary infection: antibiotics, keep fingernails/toenails cut short, teach to look for signs of infections

Eliminate rough fabrics, wool, irritants in soaps, detergents, wipes. Avoid latex. Keep cool – avoid excess perspiration & heat.

Provide support to family – very difficult situation

Sudden Infant Death SyndromeSIDSSudden death of an infant under 1 year of

age after complete postmortem exam, investigation of death scene & review of case history

3rd leading cause of death bet. 1m – 1 yrDramatic 40% decrease since 1992 since

“Back to Sleep” campaignHypothesis: brainstem abnormality in

neurologic regulation of cardiorespiratory control.

Maternal smoking– major factor in SIDS

SIDSOther risk factors: co-sleeping or bed

sharing, prone position, soft bedding, overheating during sleep (thermal stress)

Plagiocephaly – alter head position during sleep; place prone or side when awake

Autopsy findings: pulmonary edema, intrathoracic hemorrhages – similar to those found with suffocation

SIDS Family CareIn ER, ask only factual questionsAutopsy needed to find causeIf breastfeeding, mom needs infoAllow family to say goodby; make sure

room and baby are cleaned up; allow quiet time and encourage them to hold baby

Make sure referral is made for follow up home visit

Primary nursing responsibility is emotional support of the family and referral for follow up