JAUNDICE AND THE NEWBORN

1. JAUNDICE AND THE NEWBORN Abbey Rupe, MD

2. Objectives • Definitions • Prevention • Risk factors • Assessment • Treatment

3. What is jaundice? • Hyperbilirubinemia • Indirect/Unconjugated • Direct/Conjugated • Considered elevated if direct portion is >20% of total bilirubin level • Scope won’t be discussed in this lecture

4. Definitions • Unconjugated • Physiologic jaundice • Most babies develop some jaundice • Mean peak of 5-6 mg/dl between 60-72 hr of life • Breast-feeding jaundice • Decreased calories, increased enterohepatic circulation • Breast-milk jaundice • Prolonged after 2-3 weeks of age • ?? Protein in breast milk that increases enterohepatic circulation

5. Definitions • Pathologic • Jaundice appearing in first 24 h of life • TSB > 95th percentile for age • TSB increasing at rate >0.2 mg/dl/h or > 5 mg/dl/d • Elevated direct component • Jaundice persisting > 2 weeks in full-term infants • Exception: breast-milk jaundice • “Severe” hyperbilirubinemia • Generally considered to be TB >25-30

6. Complications of untreated jaundice • Bilirubin-induced neurologic dysfunction (BIND) • Bilirubin crosses BBB and binds to brain tissue • Risk increased when TB >25-30 • Acute bilirubin encephalopathy (ABE) • Initial phase: lethargy, hypotonia, decreased movement, poor suck • Intermediate phase: stupor, irritability, increased tone (retrocolis and opisthotonos), fever • Advanced phase: deep stupor or coma, inability to feed, shrill cry, apnea, seizures • **potentially reversible!

7. Complications • Chronic bilirubin encephalopathy (kernicterus) • Choreoathethoid cerebral palsy • Sensorineural hearing loss • Palsy of vertical gaze • Dental enamel hypoplasia • Up to 10% mortality • Most (but no all) infants with kernicterus previously manifested symptoms of ABE

8. Why do neonates become jaundiced? • Increased bilirubin production • More RBCs + shorter RBC life span = increased turnover and increased bilirubin • Decreased bilirubin clearance • Deficiency of UGT • UGT activity at 7 days of age is approx 1% of adult • Reaches adult levels around 14 days of age • Increased enterohepatic circulation

9. Pathologic jaundice--causes • Rh or ABO incompatibility • Enzyme deficiencies: G6PD, pyruvate kinase • Hemoglobinopathies • Infection • Increased RBC load • Cephalohematomas, etc • Polycythemia • Infants of diabetic mothers

10. Pathologic jaundice--causes • Disorders of bilirubin clearance • Crigler-Najjar, Gilbert • Metabolic and endocrine • Galactosemia, hypothyroidism • Increased enterohepatic circulation • Breast-feeding jaundice • Conditions causing GI obstruction or decreased motility

11. Risk factors for severe jaundice • Major: • Predischarge bili in high-risk zone • Observed jaundice in 1st 24 hours of life • Blood group incompatibility w/ positive DAT • Previous sibling required ptx • Cephalotematoma or other significant bruising • Exclusive breastfeeding (esp if not going well and weight loss is excessive) • East Asian race

12. Risk factors for severe jaundice • Minor risk factors • Predischarge bili in high-intermediate risk zone • GA 37-38 weeks • Jaundice observed before discharge • Previous sibling with jaundice • Macrosomic IDM • Maternal age ≥ 25 years • Male gender

13. Risk factors for severe jaundice • DECREASED risk • Pre-discharge bili in low-risk zone • GA ≥ 41 weeks • Exclusive bottle feeding • Black race • Discharge from hospital after 72 hours

14. Hyperbilirubinemia--prevention • Pregnant women: • ABO and Rh testing • Rhogam as indicated • screen for isoimmune antibodies • If mom is Rh- or ABO/Rh status unknown • Check blood type, Rh, and DAT on neonate (cord blood) • If DAT positive: needs frequent (q6-12hr) checking of TSB

15. Jaundice--prevention • Newborn nursery: • Advise mothers to nurse 8-12 times per day for first several days • Evaluate for jaundice every time vitals taken (q8-12 hr)

16. Testing for jaundice • Before discharge, ALL newborns should be assessed for jaundice (2004 AAP Practice Guideline) • Visual assessment • Need adequate ambient light or daylight fluorescent light • Subjective and not recommended as lone assessment • More difficult in darker-complected infants • TSB (total serum bilirubin) • Oftentimes drawn with metabolic screen • TcB (transcutaneous bilirubin) • Method implemented at SRHC in 2010

18. Assessment tools • AAP bilirubin nomograms • Electronically • UpToDate calculator • BiliTool.org • Online calculator • App for iPhone or iPod touch • Palm OS

19. Phototherapy

20. Using the nomograms • Use total bilirubin (don’t subtract direct component) • Risk factors: • Isoimmune hemolytic disease • G6PD deficiency • Asphyxia • Significant lethargy • Temp instability • Sepsis • Acidosis • Albumin < 3.0 (if measured)

21. Exchange transfusion

22. Discharge and follow-up Follow-up by a “qualified health care professional” typically with PCP or mid-level provider nurse/lactation specialist home health nurse visit If follow-up cannot be ensured, may be necessary to delay discharge

23. Anticipatory Guidance • Jaundice progresses head to toe, then as it resolves, disappears from toe to head • Testing for jaundice: blanch skin with finger • Call if: • Jaundiced to belly button • Not waking well for feeds or feeding poorly • Decreased wet diapers • Shrill cry

24. Follow-up appointment • Weight and % change from BW • Adequacy of po intake • Pattern of voiding and stooling • Presence or absence of jaundice • +/- checking bili level • If predischarge bili was high-intermediate risk, I nearly always recheck a bili • If predischarge bili was low-risk, I rarely do • If predischarge bili was low-intermediate, I do if concerns present

25. So . .. .you’ve reached LL.Now what? • History and physical exam • Laboratory testing • Phototherapy • Assess hydration

26. Laboratory evaluation • Approaching or at light level: • Direct and total bilirubin • Blood type and DAT • CBC • Peripheral smear • Consider: retic count, G6PD, ETCOc • Consider: UA (for reducing substances) with culture, sepsis eval

27. Lab evaluation • Approaching exchange levels or not responding to phototherapy • Retic, G6PD, albumin, ETCOc • UA • Sepsis eval • Elevated direct (or conjugated) bilirubin • UA and urine culture • Evaluate for sepsis if indicated by history and physical exam

28. Treatment—Phototherapy • Mechanism of action: • Exposes skin to light of specific wavelength (425-475 nm/blue-green spectrum) • Converts bilirubin to lumirubin • Lumirubin is more soluble than bilirubin and is excreted without conjugation into the bile and urine

29. Phototherapy options • Bili blanket • Home or hospital • Bili bed • Home or hospital • “Bank” phototherapy • Hospital only • Home vs hospital • Home is an option 2-3mg/dl below LL in infant with no risk factors and caregivers with ready access to medical care

30. Treatment--phototherapy • Technique—bank phototherapy • Infant should wear diaper only, and diaper should be pulled down as much as possible to increase skin exposure • “baby sunglasses” • Ensure that banks of lights are at appropriate distance from infant • Infant needs to remain under lights continuously, only to be taken out for feedings • Can utilize biliblanket to continue some ptx during feeds

31. Phototherapy • phototherapy typically results in a decline of TB of 2-3 mg/dl within 4-6 hours • Obviously, more lights = faster decline • Recheck TB within 2-6 hours of starting ptx • Then, recheck every 6-12 hours, if TB is falling • If doing home therapy, need to redraw daily • Hospitalize if surpasses LL, excessive rate of rise, or other concerns develop

32. Phototherapy—side effects • Generally considered safe • Transient erythematous rashes • Loose stools • Hyperthermia • Increased insensible water loss • Possible retinal degeneration (hence the sunglasses) • Parents/caregivers: • some models can cause HA and nausea • Frustrating to not get to hold baby • “bronze baby syndrome” • If used on infant with cholestasis

33. Management—assess hydration • Maintaining adequate hydration and UOP is important • Lumirubin excreted in urine > stool • Encourage breast or bottle feeding • Lactation consult prn • Supplement with EBM or formula in breastfed infants with excessive weight loss (>12%) or evidence of hypovolemia • IVF if oral intake is inadequate

34. Phototherapy . .. When to stop? • No strict guideline • when TSB <13-14 • When TSB back to, or lower-than, level at which ptx was initiated • Sometimes at lower level if started during NB stay and treatment initiated at lower LL

35. Phototherapy . . . When to stop? • “checking for rebound” • TB will typically re-increase by small amount (typically <1 mg/dl) after discontinuation of ptx • DON’T have to keep pt hospitalized to check for rebound, unless risk-factors present • DO check in hospital or clinic the next day if: • ptx discontinued prior to 5-6 days old (may not have reached peak yet) • Other concerns • For “nervous Nellie’s” (like myself): night before anticipated discharge, check TSB. Continue ptx until 2 or 3am, then d/c. Check bili with AM labs, should see if significant rebound present

36. If not improving Likely that hemolysis is occurring: • Increase # banks • Labs: • Retic, G6PD, albumin, ETCOc • Sepsis eval • UA and urine culture • Add IVF • Consult pediatrician +/- neonatologist • Exchange transfusion in NICU

37. Lab evaluation • Jaundice preset at ≥ 2-3 weeks • Total and direct bili • If direct bili elevated, evaluate for causes of cholestasis • Check NB thyroid and galactosemia; evaluate infant for signs/sx of hypothyroidism • If indirect elevated and breast-fed, potentially breast-milk jaundice • Option to give formula in place of breast milk x24 hours (controversial)

38. ?Sunny window? • Technically. . .. NO • Risk for sunburn • Risk for hypo- or hyper-thermia