Basics
Description
The 3 major categories of unconjugated hyperbilirubinemia associated with breastfeeding:
- Physiologic jaundice: occurs between 1 and 7 days of life and peaks at 3-5 days.
- Breastfeeding jaundice (BFJ): exaggerated physiologic jaundice associated with inadequate milk intake.
- Breast milk jaundice (BMJ): occurs between 1 and 12 weeks in thriving breast milk-fed infant.
Epidemiology
Prevalence
- Physiologic jaundice: 40-60% of infants
- BFJ: 10% of breastfed infants
- BMJ: 0.5-2% of breastfed infants
Risk Factors
- Jaundice in first 24 hours (pathologic)
- Predischarge elevated total serum bilirubin
- Blood type incompatibility
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency
- Gestational age <36 weeks
- Previous sibling receiving phototherapy
- Cephalohematoma or significant bruising
- Exclusive breastfeeding
- Eastern Asian race
Pathophysiology
- Normal physiology: Bilirubin is a breakdown product of hemoglobin. Unconjugated bilirubin is bound to albumin, transported to the liver, and conjugated by the hepatic enzyme uridine diphosphate glucuronosyl transferase (UGT1A1). Conjugated bilirubin is transported into the small intestines via the bile ducts, where it is modified and excreted in stool. If stooling is delayed, bilirubin is deconjugated by intestinal enzymes and returned to the liver via the portal circulation (enterohepatic circulation).
- Physiologic jaundice: bilirubin levels are elevated in newborns due to several factors:
- Increased hematocrit and red blood cell volume
- Increased red blood cell lysis due to shorter red blood cell lifespan.
- Impaired bilirubin excretion because of an immature hepatic UGT1A1 enzyme and increased enterohepatic circulation.
- BFJ: Lack of effective breastfeeding causes inadequate milk and calorie intake and results in decreased stooling and increased enterohepatic circulation. Infants may also be dehydrated.
- BMJ: Mechanism unclear. Hypotheses include factors found in the milk that may inhibit hepatic UGT1A1. BMJ is associated with East Asian infants who are more likely to have a UGT1A1 mutation and weight loss.
Diagnosis
History
- BFJ
- Weight loss: Infants should not lose more than 8% of their birth weight. Infants should gain 15-30 g/day after maternal copious milk production (around day 4 or 5).
- Frequency and duration of breastfeeding: Breastfed infants should suckle at least 8-12 times/24 hours.
- Pain with breastfeeding may indicate poor latch and therefore decreased milk transfer.
- Urination/24 hours: urinates 1 time for each day of life until copious milk production (around 4-5 days) then at least 6 urine/24 hours
- Stooling: changed from meconium to transitional to yellow seedy by 4-5 days of life. Has at least 3-4 yellow stools/24 hours.
- Maternal causes of decreased milk production: prior breast surgery, hypothyroidism, retained placenta, insufficient glandular tissue, few medications, obesity, and infertility
- BMJ
- Infants should be in good health, gaining at least 15-30 g/day and nursing well.
- Family history of neonatal jaundice? May indicate a genetic propensity for developing jaundice.
- Screen for risk factors associated with pathologic jaundice:
- Jaundice in the first 24 hours of life
- Direct hyperbilirubinemia
- Maternal blood type to screen for Rh or ABO incompatibility
- Inherited hemolytic diseases (hereditary spherocytosis). Ask about family history of severe neonatal jaundice, anemia, or splenectomy.
- Infection: maternal group B strep infection, maternal chorioamnionitis or intrapartum fever, prolonged rupture of membranes, infant fever ≥100.4 °F rectally, decreased infant feeding, increased infant sleepiness or fussiness
Physical Exam
- Infant
- General: thin, not thriving infant in first week associated with BFJ. Well appearing, thriving older infant associated with BMJ. An ill-appearing, grunting, tachypneic, sleepy, or fussy infant not interested in nursing should be evaluated for infection. Sleepy infants may be or become dehydrated.
- Mucous membranes: dry mucous membranes associated with dehydration
- Skin: Jaundice (and bilirubin levels) generally progress from head to toe but bilirubin levels estimated visually may be inaccurate. Infants with dark skin tones are especially difficult to assess.
- Cephalohematomas and bruising are associated with increased red blood cell breakdown.
- Abdomen: Hepatosplenomegaly is associated with metabolic or hemolytic process or biliary obstruction. Abdominal distension may be a result of intestinal obstruction.
- Direct observation of a feeding is crucial:
- Lips should be everted and mouth wide open approaching a 180-degree angle. As much of the areola as possible should be in the infant's mouth. The infant should have deep movement of the jaw and suck/swallow/breathe rhythmically with milk visible in infant's mouth.
Diagnostic Tests & Interpretation
Lab
- All mothers should be screened for blood and Rh type. Infants of mothers with blood type O or Rh negative need their cord blood tested for blood type and Coombs to identify risk for hemolytic anemia. These infants are at greater risk for kernicterus and need to be treated at lower total serum bilirubin levels.
- Infants should have a pre-discharge either total serum bilirubin or transcutaneous bilirubin level.
- Bilirubin levels are interpreted based on age in hours/days and risk factors (blood incompatibility, preterm, illness). Use of a nomogram tool (such as www.bilitool.com) stratifies the infants' risk based on the American Academy of Pediatrics (AAP) bilirubin guidelines.
- Generally, minimal laboratory evaluation is needed in a healthy breastfed infant with mild jaundice in the absence of risk factors. However, BFJ and BMJ are diagnoses of exclusion. The following tests should be considered, depending on the clinical presentation:
- Conjugated/unconjugated (direct/indirect) bilirubin level interpreted based on age and risk factors
- May guide treatment
- Must be measured in all infants with very early jaundice <24 hours of age
- Recommended in all infants with persistent jaundice
- Conjugated or direct serum bilirubin: Elevated level (>1 mg/dL or 10% of total serum bilirubin) may indicate infection, biliary obstructive disease, cholestasis, metabolic disease, or severe hemolysis.
- CBC and smear:
- Look for polycythemia or anemia
- Smear may assist with diagnosing hemolysis.
- Decreases in hematocrit over time may reflect ongoing hemorrhage or hemolysis.
- A mean corpuscular hemoglobin concentration (MCHC) of >36.0 g/dL may suggest hereditary spherocytosis.
- Abnormal white cell count may indicate infection.
- G6PD quantitative test
- G6PD deficiency may cause an increase in bilirubin later than other hemolytic disease and has been associated with an increased risk of kernicterus.
- Electrolytes (especially sodium) in infant, if concern for dehydration.
Differential Diagnosis
- Increased bilirubin production
- Hematologic
- ABO or Rh isoimmunization
- Erythrocyte enzyme defects (e.g., G6PD deficiency)
- Erythrocyte membrane defects (e.g., hereditary spherocytosis)
- Polycythemia
- Impaired bilirubin conjugation
- Gilbert syndrome
- Crigler-Najjar syndrome
- Decreased bilirubin excretion
- Biliary obstruction
- Biliary atresia
- Choledochal cyst
- Dubin-Johnson syndrome
- Rotor syndrome
- Intestinal obstruction
- Meconium ileus
- Hirschsprung disease
- Congenital: transient familial neonatal hyperbilirubinemia
- Metabolic
- Hypothyroidism
- Galactosemia
- Miscellaneous
- Dehydration
- Sepsis
- Cephalohematoma
- Maternal oxytocin use
Treatment
- BFJ
- Evaluate and treat for insufficient milk intake. Assess latch, position, and milk transfer. Consult a lactation expert if needed.
- Increase frequency of effective breastfeeding to 8-12 times/24 hours.
- Supplement with formula, pumped breast milk, or donor breast milk if needed.
- Phototherapy if serum bilirubin levels exceed the AAP recommended threshold levels for phototherapy for full-term healthy infants based on infant's age in hours, gestational age, and neurotoxicity risk factors
- Phototherapy may contribute to dehydration; it is essential to monitor hydration status.
- Consider partial exchange blood transfusion with phototherapy if indicated per the AAP hyperbilirubinemia guidelines.
- Full-term healthy infants 25-48 hours old with total serum bilirubin >19-22 mg/dL and for infants ≥48 hours old with total serum bilirubin >22-25 mg/dL
- Consider home phototherapy if bilirubin levels close to threshold. Home phototherapy poses less of an obstruction to breastfeeding.
- Monitor serum bilirubin levels closely until they are acceptable. Check levels after stopping phototherapy.
- BMJ
- Monitor clinically after excluding other causes of hyperbilirubinemia.
- Encourage and support breastfeeding.
- Consider stopping breastfeeding for 24 hours (with continued pumping to preserve supply) if bilirubin levels ≥20 mg/dL. Level should decrease 3 mg/dL in 24 hours.
Ongoing Care
Follow-Up Recommendations
- Follow up 2-3 days after discharge, earlier if infant at high risk for jaundice. Weight check, physical exam, assessment of hydration, and observation of feeding. Bilirubin level if clinically indicated.
- Close follow-up 1-2 days later if concern about milk intake or jaundice
- Patient education: Mother should be assisted with latch and positioning and be taught signs/symptoms of adequate milk intake, dehydration, illness, and jaundice.
- Late preterm/near-term infants need especially close follow-up as they have increased risk of poor milk intake.
- Visual assessment of jaundice may be inaccurate. Consider an objective measure of jaundice (e.g., total serum bilirubin or transcutaneous bilirubin level) for follow-up assessment.
Inpatient Considerations
If a breastfed infant is hospitalized for jaundice, encourage continued breastfeeding if possible. For infants with BFJ, consult a lactation expert to evaluate cause for insufficient milk intake and provide assistance. The mother may need a hospital grade double electric pump to increase her milk supply.
Complications
- Kernicterus (bilirubin encephalopathy)
- Acute symptoms: lethargy, hypotonia, opisthotonus, seizures, high-pitched cry
- Chronic symptoms: hearing loss, upward gaze palsy, cerebral palsy, cognitive impairment
- Unnecessary cessation of breastfeeding
- Parental and health care provider anxiety
Additional Reading
- Academy of Breastfeeding Medicine. Clinical protocols. Guidelines for management of jaundice in the breastfeeding infant equal to or greater than 35 weeks' gestation. http://www.bfmed.org/Resources/Protocols.aspx. Accessed March 14, 2015.
- American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004;114(4):297-316. [View Abstract]
- Christensen RD, Henry E. Hereditary spherocytosis in neonates with hyperbilirubinemia. Pediatrics. 2010;125(1):120-125. [View Abstract]
- Maisels MJ, Bhutani VK, Bogen D, et al. Hyperbilirubinemia in the newborn infant > or = 35 weeks' gestation: An update with clarifications. Pediatrics. 2009;124(4):1193-1198. [View Abstract]
- Lauer BJ, Nancy ND. Hyperbilirubinemia in the newborn. Pediatr Rev. 2011;32(8):341-349. [View Abstract]
Codes
ICD09
- 774.39 Other neonatal jaundice due to delayed conjugation from other causes
- 774.31 Neonatal jaundice due to delayed conjugation in diseases classified elsewhere
- 774.6 Unspecified fetal and neonatal jaundice
ICD10
- P59.3 Neonatal jaundice from breast milk inhibitor
- P59.8 Neonatal jaundice from other specified causes
- P59.9 Neonatal jaundice, unspecified
SNOMED
- 82696006 Neonatal jaundice due to delayed conjugation from breast milk inhibitors (disorder)
- 17140000 Neonatal jaundice due to delayed conjugation (disorder)
- 387712008 Neonatal jaundice (disorder)
FAQ
- Q: Will jaundice cause my baby to have developmental or neurologic problems?
- A: If hyperbilirubinemia is appropriately monitored and treated, it should not cause any developmental problems.
- Q: What can be done to prevent BFJ?
- A: Early, frequent, and effective breastfeeding at least 8-12 times in 24 hours can decrease the risk of BFJ. Do not supplement unless recommended by a health care provider. Get help from a breastfeeding expert if you have pain or the infant is not latching on or nursing well.
- Q: Should I stop breastfeeding if my baby is jaundiced?
- A: The frequency and effectiveness of breastfeeding should be increased and appropriate lactation consultation obtained for BFJ. For BMJ, temporary cessation of breastfeeding will lower total serum bilirubin; however, this recommendation should only be considered (along with formula supplementation and phototherapy) if total serum bilirubin is ≥20 mg/dL.