When severe jaundice is not treated for a long time, it can lead to kernicterus. Kernicterus is a type of brain damage that can happen when a baby’s blood has too much bilirubin in it.

There are several causes of excessive increase in bilirubin such as too much red blood cells, increased breakdown of red blood cells, birth injuries, low levels of albumin, obstruction in the biliary system, genetics, and problems with the breast milk fed to the baby.

The illness is more common in boys than girls, and it mostly affects children of African American and South Asian descent. The disease occurs in babies of both sexes but boys were found to be more affected. A higher incidence of kernicterus is also seen in premature infants compared to full-term babies.

If the baby presents with any of the following:

• Very bright yellow or orange skin color (changes start from the head and spread to the toes)
• Is unable to fall asleep or wake up
• Does not take to breast or bottle feeding
• Is very fussy
• Does not have a sufficient amount of soiled or wet diapers (at least 4-6 thoroughly wet diapers in 24 hours and 3 to 4 stools per day by the fourth day)
• Muscle weakness
• Slow reflexes

The following tests are used for diagnosing and treating kernicterus:

• Total and direct bilirubin levels
• Coombs test -by using Rho(D) immune globulin and checking the incompatibility between mother and child’s blood types 
• The complete blood count (CBC) – a standard diagnostic tool that is essential for ruling out sepsis and is performed as part of any comprehensive medical evaluation. Further, hemolysis, anemia, and hematopoiesis can be checked by counting the reticulocytes.
• Serum electrolytes- such as sodium, potassium, chloride, bicarbonate, urea nitrogen, and creatinine

Treatment

The main goal of treating kernicterus is to stop neurotoxicity by lowering the level of bilirubin. There are three main parts of treatment and prevention for hyperbilirubinemia.

• Exchange Transfusion Therapy is the fastest and best way to get rid of the bilirubin that has already formed in the blood.
• Phototherapy is the use of light to change molecules of bilirubin that don’t dissolve in water into isomers that do dissolve in water and can be passed out of the body.

Intravenous Immunoglobulins (IVIG) are used to treat conditions that are caused by the immune system or when Rh, ABO, or other blood group incompatibilities cause a lot of jaundice in babies.

Proper prenatal care can help doctors and the mother get ready for any complications that could make the baby more likely to get hyperbilirubinemia. All babies who leave the hospital within 48 to 72 hours of birth should have a medical checkup. Any baby whose family has a history of hyperbilirubinemia, preterm birth, birth injuries like cephalohematoma, or other problems should be watched more closely. The parents should be told how to feed their child properly and how important breastfeeding is. They should be taught to go to the doctor if they notice warning signs like less activity, a change in mental state, or a weak sucking reflex.

References

Reddy DK, Pandey S. Kernicterus. [Updated 2022 Aug 28]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559120/

National Organization for Rare Diseases (2022). Kirnecterus. Retrieved December 9, 2022, from https://rarediseases.org/rare-diseases/kernicterus/

Centers for Disease Control and Prevention (2022). What are Jaundice and Kernicterus?. Retrieved December 9, 2022, from https://www.cdc.gov/ncbddd/jaundice/facts.html#:~:text=Kernicterus%20is%20a%20type%20of,sometimes%20can%20cause%20intellectual%20disabilities.