Kernicterus – Why all Babies should be screened for Jaundice

At least half of babies develop jaundice in the first few days of their lives, and usually it is not a problem. However, in rare cases, if left undiagnosed and/or untreated, jaundice can lead to a condition called kernicterus (toxic deposits of bilirubin in the baby’s brain) that causes severe brain damage and life-long devastating disability. In all cases, this type of brain injury can be prevented by a simple, minimally-invasive and painless test and subsequent prompt treatment. Provinces, like British Columbia, that do not universally administer this test for hyperbilirubinemia should follow the example set by provinces that do, like Alberta, in order to practically eliminate the incidence of kernicterus and the resulting life-long disabilities in previously healthy babies.

What is Jaundice and when is it Dangerous?

Jaundice is a term used to describe the yellow color of the skin and whites of the eyes that happens when there is too much bilirubin produced in a baby’s body. Bilirubin is an orange-yellow pigment that is produced by the liver as a result of the breakdown of red blood cells. Babies’ levels of bilirubin are higher than those of adults for several reasons: 1) babies make more bilirubin because they have more red blood cells; 2) babies’ livers are still developing and they cannot remove as much bilirubin from the blood as adults’ livers; 3) because new babies do not yet stool (poo) within the first few hours of life, they reabsorb some bilirubin that in adults would be normally excreted through stool. All of these three causes are physiological, meaning they are “organic” to your baby’s maturation and adjustment to life outside the womb. Rarely, these causes result in extremely high levels of hyperbilirubinemia that can harm your baby.

In some cases, in addition to the above factors, babies can develop “pathological” jaundice as a result of blood group incompatibility (Rh or ABO). Sometimes, fetal red blood cells cross the placenta during pregnancy causing the mother’s blood cells to develop antibodies. When the antibodies cross over into fetal circulation, they attack the baby’s red blood cells and cause jaundice. The risk of this happening is highest during or at the time of birth. Hyperbilirubinemia occurs as a result of continuous hemolysis and failure of the newborn liver to handle the bilirubin load. This type of jaundice can develop on the first day of life and is more likely to develop into severe hyperbilirubinemia and cause a brain injury if left untreated.

What is Kernicterus?

Kernicterus refers to the yellow staining of the neurons and neuronal necrosis of the basal ganglia and brainstem nuclei (the structures within a brain) due to bilirubin crossing the blood barrier and depositing into these brain structures. It results in permanent and severe brain injury characterized by athetoid cerebral palsy with or without seizures, hearing deficits, developmental delay and cognitive impairments. Unlike many other causes of cerebral palsy, kernicterus is almost always preventable with proper testing and treatment.

What is the Test?

Transcutaneous bilirubin test is a simple minimally invasive and painless test that involves a health care provider placing a bilimeter (an instrument to measure the bilirubin levels) on a baby’s chest and reading the meter results. These results are then used (quire reliably) to predict the levels of bilirubin in the blood. If further testing is required, a simple blood test will measure the precise levels of bilirubin in your baby’s blood.

My Baby is not Visibly Jaundiced – Is the Test Still Necessary?

Visual assessment of jaundice is not a reliable method to either evaluate the levels of bilirubin in a baby’s blood or to predict hyperbilirubinemia.

What is the Treatment?

If your baby’s bilirubin levels are high, he or she will undergo phototherapy which helps with the breakdown of bilirubin. In rare cases, your baby may require a blood transfusion.

How can Kernicterus be prevented?

Kernicterus is almost always preventable. The Canadian Pediatric Society (“CPS”) recommends routine and universal testing for all infants regardless of whether they are visibly jaundiced, or not, in the first 72 hours of life, or at discharge from the hospital. In addition, the CPS recommends that any infant discharged before 24 hours of life be reviewed within 24 hours by someone with experience in the care of the newborn and access to testing and treatment facilities. Finally, the CPS recommends that any infant who is visibly jaundiced in the first 24 hours of life have their bilirubin levels assessed, because any neonatal jaundice that develops in the first 24 hours is usually pathological. If these CPS recommendations of universal testing are adopted by all health authorities across the country, cases of kernicterus can be virtually eliminated.

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