June 14, 2001 (Washington) -- The CDC has issued a call for better awareness of kernicterus, a serious -- but preventable -- neurological syndrome that can strike otherwise healthy newborns with jaundice or high bilirubin concentrations.
Kernicterus, a lifelong brain syndrome, can include cerebral palsy, mental retardation, and hearing loss. It's caused by hyperbilirubinemia, a severe and untreated elevation in a newborn's bilirubin concentration. Bilirubin is a natural by-product of decomposing red blood cells, but when it's present at dangerous levels, it can cause severe brain damage or even death in an infant.
Detection of high levels of bilirubin is "critical to the prevention of the irreversible effects of kernicterus," said the CDC in an article released Thursday in its Morbidity and Mortality Weekly Report.
There are about 100 known cases of the condition in the U.S., with boys appearing to suffer more severely under the syndrome.
Karen Dixon, a member of the executive board of PICK, Parents of Infants and Children with Kernicterus, tells WebMD, "This is totally preventable, and there is no reason that this should be happening."
Dixon's son has kernicterus, which he got after he was discharged after birth with elevated bilirubin. "The doctor should have been brought back in for another test, but he just said, 'We'll see you in two weeks. If the jaundice doesn't go away in seven to 10 days, give us a call.' And by day seven he was in a serious situation."
In May, the Joint Commission on Accreditation of Healthcare Organizations issued an alert to thousands of hospitals to help raise their awareness of kernicterus.
Rachel Nonkin Avchen, PhD, an official at the CDC's National Center on Birth Defects and Developmental Disabilities, tells WebMD that the risk factors for hyperbilirubinemia include jaundice within the first 24 hours of birth.
"Hopefully parents won't get to the point where they are seeing the onset of kernicterus," Avchen says. "It's the early signs of jaundice that we want them to notice and have their physicians treat."
The CDC recommends that infants discharged less than 48 hours after delivery be examined by a health provider within two to three days for routine follow-up and a jaundice assessment. That's because babies may leave the hospital without appearing jaundiced or may develop high bilirubin levels at a later point.
Offering advice for parents, Dixon tells WebMD, "Find out information about jaundice. Talk to your doctor and ask him how he feels about prenatal jaundice? Does he treat aggressively or does he take the more liberal approach? Find out what the policy is of that hospital as far as doing bilirubin tests. If there is any doubt in the parents' mind, they should request a bilirubin test if it has not been ordered by the doctor."
Why have more kernicterus cases come out?
Lois Johnson, MD, a professor of pediatrics at the University of Pennsylvania, tells WebMD, "It is related to a decreased concern about the toxic potential of bilirubin, and early hospital discharge, and efforts at cost containment. Doctors want to make sure they don't do too many test on babies who end up not needing them after all."
But Johnson says, "Doctors should be increasingly careful, even though their HMOs may be urging them to not have too many follow-up visits. [Kernicterus] is something that we can prevent."