May 2, 2001 (Washington) -- America's hospitals are being alerted to the risks of a life-threatening, yet almost always preventable condition that strikes infants in the early days of life.
The Joint Commission on Accreditation of Healthcare Organizations (JCAHO) says medical professionals should be on the alert for kernicterus -- a rare disorder resulting from toxic levels of bilirubin in the blood.
"My family knows first hand the tragic and profound consequences of kernicterus. It has challenged our lives, emotionally, physically, and financially," says Sue Sheridan, spokeswoman for PICK, Parents of Infants and Children with Kernicterus. Her 6-year-old son Cal will live with the consequences of the condition for the rest of his life.
Bilirubin, which causes jaundice, 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. Although most doctors only see one or two cases during their careers, there has been something of a re-emergence of the condition since 1984. One registry counts 90 cases since 1984 in the U.S. That's not many out of an estimated four million live births in America, so doctors may be inclined to underestimate these at-risk children.
Jaundice in a mild form affects about one-half of all newborns and is likely to go away in a few days. However, if the level of bilirubin unexpectedly ratchets up, it can cause severe brain damage similar to the type caused by cerebral palsy. There is no effective treatment once it progresses to that level.
The irony is that high levels of bilirubin are highly treatable simply by putting a jaundiced baby under a bright florescent light. A blue light has also proven an effective therapy for bringing the bilirubin down.
"They [doctors] have a lower level of concern, and that's understandable, because it's a rare problem," Jeffrey Maisels, MD, chairman of an American Academy of Pediatrics' subcommittee on neonatal health at the William Beaumont Hospital in Royal Oak, Mich., tells WebMD. Along with the JCAHO, the Academy is also issuing new guidelines for managing the disorder.
What the JCAHO is suggesting as a new preventive strategy is to monitor a large number of children who have elevated bilirubin with blood tests, even though their jaundice is likely to go away in a matter of days.
However, the trend toward shortened hospital stays means that it's easy to miss jaundice if it shows up in an apparently healthy baby after a day or two. The federal government says that insurance companies only have to pay for the first 48 hours in the hospital after the baby is born. But some parents want to go home sooner.
"Physicians didn't automatically make the adjustment that was necessary to recognize that the baby going home in 36 hours is not the same as the baby going home in 70 hours," says Maisels.
According to the JCAHO, the risk factors for kernicterus include the following:
- Jaundice within 24 hours of birth
- Poor breastfeeding
- Siblings with jaundice
- East-Asian or Mediterranean descent
The accrediting body suggests several fixes for the problem:
- Blood tests to predict the risk of extreme jaundice in babies showing any symptoms in 24 hours
- Follow-up on all newborns within 48 hours by a doctor or nurse, or consider delaying hospital discharge
- Provide parents with educational material about the condition
- Make sure adequate phototherapy equipment is available
During on-site accreditation visits, JCAHO will ask hospitals how they intend to protect newborns from kernicterus.