When Jaundice Leads to Brain Injury

Kernicterus a Rare but Severe Condition

Fortunately, the vast majority of parents whose newborn children develop jaundice will never see the condition progress to kernicterus.

According to the U.S. Centers for Disease Control and Prevention, the exact number of newborns who suffer from the condition is unknown, but it appears to be remarkably small. Preliminary research has suggested that the risk of developing the condition is only about one in 13,300. Other doctors say the chances are much lower.

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But the conditions that lead up to the rare condition are far more familiar. The root cause is excessive jaundice, a well-known condition in which the levels of a substance called bilirubin build up in a baby's blood faster than it can be broken down in the liver.

This condition generally poses little threat to health, beyond its characteristic yellowing of the skin and whites of the eyes.

But when bilirubin levels in the blood spike high enough, the toxic byproduct can move out of the blood and into brain tissue. This opens the door to kernicterus -- and, in turn, brain damage.

"Before people knew that high levels of bilirubin could cause brain damage... about 10 percent of cerebral palsy used to be due to kernicterus, [which is] due to jaundice," said Dr. Steven Shapiro, the pediatric neurologist at Virginia Commonwealth University, who, in 2004, diagnosed Lexi with kernicterus.

Using a bilirubin test, doctors can catch high bilirubin levels early. Lowering these levels may involve therapy that is as simple as phototherapy -- in other words, treatment with light -- which transforms the toxic version of bilirubin into a nontoxic form that the body can easily eliminate. In severe cases, babies can receive blood transfusions to get rid of the bilirubin before it has a chance to damage their brains.

Haas said her daughter never received such a test. So when her bilirubin levels spiked, Lexi began to suffer from brain damage without doctors or her parents knowing.

But the brain damage that Lexi suffered was highly specific to an area known as the globus pallidus, which is crucial in controlling voluntary movements. Consequently, Lexi is unable to move normally, but her mental faculties remain unaffected.

"Even though they look retarded because they can't move, can't talk normally, they are bright kids trapped in these bodies that don't work because of their dystonia," Shapiro said.