Jaundice is a very common condition that affects many newborns each year. Left untreated, children suffering from jaundice can develop kernicterus, a severe disorder that can cause permanent neurological impairment, debilitating motor retardation, brain damage, seizures and even death. It is a tragic and lifelong affliction that places severe burdens on both parent and child. And according to recent studies, the national incidence of kernicterus is on the rise (for more information, visit our related links).
What Is Kernicterus?
Kernicterus, also known as hyperbilirubinemia, is the most severe form of jaundice. The most obvious sign of jaundice is a yellow coloration of the infant's skin caused by the buildup of bilirubin in the blood. When this occurs there is also a corresponding buildup of bilirubin in the brain. In cases of hyperbilirubinemia, brain damage can occur and the outcome is similar to cerebral palsy.
Signs of Jaundice and Kernicterus
All parents should be on the lookout for changes in skin color and the whites (sclera) of their baby's eyes. These colors can range from yellow to rose to burnt sienna. In children of color the observation of sclera color is often helpful in establishing the presence of jaundice. While many babies develop a slightly yellow skin tone after birth, their skin color — and sclera — should lose the yellow coloration within the first week of life. If the coloration persists or worsens during the first one to two weeks, especially in breastfed infants after their mother's milk has come in, they must be examined by a pediatrician to determine treatment. The most sinister signs of kernicterus include arching of the back, neck and head, weak or poor suck and high-pitched cries. In such cases the baby needs immediate, emergency treatment.
How Can Kernicterus be Prevented?
A newborn's bilirubin levels can be checked with a simple $1 "heel stick" or $4.50 BiliCheck test at the time of hospital discharge. Because some health-care companies don't require these tests for all newborns, and because universal screening of newborns for hyperbilirubinemia is not practiced in the United States, it is important for parents to ask that the test be administered. Hopefully, in time, these tests will become a standard procedure for all newborns, but until then it's up to parents to make sure their child is checked.
How Jaundice and Kernicterus are Treated
Mildly elevated bilirubin levels in newborns can be treated by phototherapy, which is usually effective in preventing kernicterus when used appropriately. However, parents need to bring the baby back to the hospital, which is inconvenient and can become costly. To prevent jaundice or to rectify a mild case, breastfeeding can be an effective treatment: in many cases, the more frequently a newborn is breastfed, the less likely it will be that jaundice will persist or increase. It is also helpful to place the newborn infant in their bassinet, wearing only a diaper, in front of a closed, sunny window for an hour a day — basically, this is natural phototherapy. Parents should also discuss their baby's skin color with their pediatrician at each follow-up visit, and they should not hesitate to contact their child's health-care provider if they notice changes in skin or sclera color, or any other behavior that seems to indicate a problem.
Children with kernicterus suffer from a lifelong neurologic disability. Once brain damage has occurred it is irreversible. Other than rehabilitation efforts to maintain muscle tone and/or auditory correction, they require round-the-clock medical care for their lifetime. Estimates for the cost of this long-term care range from $22 to $40 million per child.
Kernicterus Is On the Rise
Recent studies show the number of reported cases of kernicterus has tripled in the recent years (for more information, visit our related links). The reason? It could be that newborns are being released from hospitals too soon and jaundice, which is the beginning stage of kernicterus, usually peaks several days after birth when baby is home instead of under a doctor's watchful eye.
Nearly 60 percent of hospital readmissions for babies are for the treatment of jaundice. The costs of readmission are considerable. Babies with more severe forms of the disease require treatment within Neonatal Intensive Care Units (NICU), where daily costs can reach $4,000 to $5,000. The true incidence of kernicterus may be under-reported and federally funded research is now underway to establish the current incidence of kernicterus. But the fact of the matter is that one baby with kernicterus is one baby too many, especially since affordable and easily administered preventive testing is available.
What's Being Done
In April 2001 the Joint Commission for the Accreditation of Healthcare Organizations (JCAHO) issued a Sentinal Event Alert to all its member hospitals regarding kernicterus and the management of hyperbilirubinemia. This alert essentially provides a notice to hospitals that they must exercise increased diligence regarding the incidence of kernicterus. Given the increased attention to jaundice prevention, parents should request that the heel-stick or BiliCheck test be administered to their newborn. Ideally, they should discuss jaundice testing with their prenatal health-care provider during the pregnancy so that they are educated about its symptoms before their baby's birth.
Other steps are being taken to ensure fewer children are afflicted with the disease, including new therapies and drugs. But until these new treatments are offered to newborns, it's imperative that parents are aware of the risk of kernicterus and that doctors are prepared to treat every case of jaundice as if it might develop into this heartbreaking disease.
Laura J. Vukovich is the Vice President of Human Resources & Administration, WellSpring Pharmaceutical Corp. located in Neptune, New Jersey.