Kernicterus: A Preventable Neonatal Brain Injury

Kernicterus, one of the most easily preventable causes of brain injury from severe neonatal jaundice, has re-emerged in the United States and other nations with well developed healthcare systems as a public and societal health concern. Kernicterus, in its usually recognized form, causes devastating disabilities including athetoid cerebral palsy (CP) and speech and hearing impairment. It represents the severe manifestation of bilirubin induced neurologic dysfunction (BIND) syndrome. This condition not only ranks amongst the highest cost per new case (according to the Center of Disease Control’s Financial Burden of Disability study in 1992), but also results in profound and uncompromising grief for the family and loss to siblings of healthy, talkative playmates. And for the child with kernicterus (usually remarkably intelligent, but trapped in an uncontrollable body), grief and frustration are enormous. In 2001 national healthcare organizations, including Centers for Disease Control (CDC), the Joint Commission for the Accreditation of Healthcare Organizations (JACHO) and the American Academy of Pediatrics (AAP) issued alerts to all accredited hospitals and public health professionals in the United States and affiliated organizations that all healthy infants are at potential risk of kernicterus if their newborn jaundice is unmonitored and inadequately treated. Evidentiary analysis of 125 cases, from an informal Pilot Kernicterus Registry, documents that unmonitored or inadequately treated severe hyperbilirubinemia results in kernicterus in otherwise healthy term and near term infants. On the basis of this empirical evidence, the lapses in care and root causes have been associated with the occurrence of kernicterus in these infants. The re-emergence of kernicterus in the United States is the result of interacting phenomena including a) early hospital discharge (before extent of jaundice is known and signs of impending brain damage have appeared); b) lack of adequate concern for the risks of severe jaundice in healthy term and near term newborns; c) an increase in breast feeding without adequate instruction, monitoring and support; d) medical care cost constraints leading to early discharge with loss of supervision; e) paucity of educational materials to enable parents to participate in safeguarding their newborns; and f) limitations within the healthcare systems to provide continuity of care. The current resources for clinical interventions that can drastically and efficiently reduce the increased bilirubin load, intensive phototherapy and exchange transfusions, are available for use in those infants with excessive hyperbilirubinemia. However, these interventions leave a very narrow margin of safety for babies who have rapid or unrecognized increases in their bilirubin load. Because most babies are discharged before the hyperbilirubinemia reaches its peak during the first week of life, preventive and system-based strategies offer a safer, kinder and gentler means to prevent BIND including kernicterus.