Twenty four million newborns develop jaundice every year, a condition caused by bilirubin accumulating in the blood because newborns lack sufficient liver function to excrete bilirubin efficiently. If the bilirubin level in blood becomes very high it can cause brain damage (called kernicterus). It is estimated that 120,000 babies die from kernicterus globally each year, and many more suffer varying degrees of permanent neurological damage. Most of these deaths take place in low-income countries, especially in Sub-Saharan Africa and South Asia.
Our goal is to treat jaundice before there are signs of bilirubin toxicity, but early signs usually resolve without permanent damage if treated quickly with intensive phototherapy. These signs are increased sleepiness, feeding poorly, sometimes irritability and require immediate care. Advancing bilirubin toxicity may result in permanent disability. Signs of advancing bilirubin toxicity include increasing stiffness of extremities, arching of the back and neck, a shrill cry, and occasionally seizures. Presence of any sign, early or advanced, is a medical emergency requiring immediate treatment.
Bilirubin brain injury varies considerably ranging from awkward gate to deafness, severe cerebral palsy with uncoordinated movements, inability to speak clearly or feed oneself. Even in cases of severe movement disorders and deafness, cognitive function is usually preserved since bilirubin does not attack parts of the brain governing thinking. Being locked in a body that cannot hear, speak, control movements needed to communicate by signing is frequently confused with severe mental retardation – it is not. Kernicterus is a terrible preventable tragedy.
Most newborns with jaundice are treated simply by exposing them to blue-light therapy before irreversible damage occurs. If the bilirubin level is extremely high, removing the jaundice blood and replacing it with compatible donor blood can lower the bilirubin concentration rapidly. This is called an exchange transfusion, or replacement transfusion.