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Kernicterus and Hyperbilirubinemia in Newborns
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It's safe to say that all newborns have at least a slight increase in bilirubin when they are born. This is because they are born with a lot more blood than they really need and that blood needs to be broken down so the levels are normal. The liver isn't usually mature enough to break down the bilirubin-a breakdown product of the blood cells and the bilirubin rises above normal. In some cases, the rise in bilirubin can be severe and can cause kernicterus-a condition where the brain is affected by very high levels of bilirubin.
Neonatal hyperbilirubinemia is also known as having a total serum bilirubin of 5 mg/dL or more and is extremely common. Up to 60 percent of all infants will have some degree of jaundice or yellowing of the eyes and skin within the first week of life. Most of these infants are otherwise normal and the disease is self-limiting or can be simply treated. Unfortunately, severe illnesses can be related to hyperbilirubinemia as well, such as hemolytic diseases of the newborn, metabolic problems, endocrine disorders and problems with the size or function of the liver. Infant infections can contribute to hyperbilirubinemia.
In general, normal infants rarely get bilirubin levels above 12 mg/dL and the condition is easily treatable. With risk factors for disease, the bilirubin level can get even higher and there can be more serious complications. Common risk factors include having an incompatibility between the mother's and infant's blood groups, such as Rh factor incompatibility or ABO incompatibility. Large cephalhematomas, which are blood clots on the infant head due to birth trauma, can cause elevated bilirubin levels. Bruising and other trauma to the infant during birth can increase the number of blood cells that need to be turned around to make bilirubin. Maternal valium use can be a risk factor as well as the use of pitocin during labor. Gestational diabetes and certain drugs in the infant can contribute to getting hyperbilirubinemia. Infections can make hyperbilirubinemia severe and having a sibling with the condition can mean you get neonatal hyperbilirubinemia. Preemies often get the disease as well.
Kernicterus happens when there are neurological consequences of having unconjugated bilirubin in the brain. It causes brain damage in the infant; the actual role is unknown. It happens when lipid-soluble bilirubin numbers are greater than the amount of the protein albumin, which carries the bilirubin. This means that bilirubin can cross over the blood-brain barrier, causing kernicterus. If the bilirubin is above 25 mg/dL, the risk of kernicterus is very high. Kernicterus is irreversible and can cause a condition known as chronic bilirubin encephalopathy. It results in developmental delay, motor delays, mild mental retardation and some deafness.
Early in kernicterus, there is lethargy, a high pitched cry, poor muscle tone and poor feeding behaviors. Later on, there are seizures, abnormal posture, rigid muscles, fever and eye changes.
In diagnosing neonatal jaundice, the doctor has to see the infant in a bright room. It takes a bilirubin level of about 4 mg/dL to see any kind of jaundice under the skin. The jaundice usually starts at the head and moves down toward the feet. The palms and soles are the last part of the body to become jaundiced. If it reaches the palms and the soles, the bilirubin has usually reached 15 mg/dL. Doctors also need to decide if the jaundice is just physiological or if there is a pathological reason behind having an elevated bilirubin. Doctors look for pinpoint lesions on the skin of blood, called petechiae, excessive bruising, an enlarged liver or spleen, excessive weight loss and dehydration.
Treatment of hyperbilirubinemia includes phototherapy, which is light therapy onto the infant's skin. They make chambers for light therapy and blankets that emit blue light to bring down the bilirubin. The bilirubin is broken down into water-soluble molecules that are easily excreted in the urine and the bile. Good phototherapy drops the bilirubin down about 1-2 mg/dL within four to six hours or so. Breast fed infants have a slightly harder time bringing down their bilirubin levels. Exchange transfusions where small amounts of infant blood are exchanged with healthy blood. This is the fastest way to lower bilirubin and help the infant avoid kernicterus.