Bilirubin encephalopathy (BE), also known as kernicterus, is a neurological condition that occurs when an infant has severe jaundice. BE is considered a rare condition, but is nevertheless a concern for parents whose newborn has been diagnosed with jaundice. Understanding how jaundice can lead to BE, as well as what symptoms to look for, can help parents address concerns before their baby’s health is compromised.
Terms You Should Know
It can be difficult to understand medical terminology and how it applies to your family. Here are some basic definitions of terms associated with BE that may help you if you are concerned about your child:
- Bilirubin – Bilirubin is a natural substance in the body that is found in bile. It is responsible for breaking down red blood cells shed from the liver. Bilirubin levels vary, so it is important to talk to your doctor about what is normal for your child, and what tests can be done to assess bilirubin levels.
- Jaundice – Jaundice is a condition that most often occurs during the first weeks of life, and is characterized by elevated levels of bilirubin in the bloodstream. Jaundice is most often recognized due to a yellowish coloration of the skin or whites of the eyes.
- Kernicterus – Kenicterus refers to permanent brain damage caused by BE. Kernicterus is also the medical term for the yellow staining that occurs in the brain due to excessive bilirubin levels.
Jaundice and Bilirubin Encephalopathy
Jaundice is a fairly common condition affecting newborns. High levels of bilirubin may cause the skin to look yellow in color due to the body attempting to rid itself of red blood cells. On its own, jaundice is considered a condition with few risks or side effects if treated properly. However, severe cases of jaundice with excessively high bilirubin levels can lead to dangerous complications, including neurological conditions like BE. It is really important for parents and healthcare providers to address jaundice immediately and get proper treatment.
When bilirubin levels get very high or the baby’s immune system is compromised due to illness, bilirubin may migrate outside the bloodstream and collect inside brain tissue. This is what leads to BE and can cause long-term complications for the baby. Common side effects of BE include:
- Hearing loss
- Brain damage
BE is most commonly diagnosed in children who have other medical conditions or compromised immune systems, and treatment or prevention of BE will depend on the overall health of the child.
Stages of Bilirubin Encephalopathy
BE is often measured in three stages: Early, middle, and late. These stages are classified by symptoms, such as the following:
- Early Stage – Symptoms may include severe jaundice, poor sucking or feeding, lethargy (extreme sleepiness), and a lack of startle reflexes.
- Middle Stage – Symptoms may include irritability, high-pitched crying, poor feeding, or hyperextended or arched back, also called hypertonia.
- Late Stage – Symptoms include shrill crying, seizures, muscle rigidity or arched back, coma, or no feeding at all. Late stage BE can be fatal if treatment is not administered in a timely manner, or is unsuccessful.
Treatment of Bilirubin Encephalopathy
Treatment of BE depends on the overall health of your child, as well as the stage of BE at the time of diagnosis. While mild to moderate cases of jaundice most often resolve on their own within a few weeks, severe jaundice or BE often requires more extensive treatments. The most common treatments for BE include:
- Light Therapy – Light therapy is the most common form of treatment for elevated bilirubin levels. Many parents find it necessary for their newborn to undergo light therapy at the hospital, or at home in the days following birth. Light therapy works by changing bilirubin molecules so they can be extracted through urine and stool. Light therapy is painless for the infant, and requires the baby be placed under special lights while wearing only a diaper and protective eyewear. Light therapy does not use ultraviolet lights, and is safe for your baby.
- Intravenous Immunoglobulin (IVIg) – IVIg is used to treat severe jaundice when the cause is related to differences in blood type between the baby and his or her mother. These differences can cause the baby to carry antibodies from the mother that negatively interact with the baby’s own blood cells. IVIg is a blood protein that reduces these antibodies and helps normalize bilirubin levels.
- Exchange Transfusion – Exchange transfusion is perhaps the most intensive treatment for severe jaundice and BE. This process includes repeated withdrawal of small amounts of the baby’s blood, diluting maternal antibodies and bilirubin, then transferring the blood back to the baby’s system. This process is only done in severe cases and is completed in the Neonatal Intensive Care Unit (NICU).
Bilirubin Encephalopathy and Birth Injuries
The natural causes of severe jaundice or BE vary depending on the child and his or her overall health. There are, however, also causes that are not natural, such as a healthcare provider failing to recognize jaundice, failing to treat it in a timely manner, or failing to treat it at all. As a parent, you place your trust, and your child’s wellbeing, in the hands of healthcare providers. While it is difficult to imagine, negligent healthcare is a serious problem.
If you are concerned about your child’s health, or have recognized the signs of jaundice or BE, contact your child’s doctor immediately. If you are not satisfied that he or she has addressed the issue, get a second opinion. While your doctor may not be able to prevent conditions like jaundice or BE, he or she has the responsibility to diagnose and treat such conditions in a timely, appropriate manner.
Getting Help after a Birth Injury
If you or your child has been injured due to a birth injury or negligent care in the days or weeks following birth, it is important to protect your health, and your legal rights. To find out more about how to protect your family’s legal rights after a birth injury, please contact us by filling out the form on this page and we will respond quickly.