Kernicterus refers to brain damage that may occur when neonatal jaundice goes untreated for too long. Physiologic neonatal jaundice (which can affect about 60% of all newborn babies) is a very common condition in which the skin and whites of the eyes are yellowish in color within a few days after birth, due to high levels of a yellow pigment (bilirubin) created when the body gets rid of old red blood cells. However, in some babies, bilirubin levels may rise excessively (hyperbilirubinemia), which can damage the brain cells. Risk factors for severe jaundice and higher bilirubin levels include premature birth (before 37 weeks); darker skin color; East Asian or Mediterranean descent; feeding difficulties; jaundice in a sibling; bruising at birth; and a mother with an O blood type or Rh negative blood factor.

After a few days of jaundice, toxic levels of bilirubin in certain areas of the brain may cause signs and symptoms such as respiratory distress, muscle spasms, and/or low muscle tone (hypotonia). Other symptoms can develop as the baby gets older, such as delayed motor development, seizures, lack of coordination (ataxia), muscle spasms (dystonia), involuntary movements (athetosis), sensory problems, lack of upward gaze, hearing loss, intellectual disability, and difficulty speaking (dysarthria). The term "Bilirubin-induced neurologic dysfunction (BIND)" is used for the signs and symptoms of kernicterus. In most cases, the syndrome develops by 3 to 4 years of age.

Early detection and management of jaundice can prevent kernicterus. Phototherapy is the most commonly used method to prevent bilirubin toxicity. The definitive method of removing bilirubin from the blood is exchange transfusion. While medications are not usually given to babies with physiologic neonatal jaundice, in some cases, phenobarbital (an inducer of liver bilirubin metabolism) has been used to reduce bilirubin levels in the blood during the first week of life.

Jaundice, or yellowing of the skin, is common in newborns. The condition results from an accumulation of bilirubin, a yellowish substance produced when red blood cells are broken down. Ordinarily, bilirubin is removed from the body by the liver.

For most infants with jaundice, the yellow skin color will fade after a few days and the infant won’t suffer any ill effects. In some infants, however, the liver fails to remove bilirubin rapidly enough, and potentially toxic levels accumulate. The condition is known as hyperbilirubinemia. Untreated, hyperbilirubinemia may cause severe brain injury, which could result in cerebral palsy, profound intellectual and developmental disability, blindness, and severe hearing loss.

The first line of treatment for newborn hyperbilirubinemia is phototherapy — exposure to high intensity light. The light penetrates the skin and converts bilirubin to a less toxic substance, which is eliminated through the urine.

See your baby’s doctor the same day if your baby:

• Is very yellow or orange (skin color changes start from the head and spread to the toes).
• Is hard to wake up or will not sleep at all.
• Is not breastfeeding or sucking from a bottle well.
• Is very fussy.
• Does not have enough wet or dirty diapers.

Ask your baby’s doctor or nurse about a jaundice bilirubin test.

Get emergency medical help if your baby:

• Is crying inconsolably or with a high pitch.
• Is arched like a bow (the head or neck and heels are bent backward and the body forward).
• Has a stiff, limp, or floppy body.
• Has strange eye movements.

At a minimum, babies should be checked for jaundice every 8 to 12 hours in the first 48 hours of life. It is important for your baby to be seen by a nurse or doctor when the baby is between 3 and 5 days old, because this is usually when a baby’s bilirubin level is highest. This is why, if your baby is discharged before age 72 hours, your baby should be seen within 2 days of discharge. The timing of this visit may vary depending on your baby’s age when released from the hospital and other factors.

A doctor or nurse may check the baby’s bilirubin using a light meter that is placed on the baby’s head. This results in a transcutaneous bilirubin (TcB) level. If it is high, a blood test will likely be ordered.

The best way to accurately measure bilirubin is with a small blood sample from the baby’s heel. This results in a total serum bilirubin (TSB) level. If the level is high, based upon the baby’s age in hours and other risk factors, treatment will likely follow. Repeat blood samples will also likely be taken to ensure that the TSB decreases with the prescribed treatment.

No baby should develop brain damage from untreated jaundice.

When being treated for high bilirubin levels, the baby will be undressed and put under special lights. The lights will not hurt the baby. This can be done in the hospital or even at home. The baby’s milk intake may also need to be increased. In some cases, if the baby has very high bilirubin levels, the doctor will do a blood exchange transfusion. Jaundice is generally treated before brain damage is a concern.

Putting the baby in sunlight is not recommended as a safe way of treating jaundice.

It is important to take jaundice seriously and stick to the follow-up plan for appointments and recommended care.

Your baby might be put under special blue lights (phototherapy) to lower the bilirubin level. You should not put your baby in direct sunlight; this is not a safe treatment for jaundice and could cause sunburn. A baby with a very high bilirubin level might need a blood transfusion in the hospital.

Make sure your baby is getting enough to eat. The process of removing waste also removes bilirubin in your baby’s blood. If you are breastfeeding, you should nurse the baby at least 8 to 12 times a day for the first few days. This will help you make enough milk for the baby and will help keep the baby’s bilirubin level down. Support and advice for breastfeeding mothers may increase the chances of successful breastfeeding. If you are having trouble breastfeeding, ask your doctor, nurse, or a lactation coach for help.

It might be helpful for parents of children affected by kernicterus to talk with one another. One parent might have learned how to address some of the same concerns another parent has. Often, parents of children with special needs can give advice about good resources for these children.

Remember that the choices of one family might not be best for another family, so it’s important that parents understand all options and discuss them with their child’s health care providers.

Parents of Infants and Children with Kernicterus (PICK)
PICK was formed in 2000 by a group of 7 mothers of infants and children with kernicterus. Although the founding group banded together to promote awareness, prevention and treatment of kernicterus, they are also means of support for each other. Since then, many families and medical professionals have become involved with PICK, and have formed the PICK community.

My son, Cal, was born a healthy baby boy on March 23, 1995 in a large accredited hospital that delivers over 5500 newborns a year. Cal was first noted to be jaundiced through visual assessment at 16.5 hours old, but a bilirubin test was not done.

Cal’s skin was described to be jaundiced again through visual assessment when he was 23 hours old, but a bilirubin test was not done. Cal was discharged from the hospital when he was 36 hours and was described as having head to toe jaundice, but a bilirubin test was not done. Neither was his blood typed or a Coombes test performed.

The information we received about jaundice was a simple brochure that never mentioned jaundice can cause brain damage. It was also suggested that we put Cal in the window for sunlight.

We were assured that Cal’s jaundice was normal and not to worry. We scheduled a routine follow up visit, for 7 days of age, with his pediatrician.

On day 4 Cal started to breast feed with a weak suck and became lethargic. I called the newborn nursery and told them that he was “still yellow, lethargic and was feeding poorly.” They asked me if I was a “first time mom” and then assured me there was no concern since sleepiness is to be expected. They told me to unwrap him and tickle his feet and if that didn’t work to call the pediatrician.

We immediately took Cal to the pediatrician and he noted the jaundice by visual assessment. A bilirubin test was not done. We were told to wait 24 hours to see if he would improve.

At 5 days of age, the pediatrician admitted Cal to the pediatric unit. Cal’s bilirubin was tested for the first time and it was 34.6mg/dl. An exchange transfusion, however, was not performed since the doctors recalled a recent “raising of the bar” from 20mg/dl to 30 mg/dl. Treatment was limited to phototherapy… and that phototherapy failed Cal.

On day 6 in the afternoon, Cal had a high pitched cry, respiratory distress, increased tone and he started to arch his neck in a way that is characteristic of opisthotonos; these behaviors were all acute symptoms of kernicterus.

Little did I know that my newborn son was suffering brain damage before my eyes and in my arms. I will be haunted by that memory and my failure to protect him forever.

At 18 months of age Cal was diagnosed with classic, textbook kernicterus. Cal has athetoid cerebral palsy throughout his body, neurosensory hearing loss, enamel dysplasia on his front teeth and crossed eyes and other abnormalities.

Today Cal cannot walk independently, his speech is impaired, he drools, and he has uncontrollable movements of his arms and legs just to describe his impairment a bit. When Cal gets sick he is reduced to the functional level of a 6 month old. Despite his limitations, Cal is a beautiful and inquisitive young man. He loves playing on the computer, swimming, his sister and Pokemon! He also enjoys creating movies, short stories, and poems.

Two and a half years after Cal’s birth we were blessed with the birth of our daughter, Mackenzie. At 16.5 hours, exactly the same timing of jaundice onset as Cal, jaundice was noticed. A bilirubin test was performed immediately and phototherapy was deemed necessary and initiated. Today we have a healthy daughter.

I was recently preparing a presentation that I often give to the health care community about Cal’s story and he asked me why his sister didn’t have kernicterus. I thought that was an excellent question and one that does not have an easy answer. I am incapable of explaining to my son the justification for his disability. It is precisely what motivates me to advocate for change in our health care system.

Cal’s brain damage was totally preventable and is the result of a tragic failure of our medical system. Unfortunately, the real tragedy is that Cal’s story in NOT unique. His story has replayed itself several times over the past decade.