Kernicterus

Kernicterus is a form of preventable brain damage occurring in newborns with untreated severe jaundice. It is a very rare condition and devastating for affected families. Although kernicterus can cause permanent disability, immediate treatment can stop further damage from occurring.

Kernicterus Explained

Kernicterus is a rare but extremely serious neurological condition caused by untreated newborn jaundice. Jaundice is a common condition, affecting between 60% to 80% of newborns. About 5% of babies have jaundice that is severe enough to require treatment.

When severe jaundice goes untreated, it can cause permanent brain damage called kernicterus. Kernicterus only affects about 1 in 44,000 newborns in developed countries because jaundice is usually treated before it becomes dangerous.

Symptoms of kernicterus include excessive sleepiness, high-pitched crying, muscle spasms and arching in the neck and back. The symptoms can become life-threatening if treatment is delayed. When a baby shows signs of kernicterus, doctors will immediately treat them with light therapy or blood transfusions to prevent further brain damage.

What Is Kernicterus?

Kernicterus is brain damage caused by severe newborn jaundice, a yellowing of the skin often seen in newborns. Although most cases of jaundice clear up on their own, severe cases can lead to brain damage if left untreated.

Kernicterus means there are high levels of bilirubin (a yellow pigment from the liver) in the blood and toxic levels in the brain. This affects several critical areas of the brain involved in movement and hearing, potentially leading to cerebral palsy, hearing loss, other neurological issues and even death.

Since kernicterus is preventable with proper treatment, the condition can be qualified as a birth injury. If doctors fail to recognize signs of newborn jaundice or kernicterus, it may be considered medical negligence.

What Causes Kernicterus?

Kernicterus starts with jaundice. Newborn jaundice occurs when there is an increase of a substance in the blood called bilirubin. Bilirubin is produced when red blood cells break down naturally. It is a toxic, yellowish substance that is removed from the blood by the liver and excreted from the body.

Newborn babies typically have higher levels of bilirubin than adults because their red blood cells break down quickly and their livers cannot keep up. Treatment aims to lower dangerous levels of bilirubin to prevent medical complications. When bilirubin levels get too high, the substance can enter the brain tissue and cause permanent damage or kernicterus.

Typically, newborn jaundice is a harmless condition that resolves on its own within 1 to 2 weeks. However, for 1 in 20 babies, jaundice is severe enough to require hospital treatment.

Kernicterus Risk Factors

Some factors can increase a newborn’s risk of developing kernicterus, including:

  • Premature Birth: Babies born before 37 weeks do not have fully developed livers. As a result, the liver may not be able to remove bilirubin from the blood as efficiently as livers of full-term babies.
  • Blood Type Differences: When a mother has a different blood type than her baby’s, the baby may inherit antibodies from the mother that attack the newborn’s red blood cells. This called hemolytic disease.
  • Trouble Feeding: Babies are more likely to get jaundice if they are not eating enough and eliminating waste properly.
  • Family History of Jaundice: Babies can inherit disorders that cause red blood cells to break down more quickly and increase bilirubin in the blood.
  • Dark Skin: Jaundice is more difficult to recognize when babies have a darker sink color, meaning there is a greater risk of it going untreated and leading to kernicterus.
  • Mediterranean or East Asian Descent: Babies from an East Asian or Mediterranean background are more likely to experience jaundice.
  • Bruising During Birth: A large amount of bruising increases the breakdown of red blood cells and can contribute to higher levels of bilirubin.

Kernicterus Symptoms

Kernicterus symptoms depend on the levels of bilirubin build-up in the blood. Parents should look out for signs of severe jaundice so that treatment can begin immediately. These signs include poor feeding, unusual sleepiness, poor reflexes and yellowing skin.

Early warning signs of kernicterus include:

  • Arching in the neck
  • Bending the heels backward
  • Feeding problems
  • High-pitched crying
  • Irritability
  • Trouble waking
  • Unusual eye movements

In late-stage kernicterus, infants experience severe symptoms, including:

  • Muscle spasms
  • Breathing problems
  • A stiff or limp body
  • No feeding
  • High-pitched crying
  • Seizures (epilepsy)

Diagnosing Kernicterus

Doctors should examine babies for jaundice every 8 to 12 hours after birth and before the mother is discharged from the hospital. The baby should be rechecked within 2 to 3 days of leaving the hospital.

A nurse or doctor will test bilirubin levels by holding a light meter to the baby’s skin. If the level is high, the doctor will order a blood test to accurately measure bilirubin. Babies with bilirubin levels higher than 25 milligrams will be treated in the hospital and monitored until bilirubin levels drop

Kernicterus has been known to occur even when bilirubin levels are lower than 25 milligrams. Doctors will perform a physical examination of your baby for the signs of kernicterus and whether brain damage has begun. Fortunately, prompt treatment can stop brain damage from progressing.

Kernicterus Treatment

Kernicterus is a medical emergency and requires immediate treatment. Treatment is focused on reducing bilirubin levels as quickly as possible to prevent further brain damage.

Light Therapy

During light therapy, the baby is placed under a special light or on top of a light blanket. This light changes the shape of bilirubin to help the liver remove it more easily.

When kernicterus is involved, doctors will likely use multiple lights to speed up treatment. Doctors will not stop treatment until bilirubin levels fall to a safe level. Babies will be fed through a tube or intravenously.

During this ongoing treatment, the doctor will test the baby every 4 to 6 hours until bilirubin levels begin to fall and then every 6 to 12 hours until treatment is complete. This treatment usually takes 1 to 2 days.

Plasma Transfusion

Doctors may recommend a plasma transfusion if light therapy is not effective or if the baby has very high levels of bilirubin. A plasma transfusion involves gradually replacing the baby’s blood with donor blood that is free from bilirubin. Doctors will test bilirubin levels 2 hours after treatment and may have to do another transfusion.

Kernicterus Prognosis

Kernicterus is a very serious and life-threatening condition. The outcome for a baby with kernicterus depends on how quickly medical treatment begins.

When kernicterus is caught early and treatment is administered immediately, babies can have very mild neurological effects. Children may still have some problems with motor skills as they develop.

If kernicterus is not stopped quickly, children may suffer from permanent brain damage, hearing loss and even death. Kernicterus can cause athetoid cerebral palsy and dystonic cerebral palsy.

When a doctor fails to recognize signs of jaundice or kernicterus, it can be considered medical negligence. It is the responsibility of doctors and nurses to look for signs of jaundice and monitor bilirubin levels. Failing to treat the condition before it becomes dangerous can have a lasting impact on a child.

Financial Compensation for Kernicterus

Since kernicterus is often caused by a failure to treat severe jaundice in infants, the condition may be considered a birth injury. If your child suffered brain damage due to unrecognized and untreated jaundice or a failure to immediately treat signs of kernicterus, you may be able to file a legal claim. Financial compensation can help pay for your child’s ongoing therapy and special needs costs.

Work with an attorney who has experience in kernicterus birth injury cases if you are considering filing a lawsuit. Contact the Birth Injury Justice Center today at 800-914-1562 to have your medical case reviewed for free.

Author:Birth Injury Justice Center
Birth Injury Justice Center

The Birth Injury Justice Center was founded in 2003 by a team of legal professionals to educate and empower victims and families affected by birth injuries. Our team is devoted to providing you with the best resources and legal information for all types of birth injuries.

Last modified: January 9, 2019

View 5 References
  1. “Acute bilirubin encephalopathy and its progression to kernicterus: current perspectives” Research and Reports in Neonatology. Retrieved from https://www.dovepress.com/acute-bilirubin-encephalopathy-and-its-progression-to-kernicterus-curr-peer-reviewed-article-RRN. Accessed on December 20, 2018.
  2. “Facts about Jaundice and Kernicterus” Centers for Disease Control and Prevention. Retrieved from https://www.cdc.gov/ncbddd/jaundice/facts.html. Accessed on December 20, 2018.
  3. “Newborn Jaundice” NHS. Retrieved from https://www.nhs.uk/conditions/jaundice-newborn/treatment/. Accessed on December 20, 2018.
  4. “Bilirubin encephalopathy” U.S. National Library of Medicine. Retrieved from https://medlineplus.gov/ency/article/007309.html. Accessed on December 20, 2018.
  5. “Kernicterus” National Organization for Rare Disorders. Retrieved from https://rarediseases.org/rare-diseases/kernicterus/. Accessed on December 20, 2018.