Kernicterus Lawyer

Kernicterus, also known as Chronic Bilirubin Encephalopathy, is a rare but severe complication from abnormally high levels of bilirubin in the blood (hyperbilirubinemia). Bilirubin at high levels is toxic to the cells of the brain – particularly the regions known as the basal ganglia and the brainstem. If left untreated, these high levels of bilirubin may cause severe and permanent brain injury.

Bilirubin is a yellowish byproduct of the breakdown of hemoglobin in red blood cells. Newborns have a higher rate of red cell breakdown with a level of bilirubin production leading to increased levels of bilirubin in the blood. High levels of bilirubin are deposited in the skin and the whites of the eyes causing a yellowing known as neonatal jaundice or jaundice of the newborn. 

Jaundice is typically seen 2 to 3 days after birth. Under normal conditions (physiologic jaundice), the increased bilirubin peaks on day 3 or 4 of life and normalizes within 10 to 14 days. The bilirubin is converted by the baby’s liver into a form that can be removed by the body and the jaundice resolves without any complications.

Hyperbilirubinemia is defined as bilirubin levels above 5 mg per deciliter (dL). Jaundice is abnormal (pathologic jaundice) when the jaundice is not physiologic. Features of pathologic jaundice include the appearance of jaundice within 24 hours after birth, a rapidly rising total serum bilirubin concentration (increase of more than 5 mg per dL per day), and a total serum bilirubin level higher than 17 mg per dL.

Some babies have risk factors that cause the bilirubin to rise abnormally high either due to increased red cell breakdown or the inability of the liver to convert it to an excretable form, leading to toxic blood levels. When this occurs, the bilirubin is taken up by the brain leading to brain injury known as kernicterus. The condition resulting from kernicterus is called bilirubin induced neurologic dysfunction (BIND).

There are many risk factors that may lead a newborn to have hyperbilirubinemia. They may include conditions associated with the mother and those associated with the birth.

Maternal (parents) risk factors for Hyperbilirubinemia (either and/or both parents): 

• Blood type incompatibility with baby (AB or Rh incompatibility)
• Breast feeding
• Certain drugs given to the mother such as diazepam (Valium), oxytocin (Pitocin), promethazine (Phenergan)
• Ethnic background – Asian, Native American, Mediterranean descent
• Gestational diabetes
• Genetic diseases of hemoglobin (thalassemias) 
• Prior pregnancies with newborn jaundice
• G6PD Deficiency (a condition causing red blood cells to break down in response to certain medications, infections, or other stressors)

Neonatal (childs) risk factors for Hyperbilirubinemia:

• Birth trauma including bruising to the head
• Drugs given to the baby such as Pediazole or Chloromycetin
• Infections in the baby
• Polycythemia (increased red cells in the blood)
• Prematurity
• Previous sibling with hyperbilirubinemia and/or newborn jaundice
• Male gender
• Liver disease

All newborns with jaundice should be evaluated with bilirubin levels.  While in the hospital, routine assessment for jaundice should be every 8 to 12 hours.  If the baby is discharged before 72 hours, they should be evaluated for jaundice within 2 days after discharge as the bilirubin levels may peak after the baby is discharged and jaundice missed.  

If the baby is noted to be jaundiced, a bilirubin level should be obtained. Testing can be done with a blood sample for total serum bilirubin (TSB) or using a light to assess the bilirubin level (Transcutaneous bilirubin – TcB).  

The risk for kernicterus is based on the bilirubin level and the baby’s age in hours using a chart known as a nomogram. Further testing is indicated based on the risk on the nomogram

Additionally, other laboratory tests should be obtained to determine whether the baby has risk factors for pathologic hyperbilirubinemia such as maternal fetal blood incompatibilities or genetic diseases of hemoglobin (thalassemia). 

Missing jaundice and not properly monitoring bilirubin levels may lead to toxic levels of bilirubin and result in kernicterus.

If the bilirubin levels present a high risk for kernicterus, hyperbilirubinemia can be treated to prevent a rise to toxic levels:

• Phototherapy or light therapy – the baby is exposed to a special light that converts the bilirubin into a form that can be excreted by the body.
• Blood exchange transfusion – a baby’s blood is exchanged with blood from a matched donor.

In addition to having jaundice, babies with kernicterus may present with more severe signs. These include:

• Lethargy / excessive sleepiness
• High-pitched crying.
• Lack of appetite or difficulties feeding
• Floppy or limp body
• Absent reflexes e.g., the moro reflex
• Uncontrollable movements / muscle spasms
• Arching of the head and heels back (like a bow)
• Vomiting
• Lack of wet diapers
• Unusual eye movements
• Fever
• Seizures

Signs of Kernicterus often do not arise until a few months or years into a child’s life. This is usually because when a child has not reached expected milestones. If kernicterus is suspected as a cause of neurologic dysfunction, an MRI may demonstrate brain injury.

Kernicterus is preventable. If the risks of kernicterus are missed because bilirubin levels were not properly monitored and treated, the result will be severe and involve devastating complications. These include:

• Athetoid cerebral palsy – a movement disorder caused by brain injury
• Lack of muscle tone
• Muscle spasms
• Difficulty with coordination
• Problems maintaining normal eye movements
• Hearing and vision loss
• Developmental impairments
• Communication difficulties including speaking
• Intellectual and learning disabilities
• Poor development of the teeth