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What is Kernicterus?

Kernicterus is a serious neurological condition affecting newborns, resulting from very high levels of bilirubin in the blood. Bilirubin is a yellow compound that occurs in the body as a byproduct of the breakdown of old red blood cells. Normally, the liver processes bilirubin, which is then excreted in bile. However, in newborns, especially preterm infants, the liver may not be fully developed, leading to an inability to process all the bilirubin formed. This causes high blood bilirubin levels and newborn jaundice (referred to as hyperbilirubinemia).

If not treated, those high levels of bilirubin can deposit in the brain tissues, leading to kernicterus. This condition is characterized by yellow staining of the brain (particularly the basal ganglia and brainstem nuclei), which can result in permanent neurological damage. Kernicterus can lead to lifelong disabilities, including cerebral palsy, hearing loss, vision problems, and cognitive impairments.

Kernicterus Symptoms in Babies

The initial kernicterus symptoms are jaundice—a yellow tone to the baby’s skin, white of the eyes, and mucous membranes in the mouth. These symptoms may show up anywhere from one to three weeks after birth.

Other early symptoms of kernicterus include low energy or a very sleepy baby, poor feeding and difficulty sucking, low muscle tone, and lack of a startle reflex in response to loud noises.

At the middle stage, kernicterus symptoms include fever, high-pitched crying, and irritability. In the late stage, the baby will experience arching of the back, stiff muscles, seizures, and won’t eat. At this point, kernicterus is life-threatening.

Kernicterus Risk Factors

Several kernicterus risk factors can be broadly categorized into those related to increased bilirubin production, decreased bilirubin elimination, or both. It is crucial for healthcare providers to identify these risk factors early and manage them to prevent the progression of hyperbilirubinemia to kernicterus.

Key risk factors of kernicterus are those that lead to increased bilirubin production and/or breakdown of red blood cells (which can result in kernicterus). These include:

  • premature infants born before 37 weeks of gestation, because their liver is less mature and less capable of processing bilirubin efficiently.
  • blood disorders that increase the breakdown of red blood cells, such as Rh incompatibility, ABO incompatibility, G6PD deficiency, and hereditary spherocytosis.
  • hypoxic birth injury, which can lead to increased red blood cell breakdown and/or poor liver function.
  • birth trauma causing bleeding and bruising, including causing collections of blood under the scalp (cephalohematomas).
  • neonatal infections and sepsis, which can contribute to increased bilirubin production and decreased bilirubin clearance.
  • genetic or metabolic diseases such as Crigler-Najjar syndrome or Gilbert’s syndrome; these affect how the liver processes bilirubin leading to hyperbilirubinemia.

Kernicterus Treatment

Kernicterus treatment—or more precisely, the prevention of kernicterus by treating severe neonatal jaundice—focuses on reducing the bilirubin levels in the newborn’s bloodstream before they can cause damage to the brain. Early detection and management of hyperbilirubinemia are crucial, through timely and frequent monitoring of bilirubin levels in newborns at risk, and then by providing appropriate interventions. The primary Kernicterus treatments include phototherapy and exchange transfusion.

Treatment protocols are based on clinical guidelines that consider the infant’s age in hours, total serum bilirubin levels, and the presence of risk factors.

  • Phototherapy – this is the most common initial treatment for neonatal jaundice. It involves exposing the baby’s skin to blue light, which alters the structure of bilirubin molecules, making them water-soluble without the need for further metabolism by the liver. With adequate hydration, this allows the bilirubin to be excreted in the baby’s urine and stool.
  • Exchange transfusion – this may be necessary when bilirubin levels are extremely high or do not respond adequately to phototherapy. This involves periodically removing small amounts of the infant’s blood and replacing it with donor blood or plasma. This directly removes bilirubin and antibody-coated red blood cells (in cases of immune-mediated hemolysis) and provides fresh, healthy red blood cells.

Alongside these treatments, supportive care measures are essential, including monitoring hydration and nutrition, monitoring of bilirubin levels, and maintaining an appropriate body temperature during treatment.

How is Kernicterus Caused by Medical Malpractice?

The delayed recognition and treatment of kernicterus in newborns can have severe and lifelong consequences. There are common mistakes and oversights in clinical practice that can lead to these delays and may lay the groundwork for a medical malpractice claim.

Kernicterus may be caused by medical malpractice if the healthcare provider fails to identify and manage risk factors. Medical malpractice may also occur if the healthcare provider makes the following errors, which result in delayed treatment:

  • The failure to assess jaundice adequately. Reliance solely on visual assessment without using bilirubin measurement tools can lead to underestimation of the severity of jaundice.
  • Failure to recognize or appropriately manage risk factors for severe jaundice, such as blood group incompatibility, glucose-6-phosphate dehydrogenase (G6PD) deficiency, and hemolysis; these conditions can lead to the rapid escalation of bilirubin levels. Inadequate monitoring of newborns after discharge from the hospital may cause a delay in the diagnosis of significant newborn jaundice, and failing to arrange appropriate follow-up to assess jaundice in the first few days after birth can be a critical mistake.
  • Disregarding parents’ observations about their baby’s jaundice or changes in behavior (e.g., lethargy, poor feeding) can delay necessary interventions.
  • Not adhering to well-established guidelines for the management of neonatal jaundice (starting phototherapy or exchange transfusion).
  • Inadequate education on breastfeeding, or ineffective or improper breastfeeding can lead to dehydration and increased bilirubin levels. kernicterus.

Contacting an Experienced Malpractice Attorney

If your child has developed kernicterus and you believe medical negligence occurred, contact Britcher, Leone & Sergio. Our attorneys are skilled in negotiating with healthcare providers, hospitals, and insurance companies, understand how to leverage evidence and expert testimony to secure fair settlements, and can provide guidance to families to help them access medical and support services.