Kernicterus is a clinical pathological entity comprised of a constellation of clinical symptomology and pathologic changes, anatomic pathologic changes in the brain resulting from hyperbilirubinemia.
What does that actually mean? Okay. The majority (approximately 60%) of babies born in the United States have some degree of jaundice. This is yellowing of the skin, eyes and tissues. It is a scary word - jaundiced. But it is rarely a big deal. Jaundice usually goes away within a few days as the liver has a chance to clear the excessive bilirubin from the blood and the baby goes about normal development. Kernicterus is out of control jaundice.
Excessive jaundice, due to increased levels in the blood of a substance called bilirubin (hyperbilirubinemia), can lead to a rare disease known as bilirubin encephalopathy. Infants showing signs of brain dysfunction due to increased bilirubin require immediate treatment in order to prevent/minimize brain damage. Kernicterus means that there is too much bilirubin in the blood. If this rare condition is properly treated, it can usually be easily managed. Untreated, kernicterus can cause permanent brain damage.Symptoms
Besides jaundice, the most common symptoms of kernicterus are irritability, abnormal suck response, abnormal tonic, and abnormal muscle tone. Other signs of acute bilirubin toxicity include:
- high pitched cries and decreased muscle tone with episodes of increased muscle tone causing backward arching of the back and head
- sleepiness and difficulty to arouse
- lack of wet diapers
- not feeding well
- generally fussy
Fevers may develop as the damage progresses. Feedings often decrease leading to the dehydration of the infant, further exacerbating symptoms.Risk Factors
Every child has a risk of kernicterus. Modern medicine does not have a great lens into why some infants get excessive jaundice and why some do not. But we do have some clues. Factors that may lead to increases in bilirubin in the blood and in turn jaundice, include blood group incompatibilities between mother and infant, prematurity, bruising (e.g. cephalohematomas and bleeding under the skin of the scalp (caputs), infection, ethnicity (east Asian or Mediterranean descent), siblings with jaundice at birth, genetic factors (ie. deficiency in glucose-6-phosphate dehydrogenase) and non-optimal suckling/feeding.Treatment
Treatment for this problem includes phototherapy with bilirubin lights, hydration with fluids and preparation of the infant for possible blood transfusion. Feedings should occur every one to three hours to ensure urination and defecation in order to aid in removal of bilirubin from the body. The course of treatment should be immediate for any suspect cases and should not be delayed awaiting further testing. Kernicterus is a preventable disorder if symptoms are recognized early and treated immediately. Delays in diagnosis/treatment have severe consequences in infants with kernicterus. Infants affected typically require physical and speech therapy and special education.Current Efforts
There has been - and there will continue to be - progress in both treating Kernicterus related birth injuries and in implementing guidelines to prevent kernicterus by detecting newborns with jaundice and providing appropriate treatment.Medical Malpractice and Kernicterus
What is maddening is that this tragedy can always be avoided if doctors, nurses and hospitals do the right thing. What is the right thing? Test for bilirubin levels. If the baby has obvious jaundice, the National Institute for Health and Care Excellence and the National Institute for Health and Care Excellence guidelines recommend measuring the bilirubin level. So many things with treating newborn babies is complicated. This is not complicated at all. It just requires attentive doctors and nurses.
Even without bilirubin screening, there are lots of steps that can be taken to prevent this disaster. Nurses and doctors need to remember to examine the newborn for jaundice and be able to distinguish visually between mild jaundice and clinically significant jaundice, based on the infant's age in hours and the need to evaluate risk factors and if warranted, assess bilirubin levels, appropriately treat and follow-up. As nurseries are often busy, it is plausible that not all things may be done correctly or under optimal conditions.
The following list includes some of the most common, preventable, mistakes made by physicians:
- Not treating the child immediately following initial bilirubin results. Like most health conditions, the faster you catch kernicterus the better. Sure, retest the child if you like. But don't let that delay treatment. The cost of waiting can be a lifetime of neurological problems for the child.
- Waiting to treat or interruption of phototherapy in order to determine risk of a blood transfusion. If tests are required in order to prepare the infant for transfusion, all tests should be done under phototherapy lights. Again, delay can lead to lifelong injury.
- Not properly examining the infant for signs of kernicterus or the symptoms discussed above. They are not flashing light emergency symptoms but a reasonable doctor should catch them.
- Failing to properly appreciate the test results. Total serum bilirubin is specific to the age - in hours - of the child. It is important that bilirubin be measured and compared to specific norms by hour. For example, blood work from a one-day old infant may be found to be normal or high depending if the newborn is 24 hours or 47 hours old. A level of 8.5 is considered high risk in a 24 hour old baby and low risk for a 47 hour old baby. Doctors sometimes interpret a test that should be cause for alarm as perfectly normal while the bilirubin levels continue to rise.
Below are some example verdicts and settlements in Kernicterus cases. They are not common. We went back to update this list in 2020 and did not find a reported verdicts. The last Kernicterus cases that we know of that was filed in Maryland was in 2014 against Johns Hopkins. If you read all of these, you will notice the common threads that run through all of these cases when they are successful. You will also find the same thread in literature below: follow the guidelines to treat infants with jaundice.
- 2017: Arkansas, $46,500,000 Verdict. A slightly jaundiced child is discharged from the hospital without a follow-up bilirubin test with instructions to return in 10 days. Three days later, the child was lethargic, crying and in pain. Mom called the doctor who said to call back the next morning. After a number of calls, she got in to see the doctor. They gave her a blood test and sent her home. Her phone message to the doctor's clinic was returned by a nurse who advised the mother to call back the next morning. The mom kept calling and got an appointment. The doctor took gave her a blood test and sent the child home. When the blood test results showed the child was in crisis, it was too late. She has a permanent brain injury and is bound to a wheelchair. Incredibly, the defense was that you cannot expect hospitals to have the resources to do the appropriate follow-up in this corner of the state.
- 2013, New York, $24,813,260 Verdict. A one-day old plaintiff is evaluated and found to be completely normal. He is discharged. Three days later, he returns to the hospital with a yellow discoloration of the skin and eyes. The doctors determine that he was suffering hyperbilirubinemia, which is a severe form of jaundice. Treatment was timely, but three-day delay proved costly. The child suffered severe brain damage and cerebral palsy. Depressingly, the defendant argued his damages should be less because their expert thought he would die by age 25. After a 12 day trial, the jury voiced its disapproval with a verdict exceeding $24 million.
- 2012, California, $5,000,000 Settlement. After a normal delivery, the child has a bilirubin level of 5.2. Mother and child are discharged with instructions to be seen within four days. Upon their return, the baby was seen by a medical assistant at a pediatric group. This time, the bilirubin count came back at 15.3 Another test two days later showed a count of 14.6. A normal level is 20. The office called the mother to report the results and said that since the levels were dropping, she could wait for a retest. A few days later the child stopped breathing, and her skin had turned yellow. Her bilirubin levels were now at 40.1 But, and this is incredible, the lab was to report levels over 30, so it caused the computer to interpret the blank value as noncritical. So the processing lab never calls the central call center. No report on the child's bilirubin count was provided. The next day, the child deteriorated, and they finally figure it out. But it was too late The little girl is subsequently diagnosed with cerebral palsy due to kernicterus and hyperbilirubinemia. The lawsuit was against both the lab and the pediatric group.
- 2008, New York, $6,000,000 Settlement. A 2-day-old boy has jaundiced skin. A follow-up is scheduled in 5-7 days. Three days later, the boy is taken to the hospital. His skin is still slightly jaundiced. His bilirubin level is 34. Doctors also noticed a hematoma of his scalp and discovery he had brain damage. The boy lawsuit alleged that his injury was the result of hyperbilirubinemia and the doctors should have followed up more vigorously on this symptoms before he was discharged. Defense counsel contended that the 5-7 day follow-up was within the standard of care. The doctors and hospitals' lawyers still saw fit to settle the case for $6 million.
If your precious child was injured from kernicterus, you may be able to receive compensation from a medical malpractice lawsuit against the doctors and hospital who treated your child. If you want to investigate your potential birth injury medical malpractice claim, call us at 800-553-8082 or get a free online consultation. More Information
- Information on malpractice cases generally
- Overview of birth injury claims
- Negligent Treatment of mom during or before delivery
- Feel Good Story: deep brain stimulation helps one child cope with Kernicterus injuries
- Med Mal FAQ
- Das, S, et. al: Clinicopathological Spectrum of Bilirubin Encephalopathy/Kernicterus. Diagnostics 2019, 9, 24. The study discusses the range of clinical, radiological, and neuropathological changes features you see with patients with bilirubin encephalopathy/kernicterus.
- Helal NF, et. al: Characteristics and outcome of newborn admitted with acute bilirubin encephalopathy to a tertiary neonatal intensive care unit. World Journal of Pediatrics. 2019 Feb 4; 15(1):42-8.
- Rennie JM, et al: Learning from claims: hyperbilirubinaemia and kernicterus. Arch Dis Child Fetal Neonatal Ed. 2018 May 25. When you look at malpractice claims involving kernicterus, what do you see? Doctors who are not following the National Institute for Health and Care Excellence guideline that recommends measuring the bilirubin level within at least 6 hours in all babies who are visibly jaundiced. Following this simple rule would avoid a lot of suffering and malpractice lawsuits.
- Kaplan M, et al (2011): Severe neonatal hyperbilirubinemia and kernicterus: Are these still problems in the third millennium? Neonatology. 2011;100: 354–62. Study underscores foreshadows the Rennie study seven years later (above). Kernicterus is mostly preventable with good care.
- American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 2004; 114:297. " In every infant, we recommend that clinicians 1) promote and support successful breastfeeding; 2) perform a systematic assessment before discharge for the risk of severe hyperbilirubinemia; 3) provide early and focused follow-up based on the risk assessment; and 4) when indicated, treat newborns with phototherapy or exchange transfusion to prevent the development of severe hyperbilirubinemia and, possibly, bilirubin encephalopathy (kernicterus)."