Kernicterus and Bilirubin-induced Neurological Disorders (BIND) Center

About 60% to 80% of newborn Infants in the United States are jaundiced, that is they look yellow.

Kernicterus is a form of brain damage caused by excessive jaundice.

The substance which causes jaundice, bilirubin, is so high that it can move out of the blood into brain tissue.

Join Dr. Shapiro as he speaks with Transformational Pediatrics about the diagnosis, treatment and research supporting the pediatric population with kernicterus and other bilirubin-induced neurological disorders.
Kernicterus and Bilirubin-induced Neurological Disorders (BIND) Center
Featured Speaker:
Steven M. Shapiro, MD, MSHA

Steven M. Shapiro is Division Director of Neurology for Children’s Mercy Kansas City and Professor of Pediatrics at the University of Missouri-Kansas City School of Medicine. He received his medical degree from the University of Pittsburgh School of Medicine, Pittsburgh, PA, and a Masters of Science in Health Administration (MSHA), Virginia Commonwealth University, Richmond, VA. He completed a residency in pediatrics at Rainbow Babies and Children's Hospital and University Affiliated Hospitals, Case Western Reserve University Cleveland, OH and fellowships in Child Neurology at Children's Hospital of Pittsburgh, University of Pittsburgh Pittsburgh, PA and Evoked Potentials within the Department of Neurology, University of Wisconsin-Madison Madison, WI. He is certified in Pediatrics, Neurology, Child Neurology, Neurodevelopmental Disabilities, and Clinical Neurophysiology. He leads the Kernicterus and Bilirubin-Induced Neurological Disorders (BIND) Center at Children’s Mercy Kansas City – the pre-eminent program in the world for the assessment and treatment of children with kernicterus and BIND.

Learn more about Steven M. Shapiro, MD
Transcription:
Kernicterus and Bilirubin-induced Neurological Disorders (BIND) Center

Dr. Michael Smith (Host) Our topic today is “Kernicterus and the Bilirubin Induced Neurological Disorders Center”. My guest is Dr. Steven Shapiro. Dr. Shapiro is the Division Director of neurology for Children’s Mercy Kansas City and professor of pediatrics at the University of Missouri Kansas City School of Medicine. Dr. Shapiro, welcome to the show.

Dr. Steven Shapiro (Guest):   Thank you so much, Dr. Mike.

Dr. Mike How common is jaundice in newborns?

Dr. Shapiro Jaundice is very common in newborns. Sixty to eighty percent of newborn infants have visible jaundice at some time within the first week or so of life.

Dr. Mike Of course, the main thing that we are trying to avoid is the development of kernicterus in these cases. Without appropriate intervention of the jaundice, how often does that happen? How often does it progress to kernicterus?

Dr. Shapiro Well, jaundice in newborn is very common and, actually, a little bit of jaundice is good for you. Kernicterus is a very rare condition. Kernicterus which “kern” means the kernel of the brain and “icterus” means yellow staining of the deep nuclei of the brain. In developed countries such as the U.S. or Great Britain, it’s about 1 in 100,000 or 1 in 110,000 live births. There are estimates that this is 25 times higher in counties that don’t have good systems of screening of infants for high levels of jaundice or hyperbilirubinemia.

Dr. Mike Dr. Shapiro, at your clinic what role do you play for community pediatricians and neonatologists?

Dr. Shapiro I’m a pediatric neurologist and I’ve formed this clinic. I’ve had a lot of experience seeing children with kernicterus with bilirubin-induced neurological disorders for quite some time. The role I play is generally in consulting on children usually after the excessive newborn hyperbilirubinemia has occurred as to whether the child has brain damage from that and also, if there is damage, what to do about it, how to treat it.  Also, in children who have neurodevelopmental disabilities or neurologic problems and whether this could have been caused by excessive newborn hyperbilirubinemia. Occasionally, I will get calls or contacted in the acute situation with a baby a few days of age with recommendations on how to treat, but that’s less common.

Dr. Mike How many patients do you see a year at your clinic?

Dr. Shapiro Right now, where I’m just restarting our clinic, having moved here a few years ago, we’re probably seeing about 10-15 patients a year and we’re starting to see more and more patients. The pace is picking up.

Dr. Mike Of course, as with everything in medicine, prevention is key, right?  That’s what we always want to try to do. What are you doing?  What’s some of the research you’re doing?  What’s some of the other research throughout the country that’s going on right now in the prevention of kernicterus?

Dr. Shapiro I think the first thing is to follow good guidelines. In 2004, the American Academy of Pediatrics put out guidelines that are excellent on how to manage hyperbilirubinemia. I think another thing that’s happened in recent years is measuring bilirubin levels either transcutaneously or by taking a little drop of blood and plotting that on a nomogram that will tell you the risk that the bilirubin is going to get high and then following kids who have high risk of that and treating them with phototherapy to prevent it from going high, in most cases. So, that’s the first thing in prevention. Still, some children slip through the system. Part of it is education. I think that could be very helpful – educating families and physicians about this condition. We are now embarking on research to look at genetics as a way to better predict risk of brain damage due to bilirubin. So, there are known conditions that increase your risk of having significant hyperbilirubinemia. One of them is something called G6BD deficiency. There are other things that cause hemolysis or break down of red blood cells. There are other conditions, like Gilbert’s Syndrome that decrease elimination of bilirubin by decreased function of the liver enzyme. So, if we know those things in advance, that’s another tip off on risk. Then, we’re hypothesizing that there may be some other individuals who may be more susceptible or more resistant to bilirubin neurotoxicity. So, once the bilirubin--which is bound in the blood to albumin primarily--is there, it’s really causing no harm but when it exceeds the capacity of the blood to hold it, it can go out into the brain and cause these very select and specific areas of neurologic damage. So, we have research going on to better predict the risk and to look for better biomarkers to say, if someone has a neurological condition, was this from bilirubin toxicity to neuro cells that happened as a newborn?

Dr. Mike In looking at some of the genetic research in some of the areas of your interest, are you taking on a role in educating the community pediatricians and neonatologists on some of these preventative measures?

Dr. Shapiro Well, I think the American Academy of Pediatrics has taken on a large role in prevention and education. There are other organizations that have done this. Our group is not that involved because, frankly, that’s where most of the effort in this has gone. I think it’s a wonderful thing. I’m interested in can we better assess risk so that we can capture everybody who might be at risk and prevent everybody and not let anybody slip through and do it with better tests, better resources and in both developed and underdeveloped countries. So, we are partnering with people who are doing that kind of work.

Dr. Mike Dr. Shapiro, I want to thank you for the work that you’re doing and I want to thank you for coming on this show. You’re listening to Transformational Pediatrics with Children’s Mercy Kansas City. For more information, you can go to childrensmercy.org. That’s childrensmercy.org. I’m Dr. Michael Smith. Thanks for listening.