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Community - A Parent's Perspective

A Parent's Scientific Perspective:
Living With Kernicterus

By Karen Dixon

     In September of 1989 my husband, Tom, and I were anxiously looking forward to the birth of our first child. I had had a relatively easy pregnancy with no complications and no problems expected during the birth. After 8 hours of labor, our son, Jess Andrew, was born. He was a beautiful, healthy baby. He did have several bruises on his head, face and arms from the birth and the use of the vacuum extractor, but the nurses and the pediatrician assured us this would go away within a few days.

     The next day we learned our son had jaundice. This is commonly seen in newborns with over 60% affected every year. Jaundice is caused by the breakdown of the hemoglobin found in red blood cells into a neurotoxin called bilirubin. The build up of bilirubin and its distribution into the tissues causes the yellow skin color that is indicative of jaundice. The doctors and nurses told us this was nothing to worry about and would go away in 7 to 10 days. At discharge, we were reassured that jaundice was normal, and were told to stand with our baby in a sunny window for 15 minutes a couple of times a day.

     When Jess was 7 days of age he began to have problems nursing and during the night began screaming a shrill high pitched scream and arching his back. We went to see the pediatrician right away and after a simple blood test we were told his bilirubin level was 40mg/dL. A normal level for a healthy, term newborn is less than 10mg/dL. I was told to rush my son to a hospital across town for emergency treatment. It was in the neonatal intensive care unit that I learned high bilirubin levels could cause brain damage. It was the first I had heard that newborn jaundice could cause serious and permanent neurological problems. In order to bring the bilirubin level down, it was necessary to do a double volume exchange transfusion. Jess’ blood was slowly removed and two volumes of clean blood was cycled through to help remove the bilirubin from my son’s brain and other tissues. While the transfusion was going on they also placed him under special lights which changed the bilirubin into a form that was easier for his little body to excrete.

     The weeks and months following his discharge from the NICU were a nightmare. We watched everything he did and constantly compared him to other infants his age. We were given very little information on what this brain damage could mean for him or what we might expect. It soon became apparent that Jess was falling behind on his developmental milestones, but his pediatrician continued to reassure us that Jess got off to a bad start and would eventually catch up. We were unable to get a diagnosis where we were living so we went to another state to have Jess evaluated and eventually moved there so he could receive the early intervention services he desperately needed.

     We finally had a diagnosis – kernicterus – a type of brain damage caused by high bilirubin levels. The classic clinical signs of kernicterus are choreoathetoid cerebral palsy (causes uncontrolled movement), auditory neuropathy (difficulty processing sounds), dental enamel hypoplasia, and gaze abnormalities. During those early years there were countless trips to therapists – occupational, physical and speech – as well as trips to orthopedists, neurologists, and endocrinologists. All necessary for Jess to have the best outcome possible.

     Thirteen years later, Jess is an amazing teenager. His courage, wonderful sense of humor, intelligence and sensitivity are a constant source of pride and inspiration. Jess has worked hard over the years and is now able to walk independently but is uncoordinated and unable to participate in most sports. However, he does participate in adaptive water skiing and enjoys therapeutic Kung Fu and Tai Chi. His fine motor coordination has been affected the most. He is unable to write and has difficulty feeding and dressing himself. He may never be able to button or zip his clothes or eat in public without embarrassment. Jess understands what happened to him and how he suffered an injury to his brain, but he has accepted this and does the best he can. He certainly has days when he is frustrated, angry and upset that his friends need to help him so much, but those days pass and are fairly uncommon for him.

     Over the past seven years, Jess and I have lectured at the University of Alabama at Birmingham School of Medicine about cerebral palsy, newborn jaundice and kernicterus. We’ve talked with first year medical students - future physicians - about the dangers of severe jaundice in newborns and how easy it is to prevent permanent brain damage. We also discuss how changes in our healthcare system have led to the re-emergence of kernicterus in the United States and other developed nations.

     In the last 12-15 years we have seen more children with kernicterus. Several factors have contributed to this trend, including early hospital discharge, inadequate post-discharge follow-up, the relaxation of guidelines on neonatal jaundice management, and a lack of education among healthcare professionals, as well as a lack of concern among physicians who believed that kernicterus had been eradicated in the 70’s.

     For years I was told that kernicterus was extremely rare and it was unlikely that I would ever meet another family who had a child like Jess. I eventually learned how very wrong those people were. In the summer of 2000 I made contact with an expert in the field about starting a website for expecting and new parents about the potential dangers of newborn jaundice. Dr. Steven Shapiro, a neurologist at the Virginia Commonwealth University Medical College of Virginia, knew of other families and through him I was able to contact another mother with a child with kernicterus in Boise, Idaho. When we first spoke on the phone it was very emotional for both of us. I had an incredible sense of community and understanding that I had been missing for so long.

     We soon met in person in Chicago, and along with 6 other mothers, started an advocacy and support group called Parents of Infants and Children with Kernicterus (PICK). From the outset, PICK’s goals were threefold:

To initiate a safe, system-based standard of care for management of newborn jaundice
To provide comfort, support and information on treatment options for families of children with kernicterus
To establish a model for a family-centered, system-based approach for constructive parent/health care partnerships to address preventable newborn disabilities

In the last 2 ½ years we have learned of more families like ours and are now hearing from new families weekly through our website (www.PICKonline.org).

In February of 2001, with an advisory group of nationally renowned experts, PICK hosted the first ever system-wide and healthcare industry workshop for kernicterus. Federal healthcare agencies, medical experts, medical and nursing associations and parents were present to discuss the prevention of kernicterus. Among those attending this meeting were representatives from the Joint Commission on the Accreditation of Health Care Organizations, (JCAHO), the Centers for Disease Control and Prevention (CDC), the National Institutes of Health (NIH), the Agency for Healthcare Research and Quality (AHRQ), the Health Care Financing Administration, Harvard School of Public Health, and the Maternal and Child Health Bureau. From this meeting, PICK worked with the JCAHO on the first consumer-driven Sentinel Event Alert on kernicterus prevention, released in April of 2001. We also worked with the CDC on the June 2001 report on kernicterus in the Morbity, Mortality, Weekly Report which was later reprinted in the Journal of the American Medical Association. In 2001 PICK was recognized by the Patient Safety Partnership Symposium for bringing about change through effective partnerships with the healthcare system. We have also advocated successfully to have kernicterus classified as a “Never Event” by the National Quality Forum. Currently, we are partnering with Pennsylvania Hospital on a CDC grant for research and prevention of kernicterus.

     In June 2002, we hosted a conference along with Virginia Commonwealth University Medical College of Virginia and the Children’s Hospital of Richmond, Virginia. Twenty-seven families and adults with kernicterus were present at this three-day event that featured experts in cerebral palsy, movement disorders, rehabilitation, auditory neuropathy, assistive technology and other fields. By far the most powerful element of the conference was the interaction among the families.

     Despite all our hard work, we continue to hear of new cases of kernicterus. We know the only way to truly prevent this devastating and preventable brain damage is through public outreach. This spring PICK will release educational videos and printed materials that will be sent to every hospital in the U.S., as well as a video for expecting and new parents. We hope this effort will prevent new cases of kernicterus by the end of 2004.

     Once our children have the kernicterus diagnosis, we seem to be thrown into a medical black hole. In fact, it was Jess’ kernicterus that prompted me to pursue a doctorate in molecular biology. Very few people understand the complexities or have experience treating children with kernicterus, so we continue our work to motivate medical researchers to find promising interventions for our children. Only recently have a group of “movement disorder” specialists begun to look at pharmaceutical therapies that may help children like ours.

     With the help of Dr. Michael Friedlander, Director of the Civitan International Research Center, we hope to raise awareness on the potential dangers of severe newborn jaundice and also to generate interest and funding to find promising interventions to help those with kernicterus. Our children have brain damage because of a healthcare system failure and we hope by partnering with that system we can prevent kernicterus, provide support for the children and their families, identify appropriate medical care and hope for future treatments that will improve their quality of life.

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