Newborn Screening

Neonatologist Vinod Bhutani, MD – Talks About Jaundice Management

Dr. Vinod Bhutani is a newborn specialist (neonatologist) and a pediatrician who has been based in Philadelphia, Pennsylvania for the past 30 years. He is a member of both the Society for Pediatric Research and the American Pediatric Society and was named a “Top Doc” by Philadelphia Magazine. Dr. Bhutani is presently involved in research and prevention of kernicterus (a neurologic injury in newborns that leads to severe brain damage or death) and research for the management of severe hyperbilirubinemia. He chairs the Medical Advisory Board for Parents of Infants and Children with Kernicterus (PICK) and serves on the American Academy of Pediatrics’ Subcommittee to develop Guidelines for the Management of Newborn Jaundice. We spoke with him recently about the management of newborn jaundice and the prevention of kernicterus.

Q. What is jaundice, what causes it and when does it have potential to harm newborns?

A. Most babies will develop jaundice, which is the accumulation of the yellow pigment (called bilirubin, pronounced “Billy-Roo-Bin”) in the skin. This pigment is naturally formed by the breakdown of red blood cells in our tissues and blood circulation. Excessive breakdown of the red blood cells (hemolysis) or inadequate elimination of this pigment through stooling or urination leads to an increased level of this yellow pigment, which leads to harmful effects for newborns. Hemolysis can occur when the baby’s blood type is different than the mother’s. Bilirubin at high levels can be toxic to the nervous system. Usually, the brain is protected from accumulation of bilirubin. If and when bilirubin enters the brain tissue, it can lead to brain injury or side effects. Babies born before 38 weeks of pregnancy, those with hemolysis and those with low protective levels of protein (albumin) are at high risk for such injury when bilirubin is accumulated in the body.

Q. When is a baby assessed for jaundice?

A. Jaundice in a newborn is similar to a vital sign, like determining their temperature or heart rate. Thus, just as we assess for fever with a frequent check of temperature, all infants are to be assessed for jaundice after birthing and then every 6 to 8 hours until discharged from the hospital. Presence of jaundice should be measured by measuring the tissue level of bilirubin (done by visually looking at the baby’s skin color) and by a blood test (total serum bilirubin). The results of the tests are compared to a scale of what is normal for the baby’s age in hours and then ranked by percentiles on a scale to determine severity and risk. Jaundice and high bilirubin levels usually peak between 4 to 7 days after birth when most babies are at home. Thus, all babies need to be screened for the risk of their occurrence of jaundice before they are discharged from their birthing hospital. This allows for individualized family education for prevention and management of newborn jaundice. Absence of jaundice on visual inspection is not an adequate assessment for family counseling because appearance of jaundice may be masked by skin pigmentation, blushed or ruddy skin tone or lighting that is not bright enough to assess the baby’s appearance. It is also important to have the blood test to obtain an accurate evaluation.

Q. What is kernicterus and how can it be prevented?

A. Babies with particularly high bilirubin levels that are ranked above the 99th percentile are at high risk for developing neurologic injury that includes brain damage. This condition has been known as kernicterus and may present itself suddenly and severely or silently during infancy as cerebral palsy with movement and hearing disorders (athetoid type cerebral palsy). The acute signs are of encephalopathy, a disease of the brain that commences with excessive drowsiness, poor feeding and shrill or high-pitched cries. It can progress rapidly to an irreversible state with seizures, coma and even death. In some, less severe forms or those with delayed interventions, the signs may be mild, subtle or missed and are apparent only during infancy with signs of brain damage due to jaundice, primarily to certain specific parts of the nervous system.

Almost all cases of kernicterus are preventable with screening, monitoring and following newborn bilirubin levels. Babies with high bilirubin levels (ranked above the 75th percentile for age) may be managed with nutritional support such as enhancing nutrition with breastfeeding and promoting the body’s ability to eliminate bilirubin. Babies with excessive bilirubin levels and those at risk for neurologic injury need expeditious and timely, dramatic reduction in tissue bilirubin levels with interventions such as intensive phototherapy (where the baby is exposed to certain wavelengths of light) or a blood exchange transfusion, where the baby’s unhealthy blood is replaced with good blood to prevent or minimize neurologic injury.

Q. Kernicterus was thought to be eradicated. What accounts for the re-emergence of cases in recent years?

A. Our success in preventing kernicterus with a national prenatal program to identify and manage Rh negative mothers led to the near-elimination of kernicterus in the 1980s. Because of our historical efforts to aggressively treat high bilirubin levels (hyperbilirubinemia), evidence of bilirubin toxicity to healthy term babies was never accurately documented. Now, as post-birthing care is increasingly provided at home, often without adequate health care supervision of increasing bilirubin levels, optimal nutrition (especially, lactational) and worrisome clinical signs, the diverse and multi-ethnic US population is vulnerable to a higher risk of kernicterus.

Q. What is currently being done to ensure that more newborns will not be harmed?

A. In partnership with Parents of Infants and Children with Kernicterus (PICK), we have embarked on a national campaign to change the way health care providers practice the care of newborns at risk for jaundice, hyperbilirubinemia and kernicterus. The goal is for each newborn in the US to have a safe experience with jaundice. Our approach calls for a system-based approach that educates the family, empowers our nurses to measure bilirubin without a physician order (similar to measuring blood sugar), and reminds the health care community to objectively measure the severity of hyperbilirubinemia corrected for age in hours, ranked by percentile and the risk of its rate of rise on the bilirubin nomogram. Several health care organizations have endorsed this approach, and recommended it as one of the system-based approaches by the American Academy of Pediatrics as well as the Joint Committee on Accreditation of Healthcare Organizations.

Q. What do expectant parents need to know?

A. Families caring for newborns need to feel and can be reassured that their infant will have a safe experience with jaundice. It is important to share with them that most babies will develop jaundice and most can be managed with effective feeding practices of the baby that will lead to regular elimination of bilirubin through normal bowel movements. One out of four babies will have bilirubin level that can be mostly managed with such an approach. One out of ten babies can progress to a higher bilirubin level that may need more intensive interventions. As bilirubin levels rise, families need to be knowledgeable of the clinical warning signs: excessive sleepiness, unexplained fussiness and irritability, poor feeding, inconsolable crying that becomes shriller and backward posturing of the neck. One out of 700 babies can be at risk for “dangerous” or extreme hyperbilirubinemia. It is now appreciated that such occurrences may be preventable with a safer and objective monitoring for jaundice during the first week after birth.