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Jaundice: Proceed With Caution
By Kim M. Norton
For The Record
Vol. 18 No. 1 P. 34

Hyperbilirubinemia, or severe jaundice, can lead to kernicterus, a rare disease that causes brain damage. However, with adherence to the American Academy of Pediatrics’ guidelines, the condition is preventable in almost all cases.

When a woman learns of her first pregnancy, a flurry of emotions ensues. She is excited, nervous, and apprehensive of the changes that will occur both over the next nine months and once her child arrives. Frequently, the mother takes an intense interest in everything pertaining to the growing fetus inside her. She educates herself on every possible topic, from breast-feeding to bathing her newborn.

Despite this attention to detail, sometimes something gets overlooked or doesn’t receive the attention it deserves. On occasion, this can have severe consequences on the little life growing within. This was the case with Susan Sheridan and her son Cal.

Sheridan devoured every baby preparedness book she could find. She felt reasonably educated about the topics to discuss with her doctor and she was confident with her self-education.

Cal was born at 37 weeks, 2 days, and Mom could not have been happier. Her little boy was finally here. At 16.5 hours old, Cal was observed as jaundiced through a visual assessment (transcutaneous bilirubinometer) of his skin. At 23 hours old, Cal was described as having head-to-toe jaundice, but Sheridan and her husband, Patrick, were told not to worry. At 36 hours old, the baby boy and his mother were discharged from the hospital armed with a pamphlet about jaundice and the advice to “put Cal in the sunlight.”

From her research, Sheridan knew jaundice caused a yellow staining of the skin and that was about it. She did not know that severe jaundice, left untreated, could cause brain damage. She also did not know that a blood test was available to measure her son’s bilirubin level (another term she had never heard).

She did not know that had Cal’s blood been typed, the doctors would have realized that her blood type (O+) was incompatible with Cal’s blood type (A) and that incompatibility is a risk factor for kernicterus. She was also not aware of the treatments available to reduce high bilirubin: phototherapy or an exchange blood transfusion for severely high levels of bilirubin. At 5 days old, Cal’s first bilirubin blood test was measured at 34.6 milligrams per deciliter—anything above 20 milligrams per deciliter is considered severe.

“However, an exchange transfusion was not performed since the doctors recalled a recent ‘raising of the bar’ from 20 milligrams per deciliter to 30 milligrams per deciliter,” Sheridan recalls. Treatment was limited to phototherapy, which failed due to the amounts of bilirubin in Cal’s system.

At 6 days old, Cal had a high-pitched cry, respiratory distress, and increased tone. Also, he started to arch his neck in a way that is characteristic of opisthotonos, a condition of abnormal posturing. All these symptoms are indicative of kernicterus, according to literature on the disease.

Kernicterus is considered a rare disease, with less than 200,000 Americans affected by it, according to the National Organization for Rare Disorders. Also considered a “never event” by the National Quality Forum, it is largely preventable if the American Academy of Pediatrics (AAP) clinical guidelines for the management of hyperbilirubinemia are followed.

Had Sheridan known this information, Cal may have had his bilirubin tested earlier, appropriate treatment could have been administered, and Sheridan would not have cofounded the Parents of Infants and Children with Kernicterus (PICK) as a result of her son’s preventable disability.

What Is Kernicterus?
Kernicterus is a type of brain damage that can affect full- or near-term infants of 35 or more weeks gestation. It causes athetoid cerebral palsy, hearing loss, vision and dental problems, and, sometimes mental retardation, according to the National Center on Birth Defects and Developmental Disabilities department of the Centers for Disease Control and Prevention (CDC).

Severe jaundice—anything above 20 milligrams per deciliter—is classified as severe hyperbilirubinemia and demands immediate treatment to reduce the amount of bilirubin in the infant’s system before it can cause irreparable damage.

Early warning signs of kernicterus include very yellow to orange skin tones, generally beginning at the head and spreading to the toes; listlessness; high-pitched cry; and abnormal positioning, which progresses to motor abnormalities. It is a progressive disease and most of its signs are not detected immediately, according to Thomas Hegyi, MD, a neonatologist at University of Medicine & Dentistry of New Jersey-Robert Wood Johnson Medical School.

Although bilirubin testing is not mandatory, assessing the risk a child may have in developing severe hyperbilirubinemia is critical to determine whether kernicterus is an issue. Currently, AAP Guidelines require healthcare providers to assess the risk an infant may have of developing hyperbilirubinemia and monitor them if necessary.

Risk factors associated with the disease include a gestational age of 35 to 37 weeks; having a pathological reason for an increased bilirubin count, such as a different blood type from the mother; exclusive breast-feeding; and a previous sibling with severe hyperbilirubinemia, according to Michael Giuliano, MD, chief of neonatology at The Joseph M. Sanzari Children’s Hospital-Hackensack University Medical Center in Hackensack, N.J.

Giuliano says Asians are more prone to kernicterus, but if any combination of the risk factors is present, the child is at an increased risk. “Another major risk factor is if the child has blood outside the vascular tree where it does not belong,” he says. “For example, if there is a lot of bleeding or if the child has a cephalohematoma [blood on the scalp from the birth], the child’s bilirubin levels should be carefully monitored to ensure that kernicterus is never really of concern.”

However, Giuliano is ardent in explaining, “A child should never reach the point where he is showing signs of kernicterus. Their pediatrician should monitor the jaundice very carefully. If the levels of bilirubin reach a point where they feel it should be treated, it gets treated.

“If a child does show signs or symptoms of kernicterus, you allowed the bilirubin levels to rise far too high and that is why the new policy statement from the AAP has been issued so there is an assessment of risk and follow up is carefully done so these babies never have signs or symptoms of kernicterus.”

Monitoring Hyperbilirubinemia
“Any baby with untreated jaundice is at risk for kernicterus, but this does not mean that every baby with yellow skin will have brain damage,” the CDC states.

What it does mean is that there must be increased vigilance in assessing the risk a child may have in developing severe jaundice, Hegyi explains. “Neonatal hyperbilirubinemia is a physiologic jaundice that all newborn babies have and 60% have visible jaundice,” he adds. The CDC says that, at a minimum, babies should be assessed for jaundice every eight to 12 hours in the first 48 hours of life and again before 5 days of age.

Despite the fact that pediatricians, physicians, and nurses should be aware of hyperbilirubinemia, it is also important to note that if a baby is at risk of developing kernicterus, it will develop once the child leaves the hospital. “Usually, in term babies, jaundice peaks around 3 days of age and then it starts coming down,” Hegyi says. “In premature babies, however, it peaks later, at 5 to 6 days of age, and then it starts coming down. Bilirubin peaks higher in premature babies and the danger is that there are premature babies that are forgotten because they are so big and they go to the regular nursery and are discharged at 48 hours to 72 hours while the bilirubin is still rising.” Hegyi says the important thing is to not forget about the premature infant because of his or her size. They need fairly close and immediate follow-up with their pediatrician to monitor their bilirubin levels.

“If the baby leaves the hospital before or at 48 hours, he [or she] should be seen by the pediatrician no later than one to two days after discharge,” Giuliano says. “Always when the cases of kernicterus are seen, invariably it is that the mother and child have not been seen back for five to eight days and when they return the bilirubin levels are already high and the child shows evidence of kernicterus. These are the cases we worry about because if they were evaluated and treated earlier that would have alleviated the problem.”

Educating Parents About Jaundice
Although jaundice is not hereditary and the mother can do nothing to prevent it during her pregnancy, parents must be educated about both its treatment and possible complications, Hegyi and Giuliano say. Realizing there is a disparity between what physicians know about jaundice and what parents know, Hegyi says, “One of my projects is to teach medical students, physicians, pediatricians, and even obstetricians that one has to begin educating the mothers about neonatal problems. If they can educate mothers about breast-feeding then they can educate them about the risks for jaundice.”

Hegyi emphasizes the importance of communication. “The parent should be a partner with the physician in caring for the child,” he says. “I do not think that parents should be doctors, so a mother requesting a bilirubin test is not what should happen. I think that the parents should have an intelligent discussion with the physician about their concerns about jaundice and what is his plan to monitor the baby so the parents can understand better.”
Educating the parents is essential, Sheridan says. “Complications of jaundice are not discussed in the prenatal books. There is no discussion of the risk factors associated with it and there is no discussion that jaundice can cause brain damage,” she says.

Following Cal’s diagnosis, litigation proceedings began, as they do in most kernicterus cases. “Kernicterus babies are expensive babies,” Sheridan says. “In our case, there was no assignment of blame. But, in most cases, it appears that the parents are assigned the blame. Parents are not responsible for medical diagnosis nor should doctors expect that we know the complications of jaundice.”

To help educate parents about neonatal problems such as jaundice, the CDC has created a Jaundice Alert sheet that informs parents about what jaundice is, what causes it, signs of jaundice, and how it is treated. PICK is also working with the CDC, several hospitals, and other prenatal education resources to educate mothers about jaundice and its treatment.

PICK also began an awareness campaign called “What’s Your Baby’s Bili?” On the organization’s Web site, parents can purchase a rubberized bracelet to raise awareness of bilirubin and its effects.

Further Investigation
Despite the effort to educate parents and healthcare providers about jaundice and kernicterus, there is “no real evidence out there about how many kids have kernicterus,” Sheridan says. “I realize that healthcare providers do not like to scare their patients and I also realize that physicians like to base their discussions on evidence but without any real idea about how many children are affected by [severe hyperbilirubinemia], I challenge them to define rare.”

To date, there is no statistical data available on the number of children who have kernicterus, Hegyi says, although there is a voluntary database at Stanford University School of Medicine that is maintained by Vinod K. Bhutani, MD, a clinical professor of neonatology.

In his voluntary database, Bhutani has 172 cases from the last 15 years. He has seen a steady increase in the number of cases that have been reported since 1990. “This number is a gross under representation of the cases that are out there because kernicterus is strongly associated with litigation,” he says.

“According to the data, one in 10,000 babies are at risk for kernicterus and one in 30,000 babies will develop kernicterus. These numbers are significant. Only a select number of hospitals that we are associated with across the nation are submitting cases of kernicterus to this database. Because of gag orders implemented after litigation we will not know of the case of kernicterus unless if the physician, family, or attorney on behalf of the family submits information on the case,” Bhutani says.

To complement the efforts of the voluntary database, Hegyi, PICK, and the New Jersey Department of Health and Senior Services (HSS) are working along with the CDC to create a severe jaundice registry that will collect data on any child with a bilirubin level over 20 milligrams per deciliter. Marilyn Reilly, public information officer with the New Jersey Department of HSS, says, “The department is working with a group of outside experts to coordinate the specifics of the registry and it hopes to have it up and running by the middle of 2006.”

Learning the Hard Way
When Sheridan preregistered at the hospital before the birth of her second child, Mackenzie, she made two requests: She wanted her daughter’s blood to be typed and her bilirubin to be tested after birth. “Sure enough, her numbers were very high and she had a different blood type from me,” Sheridan says. “Mackenzie received phototherapy treatment before any damage could be done.”
Once Mackenzie began her treatment, Sheridan took a shower to relax. However, while showering, she began to cry. She cried so long that a nurse needed to be called in to be sure she was all right. Sheridan knew her daughter was not going to have kernicterus like her big brother.

— Kim M. Norton is a freelance writer/journalist.

FAQ About Jaundice
What is jaundice?
Jaundice is the yellow color seen in the skin of many newborns. It happens when a chemical called bilirubin builds up in the baby’s blood. Jaundice can occur in babies of any race or ethnicity, regardless of skin color. Low levels of bilirubin are not a problem, but a few babies have too much jaundice. If not treated, high levels of bilirubin can cause brain damage and a life-long condition called kernicterus. Yet, early detection and management of jaundice can prevent kernicterus. At a minimum, babies should be assessed for jaundice every eight to 12 hours in the first 48 hours of life and again before 5 days of age.

What causes jaundice?
Jaundice can develop when red blood cells break down and bilirubin is left. It is normal for some red blood cells to die every day. In the womb, the mother’s liver removes bilirubin for the baby, but after birth the baby’s liver must remove the bilirubin. In some babies, the liver may not be developed enough to efficiently get rid of bilirubin. When too much bilirubin builds up in a new baby’s body, the skin and whites of the eyes may look yellow. This yellow coloring is called jaundice.

What are some signs of jaundice?
Jaundice usually appears first on the face and then moves to the chest, belly, arms, and legs as bilirubin levels get higher. The whites of the eyes can also look yellow. Jaundice can be harder to see in babies with darker skin color. Doctors or nurses can and should test how much bilirubin is in a baby’s blood.

Can jaundice be treated?
Yes, it can. When being treated for high bilirubin levels, the baby will be undressed and put under special lights. The lights will not hurt the baby. This can be done in the hospital or even at home. The baby’s milk intake may also need to be increased. In some cases, if the baby has very high bilirubin levels, the doctor will do an exchange transfusion of the baby’s blood. Jaundice is generally treated before brain damage is a concern. Putting the baby in sunlight is not recommended as a safe way of treating jaundice.

Will my baby become jaundiced?
Approximately 60% of all babies have jaundice. Some babies are more likely to have severe jaundice and higher bilirubin levels than others. Babies with any of the following risk factors need close monitoring and early jaundice management:

• Preterm babies: Babies born before 37 weeks, or 81/2 months, of pregnancy may have jaundice because their liver is not fully developed. The young liver may not be able to get rid of so much bilirubin.

• Babies with darker skin color: Jaundice may be missed or not recognized in a baby with darker skin color. Checking the gums and inner lips may detect jaundice. If there is any doubt, a bilirubin test should be done.

• Heredity: A baby born to an East Asian or Mediterranean family is at a higher risk of becoming jaundiced. Also, some families inherit conditions (such as G6PD deficiency) and their babies are more likely to get jaundice.

• Feeding difficulties: A baby who is not eating, wetting, or stooling well in the first few days of life is more likely to get jaundice.

• Sibling with jaundice: A baby with a sister or brother who had jaundice is more likely to develop jaundice.

• Bruising: A baby with bruises at birth is more likely to get jaundice. A bruise forms when blood leaks out of a blood vessel and causes the skin to look black and blue. The healing of large bruises can cause high levels of bilirubin and your baby may get jaundice.

• Blood type: Women with an O blood type or Rh negative blood factor may have babies with higher bilirubin levels. A mother with Rh incompatibility should be given RhoGAM.

What can I do to make sure my baby’s jaundice does not cause brain damage?
Ask your doctor or nurse about a bilirubin test.

Create a follow-up plan before leaving the birth hospital. All babies 3 to 5 days of age should be checked by a nurse or doctor because this is usually when a baby’s bilirubin level is highest. The timing of the follow-up visit will depend on how old the baby is when he or she leaves the birth hospital and any other risk factors. Babies with jaundice in the first 24 hours of life or with high bilirubin levels before hospital discharge should have an early follow-up plan.

Treat jaundice seriously.

Ask your pediatrician to see your baby the day you call, if your baby:

• is very yellow or orange (skin color changes start from the head and spread to the toes);

• is hard to wake up or will not sleep at all;

• is not breast-feeding or sucking from a bottle well;

• is very fussy; or

• does not have enough wet or dirty diapers.

Get emergency medical help if your baby:

• is crying inconsolably or with a high pitch;

• is arched like a bow (the head or neck and heels are bent backward and the body forward);

• has a stiff, limp, or floppy body; or

• has strange eye movements.

— Source: Centers for Disease Control and Prevention (www.cdc.gov/ncbddd/dd/kernichome.htm)

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