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Birth Injury Law:  Jaundice, Hyperbilirubinemia & Kernicterus

Stanate: Treating Jaundice and Hyperbilirubinemia, and Preventing Kernicterus

Kernicterus is a preventable, but life-long neurologic disorder caused by severe and typically untreated hyperbilirubinemia during the neonatal period. High levels of bilirubin are toxic to a newborn.

Bilirubin is a normal product of heme degradation. The enzyme, heme oxygenase, converts hemoglobin to bilirubin and, thus, corresponds directly to the amount of bilirubin present. Newborns produce bilirubin faster than they can dispose of it and, as a result, typically experience a mild, transient jaundice after birth. The jaundice usually peaks at 96 hours, well after the mother and infant have left the hospital. See: Attallah Kappes, M.D., "A Method for Interdicting the Development of Severe Jaundice in Newborns by Inhibiting the Production of Bilirubin," PEDIATRICS, Vol. 113, No. 1 (January 2004).

High levels of bilirubin, hyperbilirubinemia, and the related symptoms can include severe jaundice, poor appetite, and lethargy. Hyperbilirubinemia can run a severe course, first unrecognized and then unmanageable. As a result, substantial brain damage can occur. The damage can take place rapidly and produce within hours the severe neurologic deterioration known as kernicterus. Kappes, M.D., p. 119.

Every year, several hundred thousand infants, of the more than four million births annually in the United States, receive phototherapy to treat jaundice. Putting a baby "under the lights" has been around as a means of treatment for more than 40 years. From a scientific standpoint, we know that light converts bilirubin into a new molecular species that is more readily excreted by the liver. Thus, bilirubin levels decline over time, although bilirubin overproduction may continue. Kappes, M.D., p. 119.

Phototherapy is still an "after the fact" method of treatment. It involves attempting to dispose of the bilirubin pigment after it has already been formed in the bloodstream. There are other issues related to phototherapy, for example, the fact it may involve a significant number of hours of treatment and it may further involve separation of the infant from the mother. Even with phototherapy, in some cases there is a rebound of hyperbilirubinemia.

Bilirubin is basically a waste-product related to old red blood cells the body no longer needs. The liver, under normal circumstances, filters the bilirubin from the blood and passes it to the bowel so the body can remove it in bowel movements. Because babies produce bilirubin faster than they can remove it, it can become harmful and toxic. When bilirubin levels in the bloodstream become very high, the bilirubin actually begins to move from the bloodstream into the brain. In the brain, bilirubin can directly harm the brain cells and actually cause permanent cellular damage, for example, in the form of kernicterus.

Hyperbilirubinemia in full-term, otherwise healthy infants, can lead to kernicterus. The literature suggests that boys are more susceptible than girls to adverse outcomes from hyperbilirubinemia. Treating hyperbilirubinemia with phototherapy, if the treatment is initiated promptly and continued until bilirubin levels normalize, can be very effective. Blood transfusions can similarly be effective in treating the more severe cases of hyperbilirubinemia. One of the major problems today is the fact that mothers are discharged with their newborns shortly after giving birth. Serum bilirubin levels may not increase until 48 to 72 hours and jaundice may not peak until approximately 96 hours.

Because insurance carriers do not desire to pay for mothers to stay in the hospital, they are typically discharged before peak levels of serum bilirubin or jaundice appear. Once home, absent returning and receiving competent medical care, the jaundice can get worse and the bilirubin levels can continue to increase to unmanageable and thereafter severely dangerous levels.

Kernicterus is irreversible once present and, as noted, life-long. Some of the major risk factors for hyperbilirubinemia in full-term newborns may be described with the benefit of the acronym, JAUNDICE:

  • Jaundice within 24 hours after birth.
  • A sibling, who was jaundiced as a neonate.
  • Unrecognized hemolysis, such as ABO blood type incompatibility or Rh incompatibility.
  • Non-optimal sucking/nursing.
  • Deficiency in glucose-6-phosphate dehydrogenase, a genetic disorder.
  • Infection.
  • Cephalo hematomas/bruising.
  • East Asian or Mediterranean descent.

 

In 2004, the American Academy of Pediatrics issued revised recommendations for screening babies with jaundice. The new guidelines take into account that most newborns go home with their mothers, for insurance reasons, within 48 hours of being born, which is often too early for the evidence of severe jaundice to appear. Although a little jaundice is common and usually goes away on its own, it can be, as noted, very serious and, unfortunately, the insurance companies have mandated that babies and their mothers be discharged before the serious nature of jaundice typically appears.

The guidelines provided by the American Academy of Pediatrics also take into accountthe fact more women are breast-feeding. Babies, who do not get enough milk, are at a higher risk for excessive jaundice. The American Academy of Pediatrics says newborns should be breastfed at least eight to twelve times a day for the first few days, however, many women new to breast-feeding may not know how to monitor how much milk their babies are receiving and, thus, the amount may be insufficient. The better appetite the baby has the less likely there is to be a problem with bilirubin or hyperbilirubinemia. The more the baby eats, the more hydrated the baby is, and the better the baby eliminates bilirubin in his/her stools.

An additional way to defeat kernicterus is to assure that all hospitals conduct bilirubin screenings on newborns. There is a particular blood test specific to bilirubin. If the bilirubin blood test comes back higher than 20 mg/dL, you should make sure treatment is initiated immediately for your baby. You can have the test repeated while treatment is already underway. You do not have the time to waste if the blood level comes back high.

You can ask your healthcare provider for a bilirubin test. The serum or blood bilirubin test is, unfortunately, still not done by many hospitals. And, insurance companies are still reluctant to pay for such a test even though it is very inexpensive. Because of insurance guidelines concerning payment, many women are now discharged within 24 hours. According to the American Academy of Pediatrics, an infant discharged before 24 hours should be seen by a physician within 72 hours. A baby discharged 25 hours to 48 hours after birth should be seen by a pediatrician by no later than 96 hours after birth. Babies discharged from 48 hours to 72 hours after birth should be seen by a pediatrician no later than 120 hours after birth. In the old days, because babies were typically discharged later, a newborn might not have his or her first visit with a physician for two weeks. Because insurance companies now prescribe much more rapid discharge, the guidelines identified by the American Academy of Pediatrics are critical to follow to prevent the type of life-long damage kernicterus causes. Features of kernicterus include, but are not limited to, athetoid cerebral palsy, mental retardation, sensorineural hearing loss, and gaze paresis.

We are honored to be involved with a pharmaceutical company, Infacare, started by Dr. Robert Vuckovich, which has developed a medication, Stanate (Tin-mesoporphyrin), which is currently in the clinical trial phase of the approval process before the Food and Drug Administration (FDA). It is a long, expensive, and tedious process to go through to obtain FDA approval, but Stanate has the potential to be one of the most significant new drugs introduced into pediatrics in many years. The trials conducted to this point, including trials already conducted in Greece and Argentina, evaluated numerous infants with seriously high bilirubin levels. The results have been dramatic.

In the first study conducted in Greece, Tin-mesoporphyrin demonstrated a dramatic (76%) reduction in the need for phototherapy, with no evidence of adverse reactions. The second trial, also performed in Greece, resulted in none of the infants who were given a single dose of Tin-mesoporphyrin at 24 hours requiring phototherapy for jaundice, compared with greater than 30% of the untreated population that required follow-up treatment. In the third study, conducted in Argentina, Tin-mesoporphyrin was given to 166 term infants, whose bilirubin levels reached seriously high levels, before receiving the drug. The results were equally dramatic; none of the treated infants reached a bilirubin level requiring any further follow-up treatment.

The concept behind Stanate (Tin-mesoporphyrin) is to provide an inhibitor of heme oxygenase to block bilirubin overproduction until the bilirubin-disposal mechanism in the infant matures. Interdicting (intervening to control) the production of bilirubin to moderate the course of hyperbilirubinemia seems far more logical than attempting to dispose of bilirubin after it has already been formed and potentially reached a dangerous level in the bloodstream. It is rare that you have the opportunity to participate with a drug or product that can be life-altering. Stanate (Tin-mesoporphyrin) is clearly just such a drug.

In the United States, we have been allowed to use Stanate on a "compassionate use" basis. Though it provides for very limited usage until FDA approval, the results have also been dramatic. A Jehovah's Witness, for example, cannot receive a blood transfusion. In those instances where the infants of Jehovah's Witnesses have reached severely high bilirubin levels, we have been allowed to provide Stanate. In every setting where it was provided, Stanate brought the bilirubin levels back to normal and prevented damage to the newborn.

We are honored to be a part of Infacare and we look forward to the day when Stanate will be available to all parents and their children.

-Riley Allen

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