Jaundice

About 60% of newborn infants in the United States are jaundiced, that is they look yellow. Jaundice is the yellow coloring of the skin and other tissues. Jaundice can often be seen well in the sclera, the "whites" of the eyes, which look yellow. Many many babies look jaundiced (60%), but they are not deeply jaundiced, not jaundiced below the abdomen, and they act OK - they nurse, they aren't too sleepy, they have normal muscle tone, their cry is normal, they don't arch their backs.

Kernicterus

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. When babies begin to be affected by excessive jaundice, when they begin to have brain damage, they become excessively lethargic. They are too sleepy, and they are difficult to arouse - either they don't wake up from sleep easily like a normal baby, or they don't wake up fully, or they can't be kept awake. They have a high-pitched cry, and decreased muscle tone, becoming hypotonic or floppy) with episodes of increased muscle tone (hypertonic) and arching of the head and back backwards. As the damage continues, they may develop fever, may arch their heads back into a very contorted position known as opisthotonus or retrocollis.

Information for Parents: The Jaundiced Baby

Jaundice in Newborns and its Treatment

About 60% of newborn infants in the United States are jaundiced, that is they look yellow. Excessive jaundice in newborn infants may cause brain damage. Jaundice is caused by a high level of bilirubin in the blood (hyperbilirubinemia) and tissues. When bilirubin gets too high it can be treated. Norms exist for bilirubin in term and nearly term babies based on the age in hours after birth. Other factors, such as prematurity, blood group incompatibilities between infant and mother including Rh and ABO blood types, and bruising, especially cephalohematomas and caputs, can increase bilirubin production and lead to excessive jaundice.

Babies with high bilirubin levels can be effectively treated. Phototherapy (treatment with light) is usually very effective. It is the blue color that alters the bilirubin from a toxic form to a water soluble, non-toxic form that can be eliminated. At higher, more dangerous levels of bilirubin, or in certain situations where the bilirubin is expected to rise very rapidly, such as Rh or other hemolytic diseases of the newborn, a more extreme treatment may be used, exchange transfusion, to rapidly remove toxic bilirubin from the blood.

The Jaundiced Baby with Signs of Acute Kernicterus: A Medical Emergency

When signs of acute kernicterus occur in a jaundiced baby, permanent brain damage is occurring. Immediate treatment should be done to prevent further damage, and because perhaps some of the damage is reversible.

Treatment should be immediate triple-bank phototherapy lights put as close as possible to the baby, a stat measurement of blood bilirubin should be sent, but the phototherapy should be started before the bilirubin results come back. The baby should be hydrated with fluids and probably be tube fed an elemental infant formula. The baby should be blood typed for a possible exchange transfusion which should be done as soon as possible unless there is a large drop in the bilirubin and the baby improves before the blood is ready for an exchange transfusion.

Introduction: The Jaundiced Baby with a high Bilirubin and NO Signs of Acute Kernicterus

The bilirubin should be plotted on a nomogram such as the Bhutani and Johnson nomogram to see what percentile it is in. The cause of the jaundice should be determined. Measures to increase feeding and hydration, e.g. lactation couseling and increased breast feeding and/or temporary supplementation should be considered. Home phototherapy with a phototherapy blanket ("biliblanket") might be prescribed, but levels must be closely followed since the amount of phototherapy delivered by home systems is relatively small.

Jaundice and Preventing Brain Damage

When infants have signs of brain dysfunction from bilirubin toxicity, immediate treatment is needed to minimize permanent brain damage. The signs of acute bilirubin toxicity are: 1) abnormalities of tone, including increased tone (hypertonia), decreased tone (hypotonia), or a variation in tone from hypertonia to hypotonia, 2) lethargy, difficulty in arousing the baby, 3) a high-pitched cry, 4) arching the back and spine (retrocollis or opisthotonus), and 5) fever. Feeding or nursing is decreased, which makes matters worse not only because of dehydration, but because bilirubin is eliminated via the stool, and decreased feeding prevents bilirubin from being eliminated from the body. Expert neonatologists say that the most common cause of bilirubin levels rising high enough after discharge from the hospital to require readmission is inadequate feeding.

Experts have proposed a clinical scale called the BIND scale, for Bilirubin-Induced Neurologic Dysfunction. Babies are scored from 0-3 on each of three characteristics, tone, cry and mental status, with 0 being normal, and 9 the worst score. Degrees of severity of mental status, for example, would include with a normal awake baby or a sleeping baby who is easily roused, a lethargic baby who is difficult to rouse and falls back to sleep, a comatose baby responsive to only deep painful stimuli, and a comatose unresponsive baby. In any event, jaundice with any of abnormal signs such as lethargy, abnormal tone, arching, high-pitched cry, or fever, is a cause for immediate concern, and an urgent visit to a physician or hospital emergency room is required.

Some physicians have asked me, when the signs occur, isn't it too late to treat? NO! Although damage may have occurred, when the infant is jaundiced and signs are occurring, damage is continuing to occur. The sooner the bilirubin is reduced, the better, the less permanent brain damage will occur. THIS IS A TRUE MEDICAL EMERGENCY! Delay will make the damage worse.

With an excessively high bilirubin level, and with signs of acute kernicterus, arrangements should immediately be made for a double volume exchange transfusion. This may take a few hours, even in the best of medical centers. In the meantime, the baby should be given double or triple phototherapy with the lights as close as possible to the baby with maximal surface area exposed (and the eyes covered), and the baby should be fed orally or by gavage tube with Nutramigen or another elemental formula, to eliminate bilirubin via the gut. Dehydration may be corrected by intravenous infusion, but gastrointestional feeding should not be ignored unless the baby is seizing.

When bilirubin is very high, do not make or let your child's physicians make any of the following mistakes in care:

1. Not believing the bilirubin level from the lab, and delaying treatment while it is repeated. There is no problem in repeating the test, but don't delay treatment for an instant while waiting for the repeat - you have nothing to lose by treating with a huge dose of phototherapy, gavage feeding, hydrating, ordering a type and cross match and blood. If the bilirubin drops rapidly to a relatively safe level, and the child is aysmptomatic, the exchange transfusion can be cancelled.
2. Delaying treatment or interrupting phototherapy for diagnostic testing to determine the risk of an exchange. If a sepsis workup or LP is needed, or an echocardiogram etc., do it under the lights. If it's not possible, keep the lights on every possible minute. If the baby needs to go for a test out of the unit, the lights go with him or her.
3. Not examing the baby for signs of acute kernicterus
4. Using the indirect bilirubin instead of the total serum bilirubin. Although it is true that the direct (conjugated) bilirubin is non-toxic, it binds to the same serum albumin site as toxic bilirubin, displacing it into brain tissue. Use the total bilirubin.
5. Allowing the bilirubin to reach potentially dangerous levels. Visual inspection by experienced personnel, transcutaneous bilirubins, blood bilirubin are all easy to do. It's much easier to prevent bilirubin from rising too high than to treat it when it does.
6. Measuring the bilirubin and not comparing it to hour-specific norms. This is very important. A bilirubin level in a one-day-old may be normal or dangerously high depending on whether the baby is 24 or 47 hours old. A level of 8.5 would be in a high-risk zone (95^th percentile) in a 24h old baby, and in a low risk zone (40^th percentile) in a 47h old baby.

Kernicterus: Brain Damage due to Excessive Jaundice

Kernicterus is the name given to the severe form of brain damage that is caused by excessive jaundice in newborn infants. Kernicterus is from the Greek "kern" or kernal plus "icterus" or yellow.

Kernicterus refers to the yellow staining of the deep nuclei (i.e., the kernal) of the brain namely, the basal ganglia. Kernicterus involves a specific part of the basal ganglia, the globus pallidus. It also includes lesions of brainstem nuclei in auditory (hearing), oculomotor (eye movement), vestibular (balance) systems and the cerebellum (coordination). Today, abnormalities of the globus pallidus can be seen on MRI scans of infants with kernicterus.

Clinically, classic kernicterus involves: 1) specific movement disorder, 2) hearing loss or deafness, 3) impairment of eye movements especially upward gaze, and 4) abnormal staining of the enamel of baby teeth. An "athetoid" form of cerebral palsy is classic in kernicterus. Athetosis refers to the slow, writhing involuntary movements that occur. Dystonia, or abnormal muscle tone and position, also occurs. Some are deaf, some have normal hearing, and some with or without deafness have an auditory processing problem now called auditory neuropathy or auditory dys-synchrony. Auditory brainstem response (ABR) testing (also known as BAEP or BAER) is often abnormal, whereas other "hearing" tests, such as otoacoustic emissions (OAEs) and cochlear microphonic responses are normal.

Kernicterus is fortunately a very rare occurence.

Other forms of bilirubin-induced neurological damage may exist, including cognitive problems and auditory processing problems, one form of which is called auditory neuropathy or auditory dys-synchrony.

Caveats

The opinions in this article are solely mine except where I've cited others. I'm a child neurologist and medical researcher. I've been studying brain damage due to jaundice since 1982. I care very deeply about preventing brain damage, and kernicterus is a preventable form of brain damage that occurs in newborm infants. Many things are very well established about how bilirubin toxicity damages the brain, but unfortunately many other things are conjecture. I'll try to let you know which is which. Usually conservative in my clinical practice, in this area I would err on the side of more aggressive treatment. For example, if there is a possibility that subtle cognitive processing problems are caused by levels of bilirubin lower than are usually treated, and if it will take time for new studies to resolve the concern, then I'd err on the side of overtreating while there is still uncertainty because the cost is a few days or so of a very safe treatment, and the cost of not treating could be a lifetime of a neurological problem.

 Kernicterus Research

Information on Current Research Projects Worldwide
Information on Research Projects in Development
The BIND Study - Detection and prevention of Bilirubin-Induced Neurologic Dysfunction
Research's Exchange and Database
Sources of Funding for Kernicterus Research

Kernicterus Center of Excellence

Research at the Kernicterus Center of Excellence
Clinical Services available the Kernicterus Center of Excellence

Information for Parents

Survey of Newborns with Jaundice
Discussion Forum for Parents
Parents of Newborns with Jaundice
Parents who want to know if their child's problems could be due to newborn jaundice
Contact P.I.C.K., Parents of Infants and Children with Kernicterus -
   A parent's organization decicated to preventing and treating kernicterus

Information for Physicians

Information on making Donations

Donations to the Kernicterus Research Fund a supporting clinical and basic science research to detect, prevent and treat kernciterus and bilirubin-induced neurological disorders, can be made to:

Kernicterus Research Fund
MCV Foundation
Box 980234 Richmond, VA 23298-0234
804 828-9734
email: mdowdy@vcu.edu
website: www.mcvfoundation.org
The Kernicterus Research fund is a 501(c)3 educational and charitable fund

If you have any questions or comments, please feel free to contact us.

Dr. Steven M. Shapiro
Division of Pediatric Neurology
Departments of Neurology, Pediatrics, Otolaryngology and Physiology
Virginia Commonwealth University Health System
Box 980599 MCV
Richmond, VA 23298-0599
Clinical Office (804) 828-0442
Clinical Office Fax (804) 828-6690
Laboratory Office (804) 828-7416
Laboratory Office Fax (804) 828-5654
email: sshapiro@vcu.edu

Dr. Lois Johnson
Newborn Pediatrics
Pennsylvania Hospital
800 Spruce Street
Philadelphia, PA 19107
(215) 829-3301
email: johnsonl@pahosp.com

Dr. Vinod Bhutoni
Newborn Pediatrics
Pennsylvania Hospital
800 Spruce Street
Philadelphia, PA 19107
(215) 829-3301
email: vibhut@pahosp.com

Sue Sheridan
202 Sandra St.
Eagle, ID 83616
(208) 939-0260
email: psheri9110@aol.com
Co-Founder, Parents of Infants and Children with Kernicteruus (P.I.C.K.)
Son, Cal Sheridan, age 5, with kernicterus

Karen Dixon
517 Brentwood Drive
Birmingham, AL 35226
205 979-2021
email: ktdixon1@home.com
Co-Founder, Parents of Infants and Children with Kernicteruus (P.I.C.K.)
Son, Jess Dixon, age 11, with kernicterus