Pulley Watson | Kernicterus Info

What is Kernicterus?

Kernicterus is a form of brain damage that can result when jaundice is not treated properly. Kernicterus can be prevented.

Jaundice, or yellowing of the skin, affects approximately 60% of all newborns. With aggressive early discharge of mothers and infants from the hospital, some babies who become jaundiced are not having their jaundice measured and treated before it peaks. The result may be catastrophic. Severe jaundice (elevated bilirubin) can cause cerebral palsy. When cerebral palsy is caused by untreated elevated bilirubin (severe jaundice), it is called Kernicterus.

RISK FACTORS FOR SEVERE NEWBORN JAUNDICE OR EXCESSIVE HYPERBILIRUBINEMIA

PREMATURITY
DECLINING GESTATIONAL AGE
LOW BIRTH WEIGHT
MALE SEX
DIABETIC MOTHER
LARGE FOR GESTATIONAL AGE (LGA)
BREASTFED
POOR FEEDING OR INADEQUATE NURSING
BRUISING
JAUNDICED SIBLING
Rh DISEASE
OTHER HEMOLYTIC DISEASES

AN ENZYME DEFICIENCY CALLED G6PD (GLOCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY)
JAUNDICED ON THE FIRST DAY OF LIFE
INFECTION
SICK BABY
ABO BLOOD INCOMPATIBILITY BETWEEN MOTHER AND BABY
WEIGHT LOSS GREATER THAN 10%
A RAPID RISE IN SERUM BILIRUBIN LEVELS
ETHNICITY SUGGESTIVE OF INHERITED DISEASE SUCH AS EAST ASIAN OR MEDITERRANEAN

Signs of Acute Kernicterus – A Medical Emergency

Signs of acute kernicterus (brain damage from excessive bilirubin) should be treated like choking – as a medical emergency. Even when brain damage from bilirubin is occurring it can be stopped, reversed or further damage prevented. Immediate treatment may mean the difference between a child with a clumsy gait versus one in a wheelchair, a child with a mild hearing loss at high frequencies versus one profoundly deaf, or a child who can swallow versus one needing a feeding tube. Even more importantly, immediate treatment may mean the difference between a completely normal child and one with cerebral palsy and hearing loss.

Abnormal or fluctuating tone, increased or decreased
Poor feeding
Difficulty being aroused or lethargy
A high-pitched cry, sometimes described like a cat's cry
Temperature fluctuations, fever or low temperature
Arching of the back and/or spine

These are signs that REQUIRE preparations for a blood exchange transfusion to quickly bring the bilirubin which is causing the severe jaundice to a safe level. While preparations are being made the baby MUST be given intensive phototherapy with the eyes covered and the lights as safely close to as much skin as possible. At the same time the baby must be fed orally or by gavage (tube) feeding, since feeding eliminates bilirubin through the stool, must be kept hydrated, and the body temperature corrected.

At the chronic stage, kernicterus may not be diagnosed until your child is several months old and has classic symptoms: athetotic cerebral palsy, auditory neuropathy, loss of upward gaze and yellow staining of the teeth. There are also classic MRI findings.

Can Kernicterus Be Treated?

Yellow jaundice is a common occurrence in breast-fed babies and some formula babies, too. In an otherwise healthy, term infant it often stays at levels below which treatment is needed. However, at higher levels or in a pre-term or sick baby or one with an infection, phototherapy must be given to bring the levels down. The baby must be placed under phototherapy lights and given supplemental feedings to encourage weight gain. Sometimes the level of bilirubin in the blood must be reduced quickly by way of a blood exchange.

Doctors and nurses must be able to recognize at-risk infants; infants of diabetic mothers, infants delivered after prolonged rupture of membranes, premature infants or infants having cephalohematomas or other bruising are at special risk. Physicians and hospitals must err on the side of aggressive treatment by phototherapy to prevent kernicterus in the rare infant who will get it without treatment.

Both phototherapy and a blood exchange require the pediatrician, nurse or neonatologist to do a heel stick and measure the level of bilirubin. If jaundice is not measured, treatment will not be forthcoming. A visual assessment of jaundice (yellow face, or to the navel, or to the legs) is notoriously unreliable. There are 2004 American College of Pediatrics Practice Parameters to help the hospital and physician know at what level and under what conditions to aggressively treat jaundice. The failure to diagnose and treat has been serious enough that 2004 JHACO (Joint Commission for the Accreditation of Health Organization) has put out an ALERT to its members reminding them how to prevent kernicterus.

WHEN BILIRUBIN IS VERY HIGH YOUR CHILD'S PHYSICIAN MUST DO THE FOLLOWING:

Begin intensive phototherapy. There is no benefit to a baby in delaying treatment.
Keep the lights on every possible minute, such as while performing other tests or while feeding.
Examine the baby for acute signs of brain damage from bilirubin (kernicterus ): lethargy, poor feeding, arching, a high-pitched cry.
Make treatment decisions based upon total bilirubin values (TSB or total serum bilirubin ). All bilirubin, whether direct (conjugated) and non-toxic or indirect (unconjugated) and toxic, binds to the same albumin sites displacing it into brain tissue. Do not subtract.
Make preparation for an exchange transfusion. It can always be cancelled if under phototherapy the bilirubin drops rapidly to a safe level.
Keep the bilirubin from reaching a potentially dangerous level. Intensive phototherapy, hydration, gavage (tube) feeding, and frequent bilirubin checks are easy.
Measure the bilirubin frequently and compare it to hour specific norms. A bilirubin of 8 mg/dl in an 18 hour baby places the baby at high risk for severe jaundice or hyperbilirubinemia. The same level in a 50 hour baby indicates a low risk for hyperbilirubinemia. Every hour counts in measuring and treating jaundice.

AUDITORY NEUROPATHY AND SEVERE JAUNDICE FAQs

1. Could my child's hearing loss be due to his newborn jaundice?

Yes. Severe jaundice can cause hearing loss. It can also cause auditory processing problems. The kind of hearing loss caused by severe newborn jaundice (or hyperbilirubinemia) may be called Auditory Neuropathy (AN) or Auditory Dys-synchrony. With Auditory Neuropathy (or AN) the inner ear, or cochlea, seems to receive sounds normally, but the signals leaving the cochlea may be disorganized. The hearing nerve may not process sounds normally.

2. What can I expect with AN?

A child with AN or auditory processing problems may or may not be deaf. The range of hearing loss varies. Other features of children with AN include difficulty understanding speech (made worse by noise), fluctuating hearing, and coordination problems that affect such every-day activities as walking, talking, running and writing.

3. My baby passed her newborn hearing screen. How can she be deaf?

A baby with AN caused by severe newborn jaundice was not born deaf. These babies pass their newborn hearing screens. Like many babies, they become jaundiced and the jaundice, for any number of reasons, becomes severe. If it is not treated by phototherapy before the bilirubin in the blood causing the yellow color becomes excessive, or treated quickly by an exchange transfusion if necessary, the bilirubin can pass through the immature blood-brain barrier and cause brain damage. This usually happens on the third or fourth day of life.

4. Can testing distinguish AN from other kinds of hearing loss?

Yes. In AN the hair cells of the inner ear work normally. Children with AN pass the OAE (otoacoustic emissions) hearing test, the test that measures the echo response from the inner ear. But they fail the ABR (auditory brainstem response) hearing test because bilirubin has damaged their brainstem. Their auditory brainstem response is absent or abnormal.

5. Who can help my child?

Many physicians and therapists can help. An otolaryngologist who is knowledgeable about kernicterus (the preventable neurological disorder caused by severe jaundice) will discuss cochlear implants for your child with you. Developmental pediatricians who understand the kind of cerebral palsy that comes from bilirubin induced brain damage can help. Speech, physical and occupational therapists all help. Gastroenterologists may be necessary for children who have eating difficulties.