A: About half of babies have some jaundice shortly after birth, which is caused by an excess of bilirubin in the blood. Jaundice can cause brain damage when bilirubin levels get too high and the bilirubin enters the baby’s brain tissue. High bilirubin levels can cause serious forms of brain damage such as kernicterus disease, cerebral palsy, and encephalopathy. The baby may also have seizures, intellectual disabilities, developmental delays, and hearing and sight problems.
The yellow-orange skin tone characteristic of jaundice is caused by excessive bilirubin in the blood. In babies, jaundice usually disappears around 1 – 2 weeks after birth. Bilirubin is the end product of red blood cell breakdown. Everybody experiences red blood cell breakdown, but in newborns, the breakdown is at a higher rate. This is because babies have more red blood cells than adults, which is part of the transition from being in the womb. Fetal red blood cells have a short life span, which leads to a lot of cell breakdown and excess bilirubin in the blood. Furthermore, babies have a slower time removing bilirubin from their bodies. In the womb, the placenta removes the bilirubin, but after birth, the baby’s liver begins removing the bilirubin and this can sometimes take a while. Some newborns’ livers are slower to develop than others.
HOW ARE JAUNDICE AND HIGH BILIRUBIN LEVELS TREATED?
Indeed, some babies have characteristics that cause their bodies to have excessive amounts of bilirubin in their blood, called hyperbilirubinemia. This should not be a problem, however, because testing for and treating high bilirubin levels is very easy. When a baby has hyperbilirubinemia, she can be placed under special lights to reduce her bilirubin levels. This is called phototherapy. A fiberoptic blanket is an alternative to phototherapy. In more serious cases, a baby may need a blood exchange transfusion, which is when her blood is slowly removed and replaced with donor blood.
Testing for hyperbilirubinemia involves placing a light meter on the baby’s head, which is called transcutaneous monitoring. If the transcutatneous bilirubin level is high, a blood test will be ordered. This will show the total serum bilirubin level, which is a more accurate assessment of bilirubin in the blood. All newborn babies should have their bilirubin levels checked every 9 – 12 hours in the first 2 days of life, at a minimum. The levels should be checked again at 5 days of life. When the serum level is high, the baby will receive treatment. In fact, if the transcutaneous level is high, treatment may be given while waiting for the blood test results. Repeat blood test samples should be taken to ensure that the treatment is working.
High bilirubin levels require treatment when one or more of the following occurs:
- There is a very fast rise in the baby’s bilirubin level
- Bilirubin levels (total serum bilirubin) are greater than the 95th percentile of what is normal for a newborn of a certain age (in hours)
- The baby has jaundice within the first day of life
- There is a high level of conjugated bilirubin (a form of bilirubin created by the chemical addition of sugar to serum bilirubin)
WHAT ARE THE RISK FACTORS FOR HIGH BILIRUBIN LEVELS AND JAUNDICE?
Risk factors for jaundice and hyperbilirubinemia include the following:
- Preterm birth
- Feeding problems
- Having a sibling with jaundice
- Darker skin color
- Mismatch in blood type between mother and baby (Rh incompatibility)
- Polycythemia (high red blood cell level)
- East Asian or Mediterranean descent
- Certain drugs called sulfonamides (e.g. trimoxazole)
- Crigler-Najjar syndrome type I
- Gilbert’s syndrome
- G6PD deficiency
WHAT ARE THE CAUSES OF HIGH BILIRUBIN LEVELS AND JAUNDICE?
High bilirubin levels can be caused by many different conditions. Severe hyperbilirubinemia can occur when a newborn has a disorder or condition that increases the number of red blood cells being broken down. These conditions include the following:
- Rh incompatibility
- A cephalohematoma, which is when a baby has bleeding under the scalp caused by a difficult delivery, often involving vacuum extractors or forceps
- An infection such as sepsis
- A lack of certain key enzymes
- A high red blood cell level, which is more common in babies who are small for gestational age or twins
- Abnormally shaped red blood cells
*Macrosomic (large) babies who have diabetic mothers are at an increased risk of having hyperbilirubinemia due to blood cell problems.
Factors that make it harder for a baby’s body to remove bilirubin can also cause severe hyperbilirubinemia. These include:
- Certain medications
- Infections such as syphilis and rubella, as well as sepsis and meningitis
- Diseases that affect the biliary tract or liver, such as cystic fibrosis
- A lack of oxygen in the baby’s tissues (hypoxia)
- Certain genetic or inherited disorders.
Babies who are breast-fed can get the following types of jaundice:
- Newborns who have problems nursing or whose mother’s milk is slow to come in may develop breastfeeding jaundice, also called breastfeeding failure jaundice.
- When babies have jaundice (not caused by genetic problems) that lasts after the first week of life it is called breast milk jaundice. This type may last for a month or more at lower levels of bilirubin. It is thought to be caused by the way breast milk substances affect the breakdown of bilirubin in the liver.
Breastfeeding failure jaundice typically occurs within the first week of life, as lactation failure leads to poor feeding by the baby, causing the baby to lose a lot of weight and fluid. This can cause the baby’s blood volume and blood pressure to drop, which can cause hyperbilirubinemia. One reason hyperbilirubinemia occurs in this scenario is because when blood pressure drops, the body doesn’t get rid of bilirubin as fast.
HOW CAN HYPERBILIRUBINEMIA AND BRAIN DAMAGE BE PREVENTED?
Regular and frequent feeding of a newborn can greatly reduce the chance of her having high bilirubin levels. Feeding should occur 8 – 12 times a day for the first several day of life.
All pregnant women should be tested for blood type and unusual antibodies. If the mother is Rh negative, follow up testing of the baby’s umbilical cord should occur. Even if a newborn does not have risk factors for hyperbilirubinemia, she should have her bilirubin levels frequently checked. If a newborn goes home shortly after birth, a follow up visit with the physician should be scheduled within the first seven days after birth.
Other actions that can prevent brain damage caused by hyperbilirubinemia include:
- Refraining from use of forceps or vacuum extractors during delivery can help prevent brain bleeds (cephalohematomas) that cause hyperbilirubinemia.
- If the physician does not believe the accuracy of a test and wants to confirm it, treatment should be started while waiting for confirmation. There are no downsides to treating a baby with phototherapy, increasing feeding and hydration, and ordering a type and cross match of blood in the event that an exchange transfusion is necessary.
- Phototherapy should not be postponed or interrupted for just because the baby is having tests done. Most tests can be done under the lights, or the lights can be moved with the baby if she needs an outside test.
- Bilirubin levels should be compared to hour-specific norms. A bilirubin level in a baby who is a day old may be normal or dangerously high, depending on whether the baby is closer to 24 hours of age or 47 hours of age.
HOW DO JAUNDICE & HYPERBILIRUBINEMIA CAUSE BRAIN DAMAGE, KERNICTERUS, ENCEPHALOPATHY, CEREBRAL PALSY & SEIZURES?
Bilirubin is a brain toxin. When bilirubin levels get too high, bilirubin can pass through the blood brain barrier and enter brain tissue. Bilirubin then builds up in the gray matter, which contains most of the brain’s neuronal cell bodies. Gray matter plays a major role in muscle control, seeing, hearing, memory, emotions, decision-making, speech and self-control. 95 percent of the oxygen that enters the brain goes to the gray matter. Damage to gray matter can cause cerebral palsy, seizure disorders, intellectual disabilities and speech, sight and hearing problems. Kernicterus usually causes athetoid cerebral palsy.
Kernicterus can be classified into 3 different types of encephalopathy (brain injury). Acute bilirubin encephalopathy (ABE) is a sudden onset of high bilirubin in the brain. Symptoms of ABE include the following:
- The baby is too stiff (has hypertonia) of too floppy (has hypotonia).
- Decreased feeding
- High-pitched cry
- Setting sun sign (eyelids are retracted up but eyes gaze down)
- Spasmodic torticollis (neck involuntarily turns up, down, left or right)
- Opisthotonus (severe hyperextension and spasticity whereby the body is extremely arched)
If high bilirubin level are not promptly decreased, ABE quickly progresses to chronic (long-term) bilirubin encephalopathy (CBE).
Chronic bilirubin encephalopathy (CBE) is when there are severe lesions (abnormal tissue) in the brain caused by bilirubin. Once CBE occurs, reducing bilirubin levels in the baby will not reverse the progression of CBE. A baby with CBE may exhibit the following:
- Athetoid cerebral palsy and / or dystonia (involuntary muscle movement)
- Hearing problems and auditory neuropathy (ANSD)
- Eye problems such as nystagmus, strabismus, impaired upward or downward gaze, and / or cortical visual impairment.
These medical problems are associated with lesions in parts of the brain stem and basal ganglia.
Subtle bilirubin encephalopathy (SBE) is a long term state of mild brain dysfunction. A child with SBE will have neurological, movement and learning disorders, and some hearing problems are also likely to occur.
BABIES SHOULD NOT DEVELOP BRAIN DAMAGE FROM HIGH BILIRUBIN LEVELS & JAUNDICE
When a baby has hyperbilirubinemia, the only way to prevent brain damage, encephalopathy and kernicterus is to lower serum bilirubin levels either by phototherapy or exchange transfusion. There is no excuse for a baby to develop brain damage caused by high bilirubin levels. Hyprbilirubinemia is very easy to test for and treat.
Physicians must remember that when signs of bilirubin induced brain damage first appear, it is usually not too late to treat the baby. This is because when a baby is jaundiced and signs are present, damage is continuing to occur. The sooner the bilirubin is reduced, the better, and the less severe the brain damage will be, in most cases. Hyperbilirubinemia is a medical emergency, and delay makes damage worse.
ABC LAW CENTERS: ADVOCATES FOR CHILDREN WITH KERNICTERUS & CEREBRAL PALSY FOR OVER 25 YEARS
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If your child was diagnosed with a permanent disability, such as kernicterus, cerebral palsy, a seizure disorder, or hypoxic ischemic encephalopathy (HIE), the award winning lawyers at ABC Law Centers can help. We have helped children throughout the country obtain compensation for lifelong treatment, therapy and a secure future, and we give personal attention to each child and family we represent. Our nationally recognized firm has numerous multi-million dollar verdicts and settlements that attest to our success and no fees are ever paid to our firm until we win your case. Contact ABC Law Centers at 888-419-2229 for a free case evaluation. Our award winning lawyers are available 24 / 7 to speak with you.