Jaundice and Kernicterus
Jaundice is a common and easily diagnosed condition in newborns caused by elevated bilirubin levels. If jaundice is not properly handled, it can lead to kernicterus, a dangerous and permanent form of brain damage. Doctors can conduct diagnostic testing to determine a child’s bilirubin levels and take appropriate action depending on severity. They can treat jaundice using phototherapy, blood transfusions, or by treating any underlying causes. Because jaundice is so easily diagnosed and treated, kernicterus is highly preventable.
How Common is Jaundice in Newborns?
About 60% of full-term infants and 80% of preterm infants develop jaundice as a result of elevated bilirubin. In many infants, the jaundice will go away on its own. However, some infants may need extra help removing this excess bilirubin, and will be placed under a blue or purple phototherapy light, which helps the liver eliminate extra bilirubin, or be given a blood transfusion.
All babies should be checked for high bilirubin levels at a minimum every 9 – 12 hours in the first 2 days of life, and again at 5 days of life, or sooner depending on circumstances. This is done using a light meter to measure transcutaneous bilirubin levels. If the light meter shows high bilirubin, medical staff should order a blood test to show total serum bilirubin levels (a more accurate way to assess bilirubin levels in a baby’s blood). If serum levels are high, the baby must receive treatment. Often, a baby may be put under phototherapy lights if their transcutaneous levels are high while waiting for the blood test results. There are no negative side effects to phototherapy, and beginning therapy immediately helps avoid kernicterus, a form of irreversible brain damage. Medical staff should check to make sure the therapy is working with repeat blood tests.
Is Jaundice Dangerous for Babies?
While very mild levels of jaundice are not dangerous, more intense degrees of jaundice can be a red flag and cause for concern. Typically, jaundice is normal in babies, and it typically goes away around 1-2 weeks after birth. The bilirubin level peaks at about 2-5 days of age. However, babies should be treated for jaundice when:
- 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 24 hours of life
- There is a very fast rise in the baby’s bilirubin level
- There is a high level of conjugated bilirubin
In these situations, jaundice may be dangerous and should be promptly treated.
Some babies may have jaundice that is typically not serious, but still warrants monitoring. Some jaundice is related to breastfeeding:
- Babies who have trouble nursing or whose mother’s milk comes in slowly, called breastfeeding failure jaundice. Poor feeding can mean poor fluid intake, which means lower blood pressure and blood volume. Lower blood pressure can mean poorer excretion of waste products like bilirubin, which results in bilirubin buildup in the blood.
- Jaundice that lasts after 7 days of life not caused by genetic disease, called breast milk jaundice. Some researchers believe this is because breast milk can alter red blood cell breakdown.
Jaundice from breastfeeding can be avoided or treated with regular, frequent newborn feeding (8-12 times/day for the first several days of life).
What Causes High Bilirubin in Newborns?
Bilirubin is a byproduct of the body breaking down red blood cells. In adults, the liver breaks down about 1% of blood cells each day. However, newborns have many more red blood cells than adults when they are transitioning away from being in the womb. Their fetal red blood cells have a short lifespan, which means they produce a lot of bilirubin as a result. Newborn’s livers are also slower at clearing away bilirubin – In the womb, the mother’s placenta removes bilirubin, but after birth, the baby’s liver must adjust to removing the bilirubin, which can take some time. Some newborns’ livers are also slower to develop than others. This means their livers may have some trouble clearing away red blood cells at first, causing hyperbilirubinemia. In hyperbilirubinemia, bilirubin builds up in the baby’s blood and tissues. Because it is pigmented, this can cause the baby to have yellow-looking skin and eyes (jaundice). High bilirubin levels can happen if the liver is overloaded or injured, there are too many red blood cells to break down, or if a blockage prevents the bilirubin from moving through the biliary tract to the intestines.
Severe hyperbilirubinemia can occur if a baby has a condition that increases the number of red blood cells being broken down, such as:
- Blood type mismatch (Rh incompatibility) between mother and baby
- Cephalohematoma (bleeding under the scalp caused by difficult delivery, often involving vacuum extractors or forceps)
- A lack of certain important enzymes
- High red blood cell counts (polycythemia) (more common in babies who are small for gestational age or twins)
- Abnormally shaped blood cells
- Infection such as sepsis
- Macrosomic (large) babies of diabetic mothers are predisposed to blood cell problems.
Factors that make it harder for a baby’s body to remove bilirubin can also cause severe hyperbilirubinemia, including:
- Infections (such as syphilis, rubella, or sepsis)
- Certain medications
- 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
Other risk factors for high bilirubin include:
- Preterm birth
- Feeding problems
- Having a sibling with jaundice
- East Asian, Middle Eastern, or Mediterranean descent
- Certain drugs called sulfonamides (e.g. trimoxazole)
- Crigler-Najjar syndrome type I
- Gilbert’s syndrome
- G6PD deficiency
Symptoms, Diagnosis and Treatment of Elevated Bilirubin
How Do I Know if My Baby Has Jaundice?
Every baby is different and therefore may experience different symptoms at different times. However, there are certain signs and symptoms that may mean that a baby has jaundice. These include:
- Yellowing of the baby’s skin (usually starting on the face and moving down the body) and the whites of the eyes
- Poor feeding
- Lethargy, tiredness
- Hypotonia (in early stages) or hypertonia (in later stages)
- High-pitched or shrill cry
Diagnostic Tests for Jaundice and Hyperbilirubinemia
There are several diagnostic tests that can be done to confirm hyperbilirubinemia. These include:
- direct and indirect tests of bilirubin levels, checking whether the liver is passing the bilirubin so it can be excreted (direct) or is if it’s being circulated in the blood (indirect).
- red blood cell counts
- blood type and testing for Rh incompatibility (Coomb’s test)
The timing of the first appearance of jaundice helps with diagnosis and treatment. If jaundice appears within the first 24 hours, it is serious and should be treated immediately. If the jaundice appears on the second or third day after birth, it is usually “physiological jaundice,” which is the common jaundice seen in the majority of infants and is not serious. When jaundice appears on the third day to the first week, it may be from an infection. Later appearance of jaundice, is often related to breast milk feedings, but may have other causes.
Treating Jaundice and Hyperbilirubinemia
Treatment depends on many factors, including the cause of the hyperbilirubinemia and the actual level of bilirubin. Some common treatments:
- Phototherapy. The baby is exposed to a special blue-spectrum light that decreases the bilirubin levels. Blood tests are performed afterwards to check bilirubin levels to ensure the phototherapy is working.
- Fiber optic blanket. This is another form of phototherapy. The blanket is placed underneath the infant and can be used alone or along with regular phototherapy.
- Blood transfusion to increase the red blood cell count and reduce the levels of bilirubin.
- Treatment of any underlying causes of hyperbilirubinemia, such as infection.
What are the Types of Bilirubin Encephalopathy?
There are three different classifications of kernicterus:
- Acute bilirubin encephalopathy (ABE) is acute kernicterus, where bilirubin levels get too high and cross the blood-brain barrier, causing brain damage. If not promptly treated, ABE progresses to chronic bilirubin encephalopathy. This diagnosis is a medical emergency. Symptoms of ABE include lethargy, poor feeding, poor muscle tone, high-pitched crying, and seizures, among other signs. This is the stage of kernicterus where severe damage is ongoing and can be treated if diagnosed in a timely fashion. The gold standard of therapy for treatment of acute bilirubin encephalopathy includes double volume exchange transfusion, ‘triple’ phototherapy (two banks of lights from above as close to the baby as possible, and one from below), and gavage feedings to promote fecal excretion of bilirubin.
- Chronic bilirubin encephalopathy (CBE) is permanent and occurs when ABE is improperly recognized or treated, resulting in severe brain damage to the baby. CBE occurs in infants who survive the neonatal period after undergoing ABE. CBE causes cognitive impairment, movement disorders like cerebral palsy, auditory limitations, visual impairments, dental abnormalities, gastrointestinal reflux, and an impaired digestive system.
- Subtle bilirubin encephalopathy (SBE) is a chronic state which includes neurological, learning and movement disorders and hearing impairments.
Can Conjugated Bilirubin Cause Kernicterus?
There are two types of bilirubin: conjugated and unconjugated bilirubin. Unconjugated bilirubin is processed by the liver into conjugated bilirubin. Unconjugated bilirubin can cross the blood-brain barrier to cause kernicterus directly. Conjugated bilirubin by itself is nontoxic. However, both conjugated and unconjugated bilirubin are present in the blood and compete for spots in binding to albumin. Because of this competition, high levels of unconjugated bilirubin contribute indirectly to kernicterus as it outcompetes unconjugated albumin, allowing unconjugated bilirubin to cross the blood-brain barrier and cause damage. In the intestines, conjugated bilirubin can become unconjugated and be reabsorbed through enterohepatic circulation.
Conjugated hyperbilirubinemia (neonatal cholestasis) can occur in the presence of infections like E. coli and in the presence of hypoxic-ischemic injury. Conjugated hyperbilirubinemia is treated very differently (example here) than physiologic jaundice; it is key that medical professionals distinguish between the two.
Legal Help for Infant Jaundice and Kernicterus | Birth Injury Attorneys
Babies are often born with a condition called jaundice due to elevated bilirubin levels in their bodies. Easily diagnosed and highly treatable, doctors are trained to look for the signs of jaundice in newborns and treat it before the jaundice escalates into a type of brain damage called kernicterus. If your child has been diagnosed with conditions like cerebral palsy, seizures, or intellectual impairments as a result of kernicterus, it is likely a case of medical malpractice and you may be entitled to financial compensation.
At Reiter & Walsh, P.C., our attorneys handle 100% birth trauma cases, and they consistently secure multi-million dollar settlements for their clients. Reiter & Walsh ABC Law Centers is based in Michigan, but we handle cases throughout the United States. We’ve handled hypoxic ischemic encephalopathy cases in Michigan, Ohio, Washington D.C., Pennsylvania, Tennessee, Arkansas, Mississippi, Texas, Wisconsin, and more. Our birth injury team is also equipped to handle cases involving military medical malpractice and federally funded clinics.
Contact Reiter & Walsh, P.C. today to begin your free case review with our Detroit, Michigan hypoxic ischemic encephalopathy lawyers. Free of charge and obligations, we will answer your legal questions, determine the negligent party and inform you of your legal options. Our team is available to speak with you to set up an appointment in any of the following ways:
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