Neonatal jaundice describes a condition in which an infant’s skin appears yellow within the first few days of life. The yellowish appearance is a sign of an increased blood pigment called Bilirubin, which then settles in the skin. In many cases this is a normal process and occurs in about 2/3 of all healthy newborns. However, it may at times be a sign of a problem with the baby’s feeding, level of hydration or red blood cells lifespan. Other rare causes such as metabolism disorders, gland malfunction or liver disease can also present with jaundice. Only the health care provider can determine if the infant’s jaundice is normal and may order a blood test to help with diagnosis. In some cases, a specialist in liver disease or blood disorders may be called in to help take care of the newborn. Treatment can be very simple from increasing the baby’s water intake and modifying the feeding to very complex treatment. The choice of treatment is made according to the severity of the jaundice, the cause for the increase of bilirubin or the type of bilirubin.
The first symptom is yellow appearance of the skin and the eyes. The infant’s skin may appear yellow as early as the 1st or 2nd day of life. The jaundice starts around the head and the face then progresses to the shoulders, arms and the rest of the body including the legs and feet. The appearance may become more yellow when the baby is 3 to 4 days old and then slowly gets better. This is called “physiologic” or normal neonatal jaundice. Most infants have this pattern so no testing is needed.
At times, the yellow appearance may occur earlier (shortly after birth), last longer than 5-6 days or may be much more pronounced. A consultation with your health care provider is then needed to determine if testing is indicated.
Along with the skin becoming more yellow, the color of the baby’s urine can change from very light yellow or very dark brown. In the same manner, the color of the baby’s stool can vary from a yellow mustard color (normal) to light beige. These 2 color changes in the urine or the stool can indicate that the jaundice is due to different pigments. Although very rare in the first days of life, the presence of a very dark urine or light beige stool should be evaluated by a doctor immediately.
The yellow appearance comes from the accumulation of a yellow pigment called bilirubin in the skin. Right after birth, the infant body has to break down the red blood cells used while in the womb and make new ones now that the baby breathes the ambient air. The red color of the blood comes from a protein called hemoglobin, which carries the oxygen. As cells are being broken down, the hemoglobin gets modified in the liver and becomes bilirubin. Because the infant’s liver is so young and immature, it cannot keep up with all the produced bilirubin, which then leaks into the blood stream and settles in the skin.
A variety of conditions or diseases may present with an increase of the amount of pigment (bilirubin) produced. Poor feeding due to decreased breast feeding or the amount of breast milk can contribute to the increase in bilirubin. The same can happen with formula if the infant is not able to drink a sufficient amount. Maternal diabetes, exposure to some medications such as sulfa drugs or being underweight can also cause an increased bilirubin level.
Other conditions may be more serious:
- Increased production of bilirubin: In certain diseases, the red blood cells of the baby are destroyed at a faster rate than normal (this is called hemolysis). An example of such a disease is when the baby’s and mother’s blood type are different and not compatible. When this occurs, the mother’s immune system reacts and will form antibodies that attack the baby’s red blood cells. Babies also become anemic (low number of red blood cells) due to rapid destruction (hemolysis).
- Birth trauma: When vacuum extractors or forceps are used to deliver the baby a very large bruise over the scalp or the head may occur. This very large bruise will be re-absorbed. The old blood from the bruise will break down to make more bilirubin, which needs to be cleared by the liver. Some also may leak into the blood stream.
- Infection: Babies with infections may not be able to process bilirubin normally resulting in increased levels in the blood. This can occur with infection in the urine, blood, liver or other organs.
- Problems with bilirubin metabolism: In very rare cases the baby’s liver is not able to change the bilirubin into a form that can easily be removed from the body. This occurs in a condition called Crigler-Najjar syndrome. This is a very rare disease; the level of bilirubin increases very rapidly within hours. Immediate attention by a newborn specialist is then needed.
- Problems with digesting galactose: Rarely, babies cannot normally break down the sugar in breast milk (lactose) or in regular formulas made using the protein from cow’s milk. The sugar of the milk (lactose) is broken down into 2 smaller sugars called glucose and galactose. Rarely, the baby’s liver cannot process galactose. This is called galactosemia. This disease can present with jaundice in the newborn period and is associated with other severe symptoms (such as lethargy, vomiting, irritability and possibly convulsions). Galactosemia is often detected by a blood test (heel prick) before discharge from the nursery as part of the mandatory state screening for newborn diseases. Galactosemia is treated with strict dietary avoidance of galactose. This is not the same as being lactose intolerant and the two conditions should not be confused.
If the doctor is concerned about the severity of the jaundice, a blood test called a serum total bilirubin level is performed using a very small amount of blood. Other tests such as a transcutaneous (through the skin) test may be used to determine the bilirubin level in certain hospitals. This test is less accurate and needs to be confirmed with blood testing.
If the result is high, your doctor will order a blood test that will measure the different types of Bilirubin pigments, which make up the total bilirubin:
- Unconjugated or indirect bilirubin: This pigment is increased mostly in infants with neonatal jaundice. It is the bilirubin associated with normal destruction of older red blood cells. This is called physiologic jaundice. The baby’s urine is usually light yellow and the stool color is mustard yellow or darker.
In some cases, the level of indirect bilirubin can go very high. Then, a neonatal specialist or blood specialist may be called in to help care for the newborn. Doctors are concerned if the bilirubin levels are more than 20-25 mg/dl (deciliter) and will start treatment to prevent the bilirubin from getting to this level. A level of indirect bilirubin at or above 20-25 mg/dl may cause irritation in some areas of the brain. This is called acute encephalopathy (inflammation of the brain). If the bilirubin remains very high, above 25 mg/dl, babies can be at risk for significant brain damage. This very rare condition is called kernicterus. Because of that risk, the doctor will start testing early for the bilirubin level and repeat the test often to identify the trend and start treatment rapidly.
- Conjugated or Direct bilirubin: The previous pigment (indirect or unconjugated bilirubin) is packaged in the liver into a form ready for removal into the bile and the gallbladder. This pigment is called conjugated (packaged) or direct bilirubin. For a variety of reasons, the liver cannot get rid of it, the direct bilirubin leaks back into the blood and also settles in the skin. At times the urine of the baby can be dark “coca cola” color and the stool can be light beige.
The symptoms can be very different from those of normal neonatal jaundice. Babies can be very irritable, fussy, may have fever or they can have no symptoms. In addition to blood work to look for infections, other testing may be performed. A specialist in liver disease in children, called a pediatric gastroenterologist, may be called in consultation to help sort out the diagnosis. Liver disease is diagnosed with additional blood tests; the specialist may order an ultrasound or other specialized testing. These may lead to a procedure called a liver biopsy, where a small sample of liver tissue is taken for examination under a microscope.
The treatment varies based on the cause of the jaundice and the bilirubin level. For the purpose of this information we will discuss only the treatment for the elevation of the unconjugated or indirect bilirubin pigment. We will not discuss the jaundice associated with liver disease or the rare diseases causing an elevation of the pigment called conjugated or direct bilirubin.
Usually in normal physiologic neonatal jaundice, the process will be self-limiting and the baby does not need to be treated. The unconjugated bilirubin is broken down just with some exposure to the indirect sunlight. This is by far the vast majority. The baby may be able to be discharged home from the nursery within 48 hours of life without problems. The baby will need to be followed up by the pediatrician to ensure that the bilirubin level is going down and the baby’s weight is appropriate. This is especially true with breast-fed babies.
If the unconjugated or indirect bilirubin level remains high or is increasing, the baby may need further treatment to decrease the bilirubin level. Treatments might include:
- Some babies have a high indirect bilirubin level associated with breast feeding. Holding breast feeding and supplementing with infant formula for 48 hours may in some cases decrease the bilirubin in babies with “breast feeding jaundice.” A small amount of breast fed babies may continue to have elevated indirect bilirubin after 10-14 days. Once again holding breast feeding for 2 or 3 days may be sufficient and breast feeding can resume when the level of indirect bilirubin is lower. Breast feeding is by far the best option for neonates and should not be stopped completely because of a mild elevation of the unconjugated or indirect bilirubin. Families should speak with their physician or health care provider to determine if stopping breast feeding is appropriate and for instructions on how to proceed.
- Phototherapy is a treatment that allows the bilirubin under the skin to be broken down by a special light that illuminates the baby’s body. These lights are usually blue-green. They are placed about 4 inches above the baby. The more skin that is exposed to the lights, the better they work to break down a larger amount of unconjugated or indirect bilirubin. The lights do not prevent the baby from drinking formula or being breast-fed. The baby can be safely removed from the phototherapy at feeding times without decreasing the efficacy of the treatment. There are generally no significant risks with phototherapy. The eyes of the baby will have shields to prevent the light from harming the baby’s eyes and retina. There are no risks to the genitals of the baby. As long as the level of bilirubin is not very high, the phototherapy treatment can be done at home with a special blanket called a “bili” blanket. The physicians will then arrange for blood tests to be done regularly to ensure that the treatment is working. Most insurance will pay for this treatment at home.
- For a few babies, the level of unconjugated or indirect bilirubin is so high (greater than 20-25 mg/dl) that physicians are concerned about brain damage. The level has to be brought down very fast using a technique called exchange transfusion. An exchange transfusion is performed in the neonatal intensive care unit. The baby’s blood is exchanged and replaced very slowly and carefully with a donor’s blood. This allows for the indirect bilirubin to be removed faster, which will decrease the risk of further complications. This treatment is reserved for the most serious cases at risk for developing kernicterus (a condition where the indirect bilirubin is stored in areas of the brain and causes abnormal movements and seizures.)