HYPERBILIRUBINEMIA

Also called pathologic jaundice Bilirubin is a yellow substance that the body creates when it replaces old red blood cells. The liver helps break down the substance so it can be removed from the body in the stool. Characterized by a bilirubin level that exceeds 6 mg/dl within the first 24 hours after delivery and remains elevated beyond 7 days in a full-term neonate and beyond 10 days in a pre-term neonate A bilirubin level that rises by more than 5 mg/day A level thats greater than 12 mg/dl in premature or term neonates Conjugated (direct) bilirubin level that exceeds 1.5 to 2 mg/dl The prognosis for hyperbilirubinemia varies depending on the cause

Causes

It is normal for a baby's bilirubin level to be a bit higher after birth. When the baby is growing in the mother's womb, the placenta removes bilirubin from the baby's body. The placenta is the organ that grows during pregnancy to feed the baby. After birth, the baby's liver starts doing this job. This can take a while. Most newborns have some yellowing of the skin, or jaundice. This is called "physiological jaundice." It is harmless, and usually is worst when the baby is 2 - 4 days old. It goes away within 2 weeks and doesn't usually cause a problem. Two types of jaundice may occur in newborns who are breast fed. Both types are usually harmless.

Breastfeeding jaundice is seen in breastfed babies during the first week of life, especially in babies who do not nurse well or if the mother's milk is slow to come in. Breast milk jaundice may appear in some healthy, breastfed babies after day 7 of life. It usually peaks during weeks 2 and 3. It may last at low levels for a month or more. It may be due to how substances in the breast milk affect how bilirubin breaks down in the liver. Breast milk jaundice is different than breastfeeding jaundice.

Severe newborn jaundice may occur if your baby has a condition that increases the number of red blood cells that need to be replaced in the body, such as:

Abnormal blood cell shapes Blood type mismatch between the mother and the baby Bleeding underneath the scalp (cephalohematoma) caused by a difficult delivery

Higher levels of red blood cells, which is more common in small-for-gestational-age babies and some twins Infection Lack (deficiency) of certain important proteins, called enzymes

Things that make it harder for the baby's body to remove bilirubin may also lead to more severe jaundice, including:

Certain medications Congenital infections, such as rubella, syphilis, and others Diseases that affect the liver or biliary tract, such as cystic fibrosis or hepatitis Low oxygen level (hypoxia) Infections (such as sepsis) Many different genetic or inherited disorders

Babies who are born too early (premature) are more likely to develop jaundice than full-term babies. Symptoms Jaundice causes a yellow color of the skin. The color sometimes begins on the face and then moves down to the chest, belly area, legs, and soles of the feet. Sometimes, infants with significant jaundice have extreme tiredness and poor feeding. Exams and Tests Doctors, nurses, and family members will watch for signs of jaundice at the hospital, and after the newborn goes home. Any infant who appears jaundiced should have bilirubin levels measured right away. This can be done with a blood test. Many hospitals check total bilirubin levels on all babies at about 24 hours of age. Hospitals use probes that can estimate the bilirubin level just by touching the skin. High readings need to be confirmed with blood tests. Tests that will likely be done include:

Complete blood count Coomb's test Reticulocyte count

Further testing may be needed for babies who need treatment or whose total bilirubin levels are rising more quickly than expected.

Treatment Treatment is usually not needed. When determining treatment, the doctor must consider:

The baby's bilirubin level How fast the level has been rising Whether the baby was born early (babies born early are more likely to be treated at lower bilirubin levels) How old the baby is now

Your child will need treatment if the bilirubin level is too high or is rising too quickly. Keep the baby well hydrated with breast milk or formula. Frequent feedings (up to 12 times a day) encourage frequent bowel movements, which help remove bilirubin through the stools. Ask your doctor before giving your newborn extra formula. Some newborns need to be treated before they leave the hospital. Others may need to go back to the hospital when they are a few days old. Treatment in the hospital usually lasts 1 to 2 days. Sometimes special blue lights are used on infants whose levels are very high. This is called phototherapy. These lights work by helping to break down bilirubin in the skin. The infant is placed under artificial light in a warm, enclosed bed to maintain constant temperature. The baby will wear only a diaper and special eye shades to protect the eyes. The American Academy of Pediatrics recommends that breastfeeding be continued through phototherapy, if possible. Rarely, the baby may have an intravenous (IV) line to deliver fluids. If the bilirubin level is not too high or is not rising quickly, you can do phototherapy at home with a fiberoptic blanket, which has tiny bright lights in it. You may also use a bed that shines light up from the mattress.

You must keep the light therapy on your child's skin and feed your child every 2 to 3 hours (10 to 12 times a day). A nurse will come to your home to teach you how to use the blanket or bed, and to check on your child. The nurse will return daily to check your child's weight, feedings, skin, and bilirubin levels. You will be asked to count the number of wet and dirty diapers.

In the most severe cases of jaundice, an exchange transfusion is required. In this procedure, the baby's blood is replaced with fresh blood. Treating severely jaundiced babies with intravenous immunoglobulin may also be very effective at reducing bilirubin levels.

Outlook (Prognosis) Usually newborn jaundice is not harmful. For most babies, jaundice usually gets better without treatment within 1 to 2 weeks. Very high levels of bilirubin can damage the brain. This is called kernicterus. However, the condition is almost always diagnosed before levels become high enough to cause this damage. For babies who need treatment, the treatment is usually effective. Possible Complications Rare, but serious, complications from high bilirubin levels include:

Cerebral palsy Deafness Kernicterus -- brain damage from very high bilirubin levels

When to Contact a Medical Professional All babies should be seen by a health care provider in the first 5 days of life to check for jaundice.

Those who spend less than 24 hours in a hospital should be seen by age 72 hours. Infants sent home between 24 and 48 hours should be seen again by age 96 hours. Infants sent home between 48 and 72 hours should be seen again by age 120 hours.

Jaundice is an emergency if the baby has a fever, has become listless, or is not feeding well. Jaundice may be dangerous in high-risk newborns. Jaundice is generally NOT dangerous in term, otherwise healthy newborns. Call the infant's health care provider if:

Jaundice is severe (the skin is bright yellow) Jaundice continues to increase after the newborn visit, lasts longer than 2 weeks, or other symptoms develop The feet, especially the soles, are yellow

Prevention In newborns, some degree of jaundice is normal and probably not preventable. The risk of significant jaundice can often be reduced by feeding babies at least 8 to 12 times a day for the first several days and by carefully identifying infants at highest risk. All pregnant women should be tested for blood type and unusual antibodies. If the mother is Rh negative, follow-up testing on the infant's cord is recommended. This may also be done if the mother's blood type is O+, but it is not needed if careful monitoring takes place.

Careful monitoring of all babies during the first 5 days of life can prevent most complications of jaundice. Ideally, this includes:

Considering a baby's risk for jaundice Checking bilirubin level in the first day or so Scheduling at least one follow-up visit the first week of life for babies sent home from the hospital in 72 hours

Pathophysiology Hyperbilirubinemia can develop several ways Certain drugs (such as aspirin, tranquilizers, and sulfonamides) and conditions (such as hypothermia, anoxia, hypoglycemia, and hypoalbuminemia) can disrupt conjugation and usurp albumin-binding sites Decreased hepatic function can result in reduced bilirubin conjugation Increased erythrocyte production or breakdown can accompany a hemolytic disorder or Rh or ABO incompability Biliary obstruction or hepatitis may block normal bile flow Maternal enzymes present in breast milk can inhibit the neonates glucuronosyltransferase-conjugating activity As erythrocytes breakdown at the end of their neonatal life cycle, hemoglobin separates into globin (protein) and heme (iron) fragments Heme fragments form unconjugated (indirect) bilirubin, which binds with albumin for trasport to liver cells to conjugate with glucuronide, forming direct bilirubin Unconjugated bilirubin is fat-soluble and cant be excreted in the urine or bile; it may escape to extravascular tissue, especially fatty tissue and the brain, resulting in hyperbilirubinemia Unconjugated bilirubin can infiltrate the nuclei of the cerebral cortex and thalamus, leading to kernicterus (an encehalopathy) Although exact level is unknown, kernicterus may occur with serum bilirubin levels at or above 20 mg/dl (full-term) and at lower levels (about 14 mg/dl) in preterm neonates S/Sx of kernicterus: lethargy, decreased reflexes, seizures, opisthotonos, & high-pitched cry Possible causes include include hemolytic disease of the neonate, sepsis, imparied hepatic functioning, polycythemia, enclosed hemorrhage, hypothermia, hypoglycemia, and asphyxia neonatorum Glucose-6-phosphate deficiency (G6PD) increases the incidence of jaundice.

Phototherapy

Considered the treatment of choice for hyperbilirubinemia due to hemolytic disease of the neonate (after the initial exchange transfusion) uses fluorescent light to decompose bilirubin in the skin by oxidation Usually discontinued after bilirubin levels fall below 10 mg/100 ml and continue to decrease for 24 hours Albumin administration (1g/kg of 25% salt-poor albumin) to provide additional albumin for binding unconjugated bilirubin; done 1 to 2 hours before exchange or as a substitute for a portion of the plasma in the transfused blood Treatment of anemia caused by hemolytic disease To prevent hyperbilirubinemia encourage the mother to breastfeed at least 8 to 12 times per day. Dont skip feedings because fasting stimulates the conversion of heme to bilirubin. Also, dont supplement non-dehydrated breastfed infants with water or water and dextrose Assess and record the neonates jaundice in the first 24 hours after birth, and note the time it began; immediately report the jaundice and serum or transcutaneous bilirubin levels Obtain lab values as ordered, which may include blood type, Coombs test, CBC, reticulocyte count, G6PD, U/A & total and direct bilirubin Institute phototherapy as ordered Clean the neonates eyes periodically to remove drainage Offer extra water to promote bilirubin excretion Explain that the neonates stool contains some bile and may be breenish Assist with an exchange transfusion if indicated Administer Rho (D) immune globulin (human), as ordered, to an Rh (-) mother after amniocentesis or to an Rh (-) mother during the third trimester (for the purpose of preventing hemolytic disease once the neonate is born), after the birth of an Rh (+) neonate, or after spontaneous or elective abortion Reassure parents that most neonates experience some degree of jaundice Explain hyperbilirubinemia, its causes, diagnostic tests, and treatment; provide written information

Assess all neonates for risk of hyperbilirubinemia before discharge Explain the importance of follow-up visit to assess for hyperbilirubinemia Place the opaque eye mask over the neonates closed eyes, & fasten securely Undress the neonate, & place a diaper under him. Cover male genitalia with a surgical mask or small diaper to catch urine & prevent possible damage from the heat & light waves Take the neonates axillary temp every 2 hours & provide additional warmth by adjusting the warming units thermostat Monitor elimination, & weigh the neonate twice daily. Watch for signs of DHN (dry skin, poor turgor, depressed fontanels), & check urine specific gravity with a urinometer to gauge hydration status

Performing Phototherapy Set up the phototherapy unit about 18 inches (45.7 cm) above the neonates bassinet & verify placement of the lightbulb shield If the neonate is in an incubator, place the phototherpay unit at least 3 (7.6 cm) above the incubator, and turn on the lights Place a photometer probe in the middle of the bassinet to measure the energy emitted by the lights. The average range is 6 to 8 w/cm2/nanometer Explain the procedure to the parents Record the newborns initial bilirubin level and his axillary temp. Place the opaque eye mask over the neonates closed eyes, & fasten securely Undress the neonate, & place a diaper under him. Cover male genitalia with a surgical mask or small diaper to catch urine & prevent possible damage from the heat & light waves Take the neonates axillary temp every 2 hours & provide additional warmth by adjusting the warming units thermostat Monitor elimination, & weigh the neonate twice daily. Watch for signs of DHN (dry skin, poor turgor, depressed fontanels), & check urine specific gravity with a urinometer to gauge hydration status

 Take the neonate out of the bassinet, turn off the phototherapy lights, & unmask his eyes at least every 3 to 4 hours (with feedings). Assess his eyes for inflammation or injury Reposition the neonate every 2 hours to expose all body surfaces to the light and to prevent head molding and skin breakdown from pressure Check the bilirubin level at least once every 24 hours more often if levels rise significantly. Turn off the phototherapy unit before drawing venous blood for testing because the lights may degrade bilirubin in the blood. Notify the health care provider if the bilirubin level nears 20 mg/dl fullterm or 15 mg/dl if premature.