Hyperbilirubinaemia is a common condition of newborns, including those that are otherwise healthy. If severe and untreated or poorly treated, the bilirubin can become deposited into the brain, causing brain damage called kernicterus which can cause a form of cerebral palsy that results in uncontrollable movements of the body, face, legs and arms, called athetoid cerebral palsy.

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Neonatal Hyperbilirubinaemia

In neonatal hyperbilirubinaemia, the total serum bilirubin level is greater than 5 mg/dL. Some degree of jaundice is present in up to 60 percent of newborn infants in the first week of life. Few have much problem with it and the condition is self limited. On the other hand, neonatal hyperbilirubinaemia can become associated with more severe illnesses such as metabolic disorders, endocrine disorders, anatomic problems of the liver, infections and haemolytic disease of the newborn.

The jaundice itself comes from unconjugated bilirubin, which is deposited into the skin and mucus membranes. The result is a yellow or orange colouration to the skin. The bilirubin is unconjugated, meaning that it hasn’t been processed by the liver, usually because the liver is overwhelmed and can’t handle the load of bilirubin.

There are many risk factors for hyperbilirubinaemia of the newborn. These include the following: -

• ABO or Rh incompatibility between the mother and the fetus
• Breastfeeding
• Taking Valium or oxytocin in pregnancy
• Having gestational diabetes
• Being of Asian or Native American ethnicity
• Birth trauma
• Birth infections
• Prematurity
• Family history

The bilirubin comes from haeme degradation. When haeme degrades, it eventually turns into bilirubin, which is insoluble in plasma and needs a binding protein in order to remain in solution. Normally, it is conjugated in the liver and passes through the stools. Babies make about 6-8 mg per kg of bilirubin per day in the neonatal period. This rate rapidly returns to normal adult levels by two weeks of age.

Kernicterus

Kernicterus is a type of brain damage in babies caused by jaundice. Jaundice is a condition that turns the skin and eyes yellow due to high levels of bilirubin. It is common for babies to be jaundiced; however, deeply jaundiced babies have very high amounts of bilirubin that move out of the bloodstream and into the brain’s tissue. When this happens, they become very lethargic and can begin to develop brain damage. This is noted by excessive sleepiness, they are difficult to wake up from sleep, they are unable to awaken fully and they cannot stay awake. These babies often have a high pitched cry and decreased muscle tone issues, such as being very floppy or hypotonic. They may also develop increased muscle tone, which is evidenced by backward arching of the head and back. As the brain damage progresses, the baby may develop a fever and hold his or her head in a very contorted position called “retrocollis” or “opisthotonus”. If you think that your child's condition has arisen as a result of medical negligence, contact our kernicterus solicitors for compensation advice at no cost.

The name kernicterus is derived from the Greek “kern” (meaning kernel) and “icterus” (meaning yellow). The term refers to the deep nuclei (the kernel) of the brain, more specifically the basal ganglia, becoming stained yellow. Kernicterus affects the globus pallidus, a very distinct part of the basal ganglia. It can also include brainstem nuclei lesions that affect hearing, eye movement, coordination and balance. These damaged areas are viewable on MRI scans.

It is important to note occurrences of kernicterus cases are quite rare. That said, acute kernicterus is a very serious issue that needs to be addressed quickly. When signs of this condition are present, brain damage is occurring. Treatment must be administered immediately to stop the damage, prevent further damage and, perhaps, reverse some of the damage already done.

In kernicterus, unbound lipid-soluble bilirubin is able to cross the blood brain barrier. Sometimes, albumin-bound bilirubin can make it across the blood brain barrier. The exact toxic level of bilirubin in the blood varies from infant to infant so there not just one number that does or does not lead to kernicterus. There should be a significant worry, however, with a bilirubin level above 25 mg/dL. If the infant is suffering from haemolysis, then the concern should be present if the levels reach 20 mg/dL.

Unfortunately, the effects of bilirubin toxicity on the brain are irreversible. The early signs of trouble are quite subtle. They usually start around day 3-4 after birth. If the baby survives the initial insult of bilirubin, there is the gradual development of motor delays, developmental delays, deafness and mild mental retardation. In newborns, one can see lethargy, seizures, irritability, feeding problems, a high pitched cry, low motor tone, hearing loss, apnea difficulties, high motor tone (later) and eventually athetoid cerebral palsy and mild mental retardation.

In the newborn, the physical exam reveals skin and mucus membrane jaundice that starts in the head and works its way down to the feet. If it has reached the soles of the feet, the bilirubin level is likely to be at least 15 mg/dL. If the bilirubin is less than 4 mg/dL, you will likely not notice it. On the other hand, if the jaundice is at the level of the nipples or below, it is increasingly difficult to predict accurately the level of the bilirubin.

The treatment of jaundice in the healthy newborn focuses on promoting fluid intake and using bilirubin lights to bring the bilirubin down. Bilirubin is sensitized to light and is conjugated by its presence. Bilirubin lights can involve lights placed in an isolette or a bilirubin blanket that the baby is draped in for several days until the bilirubin drops to normal levels. If the bilirubin level is toxic and kernicterus is a concern, then exchange transfusions are done to exchange blood with high bilirubin levels with blood that has low bilirubin levels. In cases of hyperbilirubinaemia associated with breastfeeding, the woman can continue breast feeding but should supplement the baby’s intake with fluids to reduce the level of bilirubin in the blood.

Four Main Elements

Kernicterus typically involves four main elements: movement disorders; deafness or some level of hearing loss; an upward gaze or other impairment of eye movement; and, the abnormal staining of teeth enamel. Kernicterus falls into the dystonic or athetoid categories of cerebral palsy. Athetoid cerebral palsy is classified by slow, involuntary movements. Dystonia refers to abnormal muscle tone and can occur without athetoid symptoms. Some individuals affected with kernicterus are completely deaf, while others have no hearing issues. Some may have problems with auditory processing, known as auditory “dys-synchrony” or “auditory neuropathy”.

Treatment

Babies diagnosed with high bilirubin levels can be effectively treated before brain damage occurs. Treatment with lights, known as “phototherapy”, is typically used and has been found to be very effective. This type of treatment makes use of the blue color in visible light, which alters the bilirubin to a non-toxic form that is water soluble and easily eliminated from the body. The treatment is carried out by placing phototherapy lights as close the baby as possible, while regularly monitoring bilirubin levels in the blood. Phototherapy is safe and should be commenced if kernicterus is suspected, without awaiting results from the first lab tests. The baby should be hydrated with plenty of fluids and fed formula through a tube. At this time, the baby should also be blood typed in case an exchange transfusion is required.

If bilirubin has reached excessively high, dangerous levels or in situations where bilirubin is predicted to rise quickly, an exchange transfusion, which is a more drastic treatment, will most likely be necessary. This procedure promptly removes toxic bilirubin from the blood and may be necessary when Rh or other hemolytic diseases are present in newborns.

Medical Negligence Solicitors

Our medical negligence solicitors deal with personal injury compensation claims arising from medical negligence by a healthcare practitioner involving kernicterus and hyperbilirubinemia. Our solicitors deal with compensation claims using the no win no fee scheme. To speak to a medical negligence solicitor about kernicterus and hyperbilirubinemia just email our lawyers offices or complete the contact form or call our solicitors helpline. Our solicitors offer advice at no cost and with no further obligation.

Kernicterus Overview

Kernicterus is a rare complication of childbirth that occurs in the newborn, who has a severely elevated bilirubin level. Hyperbilirubinemia results in jaundice or yellowing of the skin and eyes. If very severe and left untreated, the problem causes brain damage in the form of Kernicterus.

The cause of Kernicterus is an extremely elevated bilirubin level. Bilirubin is a pigment, which is yellow in color and which builds up if the liver can't process the breakdown product of red blood cells and hemoglobin. The body begins to build up the bilirubin and the end result is a yellow child. If the bilirubin count is very high for a period of time, the bilirubin builds into the brain, resulting in brain damage, which can be permanent.

Kernicterus is usually a problem that occurs in the first week of life but in can be seen up to three weeks of age. It is especially common in infants who have Rh incompatibility with the mother. This can lead to a condition known as hydrops fetalis, a sometimes fatal condition in which the infant makes large amounts of antibodies against maternal red blood cells and the red blood cells are broken up quickly and dangerously. Kernicterus is usually seen in babies who are sick or have some kind of blood condition but it can be seen in normal infants as well.

The symptoms of kernicterus include extreme yellowing of the skin (jaundice) as well as an absent startle reflex, marked lethargy or sleepiness and poor sucking reflex or inability to nurse. This is seen in the early stages of kernicterus. If the kernicterus is allowed to become more moderate, there is often an arched back with a hyper extended neck and a high pitched cry. The soft spot or fontanelles are bulging and there can be seizure behaviour. If the kernicterus is allowed to become severe or end stage, there is often hearing loss of the high frequencies first, mental deficiency, speech difficulties, muscle rigidity, and various movement disorders.

Doctors can do various tests to determine the presence of neonatal hyperbilirubinemia and kernicterus. The hyperbilirubinemia is checked for in a blood test, which will show a hemoglobin level of about 20 mg/dL or more. This usually persists, in spite of light therapy and other therapies. The doctor can also do a physical examination to see if there are signs that the elevated bilirubin is causing brain injury or if it is just causing minor symptoms such as lethargy. The bilirubin levels are often tested at regular intervals to see if it is increasing, decreasing or responding to therapy.

There are a couple of good ways to lower the bilirubin level and reduce the risk of full blown kernicterus. This involves phototherapy. Light is used to manage the levels of bilirubin in the bloodstream and can come in the form of a light box that the baby sleeps in or as a light blanket or light vest that goes up against the skin and shines a blue light onto the skin.

In severe cases, the infant can receive an exchange transfusion, which exchanges the bad blood along with the elevated bilirubin with normal blood that has normal levels of bilirubin in it. It rapidly decreases the bilirubin within the bloodstream. The treatment used depends on how old the baby happens to be and on the level of absolute bilirubin in the blood.

The outcome of kernicterus is guarded if it develops into its later stages. The outlook can mean that the infant has permanent brain injury or even death.

Prevention of kernicterus depends on checking and treating hyperbilirubinemia when it is in its early stages and before kernicterus can happen. If there is any sign of jaundice within the first day of life, a bilirubin should be performed and light therapy started if the infant has an elevated bilirubin. If the bilirubin is markedly elevated, both light therapy and exchange transfusions should begin before brain damage has had a chance to occur.