Kernicterus Solicitors - Medical Negligence Compensation Claim Lawyeys
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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.
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”.
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.
Kernicterus Medical 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.
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The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here