Kawasaki Disease
Posted: January 10th, 2009 | Filed under: Current Health News | Tags: kawasaki disease, kawasaki syndrome |
Kawasaki Disease is in the media these days because of its possible link to the sad death of John Travolta’s 16 year old son Jett. Kawasaki’s is not at all a common disease, and it causes death only very rarely.
About 30 years ago I was working as an RN on the pediatric floor at Cedars Sinai Medical Center in Los Angeles and I took care of a little Asian boy who was diagnosed with the disease. I can recall that we were treating him with large doses of aspirin. He was about five years old and I remember asking his doctor about the disease. The pediatrician pointed out the visible signs which are: fevers, irritated-looking eyes, very reddened lips and inside of his mouth, red inflamed looking hands and fingers, a reddish rash, and enlarged lymph nodes (ie. lymphadenopathy) in the neck area.
Kawasaki disease is also known as Kawasaki syndrome, syndrome meaning a collection of symptoms occurring together. The cause is not known, it is not considered to be contagious, but there may be a genetic component making siblings more likely to come down with the disease. It tends to occur more often in boys, under 5 years old, and more commonly in children of Asian heritage. Another name for it is mucocutaneaous lymph node syndrome, with ‘muco’ referring to the mucous membranes like inside the mouth, ‘cutaneous’ meaning the skin, and ‘lymph nodes’ which when enlarged are sometimes easily seen in the neck area. Persistant fever for more than a few days is usually the symptom that prompts the parents to seed medical care.
Those are the early outward signs of Kawasaki’s. What’s going on inside is vasculitis, inflammation of medium and small sized arteries. The vasculitis can be especially damaging to the coronary arteries which feed the heart muscle. For that reason, these little kids can get heart damage, even myocardial infarctions (heart attack). Kawasaki disease is now the most common cause of heart disease in children. It used to be Rheumatic fever. Most children recover from the disease without any serious problems.
Treatment is with a lot of aspirin, which decreases inflammation and fever. Additionally, aspirin thins the blood, thereby also helping avoid blockage in the coronary arteries. With the aspirin, Intravenous Gammaglobulin, IVIG, is given ideally within the first 5 to 10 days of illness onset. IVIG decreases coronary artery abnormalities caused by Kawasaki disease.
The greatest danger to the child is during the first 15 to 45 days after onset of the fever. In the small percentage of cases where the coronary arteries are heavily damaged, long term close medical attention is needed.
Here are two good references for more information:
MayoClinic.com Children’s Health Kawasaki Disease
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