This is interesting in light of Jett Travolta's death
CHILDHOOD; A Heart Disease That Slumbers
By ELIZABETH OLSON
ON March 1, Chris Waddell, an 18-year-old college freshman, collapsed and died after football conditioning practice at Northwestern State in Natchitoches, La.
He had played football in high school, and after a physical had been cleared for college play, but the coroner later found that he had died from an acute myocardial infarction, or heart attack, stemming from a little-known disease he contracted as a baby.
At 8 months, Mr. Waddell was found to have Kawasaki syndrome, a mysterious fever that mostly strikes babies and toddlers, usually boys. The disease -- named after Dr. Tomisaku Kawasaki, the Japanese pediatrician who first identified it -- has no known cause and a range of symptoms that can make it difficult to diagnose. Doctors worry that it may have long-term cardiovascular effects, ones that are hard to monitor or predict.
Many patients who had the syndrome are now entering adolescence and adulthood, and studies are beginning to show that the disease may cause scarring of vessels supplying blood to the heart, creating cardiovascular risks in otherwise seemingly healthy adults.
Kawasaki syndrome is the leading cause of aneurysms -- inflammation and widening of the blood vessels -- among children in the United States. Last year, doctors reported 4,250 hospitalizations for the syndrome, 77 percent of which were for children younger than 5 years old.
Yet disease specialists say that Kawasaki syndrome is underreported. While the symptoms can be dramatic and nasty -- fever, rash, red eyes, swelling of hands and feet, swollen lymph glands, inflamed mouth, lips and throat and peeling skin -- many patients do not exhibit all, or even most, of them.
Some symptoms can appear with other illnesses, and there is no test for the syndrome, which also makes it difficult to pinpoint. Experts estimate that there may be as many as 10,000 cases in this country every year, and as many as one in four children who have it will develop heart complications, mostly from damage to the coronary arteries.
No one knows its cause, despite investigations into varied sources, from carpet mites to viruses. But it does not appear to be contagious.
''We have looked into a number of infectious agents, and none has panned out, so it's a mystery,'' said Dr. Ermias Belay, an epidemiologist at the National Center for Infectious Diseases of the Centers for Disease Control and Prevention.
Slightly more than 60 percent of victims are boys. The illness -- first diagnosed in the United States in the 1980's -- does not necessarily run in families, but there have been community outbreaks, the latest about four years ago in the Denver area.
So far, only in Japan -- where there are an estimated 6,000 cases a year -- have researchers been able to follow patients longer term. Among those now in their 30's who suffered damage to their blood vessels as a result of the syndrome, there appears to be a greater risk of hardening of the arteries.
''We suspect that those who have structural change in the arteries may be at risk,'' said Dr. Robert P. Sundel, program director of the rheumatology department at Children's Hospital Boston.
The problem is that doctors are not sure how to measure that risk. When blood vessels are altered in shape and quality, they can heal but will not function normally, Dr. Sundel said. Unseen irregularities or turbulence affects the vessels and weakens them. Dr. Sundel compared it to tearing the elastic smooth surface of a silk stocking.
''It does not stretch and expand normally,'' he said. ''It is more likely to get pulled and torn.''
The risk of death during the initial infection has fallen in recent years. Treatment with intravenous gamma globulin, which is blood plasma extract that contains antibodies, has helped to lower the mortality rate to less than 0.1 percent from about 2 percent. Some centers are also experimenting with using steroids in addition to gamma globulin.
Affected children fall into roughly three categories: those with large aneurysms; those with medium-size aneurysms; and those with no noticeable anatomical change. Without treatment with gamma globulin, those with larger aneurysms (8 millimeters or more) have a one-third chance of a heart attack in the year after infection.
The largest group of patients have no visible aneurysms, but their blood vessels do not dilate and restrict normally, Dr. Sundel said. Because the blood is flowing turbulently through the affected area, blood cells clump and raise the possibility of clotting which, depending on the area of the body, could lead to ruptures and heart attacks.
Chris Waddell, an offensive lineman who was 6-foot-1 and 293 pounds, had both coronary arteries blocked and a coronary artery aneurysm, a result of Kawasaki syndrome. The irregularities were not detected, and he practiced and played in 2003 without incident.
Dr. Darlene Ifill-Taylor, Mr. Waddell's stepmother and a pediatric psychologist in Greenbelt, Md., said she and her husband, Gregory, who is Chris's biological father, are concerned that not enough is known about the risks of the disease.
''Was Chris's death a fluke?'' she asked. ''Or should he have been on a special diet, or not played football?''
So far there is no answer.
''This could be a self-limiting disease that people outgrow,'' Dr. Belay said, ''but it should be monitored and investigated because it can cause cardiac complications. Small-size aneurysms could regress and disappear with time, but they could also grow and burst.''
At the moment all doctors can recommend is that children who had the disease in infancy be tested with electrocardiograms on a regular basis, and as they get older have cardiac stress tests. Some hospitals are beginning to use injectable dyes with CAT scans to assess the condition of arteries in Kawasaki patients.
All in all, it is a condition with no shortage of mysteries. For instance, researchers have noticed that Kawasaki syndrome peaks between late winter and spring.
But why? Researchers do not know.
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