High fever. Rash. Redness of the eyes, lips and tongue. Painful joints. Swollen lymph nodes. An inconsolable, crying child.
These are the symptoms that can add up to something called Kawasaki syndrome, an inflammatory illness that appears almost only in young children and threatens their cardiovascular systems.
Kawasaki syndrome affects roughly 50 children in Hawaii every year. It occurs most frequently in Asian children, especially those of Japanese or Korean ancestry, so Hawaii sees more of the disease than other U.S. states.
Hawaii has been a center of research into the cause of Kawasaki syndrome and the best means of treatment. A symposium last month on the Big Island brought together doctors and other experts to discuss the latest advances (see adjoining story).
When 9-month-old Brayden was diagnosed, Pam Kutaka didn't know what to think, only how to feel.
"I was a wreck," the Honolulu mom said. "I started to cry, right there."
A few weeks earlier, the Yafusos from Pearl Ridge were hit with a double whammy. Both Bryce, 5, and Cody, 7, developed the disease.
That's rare, doctors say, but tearful second-graders in pain like Cody don't care much about being statistics.
"My head really hurt," recalled Cody. "And my eyes were really sore."
Fortunately for these and most children who develop Kawasaki syndrome, prompt treatment with aspirin and the blood component gamma globulin usually leads to a speedy recovery. However, the disease can cause coronary arteries to develop swellings aneurysms, and can cause heart disorders, especially if diagnosis and treatment are delayed. Blood clots can develop. If undetected, they may cause heart attacks.
The recent four-day symposium on Kawasaki syndrome at the Hilton Waikoloa Village offered little hope that the medical world was closer to identifying the trigger that sets off Kawasaki syndrome, said Dr. Marian Melish, infectious diseases specialist at Kapiolani Medical Center for Women and Children.
However, several scientific papers highlighted new treatment ideas that could lessen the damage done by the syndrome, also known as Kawasaki disease.
Melish was among the first to identify the syndrome in the United States; it was discovered in 1967 by Japan's Dr. Tomisaku Kawasaki.
At the symposium, a noted Atlanta adult cardiologist suggested that the disease may damage the protective inner lining of coronary arteries even in children who show no visible signs of aneurysms. This, said Dr. R. Wayne Alexander, could leave them with a heightened risk of developing heart disease later.
Melish said after the symposium that she feels the best course for Hawaii patients is to keep in touch with former patients and to advise them to adopt a heart-healthy lifestyle, including a low-fat diet and exercise.
The symposium spotlighted many high-caliber research efforts, she said, adding that technology will encourage more collaboration among researchers, who plan to communicate more regularly through a new, private Internet site.
"This is a group of people that's been meeting over 15 years, and this group is going to make more progress," Melish said.
Additionally, she said, University of Hawaii researchers have begun work on a test that could lead to quicker diagnosis of the disease. The test would detect two chemical factors in the blood that are elevated during the inflammation of blood vessels.
A few parents of children with Kawasaki syndrome also attended the symposium, including Mitsuru Asai, who formed a support group in Japan 17 years ago after the death of his son, before adequate treatment had been instituted.
In Hawaii, parents worry most about the long-term recovery of their children.
Junko Cameron of Hawaii Kai is happy that at least today's therapy restored her wailing infant daughter Leilani to her serene, healthy self.
"Twenty years ago," she said, "it was much more dangerous."
A few of the findings from the February symposium on Kawasaki syndrome held on the Big Island:
1. In patients with heart disease, the anti-clotting drug heparin in combination with an exercise program stimulated the development of new arteries, improving circulation to the heart. This can accomplish the same benefit as a bypass operation in some cases, without the need for surgery.
2. Patients who have the largest aneurysms are helped by a daily dose of warfarin (the "blood thinner" best known by the brand name Coumadin) and aspirin. Researcher Dr. Masato Takahashi of Los Angeles acknowledged that more study is needed, but the evidence so far suggests the drugs help prevent clots and stenosis, a dangerous thickening of the arteries.
3. A preliminary study of an anti-platelet agent called abcixomab indicates it helps with the shrinkage of aneurysms and may have other benefits.
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