The Kawasaki Disease Foundation, comprised of parents and former patients, has declared January 26, 2011, as the first annual National Kawasaki Disease Awareness Day. People are urged to learn about the disease because prompt diagnosis and treatment can save lives and prevent complications.
More than 2,000 people in Hawai‘i have had the mysterious illness that mostly strikes young children, with 60 to 120 new cases diagnosed here annually, says Dr. Marian Melish, world-renowned infectious disease specialist at the University of Hawai‘i of Mānoa John A. Burns School Of Medicine.
Kawasaki disease, or KD, involves serious inflammation of blood vessels throughout the body that can affect the heart arteries. The cause is not known and there is no specific test to diagnose it. Often misdiagnosed or overlooked in infants and teenagers, it can result in death and heart attacks in the recovery period and later in life if not diagnosed in the first seven days.
KD is more common in Hawai‘i and Japan than anywhere else, says Melish, explaining the life-threatening illness is more prevalent among the Japanese although it has been reported in all ethnic groups. The incidence is about 16 times higher for Japanese and Koreans with 170 cases per 100,000, compared with 12 cases per 100,000 Caucasians. More than 80 percent of patients are under the age of 5.
Major symptoms include fever that lasts for five or more days; a rash, often worse in the groin area; red bloodshot eyes without drainage or crusting; bright red, swollen, cracked lips; “strawberry” tongue; swollen hands and feet and redness of palms and soles of feet; and swollen lymph nodes in the neck.
The disease was first described in Japanese medical literature in 1967 by Dr. Tomisaku Kawasaki of Tokyo, who saw his first patient on January 26, 1961. Not knowing of Kawasaki’s discovery, three Hawai‘i physicians—Professor of Pediatrics, Tropical Medicine and Medical Microbiology Melish, pediatric rheumatologist Raquel Hicks, and pediatric pathologist Eunice Larson—recognized the first KD cases outside of Japan in 1971 at Kauikeolani Children’s Hospital, now Kapi‘olani Medical Center for Women and Children.
The first international meetings on KD were held in Hawai‘i, and successful treatment—reducing the heart damage rate from 25 percent to 2 percent—was developed in the state. Melish continues to head and participate in research groups studying the disease.
Among recent isle patients are Hoyt Fujihara, 4-year-old son of Troy and Geri Fujihara of Salt Lake, and Ryder Hurtado, 3-year-old son of Dr. Greg and Stacie Hurtado of Mililani.
Fujihara was diagnosed last November 11, the day before his fourth birthday, after spots appeared across his body, a persistent fever and other signs. Hurtado was diagnosed on Christmas Day after developing a low-grade fever and other symptoms initially believed due to flu. According to their parents, both children are back in preschool.
“They have recovered without evidence of coronary disease because they were diagnosed and treated in the first week of illness,” Melish pointed out. “However, there may be serious problems if patients see multiple doctors in their illness or are initially seen by doctors unfamiliar with the disease.”
That is what happened to 19-year-old Juliette Machado of Kaneohe, daughter of Vicki and Dale Machado. Now a sophomore at Western Washington University, she was diagnosed with KD when she was 4½. However, she was not diagnosed and treated until the eleventh day of the illness when she already had three coronary aneurysms on her echocardiogram. Some markers of Kawasaki disease were missing and her symptoms weren’t recognized in the emergency room.
“A checkup a month after the diagnosis showed one of the aneurysms had enlarged with a clot forming that could have caused a fatal heart attack,” said Melish. “Machado was successfully treated with clot-busting medicine and kept on anticoagulants, which have reduced artery damage. Her latest tests this summer showed no sign of an aneurysm.”
Melish is striving to start an island chapter of the KD Foundation to create awareness of the disease in the general public and among doctors, and provide support and improved educational material to help parents and patients cope “with the scary unknowns of KD.” She also hopes to increase research support. The Hawaii KD Treatment Program and many island families have been participating in an International Genomics Consortium project studying genetic aspects of the disease. However, the National Institutes of Health funding will lapse on January 31.