Kawasaki disease expertise crucial in hard-to-treat cases
When 16-month-old Brian developed a fever and a rash on his upper body, his father, James, and mother, Ning, initially thought it was the result of an allergic reaction to his first taste of crab meat. The next day his temperature soared to 105 degrees. After examining Brian, the family's pediatrician suspected his symptoms were the result of a food allergy. But the boy's high fever persisted. He developed sunken eyes, hives and became too weak to even sit.
His symptoms continued, and four days later he was diagnosed with Kawasaki disease in the emergency department of Northwest Community Hospital in Arlington Heights. Brian was immediately transferred to Children's Memorial Hospital, where the Center for Kawasaki Disease within the Division of Infectious Diseases is considered a worldwide leader in care, research and education to help children with the condition, which most commonly affects children 2 years of age or younger.
“Brian's diagnosis was the first time we'd ever heard of it,” says James.
James and Ning would learn much more during their young son's two-month hospitalization, which included three weeks in the pediatric intensive care unit (PICU ) at Children's Memorial.
“Brian's case was the most difficult to treat of the 1,300 cases we have treated here at Children's Memorial over the past 28 years,” says Stanford T. Shulman, MD . In fact, the search for an effective therapy led Shulman, one of the nation's top experts in this area, to consult with peers around the world.
Early diagnosis is critical
Kawasaki disease, which was first identified by Japanese physician Tomasiku Kawasaki in 1967, is a serious pediatric illness that causes inflammation of the blood vessels and can damage the coronary arteries of the heart. Early detection and diagnosis is critical, as one in four children with the disease develops heart complications without proper treatment. The disease is the most common cause of acquired heart disease in children in developed nations, and is diagnosed in approximately 3,500-5,000 children in the U.S. each year.
While Kawasaki disease is relatively uncommon, Shulman says he is seeing increasing numbers of patients each year, and that the center treated 73 children with Kawasaki disease in the past year. Symptoms include prolonged high fever, redness of the white parts of the eyes, dry cracked lips, red mouth and tongue, redness and swelling of the hands and feet, body rash and swollen lymph glands in the neck.
Although research has led to effective treatments, the cause of the disease remains unknown. Researchers like Children's Memorial's Anne Rowley, MD , are making important strides in solving the mystery. According to Shulman and Rowley, once the cause is known, a diagnostic test can lead to better treatments and perhaps the development of a vaccine to prevent it.
Seeking the best treatment
At least 90 percent of children with Kawasaki disease respond to the standard treatment of intravenous immunoglobulin (IVIG) and high-dose aspirin within a few days. Shulman was part of a team that first demonstrated the effectiveness of this treatment 20 years ago, a therapy that has saved thousands of children from developing heart disease. The majority of those who do not show improvement within 48 hours will respond to a second IVIG treatment.
Unfortunately, Brian was one of the 1 to 2 percent of patients who require additional therapies, which included courses of pulse steroids by IV and Remicade, an anti-inflammatory drug being used in a clinical trial at the hospital.
“It was a devastating experience, because we thought he would be released in a day or two,” says James. “But when you see one treatment after another that doesn't seem to be working, it scares you. We thought, ‘Maybe we're going to lose our baby, the most precious thing in our lives.'”