Top Docs 2011

Mystery Disease: Inflammatory Response

For more than 35 years, doctors at Children’s Hospital Colorado have been examining an illness that incites unpleasant symptoms in the youngest of patients.

About once a week at Children’s Hospital Colorado in Aurora, Dr. Mary “Mimi” Glodé sees a patient with the roster of telltale symptoms she’s come to know well: fever, irritability, swollen lymph nodes, rash, bloodshot eyes, a red tongue with small raised bumps, cracked lips, bright red palms of the hands and soles of the feet, stiff neck and joints, and peeling skin around the toenails and fingernails. It’s not a pleasant sight to behold. Especially since most of Dr. Glodé’s patients are younger than five years old.

About 50 children with these symptoms land at the Children’s Hospital each year. They are the fortunate few who end up at a health-care organization that has a long history dealing with their unusual illness. Dr. Glodé and Dr. James Todd are infectious disease doctors who’ve worked together for more than three decades. Glodé is an expert in treatment; Todd was the first American physician to describe the disease. “We published a paper back in 1976, almost simultaneously with three other papers from around the country, that this disease existed here in the United States,” Todd says. “The disease had been previously described by a Japanese pediatrician as mucocutaneous lymph node syndrome.” Today, it is more commonly known as Kawasaki Disease, an illness characterized by the inflammation of blood vessels in the body.

Although the mortality rate for the disease is generally listed as less than one percent, that statistic may be misleading. The most troubling—and lasting—effect of the disease (which affects about 5,000 Americans annually) is that about a quarter of those who contract it develop heart problems in the later stages of the typically three-week-long illness. “This is a disease that can cause coronary artery disease in young kids,” Glodé says, explaining that the prolonged inflammation can damage the heart’s vessels. “The initial symptoms of the disease burn out after a while, but we may actually be seeing adults having fatal heart attacks from having untreated Kawasaki Disease in childhood.”

Which is why Dr. Glodé is admittedly obsessed with not only treating everyone with the disease, but also seeking the cause of this mysterious syndrome. While experts agree there is likely a genetic predisposition—which would explain the higher frequency among those of Asian descent—they also posit the disease is triggered by an as-yet-to-be-identified agent, such as a virus or bacteria.

Kawasaki Disease often presents as a normal infection, something kids might pick up at preschool. They’ll have a fever. They’re irritable. They get a rash. Their lymph nodes are swollen. Even if parents are concerned enough to visit the pediatrician, the syndrome can be mistaken for scarlet fever, a staph infection, or even mononucleosis. And, to make it that much more difficult on your everyday pediatrician, there is no test for Kawasaki Disease. It is typically a diagnosis of exclusion, although doctors can check for mild anemia, a high white blood cell count, and an elevated erythrocyte sedimentation rate, which indicates blood vessel inflammation. All of which means many people may be suffering through the disease’s early symptoms as youngsters, only to experience dire consequences from the disease later in life as well. The only way to prevent this from happening? “If your child has a fever for more than five days and has two or more of the other symptoms,” Glodé advises, “get him to the doctor and mention Kawasaki Disease.”

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