Last spring my toddler came down with Kawasaki's disease. We are very lucky, because his pediatrician recognized his symptoms on the 5th day and he was treated. Six months have passed, and there are no signs of heart involvement. Our cardiologist says he will not be certain that there was no small vessel involvement until he is old enough for a stress test. I've joined the Kawasaki family support list on-line, and unfortunately there are many families out there that were not as lucky as us. When doctors don't find the diagnosis in time and treat with IVIG, the risk of heart involvement increases. There are some families whose children have DIED, because they didn't get therapy. This disease also seems to be becoming more common.

When my son had the first signs of the disease, I searched through all my children's care books and internet Web sites to see if there was any information. The only mention of Kawasaki's that I saw was in "What to Expect" and that mentioned that it happened primarily to Japanese/Asian descent children (my son is blond/blue-eyed). I realize that you are very busy and there are many questions like this directed at you, but please consider putting in an article on Kawasaki's. Your advice may convince someone to take their child to a doctor or prompt a doctor for a diagnosis that could save a child from having a severely damaged heart. Thanks!

A 3-year-old boy who loves trucks and ice cream is acting more irritable and easily frustrated than usual. Is this a phase--an echo of the "terrible twos"--or might he not be feeling well? Mom decides to check his temperature and can tell he is hot when she touches him. The thermometer shows a fever of 104 degrees. Is this a virus? Or might he need an antibiotic? A "quick" trip to the doctor's office, an hour in a waiting room crowded with sick children, and she has the answer--she thinks.

Her son's throat was red, and the glands in his neck were swollen, so the doctor started him on some amoxicillin. No strep test was performed. Ibuprofen (Motrin) brought the fever down, but every time the ibuprofen wore off, the fever came back. When Mom called to report this, the advice nurse suggested waiting a couple more days for the antibiotic to work. Two days later, another phone call prompted a switch to a stronger antibiotic.

But this child didn't have a throat infection; he had Kawasaki disease.

Kawasaki disease is an uncommon but important cause of fever in children. It was first noticed in Japanese children following World War II. While it is more common among the Japanese, we now know that it occurs in all racial groups, primarily in children fewer than 5 years of age. Sometimes Kawasaki disease appears in clusters of children, suggesting that infectious organisms may trigger the condition.

Kawasaki disease is an inflammation of the child's blood vessels. The most important blood vessels involved are those of the heart, including the coronary arteries. Kawasaki disease has become the most common cause of heart disease in children born without birth defects. If Kawasaki disease is not recognized and appropriately treated within 10 days, the risk for permanent heart damage increases. Children can die from heart problems during this illness. They can also die from heart attacks later in childhood or as young adults. Early treatment is effective in preventing this, but first Kawasaki disease must be recognized.

There is no specific blood test for Kawasaki disease. The diagnosis is based on symptoms. The disease is also called mucocutaneous lymph node syndrome because the classic symptoms are in the mucus membranes, skin, and lymph nodes. At an international symposium in Tokyo 10 years ago, expert participants agreed on a definition for Kawasaki disease (Pediatric Infectious Disease Journal 1989; 8:663):

A. Fever lasting for at least 5 days.

B. Presence of any four of the following five conditions:

1. Red eyes without discharge.
2. Changes of the mucus membranes of the mouth, including red throat or red and/or dry and cracked lips, or strawberry tongue (a white coat on the tongue through which swollen red bumps protrude).
3. Changes of the hands or feet, such as swelling and/or redness and/or peeling of the skin, usually beginning around the nails.
4. Rash on the trunk. May take many forms, but without blisters. Perhaps also present on the limbs or face.