Kawasaki Syndrome Treatment : Part 2

by Food and Drug Administration (FDA)

(Page 2 of 2)

Aspirin and IGIV

Early diagnosis and treatment helps minimize the danger from Kawasaki syndrome. Although the cause is unknown, it can be treated effectively, says Mary Glode, M.D., an infectious disease specialist with the Department of Pediatrics of the University of Colorado in Denver.

Researchers have found that a combination of aspirin and intravenous immune globulin (often abbreviated IGIV) is highly effective in preventing long-term coronary artery problems from Kawasaki syndrome. Initially, patients were given aspirin alone, but the findings of researchers prompted FDA to recently approve the addition of IGIV to the treatment of Kawasaki patients.

The role of IGIV is unclear. IGIV is a product made by purifying the naturally occurring antibodies in human blood plasma. Several pharmaceutical companies produce IGIV, but only one product, produced by Oesterreichisches Institut fuer Haemoderizate (OIH), a pharmaceutical company based in Austria, has been approved by FDA for use in treatment of Kawasaki disease.

According to John Finlayson, Ph.D., a protein specialist with FDA, "We don't really understand why immune globulin works, but the major benefit is in minimizing coronary aneurysms."

The immune globulin is given intravenously in either a single large dose of 2 grams per kilogram of body weight over 12 hours, or four smaller daily doses of 400 milligrams per kilogram (1 kilogram is about 2.2 pounds).

Besides minimizing the potential for cardiac damage, this treatment regimen seems to work to "turn off" the disease. If treatment starts within 24 hours of the onset of the disease, the child is usually feeling much better by the next day.

According to Newburger, aspirin provides both an anti-inflammatory and anti-platelet effect. The anti-inflammatory effect keeps the arteries from swelling, and the anti-platelet effect prevents clots from forming in arteries.

Aspirin is given is fairly high daily doses — from 30 to 180 milligrams per kilogram (for example, 405 to 2,430 milligrams per day for a 30-pound child) of the patient's body weight — for the first two weeks of the disease. (For comparison, one regular aspirin is 325 milligrams.) The dose is then lowered to 3 to 5 milligrams per kilogram for an additional six to eight weeks.

Patients who have some heart damage may be given low doses of aspirin for several months; those with more severe damage, for years.

Aspirin use in the treatment regimen for children is somewhat controversial. The association between aspirin and Reye syndrome in children with flu and chickenpox has made parents and doctors wary of using aspirin to treat children with any acute illness.

However, no association has been reported between Reye syndrome and the use of aspirin in children with Kawasaki syndrome. Therefore, the benefits of its use in this instance, under a doctor's direction, outweigh its risks. Children who need to continue low doses of aspirin for long periods after the acute stage of the disease are monitored closely for signs of chickenpox or flu, and the aspirin can be stopped for a few days if a child develops symptoms of these illnesses.

Inexplicably, a possible connection has been made by some studies between children contracting Kawasaki syndrome and recently cleaned carpets. Researchers at Cornell University Medical Center were the first to find the apparent association in a survey of the families of Kawasaki patients and a separate control group.

Researchers asked questions about other family members with the disease, the type of foods the patient had been eating, and whether or not the child was breast-fed.

The only factor that clearly differed between the two groups is that children with Kawasaki disease were more likely to have been exposed to carpets and rugs that had been shampooed, beaten, or vigorously cleaned within a month of the onset of illness. Most cases of Kawasaki syndrome occur between December and May, prompting some experts to recommend that families with infants and toddlers not clean carpets during winter and early spring.

Since the Cornell study, 13 additional studies have tested the connection. "Four studies have found an association between exposure to shampooed carpets and development of Kawasaki," says Shulman. "Nine of the studies failed to find an association."

Shulman explains that while the association is generally unproved, it may be valid in certain geographic locations. To be on the safe side, he recommends keeping children away from just-shampooed carpets for 24 to 48 hours.

Long-Term Outlook

According to Newburger, a Kawasaki patient's long-term outlook depends on how much damage is done to the heart. Patients who receive treatment early will likely suffer no damage to the arteries. More than half the children who do develop aneurysms recover within a year.

Patients who don't fare as well are those who don't receive treatment and develop extremely large aneurysms. "The most [severely affected] patients — those with aneurysms of at least 8 millimeters — those patients will probably not return to normal," says Newburger.

Because the disease was so recently identified, doctors don't yet know if there are any long-term effects that aren't apparent when the acute phase of the disease ends.

While most childhood fevers, sniffles, and watery eyes are no reason for alarm, parents should be aware that accompanied by other symptoms, the illness may require immediate medical attention. Recognized and treated early, Kawasaki syndrome can be stopped before it becomes a life-threatening problem.

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