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Children who have been diagnosed with Kawasaki disease are usually admitted to hospital, where gamma globulin is typically administered through an intravenous (I.V.) line for 10 hours. This dramatically reduces the risk of damage to the coronary arteries and diminishes the inflammatory process in general. The patient is also given relatively high doses of aspirin for the first two weeks to reduce fever, swelling and inflammation. Some other medications may also be employed depending on the response of the child to the initial treatment. These may include steroids and medications that inhibit clot formation.
This combination of medications tends to show good results within 24 hours. Because gamma globulin may make the child less responsive to viral vaccines, common childhood vaccines are generally delayed for a number of months after treatment. For example, the measles-mumps-rubella vaccine is delayed for 11 months, and the chicken pox vaccine is delayed for at least five months.
After the initial course of high-dose aspirin, low-dose aspirin (or another antiplatelet) will likely be prescribed for another four to six weeks to prevent the formation of blood clots. Physical activity is also restricted during this time.
According to the American Heart Association, any further treatment is determined by which of the following five risk levels best characterizes the young patient:
| Risk Level |
Definition |
Further Treatment |
|
I
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No cardiovascular damage seen on the echocardiogram at any stage of the disease.
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Follow-up tests by a pediatric cardiologist for a year, after which no further medical management is necessary unless otherwise determined by a physician.
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II
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Cardiovascular damage seen on the echocardiogram only while symptoms are present, after which the damage heals.
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Follow-up tests by a pediatric cardiologist for a year, after which the cardiologist may either determine that the child needs no further medical management or may ask the child to return every three to five years for additional follow-up.
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III
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One small to medium aortic aneurysm seen on the echocardiogram or coronary angiogram.
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Long-term aspirin therapy until the aneurysm has been treated. Follow-up tests by a pediatric cardiologist every year until the child turns 10, after which a stress test will be done to determine the child’s tolerance for physical activity. This test may be repeated every other year or as necessary. An abnormal stress test may prompt the cardiologist to order a coronary angiogram
|
|
IV
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One or more large aortic aneurysm or a number of smaller aneurysms.
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Long-term aspirin therapy, with or without anticoagulants. Follow-up tests by a pediatric cardiologist every six months to a year until the child turns 10, after which a stress test will be done every year. An abnormal stress test may prompt the cardiologist to order a coronary angiogram.
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| V |
Blockage(s) somewhere in the coronary arteries, as confirmed by a coronary angiogram.
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Long-term aspirin therapy, with or without anticoagulants. Restricted physical activity (e.g., no weight-lifting). Follow-up with a pediatric cardiologist every six months. A Holter monitor should be worn for a period every year until the child turns 10, after which a stress test will be done every year. Cardiac catheterization to evaluate the need for a catheter-based procedure such as balloon angioplasty with stenting.
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The long-term aspirin therapy recommended for children in risk levels III to V should not cause problems in an otherwise healthy child, but there is a small risk of developing Reye syndrome if the child comes down with the flu or chicken pox while taking aspirin. Children on long–term aspirin therapy may be given the flu vaccine to help reduce this risk. Parents of children on aspirin therapy are urged to contact their children’s pediatrician as soon as possible if their children show any signs or symptoms of the flu or chicken pox.
Some research has indicated that adding steroids to the treatment regimen may reduce the risk of long-term complications. However, more research is needed on this subject.
Data has shown that there may be microscopic changes in the walls of the coronary arteries or in the tissues around the coronary arteries even years after Kawasaki disease. Some pediatric cardiologists recommend that all patients with this condition be rechecked every two to three years indefinitely.
Patients and parents are advised to discuss any questions they may have about Kawasaki disease with their pediatrician or cardiologist.
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