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Kawasaki Disease

Posted Jun 23 2009 6:54pm

What Is Kawasaki Disease?

Kawasaki (KAH-wah-SAH-ke) disease is a rare childhood disease. It's a form of a disease called vasculitis (vas-kyu-LI-tis).

In Kawasaki disease, the walls of the blood vessels throughout the body become inflamed. The disease can affect any type of blood vessel in the body, including the arteries, veins, and capillaries.

Overview

The cause of Kawasaki disease isn't known. The body's response to a virus or infection combined with genetic factors may cause the disease. However, no specific virus or infection has been found, and the role of genetics isn't known.

The disease can't be passed from one child to another. Your child won't get it from close contact with a child who has the disease. Also, if your child has the disease, he or she can't pass it on to another child.

Kawasaki disease affects children of all races and both genders. It occurs most often in children of Asian and Pacific Island descent. It's more likely to affect males, and most cases occur in children younger than 5 years.

One of the main symptoms of this disease is a fever that lasts longer than 5 days. The fever remains high even after treatment with standard childhood fever medicines. Children with the disease also may have red eyes, red lips, and redness on the palms of their hands and soles of their feet. These are all signs of inflamed blood vessels.

Early treatment is important. It helps reduce the risk that Kawasaki disease will affect the coronary arteries and cause more serious problems.

Outlook

Kawasaki disease can't be prevented. However, most children who develop Kawasaki disease fully recover—usually within weeks of getting symptoms. Further problems are rare.

In some children, the disease affects the coronary arteries. This can cause serious problems. These children need long-term care and treatment.

How Is Kawasaki Disease Treated?

Kawasaki disease is mainly treated with medicines. Rarely, medical procedures and surgery may be used for children whose coronary arteries are affected.

The goals of treatment are to:

  • Lower fever and reduce inflammation to improve symptoms
  • Prevent the disease from affecting the coronary arteries

Initial Treatment

Kawasaki disease can cause serious complications. Therefore, your child will likely be treated in a hospital, at least for the early part of the treatment.

The standard treatment during the disease's acute phase is high-dose aspirin and immune globulin. Immune globulin is a medicine given intravenously (injected into a vein).

Most children who receive these treatments improve greatly within 24 hours. For a small number of children, fever remains. In these cases, a second round of immune globulin may be given.

At the start of treatment, high doses of aspirin are given. As soon as your child's fever goes away, a low dose of aspirin is given. The low dose helps prevent blood clots, which can form in the inflamed small arteries.

Most children treated for Kawasaki disease fully recover from the acute phase and don't need any further treatment. They should, however, follow a healthy diet and adopt healthy lifestyle habits to lower their risk for future heart disease (this is advised for all children, not just those who have the disease).

Children who have had immune globulin should wait 11 months before having the measles and chicken pox vaccines. Immune globulin can prevent those vaccines from working properly.

Long-Term Care and Treatment

If Kawasaki disease has affected your child's coronary arteries, he or she will need ongoing care and treatment. It's best if a pediatric cardiologist provides this care to reduce the risk of severe heart problems. A pediatric cardiologist is a doctor who treats children who have heart problems.

When Kawasaki disease affects the coronary arteries, they may expand and twist. If this happens, your child's doctor may prescribe anticoagulants (like warfarin) to prevent blood clots from forming in the affected coronary arteries.

Anticoagulants usually are stopped after the coronary arteries heal. Healing may occur about 18 months after the acute phase of the disease.

For the small number of children whose coronary arteries don't heal, routine tests are needed. These include:

  • Echocardiography . This test uses sound waves to create images of the heart.
  • EKG (electrocardiogram). This test detects and records the heart's electrical activity.
  • Stress test . This test gives the doctor information about how the heart works during physical activity or stress.

Rarely, a child may need cardiac catheterization (KATH-e-ter-i-ZA-shun). This procedure is used to diagnose and treat some heart conditions. A long, thin, flexible tube called a catheter is put into a blood vessel in the arm, groin (upper thigh), or neck and threaded to the heart. Through the catheter, doctors can perform diagnostic tests and treatments on the heart.

Very rarely, a child may need to have other procedures, or even surgery, if inflammation narrows his or her coronary arteries and blocks blood flow to the heart. Coronary angioplasty (AN-jee-oh-plas-tee), stent placement , or coronary artery bypass grafting (CABG) may be used.

Coronary angioplasty restores blood flow through a narrowed or blocked coronary artery. A thin tube with a balloon on the end is threaded through a blood vessel in the arm or groin (upper thigh) to the narrowed or blocked coronary artery. The balloon is then inflated to widen the artery and restore blood flow.

A stent may be placed in the coronary artery during angioplasty. Stents are small mesh tubes. They're used to keep narrowed or weakened arteries open. Stents can help improve blood flow and prevent the artery from bursting.

Rarely, a child may need to have CABG. This surgery is used when a coronary artery is severely blocked. During CABG, a healthy artery or vein from another part of the body is connected, or grafted, to the blocked coronary artery. The grafted artery or vein bypasses (that is, it goes around) the blocked part of the coronary artery. This improves blood flow to the heart.

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