What is Kawasaki disease?

Kawasaki disease is an uncommon but important cause of fever in children. It most often occurs in children 6 months to 5 years of age. Kawasaki disease belongs to a family of illnesses where vasculitis or inflammation of the blood vessels can occur. The most important blood vessels that can be involved in Kawasaki disease are those of the heart, specifically the coronary arteries that provide the heart with blood.

In Kawasaki disease, inflamed blood vessels may become weakened and stretch in size, which can lead to aneurysms of the coronary arteries. These can be seen by a test called an echocardiogram, which uses an ultrasound technique usually from the surface of the chest to view the heart and arteries.

What causes Kawasaki disease?

The cause of Kawasaki disease is unknown, although more cases occur in late winter and early spring. Kawasaki disease is not spread by person-to-person contact.

What are the symptoms of Kawasaki disease?

Features of Kawasaki disease can include:

• Fever lasting for at least 5 days
• Irritability
• Red eyes without discharge
• Redness or cracking of the lips, tongue, or throat
• Swelling and/or redness of the hands or feet. Peeling of the skin, usually beginning around the nails, is often a later finding
• Rash mostly on the trunk, but sometimes elsewhere
• Swollen lymph node(s) in the neck

What diagnostic tests will my child undergo?

The diagnosis is mostly based on compatible symptoms and signs. Other tests -- such as blood tests and an echocardiogram -- are also usually obtained. It is most important that other causes of fever be ruled out before the diagnosis of Kawasaki disease is made.

What treatments/management approaches will be considered to care for my child with Kawasaki disease?

• Immunoglobulins which are human blood proteins are given by vein (this is also called IVIG). About 10 percent of children may not respond to the first dose of IVIG and will require a second dose or other adjunctive medications.
• Aspirin is commonly given
• Fluids are given by vein for hydration
• Medications for pain and swelling
• Cold compresses

When should a child be hospitalized for Kawasaki disease?

The reasons for admission vary, but often a child who is being evaluated for Kawasaki disease is often evaluated in the hospital.

When will my child be ready for discharge if he or she has been hospitalized for Kawasaki disease?

Your child will be ready for discharge when he/she:

• Is drinking fluids
• Is tolerating medicines by mouth
• Is no longer having a fever or signs of inflammation

What will be the follow-up for my child after discharge from the hospital for Kawasaki disease?

• You will need to follow up with your primary healthcare provider, an infectious disease doctor and a cardiologist after discharge. Your child will be followed closely, including repeating lab work, until there is no longer any need for further therapy.
• Follow-up echocardiograms will be ordered by cardiology after discharge.

What is the outlook for someone with Kawasaki disease?

Kawasaki disease is the most common cause of acquired heart disease in children in the United States and Japan. Fortunately, treatment is usually effective in preventing heart disease from occurring.

What other important considerations need to be made for children with Kawasaki Disease?

• Ensuring vaccinations are up to date, including influenza and varicella, is especially important while a child is on aspirin therapy. There is a small risk of Reye's Syndrome in children who take aspirin with a viral illness.
• Children should not receive live virus vaccines (i.e., the measles-mumps-rubella, varicella, and the nasal live virus influenza vaccines) for 11 months following IVIG. This is because the IVIG may interfere with the body’s ability to develop antibodies to these viruses.
• Restriction of certain activities may be recommended by cardiology in children with evidence of abnormalities on their echocardiogram.

When should you call your healthcare provider if your child has had Kawasaki disease?

Call your healthcare provider if your child has:

• A recurrence of fever
• Any return in previous symptoms that are worsening