Pediatric Kawasaki Disease

Kawasaki disease is a rare illness that most commonly affects children ages 0 to 5, but can sometimes affect children up to the age of 13. It is a type of vasculitis. Vasculitis means inflammation of the blood vessels. It can affect the whole body, including the blood vessels of the heart (coronary arteries). The cause of Kawasaki disease is unknown. Without treatment, affected children are at higher risk of developing problems with the coronary arteries. Other areas of the heart may also be affected. With timely treatment, most children recover with no lasting problems.

Children of any race or ethnic group can get Kawasaki disease. It's more common in children whose families are from East Asia or Asian ancestry. Most children who get Kawasaki disease are younger than 5 years old. It occurs in boys more often than in girls.

The cause of Kawasaki disease is not known. Researchers think it may be the result of an infection.

Most children with Kawasaki disease get better within a few weeks. But serious complications may occur. Those involving the heart include:

• Weakening of one of the heart's arteries (coronary artery aneurysm)
• Heart muscle that doesn't work well or heart attack
• Inflammation of the heart muscle (myocarditis), lining of the heart (endocarditis) or covering of the heart (pericarditis)
• Heart valves that don't work well
• Heart failure

 Kawasaki disease may also affect other body systems. This includes the nervous, immune, digestive and urinary systems.

Call your child's healthcare provider if your child has the symptoms of Kawasaki disease. If your child is diagnosed with Kawasaki disease, keep all follow-up appointments. Also watch for signs or symptoms of complications, including:

• Chest pain
• Fever
• Poor feeding or eating
• Swelling
• Tiredness
• Trouble breathing

Your child's healthcare provider can usually diagnose Kawasaki disease by his or her symptoms, physical exam and basic laboratory evaluation.

To diagnose Kawasaki, other causes for the symptoms must be ruled out. A fever for five days must be present in addition to having four out of five of the following:

• Changes in the lining of the mouth
• Rash
• Red eyes
• Skin changes in the hands and feet
• Swollen lymph nodes

Other recommended tests include:

• Lab tests. Blood and urine samples are taken to check for signs of inflammation. These are also used to help rule out other health problems.
• Electrocardiography (ECG). This test records the electrical activity of the heart through small, sticky patches on the child's chest. The patches are connected to a machine with wires. The machine records the electrical activity. This helps check for problems with heart rhythm and heart structure.
  
• Echocardiography (echo). This test uses sound waves to create a picture of the heart. This can show problems with heart structure, valves and heart function.
• Cardiac catheterization. This test uses a small tube that goes into the blood vessels and takes pictures of the coronary arteries using contrast and X-ray.

These are common symptoms of Kawasaki disease:

• Fever of 102.0°F to 104.0°F (38.8°C to 40.0°C) that lasts for at least five days
• Red rash
• A swollen lymph node, usually in the neck
• Swollen hands and feet
• Red eyes
• Red and dry cracked lips
• Red tongue with white spots (called “strawberry tongue”)
• Irritability
• Fast heart rate
• Diarrhea or vomiting
• Skin peeling

The symptoms of Kawasaki disease can look like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

Treatment will depend on your child’s symptoms, age and general health. It will also depend on how severe the condition is. Treatment typically starts as soon as the problem is suspected. Your child may need to stay in the hospital for a few days or longer.

Your child's healthcare provider may prescribe aspirin or IV (intravenous) gamma globulin (IVIG). Infliximab, corticosteroids and other medicines may also be prescribed if aspirin and IVIG don't work well. Once your child is home, he or she may need to take low-dose aspirin for six to eight weeks. Don't stop your child aspirin without first talking with the child's healthcare provider pediatric cardiologist. This is a doctor with special training to treat children’s heart problems. Your child may need medicine, procedures or surgery.

If your child has a coronary artery aneurysm, he or she will need echocardiograms, sometimes for several years after the illness. Your child may need more treatment, including blood thinners to prevent clots. It's important to keep follow-up visits with your child's healthcare provider, even if your child is feeling well.

There is a risk for early coronary artery disease after having Kawasaki disease, including early heart attacks. Your child will need to follow a heart-healthy lifestyle for life. This includes eating healthy foods, getting regular exercise, and not smoking. Your child should have regular follow-up with a cardiologist throughout his or her life.

Talk with your child's healthcare provider about what to expect for your child.