FAQ on Kawasaki's Disease

by Dr. Y.L.M

I read with interest and sadness the case of John Travolta's son who died from seizures. It was mentioned that he also had Kawasaki's disease. I have never heard of this disease before. What is it?

Kawasaki's disease is also called Kawasaki's syndrome. It is a disease of childhood. It was first described by a Japanese doctor named Tomisaku Kawasaki in 1967.

He noted a number of Japanese children coming in with fever and rash. They initially thought it was benign, but then some of the children who were younger than two years old who had been admitted for that condition died a few years later. And when they were initially admitted, they recovered completely or had improved tremendously.

When post-mortems were done on these children, it was noted that in a lot of their heart vessels (coronary arteries), there were complete blood clots which had prevented blood from flowing to the heart. So basically, these very young children died of heart attacks!

No one to this day quite knows what causes Kawasaki's disease. This disease is most common among people of Japanese descent and in boys rather than girls. It is also most common in infants aged from 6 to 12 months. However, it can also occur in children older than 5 years.

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But how did these children's coronary arteries get blocked? What happened to them?

Kawasaki's disease is described as a 'vasculitic' syndrome, which means a disease of the blood vessels. Although no one quite knows the cause of it, plenty of researchers suspect something infectious such as viruses or bacteria. There apparently have been epidemics.

These suspected infections may trigger off immune reactivity in these children, and the child's own 'altered' immune system then turns against his own body to attack his blood vessels.

There is even one researcher who suggested a link between Kawasaki's disease and the use of a humidifier in the child's bedroom (usually an asthmatic child). Another researcher suggested a link with the use of carpet shampoo.

How would we know if our child is having this disease?

Sometimes it can be very difficult. You might observe your child having a prolonged fever of at least 5 days, which began suddenly or abruptly.

As a parent, you might attribute it to a normal childhood viral illness, but you become concerned that despite antibiotics from the doctor, your child's fever has not subsided.

Your child is also extremely irritable and cries fitfully. This irritability is out of proportion from the degree of the fever.

You would have been worried enough to bring your child to a paediatrician by then. The paediatrician requires to diagnose at least :

i) A fever of more than 39 degrees Celsius for at least 5 days which had been unresponsive to antibiotics, and

ii) 4 of the following :

* Changes in your child's hands and feet such as swelling, redness or rash and peeling of skin (desquamation). Your child may not wish to move his hands and feet much because of this and may find it difficult to walk. This usually happens in 75% of all cases 1 or 2 weeks after the fever's onset.

* Conjunctivitis of both eyes (85% of cases).

* A rash which usually occurs throughout your child's body (80% of cases). Sometimes this rash only occurs in your child's groin or legs.

* Enlarged neck lymph nodes.

* Changes in your child's lips like swelling, redness or dryness and fissuring. Your child may also have a strawberry tongue – meaning the tongue is very red and the papillae stands clearly out as in the case of a strawberry. (These collective changes occur in 90% of all cases).

This fever and rash phase will last for approximately 1 to 2 weeks. During this time, your child's heart muscle and the layer surrounding it may also be inflamed.

After the fever comes another phase until week 4. Your child remains irritable. He has no appetite. He continues to have conjunctivitis. It is during this phase that the skin changes in your child's hands and feet are noted. Some of your child's internal blood vessels (especially the coronary arteries) develop aneurysms – weakening of the vessel walls leading to a bulge. This phase is at the highest risk of death.

Then after the second phase comes the recovery phase. Unfortunately, the aneurysms are still present. Your child may have to live with these aneurysms for the rest of his life, and they may come back to haunt him when they rupture during adulthood.

Can Kawasaki's disease be treated?

The treatment is actually to decrease the severity of the immune reaction. Intravenous gamma globulin in high doses is the standard treatment. Your child usually improves within 24 hours. If treated, only 10 – 15% of the children will develop coronary artery problems as opposed to 25%.

Most children recover fully with early treatment. There are 2% of children who die however of heart attacks or heart complications.

More info on KAWASAKI DISEASE here.

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