Kawasaki Disease Can Have Serious Complications

Question: Our three-year-old daughter was very sick recently. She developed a high fever, an enlarged lymph gland in her neck, and a rash on the body. She was admitted to the hospital and was given IV antibiotics. Instead of improving, she gradually got worse and continued to spike high fever. After several days in the hospital, her hands and feet became swollen and she refused to walk. She was seen by several doctors. At last one doctor suspected she has Kawasaki Disease and treated her with IV immunoglobulin and aspirin by mouth. She started to improve within a few hours after these were started. She is now back to her normal self, playing and eating just like before she got sick. She is still taking one baby aspirin a day, and we are waiting for a special ultrasound of her heart. We have a younger son who is six months old. We want to know whether he can catch this mysterious illness, and whether there is anything that we can do to prevent it.

Answer:

From your description, your doctor is correct, your daughter most likely is suffering from Kawasaki Disease (KD). Before I answer your question, let me explain a little more about this condition so that other readers can understand it better.

KD was first reported by Dr. Tomisaku Kawasaki in 1967, after he observed a number of children in Japan who developed an illness that involved many organ-systems that I will describe later. He initially called this mucocutaneous lymph node syndrome because most children had involvement of mucous membranes, skin, and lymph nodes. Since then, this condition has been diagnosed in almost all the countries around the world. Dr. Kawasaki was recognized as the first physician to describe it, and the official name was changed to Kawasaki Disease to honour his observation.

KD affects children mostly between one to 8 years of age. It can occur in outbreaks, mostly in winter and early spring, although KD can be seen any time of the year. Orientals, especially Japanese, seem to be most susceptible to this illness.

We still don’t know what is the cause of KD. However, some kind of infection seems to trigger this illness, although researchers have not been able to identify any germ thus far.

All the children with KD have high grade fever for at least five days. Most of them are extremely irritable and refuse to eat because they are not feeling well. In addition, most of these children develop redness in the white part of their eyes, on the lips, the tongue, and the throat, which is a sign of inflammation. There is usually enlargement of at least one lymph gland which can be very tender. A skin rash can appear on the body. The hands and feet can become red, swollen, and tender; some children may refuse to walk. Over time, there is peeling of the skin, especially in the groin and around the fingernails and toenails.

Although we don’t know what causes KD, the pattern of these children’s illness suggests that an infection triggered an overreaction of the immune system. Some of the chemicals that are produced as a result of the inflammation weakens the wall of the coronary arteries that supply blood and oxygen to the heart. As a result, these arteries can become dilated.

Another important feature of KD is a dramatic increase in the number of platelets. Platelets are tiny blood cells that form blood clots to stop bleeding in the presence of an injury. However, if there are too many platelets, they can clump together and form blood clots much more readily, especially in the dilated coronary arteries which have been damaged by inflammation. These blood clots can prevent blood from flowing through the heart, essentially causing a heart attack. This unfortunate complication has actually happened to a small number of children who contracted KD. That is the reason why your daughter needs a special ultrasound of the heart, which is called echocardiogram, to look for any abnormal dilation of coronary arteries and blood clots.

The most effective treatment for KD is the use of aspirin and IV immunoglobulin. Although we don’t completely understand how they work, this combination of medicine seems to halt the inflammatory process in the body that is spiraling out of control. Aspirin also has the additional benefit of preventing platelets from clumping together to form blood clots, essentially preventing heart attack like in adults.

Although siblings have a slightly higher chance of contracting KD than the general public, there is really nothing that one can do to prevent it from happening at the present time, since we don’t know what is the cause of this illness, and how it is being spread. What you should know is that children under one year of age can present with fewer symptoms than your daughter, therefore, making it even harder for doctors to recognize this condition. Furthermore, they have a higher chance of developing coronary artery disease than older children.

If your younger son develops fever, you should have him examined by your doctor earlier, so that he can be observed more carefully for symptoms suggestive of KD.