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The following are some of the questions most frequently asked by parents of children who suffer
from Kawasaki disease. This information has been adapted from the U.S. Foundation’s KD Awareness Day package for Australian families.
WHAT IS KAWASAKI DISEASE?
Kawasaki Disease is an unusual illness characterised by inflammation of arteries throughout the body. lt is characterised by fever for at least 4-5 days and some or all the following features: rash, swelling of the hands and feet, bloodshot eyes, irritation and redness of the mucous membranes of the mouth, lips, and throat, and swollen lymph nodes in the neck.
Kawasaki disease may damage the coronary arteries that supply blood to the heart, which may result in long term complications. The risk of coronary artery damage is reduced by current treatments. Kawasaki Disease almost exclusively affects children; most patients are under 5 years of age. It is about 1.5-2 times more common in boys.
The disease is named after the Japanese paediatrician, Tomisaku Kawasaki, who described this particular pattern of signs and symptoms in 1967. Since then, Kawasaki disease has been reported in almost every country and racial group but it remains most frequent in Japanese children. About 12,000 Japanese children have Kawasaki disease each year and it is thought that about 1 in 150 Japanese children have had Kawasaki disease by the age of 10 years. ln other countries Kawasaki disease occurs most commonly in children of Asian descent. In Australia it is estimated that the disease occurs in about 10 out of every 100,000 children less than 5 years of age, giving an estimate of between 150-200 cases per year in Australia. Kawasaki Disease is not a rare illness and the exact number of cases that occur remains unclear. The disease can occur in clusters or localised outbreaks, usually in the winter and spring.
WHAT CAUSES KAWASAKI DISEASE?
To date, no cause of Kawasaki Disease has been identified. Most experts agree that an infectious cause or causes (such as a virus or bacteria) is likely, although a hereditary tendency also exists and explains why the disease occurs more frequently among children of Asian ancestry. Younger brothers and sisters of a KD patient have a 5 to 10-fold increased risk of KD because of a shared genetic predisposition. When children with Kawasaki Disease grow up, their children are at higher risk of developing KD. There is no evidence that Kawasaki disease is contagious.
WHAT ARE THE SIGNS AND SYMPTOMS?
Fever and irritability often occur first. The fever has a rapid onset and fluctuates from moderate (38 degrees) to high (above 39 degrees). A rash usually appears early in the illness; some patients may develop an accentuated rash in the groin. Often vivid red in appearance, the rash is composed of either poorly defined spots of various sizes or larger masses of merging spots. Fever continues to rise and fall, possibly for up to three weeks. Bloodshot eyes (conjunctival injection), usually without discharge, develop during the first week of illness.
A child's tongue may be red and show small, raised bumps, called a "strawberry tongue" because it resembles the seeds on a strawberry's surface. The lips become dry and cracked and often take on a bright red colour, as though the child is wearing lipstick. Mucous membranes inside the mouth turn a darker red than usual.
The palms of the hands and soles of the feet often turn bright red. Hands and feet can swell. In older children one or more lymph nodes in the neck may become swollen. Occasionally, a child may develop a stiff neck. The child usually has great difficulty getting comfortable and may be very irritable. Joint symptoms include stiffness and pain in the hips and knees and sometimes the small joints in the hands. The child may refuse to walk. Many parents also notice hoarseness during the acute illness. The features of Kawasaki disease may not all be present at the same time, but come and go over the course of up to a week or more. Some of them may have disappeared by the time the child is seen by a doctor.
When the fever subsides, the rash, red eyes and the swollen lymph nodes usually disappear. Skin often peels around the toenails and fingernails, often beginning during the third week of illness. The skin on a hand or foot may peel off in large pieces or even a single piece (much as a snake sheds its skin). Knees, hips, and ankles can become more inflamed and painful.
Occasionally, joint pain and inflammation persist after other symptoms have disappeared. Transverse (horizontal) depressed lines on fingernails and toenails, which occur during the illness, may be visible for months afterward until the nails grow out. Hair loss may occur. Eczema may flare and require treatment. Psoriasis may appear for the first time.
HOW DOES A DOCTOR DETERMINE IF A CHILD HAS KAWASAKI DISEASE?
A doctor makes a diagnosis of Kawasaki Disease after carefully examining a child, observing
signs and symptoms (and asking about those that may have been present earlier in the illness and now disappeared), and after ruling out the possibility of other diseases that can cause similar signs. There is no diagnostic test for Kawasaki disease. Blood tests are used to detect mild anaemia, an increased white blood cell count and other signs of inflammation. Blood tests may also be helpful in excluding other possible causes for the illness. A sharp rise in the number of platelets, the major clotting element in the blood, may also be detected, but this is usually in the second or third week. Urine tests may reveal unusual white blood cells in the urine. Echocardiography (an ultrasound test of heart and blood vessel structure and function) is necessary to evaluate possible damage to the coronary arteries, heart or large blood vessels.
HOW IS KAWASAKI DISEASE TREATED?
A high dose of intravenous gamma globulin (lVlG, a protein fraction of human blood) is the treatment of choice for patients with Kawasaki disease. This treatment is most effective in reducing inflammation and preventing coronary artery damage if it is started within the first 10 days of illness, but it may still be helpful if given later. Aspirin is also given with gamma globulin and continued for about 6 weeks, if the echocardiograms are normal. Either high dose or low aspirin doses may be used at different stages of the Kawasaki disease. The aspirin is switched to low dose (from high dose, if this was used initially) once the fever has subsided. Complications from treatment with either IVIG or aspirin are rare. Viruses such as HIV (the AIDS virus) and Hepatitis C virus cannot be transmitted by currently available lVlG products. Occasionally, chills, fever, and a drop in blood pressure may occur during the infusion. This is treated by interrupting the infusion and giving an antihistamine before restarting. High doses of aspirin may sometimes cause abdominal pain, gastrointestinal bleeding, and ringing in the ears. Aspirin should be discontinued if any of these signs or symptoms appear. Reye syndrome is a very rare complication of aspirin therapy that can occur in children exposed to chicken pox or influenza virus while they are taking high doses of aspirin. Low dose aspirin carries no risk of Reye syndrome.
In about 10-15% of children, the fever and inflammation either does not settle with one dose of IVIG, or fever reoccurs. Usually a second dose of IVIG is given and other treatments may be considered.
lf diagnostic tests reveal the presence of an aneurysm (dilated segment of the coronary artery) or any other heart or blood vessel abnormality, medical or surgical treatment may be needed. Your doctor may recommend that a cardiologist (a doctor who specialises in heart problems) monitor a heart or blood vessel problem following recovery from Kawasaki disease.
WHAT ARE THE CONSEQUENCES IF THE DISEASE IS NOT PROPERLY TREATED?
Fever, swollen lymph nodes (also called "swollen glands") in the neck, rash, and mucous membrane inflammation can be extremely uncomfortable and last for 1 to 3 weeks without treatment. With treatment, the fever and other symptoms usually subside within 24 hours.
About 25 percent of untreated children who are affected by Kawasaki disease develop heart problems in the latter stages of the illness. In children who receive IVIG within 10 days of the onset of fever, coronary artery damage occurs in about 5% (1 in 20 children). A weakening of the large vessels in the heart (coronary arteries) can result in an enlargement or ballooning (aneurysm) of the blood vessel wall.
Full recovery can be expected in most cases, but the possibilities of long term blood vessel and heart
disease in later life is the subject of ongoing research. lnfants less than 1 year old usually become most seriously ill and are at greatest risk for coronary artery damage. The risk of death from Kawasaki disease is low; in American studies it is about 0.15-0.2%, or less than 1 in 500 cases.
ARE THERE ANY COMPLICATIONS ASSOCIATED WITH KAWASAKI DISEASE?
Heart and blood vessel problems can make Kawasaki Disease unpredictable. Most often these problems are not serious and disappear in time. However, aneurysms of coronary or other large arteries can be serious and may require medical or surgical treatment. Severe blood vessel and heart complications can rarely prove fatal. If there are aneurysms then later after Kawasaki disease there may be scarring of the blood vessels and heart muscle that may cause problems in some older children and young adults.
Heart muscle inflammation (cardiomyopathy) and congestive heart failure may rarely accompany the fever. An abnormal and painful accumulation of fluid in the gall bladder (hydrops of the gall bladder) resulting in severe abdominal pain, sometimes occurs during the period of fever. lnflammation of the membranes around the brain may cause a sterile meningitis, usually with full recovery. On rare occasions, damage to the nerve involved in hearing can rarely occur and result in deafness. Therefore, children with Kawasaki disease may require a hearing test performed after recovery if there is any question about their hearing.
WHAT CAN I EXPECT ONCE MY CHILD COMES HOME FROM THE HOSPITAL?
After coming home from the hospital, you may notice that your child continues to be tired and has a poor appetite for about 1 to 2 months. However, unless you have been specifically told otherwise by your child’s doctor, you should not limit your child's activity or diet.
Children will usually be prescribed a low dose of aspirin to be taken for six to eight weeks. Aspirin can cause children to bruise easily, so certain activities may need to be avoided.
You should call your doctor immediately, however, if any of the following symptoms occur:
1. Signs of aspirin toxicity (while on high dose aspirin).
This is characterised by the following symptoms:
a. shallow rapid breathing
b. complaints of stomach pain
(with or without vomiting blood, which looks like coffee grounds)
2. Return of fever and other signs of Kawasaki disease (e.g. rash, red eyes - see Signs
and Symptoms section above).
Note: Tenderness or swelling of the large joints (elbows, knees) and peeling of the fingertips
and toes are a normal part of the recovery, but should resolve after approximately 3 weeks.
CAN MY CHILD CONTRACT THIS DISEASE AGAIN IN THE FUTURE?
Rarely, Kawasaki disease may reoccur months to years after the initial disease, but this is rarely reported outside of Japan. Should the signs and symptoms described earlier in this pamphlet reoccur, your child should be seen promptly by a doctor familiar with Kawasaki disease.
CAN KAWASAKI DISEASE BE PREVENTED?
Unfortunately, at this time, Kawasaki Disease cannot be prevented. However researchers in many countries, including Australia, are working to understand this mysterious disease and improve diagnosis and treatment.
Immunisation Guidelines following Kawasaki Disease
The following information on immunisation sourced from The Australian Immunisation Handbook 10th Edition 2013 (updated January 2014)
Patients who have been diagnosed with Kawasaki Disease fall into the groups with special vaccination requirements.
Recommended intervals between IVIG and measles-mumps-rebella (MMR) and measles-mumps-rebella-varicella (MMRV) or varicella vaccination (chicken pox) is 11 months,
Activity restrictions if your child has aneurysms
Children may have some activity restrictions depending on the size of their aneurysm and whether or not they are on medicines that slow blood clotting. Most children with coronary aneurysms take part in normal activities.
TO LEARN HOW YOU CAN PARTICIPATE IN KAWASAKI DISEASE RESEARCH, PLEASE CONTACT
THE KAWASAKI DISEASE FOUNDATION