FAQ

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.

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.

No, as yet there is no specific test to help doctors diagnose Kawasaki Disease. A number of tests may be performed by doctors as they work to establish a diagnosis for your child and these could include:

• blood tests – looking for signs of inflammation
• temperature checks – as Kawasaki Disease has a distinctively high and persistent fever
• an electrocardiogram (ECG) which allows doctors to check the rhythm of the heart
• a heart scan using ultrasound (echocardiogram or cardiac echo) from which doctors can check if the heart is affected by KD

Doctors also look for the specific symptoms of Kawasaki Disease – and they may ask you if you have noticed symptoms over the last few days – as Kawasaki Disease symptoms can appear one after the other over a number of days sometimes and not always all at once.

Kawasaki Disease primarily affects children between the ages of 6 months to 7 years, and is more common in male children and those of Asian descent. However, KD has been known to affect individuals of any age, gender, ethnicity, race, or country of residence.

On average, there is one child being treated for Kawasaki Disease every day in Australia.

Kawasaki Disease is quite commonly misdiagnosed by Doctors as a common virus or other childhood illness. Other childhood illnesses that may be considered is:

• Scarlet Fever
• Staphylococcal infection
• Measles
• Meningitis
• Slapped Cheek
• General viral infection
• Hand, Foot & Mouth Disease

A high dose of intravenous gamma globulin (lVlG, a protein fraction of human blood) is the 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 dose aspirin may be used at different stages of Kawasaki Disease.

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.

IVIG (intravenous immunoglobulin) is one of the treatments given in Kawasaki Disease to “dampen down” the inflammatory processes which occur in the first few days of the illness (the acute phase). One key aim in treating with IVIG as early as possible is to reduce the risk of coronary artery aneurysms developing, and to help prevent damage to the heart. Without treatment, inflammation from Kawasaki Disease might otherwise continue, possibly doing further damage to the heart in some children, until the illness eventually resolves. When a child is diagnosed late, doctors should still consider treatment with IVIG if there are signs of ongoing acute inflammation – as suggested by a persistent fever and a continued significantly raised CRP.  CRP is C-reactive protein, measured by a blood test, and this will stay raised if the acute disease action is still ongoing.

In practice, IVIG is rarely administered beyond 15 days after a child’s fever first began, because the fever and other inflammatory processes will have usually resolved – though, too often, this may have caused significant heart damage in the process. That’s why early treatment with IVIG, is critical for Kawasaki Disease within the first few days of the illness, to reduce the risk of heart damage.

Rarely, Kawasaki Disease may reoccur months to years after the initial illness, but this is rarely reported outside of Japan. Should the signs and symptoms reoccur, your child should be seen promptly by a doctor familiar with Kawasaki Disease.

There is so much that you can do to help raise awareness.

• • Join the Foundation
  • Join our social media groups
  • Share your story
  • Share KD news and information
  • Fundraise through your daycare, school, sporting events, BBQs
  • Grill’d burgers fundraiser
  • Birthday or special event fundraising
  • Morning tea
  • Hand out posters and leaflets to your local community
  • Display posters and leaflets in your local health and medical centres, daycares, school etc

Please refer to our resources to download fact sheets, posters, flyers etc. Or you can get in touch with the Kawasaki Disease Foundation to find out more.

You can donate via our Go Fundraise Page by clicking on the link.

There are also a number of events during the year that we get involved with. If you’d like to know more on how you can help get involved please contact the Kawasaki Disease Foundation.

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:

• Signs of aspirin toxicity (while on high dose aspirin).This is characterised by the following symptoms:
  • shallow rapid breathing
  • complaints of stomach pain (with or without vomiting blood, which looks like coffee grounds)
• Return of fever and other signs of Kawasaki Disease (e.g. rash, red eyes – see Signs and Symptoms section).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.

The following information on immunisation sourced is 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.

EXPOSURE TO MEASLES:

The protection offered by intravenous immunoglobulin (IVIG) given for KD against measles varies with both the batch of IVIG (as the amount of anti-measles antibody is likely to vary between batches) and also protection decreases over time as the IVIG is eliminated from the circulation. Protection may be good for a few weeks initially, then wanes.

If a non-vaccinated child aged over 12 months is exposed to measles, even if they’d had IVIG for KD, your doctor may decide that the measles vaccine is needed within 72 hours of exposure, however the vaccine is less likely to be fully effective.

If an individual has not yet received MMR vaccine and is less than 12 months of age, or is at risk of severe complications of measles (e.g. compromised immune system, a pregnant woman) then immunoglobulin (usually given intramuscular (IM), at a lower dose than for KD) may be offered for additional protection. The management decisions of a child or adult exposed to measles are made by the treating doctor.

INFECTION WITH MEASLES:

Once a child has measles there is little specific treatment, apart from vitamin A for more severe cases. The care is supportive and reducing further spread of infection.

More information about measles is available here:

https://www2.health.vic.gov.au/public-health/infectious-diseases/disease-information-advice/measles

http://www.health.gov.au/internet/main/publishing.nsf/Content/cdna-song-measles.htm#toc12