Dr Naqvi has 20 years experience of looking after children with Kawasaki Disease. She is a member of the Pan London Kawasaki Expert Group.
What is a Kawasaki Disease?
This is a rare disease of unknown cause. It is the commonest cause of acquired heart disease in children in developed countries. It is named after a Japanese doctor (Dr Tomisaku Kawasaki). There is widespread inflammation in the body, particularly affecting blood vessels (vasculitis). The most important blood vessels to be affected are the coronary arteries. These are the small tubes which supply oxygenated red blood to heart muscle. In Kawasaki Disease, the coronary arteries can enlarge, developing aneurysms. Blood flows slowly in these aneurysms and there is a risk of dangerous clot formation without treatment. It is not contagious.
Coronary Arteries
Who gets Kawasaki Disease?
Children and babies particularly those below 5 years of age. It is more common in Japan and East Asia and in children whose ancestry is from this region but any child can get it. Boys are more frequently affected than girls, but both sexes can be affected. The illness tends to be more severe in babies under 1 year of age.
Symptoms
These include FEVER and:
- Red eyes
- Swollen glands (lymph nodes) particularly in the neck
- Rash
- Red cracked lips and a swollen coated tongue (strawberry tongue)
- Swollen red hands, fingers, toes and feet with later pealing of the skin especially from the fingertips.
Diagnosis
There is no single diagnostic test for Kawasaki disease. The assessing doctor will take a history and perform a clinical examination. If the fever has persisted for 4-5 or more days with 4 of the above features Kawasaki Disease will be a possible important diagnosis. Children will be admitted to hospital. Blood tests will also be performed. An ECG and echocardiogram(heart ultrasound) will be required at some time during the initial assessment. However, treatment must not be delayed whilst awaiting an echocardiogram.
Treatment
Standard treatment is with high dose aspirin and intravenous immunoglobulin. Other medicines may also be given if the fever does not resolve. Patients will be discharged home on aspirin which will be continued (usually at a lower dose) for 6weeks or more. Another echocardiogram will be performed after discharge in the outpatient clinic.
Aspirin
Bed Rest
Immunoglobulin
Prognosis
Atypical cases and complications affecting other organs can occur. Complex cases may also be discussed with a paediatric infectious diseases expert such as those at St Mary’s Hospital in London.
The outlook for children with Kawasaki Disease is very good in the majority of cases. Dr Naqvi has cared for many babies and children with Kawasaki Disease.
She says “Having a child with Kawasaki Disease can be a frightening experience for parents and traumatic for children, particularly because repeated blood tests and intravenous cannulation are necessary. Initially children can be very sick and are often unusually miserable. This is a feature of the illness and it can take some time for them to return to their usual happy state. In the longer term, however, as long as the coronary arteries are not significantly affected including at follow up appointments, the prognosis for complete recovery is good. Babies and children who have recovered can go on to do well in sports and have no limitations on their activities. “
Even children whose coronaries are affected can do well in the future but they should have long term expert paediatric cardiology follow up and may need long term medication particularly to thin the blood to prevent clot formation in coronary aneurysms. Relatively frequent medical imaging tests will be performed throughout life. In rare cases intervention on the coronary arteries may be needed either via cardiac catheter (balloon dilatation of narrowed coronaries) or major cardiac surgery.
For more information about Kawasaki disease please see the following links:
