The first phase, which can last for up to 2 weeks, usually involves a persistent fever that is higher than 38°C (100.4°F), and lasts for at least 5 days. The other symptoms that typically develop include:
During the second phase of the illness, which usually begins within two weeks of when the fever first begins, the skin on the child's hands and feet may begin to peel. Not all the features may be present.
Kawasaki disease is probably triggered by a virus infection.
Kawasaki disease was described by Dr Kawasaki in Japan in the 1960s and is an inflammation of the arteries of the body - probably triggered by a virus infection.
The danger in the disease is that the inflammation can cause damage to the coronary arteries and affect the heart muscle.
Kawasaki disease is an illness that involves the skin, mouth, and lymph nodes, and typically affects children who are under the age of 5. The cause is unknown but if the symptoms are recognised early, kids with the disease can fully recover within a few days. If it goes untreated, it can lead to serious complications that can involve the heart.
It is more common among children of Japanese and Korean descent, but the illness can affect all ethnic groups.
There is no one test to detect Kawasaki disease, so a doctor typically diagnoses it by evaluating the child's symptoms and ruling out other conditions.
Typically, a child who is diagnosed with this illness will have a fever lasting 5 or more days and at least 4 of the following symptoms:
If Kawasaki disease is suspected, a doctor may order blood and urine tests. An ultrasound of the heart (echocardiogram) may be ordered to evaluate the coronary arteries of the the heart. Treatment is with an infusion over a few hours of immunoglobulin (special blood product) and oral aspirin - these dampen down the inflammation and reduce the likelihood of long term coronary artery damage.
Doctors can manage the symptoms of Kawasaki disease if they catch it early. The symptoms typically disappear within two days after the start of treatment. Usually, if Kawasaki disease is treated within 10 days of when the first symptoms begin, no heart problems will develop.
However, if the illness goes untreated (time period can vary, but likely for more than 12 to 14 days), it can lead to more serious complications that involve the child's heart. Kawasaki disease can lead to inflammation of the blood vessels. This can be particularly dangerous because it can affect the coronary arteries, which supply blood to the heart
Once the fever has resolved the child can go home. Aspirin is usually continued for a few weeks.
Children and adults with congenital heart disease are at an increased risk of having a heart infection. Whilst this is rare the chances of it occurring can be reduced by taking precautions.
Infections in the heart can occur for no apparent reason but are more common if the teeth are rotten - germs spread into the blood stream and infect the heart. Good dental hygiene is therefore important as are regular visits to the dentist.
If dental treatment is required then some procedures can cause germs to spill into the blood and infect the heart.
It is therefore important that the dentist is informed about the heart condition before treatment. The usual method of avoiding this problem is to give a single dose of antibiotics one hour prior to the treatment to kill any germs beforehand.
Children who have Kawasaki disease with persistent coronary artery problems are restricted to light exercise (like golf, cricket). Exercise is important even in those with heart disease. It improves the heart function and general sense of well being. It is associated with increased life expectancy and a reduced risk of heart disease in later life. In addition physical activity helps with controlling weight and reducing blood pressure.
There are different types of exercise. In static exercise the muscles contract but there is little joint movement eg weight lifting. In dynamic exercise the muscles contract and also move the joints eg running. Each places a different stress on the body and cardiovascular system. In general most types of sports are a mix of the two.
Children usually take part in more rigorous exercise at school as they grow older. In Singapore, physical education (PE) tends to teach games skills rather than competitive sports. However, pupils may take sports for their co-curricular activities (CCA) where training is more intense and competitive. Training for the NAPFA test is also intensive and some pupils with heart problems may have difficulty with the 2.4km run as they often do not perform as well at endurance-type activities.
As always parents or patients must seek the advice of their own doctor when deciding how much exercise and to what level is safe - particularly as there are no published guidelines for activity levels in children.
The Bottom Line
Most children with heart disease can have all the normal vaccinations at the appropriate time.
However some children with an immune deficiency (DiGeorge syndrome or an isomerism) and those who are receiving immunosuppression - for example following transplantation require a different vaccination schedule.
Before travelling anywhere unusual or a long distance make sure that you:
Those with cyanotic heart disease ("blue" due to reduced oxygen in the blood) can still travel but aircraft at altitude have less oxygen in the air than at ground level and so the blueness may be more apparent. This does not usually cause symptoms but if necessary, airlines can arrange for additional oxygen to be available on the aircraft.
For long aircraft flights, it is sensible to use the support stockings and take aspirin or an equivalent unless your doctor advises against it.
Special diets are not normally necessary for those with heart disease. As with everyone, it is important to have a balanced diet and not to eat to excess. It is however important to maintain a normal weight - excess weight means more work for the heart.
Most children with heart disease are no more prone to infections than any other children. Some however are likely to get chest infections - particularly those with holes in the heart (ASD, VSD, PDA).
In addition, some heart diseases are also associated with an immune deficiency and infections and therefore are more common. The majority of childhood infections are viruses and get better without antibiotics. In any case of doubt, professional medical help should be sort and the doctor will decide if antibiotics are necessary.
Most children with heart disease do not require medication. Some however need them to:
The majority of these medicine have been used for many years and are very safe but like all drugs, side effects may occur - especially if there is another illness or a change in other medication. If unusual symptoms or side effects occur whilst on medication, it is important to inform the doctor immediately.
Most women with heart disease are able to undergo a normal pregnancy and delivery.
The exceptions are those with severe cyanosis when there is usually insufficient oxygen in the blood supply to the placenta to sustain the baby. Most of these pregnancies spontaneously abort.
Women with pulmonary hypertension (high lung artery pressure) are not usually advised to get pregnant as there is a serious danger to their life.
As with any medical problem, it is essential that medical advice is taken - preferably before the event so that the pregnancy can be monitored and if treatment is necessary it can be undertaken early.
If a women herself has a heart problem, there is an increased risk that her child will have a heart problem too. The risk is around 6%. If a man has a heart problem then the risk is 2%. If the couple have had a previously affected child then the risk is about 2%. The risks quoted above are averages - the actual risk varies considerably between the various heart conditions. There is also some evidence that taking vitamins for 3 months prior to and the first 3 months of the pregnancy can reduce the chance of heart disease occurring in the baby.