Kawasaki Disease

1 What is Kawasaki Disease?

Kawasaki disease is an autoimmune disorder which affects children, often under 5 years of age. In this condition the walls of the blood vessels including coronary artery, which supplies oxygenated blood to the heart, gets inflammation which can result in severe heart problems.

The inflammation could sometimes be spread to the lymph nodes, skin and mucous membranes inside a mouth, nose and throat thus it is also known as mucocutaneous lymph node syndrome.

The disease is rare but serious and can cause death if left untreated. With early treatment of the disease, the severe effects of the disease can be prevented and the children can usually recover in few weeks.

2 Symptoms

The prognosis of Kawasaki disease involves three phases which are characterized by different signs and symptoms as given below:

Phase-I:

  • Fever, often higher than 102.2 F (39 C) which can last more than five days
  • Extremely red eyes (conjunctivitis) without any thick discharge
  • A rash on the main part of the body (trunk) and in the genital area.
  • Red, dry and cracked lips
  • An extremely red, swollen tongue (strawberry tongue)
  • Swollen, red skin on the palms of the hands and the soles of the feet.
  • Swollen lymph nodes in the neck and perhaps elsewhere and

Phase II:

  • Possibility of peeling of the skin on the hands and feet, especially the tips of the fingers and toes, often in large sheets
  • Joint pain
  • Diarrhea
  • Vomiting
  • Abdominal pain

Phase III:

In the third phase of the disease, the signs and symptoms slowly disappear if there are no serious complications. This phase may be as long as eight weeks before energy levels seem to be normal again.

Kawasaki disease is alarming. So, it is suggested that the doctor should be consulted immediately if any of the above-mentioned symptoms including prolonged fever is seen in the children.

Treating Kawasaki disease within 10 days of its onset greatly reduces the chances of severe damage.

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3 Causes

Kawasaki disease is believed to be non-contagious and has an unknown root cause.

There are many unproved theories for the origin of the disorder like the disease is caused due to bacterial infection, fungal infection or environmental factors. But none of the theories has been validated.

4 Making a Diagnosis

While making a diagnosis of Kawasaki disease, the doctor will ask several questions to know every detail of the signs and symptoms of the disease.

There is no specific test available for the disease, however, the diagnostic process involves a physical examination and ruling out other diseases which have similar signs and symptoms like scarlet fever, juvenile rheumatoid arthritis Stevens - Johnson syndrome, toxic shock syndrome, measles, Rocky Mountain spotted fever, etc.

This may involve:

  • Urine test: To rule out other diseases
  • Blood test: To clearly identify the indications of the disease like elevation in white blood cell count, a presence of anemia and inflammation.
  • Electrocardiogram: to measure patients heart beat
  • Echocardiogram: to know about how the heart and coronary arteries are functioning.

5 Treatment

After diagnosing Kawasaki disease the doctor will begin the treatment as early as possible to reduce the risk of complications. Initial treatment of the disease aims at lowering the fever, reducing inflammation and preventing any damage to the heart.

The doctor may recommend:

  • Gamma globulin - Infusion of an immune protein known as gamma globulin intravenously lowers the risk of coronary artery problems.
  • Aspirin - High doses of aspirin may help to treat inflammation, decreasing pain and reduces the fever.

Due to the high risk of complications involved, the initial treatment of the disease is administered in the hospital. Once the fever goes down, aspirin doses are needed to be reduced but may be continued for about six weeks or longer if coronary artery aneurysm develops. However, if the patient develops flu or chickenpox during the treatment, he or she may need to stop taking aspirin.

Without treatment, Kawasaki disease lasts for an average of 12 days, though heart complications can be evident later and can last longer. With treatment, the patient may start to improve soon after the first gamma globulin treatment.

Kawasaki disease is a leading cause of acquired heart disease in children. But with effective treatment, only a small percentage of children have lasting damage.

Heart complications may include:

  • Vasculitis: Inflammation of blood vessels, usually the coronary arteries
  • Myocarditis : Inflammation of the heart muscle
  • Mitral regurgitation: Heart valve problems
  • Dysrhythmia: Abnormal heart rhythm

Any of these complications can damage patient’s heart. Inflammation of the coronary arteries can lead to weakening and bulging of the artery wall (aneurysm).

Aneurysms increase the risk of blood clots forming and blocking the artery, which could lead to a heart attack or cause life-threatening internal bleeding. For a very small percentage of children who develop coronary artery problems, Kawasaki disease is fatal, even with treatment.

Treatment of complications:

  • 1. If the child has any indication of heart problems, the doctor will recommend follow-up tests to monitor heart health at regular intervals, often at six to eight weeks after the illness began. If the problem continues, the doctor may refer the patient to a pediatric cardiologist.
  • 2. If the patient has developed coronary artery aneurysm he or she may require anticoagulant drugs such as aspirin, clopidogrel (Plavix), warfarin (Coumadin) and heparin. These medications help in preventing clot formation.
  • 3. In some cases of coronary artery aneurysm, the doctor may need to go for coronary artery angioplasty to open the arteries that have narrowed to the point that they impede blood flow to the heart.
  • 4. Stent placement may be opted in which a small metal cube, known as a stent, can be implanted in the coronary artery to prevent re-blockage. This may be accompanied with coronary artery angioplasty.
  • 5. Coronary artery bypass graft can be recommended which involves rerouting the blood around a diseased coronary artery by grafting a section of blood vessel from the leg, chest or arm to be used as the alternate route for blood.

6 Lifestyle and Coping

Knowledge and awareness about Kawasaki disease help in coping with the disease better. Thus, collect all the needed information so that you can make informed choices with your child's health care team about the treatment options.

Early and proper treatment of the disease is very important for complete recovery from the disease so, consult your doctor immediately if you see any symptom of Kawasaki in your child.

Initially, you can give your child acetaminophen (Tylenol, others) or ibuprofen (Advil, Children’s Motrin, others) to reduce the fever to make him or her more comfortable. However, these products are not recommended for low fevers.

Treating the fever will make it difficult to accurately measure the severity and the actual duration of the fever. Aspirin should not be given to a child without consulting the doctor.

In children and teenagers, taking aspirin during certain viral infections, such as chickenpox, has been associated with the development of Reye's syndrome which is a rare but very serious disorder.

7 Risks and Complications

Three things are known to increase your child's risk of developing Kawasaki disease, including:

  • Age: Children under 5 years of age are at greater risk to develop Kawasaki disease. 
  • Sex: Boys are more likely to develop Kawasaki disease than girls.
  • Ethnicity: Children of Asian descent, such as Japanese or Korean, have a higher risk of developing Kawasaki disease.
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