Our hearts and prayers go out to John Travolta, Kelly Preston and their entrie family on the sudden death of their son Jett. And to Brian Littrell ( Back Street Boys ) who's 6 yrear old son was diagnosed with Kawasaki Disease, the disease cause is still unknown. According to the American Heart Association, more than 4,000 cases of the disease are diagnosed annually in the United States. It occurs more often in boys of Japanese and Korean descent, but has been identified in children of all ethnicities and races. According to the Children's Hospital Boston's Kawasaki Disease Program Kawasaki disease causes inflammation of many tissues of the body, including the hands, feet, whites of the eyes, mouth, lips and throat. The most concerning aspect of Kawasaki disease is its potential effect on the heart and blood vessels. Heart-related complications can be temporary or may affect children long-term. Because Kawasaki disease can be so difficult to diagnose, Children's Hospital Boston keeps at least one Kawasaki Disease Program team member on call at all times to assist pediatricians and parents in determining the appropriate diagnosis and treatment.A high fever -- lasting for at least four days -- is common to all cases of Kawasaki disease, and five additional symptoms point to the presence of the disease.
To meet the classic diagnostic criteria, a child must have at least four days of fever and four out of five of the diagnostic signs. It is possible to have Kawasaki disease without showing all of the diagnostic signs. Such cases are called atypical or incomplete Kawasaki disease.
Infants younger than 6 months are most likely to have atypical Kawasaki disease. They also have the highest risk for developing coronary artery damage. Therefore, pediatricians should consider treating a baby for Kawasaki disease whenever he or she has prolonged fever and any of the diagnostic criteria.
The primary goal of treatment of Kawasaki disease in the acute phase (rapid onset followed by a short, severe course) is to reduce inflammation and control platelet activity.
The standard treatment for Kawasaki disease is the administration of intravenous immunoglobulin (IVIG). Giving IVIG (2 gms/kg over 8-12 hours) during the first 10 days of illness shortens the duration of fever and decreases the risk of aneurysm formation.
In addition to IVIG, high doses of aspirin help control fever and decrease joint pain. Doctors will usually stop high-dose aspirin therapy shortly after a child's fever has declined, but will continue a lower dose of aspirin for several weeks. Lower dose aspirin helps prevent clotting during the time when the coronary arteries can be enlarging or developing aneurysms.
Children who do develop coronary abnormalities may continue aspirin therapy indefinitely.
Doctors use echocardiograms to examine coronary artery size, heart function and valve function at the time of diagnosis, one to two weeks after treatment and four to six weeks after treatment.
Blood work--including a complete blood count, platelet count, liver function tests and tests for inflammation in the blood--is repeated at the same intervals.
Some studies suggest that the addition of steroids to the aspirin and immunoglobulin may shorten the course of fever and calm the immune reactions in Kawasaki disease. It is not yet known if steroid treatment helps prevent or reduce cardiac abnormalities. Children's Hospital Boston is part of a multi-center research study designed to answer this question
The long-term management of children who have had Kawasaki disease depends on the degree of coronary artery involvement.
Children who never had any abnormalities of the coronary arteries have an excellent outlook. With almost 30 years of follow-up, children in whom aneurysms were never detected at any stage of the illness do not show a higher incidence of heart disease or any other disorders. Some small research studies suggest that their blood vessels may be stiffer and less reactive than those of people who never had Kawasaki disease. Until more research is completed, children should be screened for any additional risk factors that are known to affect coronary arteries. A cholesterol profile is recommended at the one-year follow-up visit. In addition, children should eat a heart-healthy diet, exercise regularly and avoid smoking.
Recent research carried out at Children's Hospital Boston examined the psychosocial and physical functioning of children who have recovered from Kawasaki disease. Those without aneurysms were similar in overall health and psychosocial status to the general population.
Children who have developed aneurysms are monitored more frequently. The severity of coronary artery abnormalities determines the frequency and type of testing. Chronic management of patients with coronary artery aneurysms is focused on preventing clots and monitoring for signs/symptoms that the heart is not getting enough blood flow. Children with coronary aneurysms require long-term therapy to decrease the likelihood of clotting within the aneurysm.
Aspirin therapy reduces platelet activity. Other anti-clotting medications such as heparin, warfarin (Coumadin), or clopidigrel (Plavix) may be added to aspirin for patients with larger aneurysms. The cardiologist will balance the risks and benefits for the individual child when determining a medication regimen to prevent clotting in coronary aneurysms.
Children who are on long-term aspirin therapy should receive yearly flu shots to reduce the risk of Reye syndrome, a disease linked to the combination of aspirin use (usually high dose) and viral illness, such as chicken pox or influenza. Aspirin therapy should be stopped for six weeks after the chicken pox vaccine is given, but other anti-platelet drugs can be substituted. If a blood clot occurs within a coronary aneurysm, either as detected by echocardiography or by symptoms of a heart attack, doctors will start special treatments to dissolve the clot and to prevent further clotting in order to protect the heart muscle.
The aneurysms formed in Kawasaki disease may heal gradually over time, with the highest likelihood of return to normal for patients whose aneurysms were small to begin with. On the other hand, stenosis (the narrowing or blockage of coronary arteries in patients with aneurysms) tends to worsen with time. Again, the highest likelihood of stenosis occurs in those children with the largest aneurysms (giant aneurysms).
Stress tests, cardiac MRI's and other imaging studies to assess blood flow to the heart should be part of disease management for any child with significant cardiac abnormalities resulting from Kawasaki disease.
If there are signs or symptoms that the heart is not getting enough oxygen, a cardiac catheterization may be needed. Results for these procedures are much better in institutions with specific experience in coronary interventions, such as Children's Hospital Boston. Cardiologists also may recommend procedures such as angioplasty and the placement of stents (a device implanted in a vessel used to help keep it open), which prop open the arteries. Time is critical in these situations, so the best therapy will be the one that can be administered quickly with the most expertise. Coronary artery interventions, such as angioplasty or rotation ablation, stent placement or bypass surgery, should be performed by a team of pediatric and adult cardiovascular specialists. In very rare cases, if severe blockages have caused irreparable damage, heart transplantation may be appropriate.
