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Kawasaki disease, although common among children of Japanese and Korean descent, can strike children of any ethnic group. This illness if left untreated can cause significant damage to the heart which is why it is so important to be able to identify warning signs before coronary damage becomes too severe. In this article we will discuss how you can spot Kawasaki disease in time for treatment to be most effective; covering signs, symptoms, complications, statistics and treatment.
What is Kawasaki Disease?
Kawasaki disease is also recognized by the names: Kawasaki syndrome, lymph node syndrome and mucocutaneous lymph node syndrome. This autoimmune disease is most commonly found in children under the age of five years old and can cause significant damage and potentially death if left untreated for an extended period of time. Named after Dr. Tomisaku Kawasaki of Japan, this disorder was first described by him in 1967 after first being observed in 1961. While this Japanese pediatrician was able to identify this mystery disease and the symptoms which characterize it, he – nor any other researcher, has been able to determine why Kawasaki disease actually develops.
What Does Kawasaki Disease Do?
As Kawasaki disease sets in the patient experiences inflammation of the medium sized blood vessels within the body. Patients may also commonly experience inflammation of the skin, lymph nodes, mucous membranes and the heart. When treated promptly Kawasaki disease does not tend to affect the heart; however, when left unidentified and untreated for an extended period of time, this illness can cause aneurysms of the coronary artery. In such young children these aneurysms are often fatal but fortunately with increased exposure to this disorder, many doctors are able to identify and begin treatment before the disease progresses to this point.
The Demographics of Kawasaki Disease
Kawasaki disease is estimated to affect 19 out of every 100,000 children in the United States. Since this disorder is significantly more prevalent in children of particular Asian descents however, rates of occurrence in foreign countries tend to vary. No one yet understands why this disorder develops or why it is more prevalent in children of particular descent, most specifically Japanese and Korean. Individuals of Afro-Caribbean descent are also at an increased risk for developing this autoimmune disorder. In more recent years increasing numbers of children of Caucasian descent have been diagnosed with this disorder as well.
Currently the highest rate of Kawasaki disease diagnosis exists within Japan with a rate of occurrence at around 218 out of every 100,000 children under the age of five developing this disorder. 218 is a far cry from the 19 out of every 100,000 diagnosed children within the United States. With these statistics holding steady it is estimated that more than 1 of every 150 children in Japan will develop this disorder. Of all diagnoses of this disorder made across the world, statistics show that boys are more commonly diagnosed than are girls.
What is Kawasaki Disease? [VIDEO]
Here’s a video by the KD Foundation illustrating Kawasaki disease in detail and how it affects an individual’s body.
How Does Kawasaki Disease Manifest?
There are a number of symptoms and signs of Kawasaki disease that are labeled as “telltale” signs of the disorder which often tip off parents and pediatricians before the disorder progresses.
The primary symptom is a high fever which persists and does not respond to traditional fever reducing medications. This characteristic fever can continue for as long as two weeks. The fever that comes with Kawasaki disease often tends to reach 104 degrees Fahrenheit or higher and lasts for as long as five days. Fevers this high can cause significant complications for younger children. Such persistent high temperatures should always be treated by a medical professional.
Eye redness often occurs as a symptom of Kawasaki disease as the tissue around the eye becomes swollen and irritated. This eye redness can be diagnosed as conjunctivitis, iritis or uveitis.
Swelling in the Mouth
As tissues become swollen and irritated so too do the mucous membranes within the mouth. This type of swelling can be particularly painful and is often accompanied by a variety of other symptoms affecting this area including: redness, swelling, cracking, peeling and fluid buildup in the lips.
Strawberry tongue is actually a symptom that falls under oral signs of Kawasaki disease; however, it is such a recognizable symptom that it is often identified alone. Strawberry tongue is a result of swelling of the tongue and an extremely deep red color.
Lymph Node Swelling
Swelling of the lymph nodes is also common when Kawasaki disease sets in. This swelling often leads parents to think that their child has a simple infection when in fact the condition is much more serious.
A skin rash is commonly noted in the beginning phases of Kawasaki disease. This rash can take a number of shapes and appearances and is often difficult to pinpoint as part of this autoimmune disorder. This rash is most commonly noted within the first five days of the appearance of the fever.
Another common sign of Kawasaki disease is peeling of the skin. This peeling is most commonly noted on the hands, feet and anal regions. The palms, soles and nails are the most likely affected areas. Skin peeling is often a more advanced sign of Kawasaki disease.
As the progression of Kawasaki disease continues, internal organs may begin to be affected by the inflammation that is visible on the outside of the body. Some internal symptoms of this disease that have been noted include: pneumonitis, myocarditis, pericarditis, aseptic meningitis, valvulitis, lymphadenitis and hepatitis. Systemic symptoms are an advanced sign of this autoimmune disorder and all of these symptoms are serious and require immediate medical attention. Most systemic symptoms can be addressed by medical professionals and will eventually relent; the exception to this rule is when the coronary artery is affected. When the coronary artery is affected by Kawasaki disease damage can be significant and can lead to death by heart attack. The good news about the affect of this disease is that in most cases it is caught before significant damage to internal systems.
As swelling occurs with Kawasaki disease, patients may begin to experience swelling within the joints which causes significant pain particularly in children. This type of joint pain or arthritic symptom is generally experienced symmetrically over the body.
The combination of symptoms that present with Kawasaki disease frequently cause irritability in those diagnosed. Irritability can be lessened by making the patient feel as comfortable as possible while they are being treated for the disorder.
Frequently children who experience Kawasaki disease, tachycardia or a rapid heartbeat are noted.
Beau’s lines are deep horizontal grooves or ridges that run across the fingernail. It is important to note that these lines move horizontally rather than vertically like they do naturally as individual’s age.
The swelling that is characteristic in Kawasaki Disease can often cause difficulty in breathing.
Complications Arising From Kawasaki Disease
One of the more concerning factors of Kawasaki disease is the complications that result from the manifestation of symptoms. When left untreated for an extended period of time complications tend to arise from the disease. These complications are generally avoided by treating the disease as soon as symptoms develop. In most cases symptoms of Kawasaki disease disappear completely after two to three days of treatment. If treated within ten days most severe complications such as heart problems will not develop. It should be noted here that no case of Kawasaki disease is “by the book” and progression can be much slower or much faster than “average.”
Swelling of the Heart
One of the most concerning side effects of untreated Kawasaki disease is the swelling of coronary arteries, the lining of the heart, the valves of the heart, the muscle of the heart itself and the membrane that surrounds the heart. This type of swelling in such a crucial organ can contribute to a number of undesired effects such as arrhythmia and an inability of the heart to function normally resulting from heart valve concerns.
Another concern that results from untreated Kawasaki disease is the inflammation of blood vessels that can affect the crucial vessels within the body like those that supply blood to major organs.
Can Doctors Test For Kawasaki Disease?
Doctors do not have a test at the moment that can detect Kawasaki disease and instead they must focus on the observable symptoms to make a diagnosis. When symptoms characteristic of this disease such as strawberry tongue are present it can be much easier to identify than when symptoms are more “generic.” Like many disorders and diseases that pose something of a mystery to the medical community, diagnosis of Kawasaki disease is dependent for the most part on ruling out other potential causes for symptoms.
If a doctor suspects the presence of Kawasaki disease various tests will be performed to monitor its potential progress in addition to ruling out other common diseases. One of the most important tests performed in suspected cases of Kawasaki disease is heart function monitoring. Monitoring of the heart function allows the doctor to determine whether the disease has advanced so far that heart function has been compromised. Heart function monitoring is less of a diagnostic procedure and more of a monitoring procedure. Diagnostic procedures include urinalysis and blood tests to rule out other diseases such as an allergic reaction to a drug, scarlet fever, Rocky Mountain spotted fever, measles or rheumatoid arthritis.
In order to diagnose this disease doctors most often look for a fever that has lasted for five or more days plus four of the following five criteria: fluid buildup in the lips or mouth or cracking of the lips, swelling or peeling of the hands or feet, a rash on the body trunk, red eyes and a swollen lymph node in the neck.
How is Kawasaki Disease Treated?
Due to the fact that Kawasaki disease is still a disease that is not universally recognized or understood it is important that a child suspected of having this disease be seen by a doctor with experience in the area. If a child has been diagnosed with Kawasaki Disease they should be hospitalized and treated by a knowledgeable physician. Treatment should begin as soon as the child is hospitalized in order to prevent the disease from affecting the heart.
The first stage of treatment for Kawasaki disease is the administration of Intravenous immunoglobulin. Intravenous immunoglobulin (IVIG) is a blood product that is extracted from blood plasma of blood donors and is most commonly utilized to treat autoimmune diseases, immune deficiencies and acute infections. When the body no longer has the ability or has a decreased ability to produce antibodies IVIG therapy is used to replace this ability. In cases where a child has Kawasaki disease high doses of IVIG are given and their condition should show improvement within 24 hours. If Kawasaki disease is the only disease being considered and a response is not shown to the initial IVIG therapy a second dose may be administered. Rarely when the disease has progressed to a considerable extent or when it simply shows much resistance to treatment, a third round of IVIG may be administered.
Salicylate therapy is treatment with a product containing salicylic acid, a compound similar to that found in aspirin. Aspirin therapy remains a recommended stage of treatment in bringing down a prolonged fever. High doses of aspirin are administered to bring down the initial fever and following fever reduction lower doses are administered for approximately two months to ensure blood clots are avoided. Many individuals question the use of aspirin therapy in young children because of the incidence of Reye’s syndrome associated with aspirin use. Others believe that the administration of aspirin in conjunction with IVIG administration is the most effective method for treating Kawasaki disease. For those who do believe in the use of aspirin therapy it is important to avoid vaccinations which are tied in to the development of Reye’s syndrome, these include: influenza and varicella. These injections should be avoided for the time when a child is undergoing aspirin therapy.
When Kawasaki disease sets in and shows no signs of leaving, such as recurring symptoms or failure of other treatments, some advocate the use of corticosteroids. While this theory still abounds there is currently much more evidence to indicate that the use of corticosteroids in treatment is contraindicated due to the increased risk it creates for a coronary artery aneurysm. It should also be noted that a controlled trial used to investigate the implementation of corticosteroid use in cases of Kawasaki disease, that the implementation of corticosteroids in addition to IVIG and aspirin therapy showed no significant difference in treatment outcome.
A handful of other treatments are often implemented in cases of Kawasaki disease; however, these are not specific to the disease, rather they are used to control symptoms. Most commonly eye drops and skin creams can help to improve physical symptoms of the disease.
What is the Prognosis for a Kawasaki Disease Diagnosis?
When discussing the prognosis for an individual affected with Kawasaki disease it is important to consider the individual case. Depending upon how long the disease has been present and how much damage it has caused to the patient, the prognosis can vary. For cases where the disease was caught in its early stages before significant systemic symptoms developed there is a high likelihood that coronary artery aneurysms will be avoided. For cases where treatment is delayed, the potential for coronary artery damage increases. While the majority of symptoms of Kawasaki will eventually subside, damage that is done to internal organs such as the coronary artery cannot be repaired without medical intervention. In cases where coronary arteries are involved prognosis is not as great. It is estimated that approximately 2% of patient’s diagnoses with Kawasaki disease die as a result of coronary vasculitis. Children who successfully recover from Kawasaki disease should follow up with regular echocardiograms every few weeks to ensure no further progression of the disease. As time goes on these patients should begin to have echocardiograms every year or two to ensure that the cardiac system is still functioning healthily.
Individuals who have been “successfully” treated for Kawasaki disease may also experience a relapse of symptoms following their treatment which requires a second round of treatment. There are also a few rare cases where treatment with IVIG can cause reactions in patients; however, the medical community asserts that this risk is much less than the risk of leaving Kawasaki disease untreated.
Finally, it should be noted that current medical research suggests that the ability to metabolize lipids is altered even after the resolution of Kawasaki disease. This alteration is believed to be persistent long after the disease itself has been resolved.
Is Kawasaki Disease a Real Concern?
So is Kawasaki disease a real concern for parents? It is certainly a disease that should be tucked away in to the “what if” files for every parent but the actual chances of a child developing this illness are remote. With that said any time a parent suspects a child of showing symptoms of this devastating disease it is always better to be safe than sorry and a visit to the pediatrician is always recommended. Kawasaki disease may not be a significantly widespread or even well known disease but the damage it can inflict is life lasting. Education is the key to prevention in the case of this autoimmune disorder because while there is not currently a preventative vaccination available, rapid treatment at the onset of the disease makes all of the difference in a child’s prognosis.