For actress Sarah Chalke, her role on last week's Grey's Anatomy hit very close to home.
Chalke, who is best known for her role as Dr. Elliot Reid on the hit comedy Scrubs, played Casey, a mother desperate to find out what medical illness her son has. Just so happens that Sarah had a very similar medical mystery at home.
About 2 years ago, her own 1-year-old son Charlie became very ill and was thought to have a viral infection. Chalke was not convinced and extensively researched the symptoms herself. She came to believe that her son had a difficult to diagnose disease called Kawasaki disease (KD), but had a hard time getting doctors to listen to her. As she told :
"You present with all these symptoms, you get a lot of repeated misdiagnoses, you keep getting sent home [by doctors]… We had a really hard time getting him diagnosed."And with Kawasaki disease, there is an added wrinkle: treatment needs to be started in the first 10 days to prevent more serious complications (see below). Fortunately, although Charlie started treatment on day 10-1/2, he is fine today. After going through this ordeal, she and fiancé Jamie Afifi wanted to figure out a way to help other families going through the same thing:
"We thought, ‘What can we do to help other parents and kids not have to go through what we went through? What about an episode on a medical show?’"Thus came the Grey's Anatomy episode. Chalke pitched the idea to Grey's producer Shonda Rimes with the that "symptoms would be mentioned and shown so that as a parent when you're watching, you're able to see the symptoms and have this visual trigger."
Besides educating the public about the symptoms of KD, Chalke also wants parents to take home this message:
"Fight for your kid, don't be scared to get a second, third, sixth opinion or go into an ER and put your foot down. That's certainly how I felt in my experience."Chalke also filmed a public service announcement which aired at the end of the episode on behalf of the .
What is Kawasaki Disease?
Kawasaki disease is a rare childhood disease . It's a form of vasculitis and can affect any type of blood vessel in the body, including the arteries, veins, and capillaries.Sometimes Kawasaki disease affects the coronary arteries. As a result, some children who have Kawasaki disease may develop serious heart problems.
In the United States and other Western countries, Kawasaki disease occurs in approximately 1 in 10,000 children under 5 each year. The condition is 10 to 20 times more common in East Asia, including Japan, Korea, and Taiwan.
What Causes KD?
The causes of Kawasaki disease are not well understood. Although the disorder is generally regarded as an abnormal immune system activation, the triggers of this abnormal response are unknown. As cases of the disorder tend to cluster geographically and by season, researchers have suggested that an infection may be involved. However, no infectious agent has, as of yet, been identified. The disease does not appear to be contagious.A variation in the has been associated with an increased risk of Kawasaki disease. The ITPKC gene provides instructions for making an enzyme called inositol 1,4,5-trisphosphate 3-kinase C. This enzyme helps limit the activity of immune system cells called T cells. T cells identify foreign substances and defend the body against infection. Reducing the activity of T cells when appropriate prevents the overproduction of immune proteins called cytokines that lead to inflammation and which, in excess, cause tissue damage. Researchers suggest that the ITPKC gene variation may interfere with the body's ability to reduce T cell activity, leading to inflammation that damages blood vessels and results in the signs and symptoms of Kawasaki disease.
Kawasaki disease affects children of all races and ages and both genders. It occurs most often in children of Asian and Pacific Islander descent. The disease is more likely to affect boys than girls. Most cases occur in children younger than 5 years old.
What Are the Signs and Symptoms of Kawasaki Disease?
Major Signs and Symptoms
One of the main symptoms during the early part of Kawasaki disease, the acute phase, is fever. The fever lasts longer than 5 days. It remains high even after treatment with standard childhood fever medicines. Other classic signs of the disease are:- Cervical lymphadenopathy
- A rash on the mid-section of the body and in the genital area
- Red, dry, cracked lips and a red, swollen tongue
- Red, swollen palms of the hands and soles of the feet
- Redness of the eyes
- During the acute phase, your child also may be irritable and have a sore throat, joint pain, diarrhea, vomiting, and stomach pain.
- Within 2 to 3 weeks of the start of symptoms, the skin on your child's fingers and toes may peel, sometimes in large sheets.
Without treatment, about 25% of children develop heart disease involving the coronary arteries. Inflammation in the walls of these arteries cause them to weaken and balloon out, forming coronary aneurysms. Often, the disease's effects on the coronary arteries don't show up until the second or third week after the first symptoms appear. Treatment is most effective in preventing coronary aneurysms if given within the first 10 days of the disease.
How Is Kawasaki Disease Diagnosed?
Diagnosing Kawasaki Disease is not easy. Early on, the illness looks like a simple viral illness or perhaps strep throat. It is only as the disease progresses -- i.e. the fever doesn't go away, the rash progresses, the hands and feet look puffy -- that a physician may suspect something else is going on.The diagnostic criteria for Kawasaki has been set by the American Heart Association:
Other diseases which cause similar symptoms must also be ruled out, including Rocky Mountain spotted fever, scarlet fever, and juvenile rheumatoid arthritis. Tests and procedures, including an echocardiogram, blood tests, chest x-ray and EKG may help confirm whether a child has the disease.
Unfortunately, not all children have classic signs and symptoms of Kawasaki disease. Incomplete Kawasaki disease refers to patients who do not fulfill the classic criteria of at least four of the five findings. It is more common in children younger than 1 year. Unfortunately, the rate of coronary artery aneurysms is higher in this group if untreated.
Some promising research at Boston Children's Hospital may make the diagnosis of Kawasaki disease easier and faster. Drs. Alex Kentsis, Hanno Steen and Susan Kim have been looking for biomarkers in urine that may help make the diagnosis of KD. Their 2012 paper in the journal "identified signatures of distinct biological processes that are associated with Kawasaki disease. Using tests readily amenable for routine medical use, [they] found that two discovered markers of Kawasaki disease, meprin A and filamin C, can be used to identify patients with Kawasaki disease with excellent accuracy."
How Is Kawasaki Disease Treated?
Medicines are the main treatment for Kawasaki disease. Rarely, children whose coronary (heart) arteries are affected may need medical procedures or surgery. The goals of treatment include:- Reducing fever and inflammation to improve symptoms
- Preventing the disease from affecting the coronary arteries
Initial Treatment
Kawasaki disease can cause serious health problems. A child will likely be treated in a hospital, at least for the early part of treatment.The standard treatment during the disease's acute phase is high-dose aspirin and immune globulin.
Most children who receive these treatments improve greatly within 24 hours. For a small number of children, fever remains. These children may need a second round of immune globulin.
At the start of treatment, the patient will also receive high doses of aspirin. As soon as his or her fever goes away, a lower dose of aspirin is given to prevents blood clots which can form in the inflamed small arteries.
Most children treated for Kawasaki disease fully recover from the acute phase and don't need any further treatment.
Children who have had immune globulin should wait 11 months before having the measles and chicken pox vaccines. Immune globulin can prevent those vaccines from working well.
If Kawasaki disease has affected a child's coronary arteries, he or she will need ongoing care and treatment with a pediatric cardiologist.
For more information on Kawasaki disease, go to the