It’s been a big year for Kawasaki Disease, but this hasn’t always been the case. KD has been a pretty steady bench warmer on the team of rare diseases. Every once in a while, we’re called in to pinch hit. We bat a marathon here, a House episode there. But all jokes aside, Kawasaki Disease is just not very well known. I’ve had nurses ask me what it is when giving them my daughter’s history.
According to Seattle Children’s Hospital, doctors in America diagnose approximately 4,000 cases of KD each year. And this, of course, doesn’t consider the countless cases that go misdiagnosed. The Kawasaki Disease Lab at UCSD tells parents as much by stating, “Kawasaki Disease is not a rare illness and the exact number of cases which occur in the United States has not been determined,” They estimate that 10 out of every 100,000 children will be affected and have observed KD occurring in “clusters or localized outbreaks - usually in the winter and spring.” I’ve seen other statistics as high as 19 or 20 out of every 100,000.
The only time I’d ever heard of this illness before 2008 was in an episode of the TV show ER. Then my 22 month old started displaying the classic symptoms of Kawasaki Disease and I got very familiar very quickly. If that wasn’t enough, I became even more intimately involved when she was diagnosed again at 6 years old with Recurrent Kawasaki Disease.
From a medical standpoint, KD is defined as a pediatric self-limited vasculitis that is the leading cause of acquired heart disease in children. The complication that raises the most concern is inflammation in the coronary arteries of the heart. If left untreated, KD can lead to coronary artery aneurysms in up to 25% of reported cases. When treated with Intravenous Immunoglobulin (IVIG) within the first 10 days after symptoms start, chances of heart complications are reduced. The primary symptom of KD is a fever that lasts more than five days. The other symptoms are visually alarming and include red and swollen feet and hands, red eyes, red/chapped/cracked lips, strawberry tongue, rash, and swollen lymph nodes in the neck.
From a kid engulfed in its blaze standpoint, KD is defined as pain - an awful, burning pain. My daughter has a hard time describing it. She will talk about how much it hurt, but can’t tell me where. It’s like it hurt everywhere at the same time, and then it hurt worse because someone was always “doing something to me.”
From a parent sitting helplessly at the foot of the bed standpoint, KD is defined as watching my child burning from the inside, out. Think about the last time you had a sunburn and how much that hurt. Now imagine having a sunburn on the inside of your body. It’s like every cell in her body was on fire, and nothing could quench it. You want desperately to soothe your baby, and your baby wants so desperately to be soothed. But there is no soothing. There is no comforting. There is only burning.
And the hits just keep on comin’. No one knows what causes KD. There is no diagnostic test for KD and no test to show it’s “cured.” No one knows how to prevent Kawasaki Disease and many cases of a-typical or incomplete KD are never diagnosed. Many other cases of KD aren’t diagnosed within the 10 day window, which often leads to further complications.
But, I don’t mean to be quite so dire. Even though there are still many confusing questions, the quest for answers has never been more fervent than in the past few years. In November of 2011, a group of Singapore scientists published a study in Nature Genetics that identifies a specific gene (a protein called FCGR2A) associated with an increased risk of KD. That same month, a group of doctors, including Dr. Burns, published a study that suggested the environmental trigger for KD could be wind-borne.
Many more hospitals are now focusing in on Kawasaki Disease. Seattle Children’s Hospital’s Portman Research Group Kawasaki Disease Program is currently trying to understand and improve the treatments for KD and is also pursuing a hypothesis that soy consumption may be associated with KD risk. Rady’s Children’s Hospital’s Kawasaki Disease Clinic follows the status of 1,200 KD patients in San Diego and treats 80 to 90 cases every year. Dr. Burn’s Kawasaki Disease Lab at UCSD has information about 12 separate studies on their website. Many of the research studies conducted by these groups are still accepting participants.
There have also been media sightings of a slightly flashier nature. In April of 2011, chef Nate Appleman chose the Kawasaki Disease Foundation as his charity when he won the Food Network’s Chopped All-Star competition because his son had been affected. After his own son recovered from KD, Gary Busey joined the campaign and started The Busey Foundation. Busey went on (and was fired from) the latest season of Donald Trump’s Celebrity Apprentice, in the hopes of winning money for KD.
The biggest spotlight yet came on March 8, 2013, when Sarah Chalke guest starred as the mom of a little boy sick with Kawasaki Disease on an episode of Grey’s Anatomy. The prominent storyline not only mentioned the classic symptoms of KD, but also illustrated the kind of cat-scratch fighting a parent often has to endure when Kawasaki Disease invades their child and how important it is to listen to your intuition when it comes to the health of your child.
The wonderfully hilarious actress got serious when she opened up a small window into her private life and told the world that her own son was a Kawasaki Disease survivor. Ms. Chalke explained that after meeting other KD survivors and their parents, she felt compelled to join the KD awareness campaign. She approached Grey’s creator, Shonda Rhimes, with the KD storyline idea and made the round of popular talk and news shows, giving millions of people their first glimpse at this unusual disease.
Promising research and a larger public spotlight have now collided into the most hopeful KD news yet. We may soon have a diagnostic test for Kawasaki Disease! The combined efforts of Dr. Burns' team, The Kawasaki Disease Foundation, and Sarah Chalke have culminated into the Kawasaki Disease Challenge Fundraiser on CrowdRise.com. On the fundraiser’s website, Sarah Chalke writes:
When my son had Kawasaki Disease and was misdiagnosed for ten days, I can’t tell you how many times I said, “I just wish there was a test. A test that could tell us if this is KD.”
And now there might be. A team of doctors at Stanford University and UCSD have devised a prototype for a diagnostic test. This is a huge deal. This test could be 95% accurate but it needs to be tested for two years before it can be used. We NEED this test because of the ticking clock to get treatment to prevent heart damage that can lead to a heart attack.
The disease that didn’t even get its name until 1967 may yet be considered a rare childhood disease to some and many have still never even heard of it, but once Kawasaki Disease enters your life, you never forget it. So many moms, just like myself, have gone through this nightmare and felt compelled to reach out and make sure other parents have indeed heard of Kawasaki Disease. Sarah Chalke is one of these moms. We want the misdiagnosing to stop. The best way to do that is with a diagnostic test. We’re now closer than we’ve ever been before. Please go to Sarah Chalke’s Kawasaki Disease Challenge today. It has already raised over $5,000 in its first 2 days, and the Gordon and Marilyn Macklin Foundation will match dollar for dollar up to $100,000! Let's raise $200,000 for a Kawasaki Disease diagnostic test. Donate. Share. Tell your coworker over coffee. Mention it to your neighbor over the fence. Save a heart. Save a life.
For an organized list of all the links in this article and many more, please check out my KD Resource List.