Recognizing and treating an atypical case of Kawasaki disease

It was a Saint Paddy’s Day that Jake and his mother, Nancy O’Connor, will never forget. While everyone else was sporting green, Jake’s skin and tongue started turning red, and the whites of his eyes eventually turned yellow. It was a medical problem that eventually led him to Boston Children’s Hospital where he was diagnosed with an atypical case of Kawasaki disease, a rare condition that can cause a rash, red eyes and mouth, and other inflammatory symptoms.  Most importantly, Kawasaki disease can lead to serious complications of the heart, especially if not treated in time.

Difficult diagnosis

feverJake woke up that March morning not feeling well. His skin was itchy and irritated, his tongue was swollen and his forehead was hot to the touch. Being a nurse and mother, Nancy has seen her fair share of flus, colds and stomach bugs—but the moment she laid eyes on her son Nancy knew there was something more seriously wrong with Jake.

“He looked so terrible, like he wasn’t even himself,” she says. “I wasn’t sure what was wrong, but something told me this wasn’t your typical childhood illness. Something very different, and very bad, was happening to Jake.”

Nancy took her son to a pediatrician, but Jake’s doctor was far less worried than his mother. After a quick physical exam the doctor told Nancy not to worry—her son had probably just come down a virus, which was very common for that time of year. He prescribed plenty of fluids and rest and sent the O’Connors on their way.

But nearly a week passed and Jake went from bad to worse: his rash was spreading, the fever had intensified and his eyes started turning a faint shade of yellow. Recognizing the yellowing as jaundice, Nancy took Jake to the local Emergency Department (ED). The ED staff believed Jake had scarlet fever, so he was given a strong dose of antibiotics and Nancy was told it would clear up soon.

The days passed but Jake failed to get better. Nancy began searching Jake’s symptoms online and speaking with family and friends, hoping to connect with anyone whose child had come down with a similar illness. Everything she read or heard seemed to indicate Jake had Kawasaki disease, but there was one problem with the homespun diagnosis—Jake was almost half a decade older than the typical Kawasaki case.

“Everything I learned about Kawasaki’s symptoms sounded exactly like what was happening to Jake, but it usually only affects babies or toddlers, not 10-year-olds,” Nancy says. “Because of his age none of Jake’s doctors had even considered Kawasaki. But the more I researched it, the more convinced I was that that’s what Jake had.”

An unusual case

Newburger_Jane_2PreviewlargeWhen Nancy and Jake went back to his pediatrician a third time, his skin was now completely covered in an itchy, peeling rash, and he was still running a fever. Nancy shared all the information she had gathered on Kawasaki and its symptoms with the doctor, who agreed that even though Jake’s age made Kawasaki unlikely, clearly he was suffering from more than a virus. He suggested she take Jake to one of the Boston hospitals that had specialized staff to treat such rare diseases. Within a few hours Jake and Nancy were on their way to Boston Children’s to meet Jane Newburger, MD, MPH, one of the country’s leading Kawasaki researchers and experts.

A better understanding of Kawasaki

At Boston Children’s, Jake was immediately admitted and had a series of tests done to confirm the diagnosis.

“Only about 25 percent of all Kawasaki cases happen in children over 5, but they do happen, even in teenagers, so you can never really use age to rule out a Kawasaki diagnosis,” says Newburger, who is director of Boston Children’s Kawasaki Disease Program. “Once the correct diagnosis is made, treatment and cardiac monitoring needs to begin quickly, because people whose Kawasaki disease goes unrecognized and untreated for too long are at a much higher risk for development of coronary aneurysms.”

Once it was determined that Jake did have Kawasaki, he received an infusion of intravenous immunoglobulin (IVIG), which calms the inflammatory response in the wall of the coronary arteries. He had only been in the hospital for a few hours, but already the immunoglobulin infusion had him looking much better.

“All of a sudden the rash started to subside and his skin started to clear up,” Nancy says. “After all the time we spent getting to Boston Children’s, it was remarkable how fast he started to look like himself again once his treatment began.”

jake-IBut Jake’s road to recovery wasn’t without a few speed bumps. Even though his skin had begun to clear and his eyes returned to their normal color, his fever refused to break. Nancy’s fear of the worst returned, but the Boston Children’s nursing staff was quick to comfort her, letting her know that it wasn’t uncommon for kids to need two, sometimes even three infusions before they were well again. Sure enough, they were right—two days after they arrived at Boston Children’s, Nancy and Jake were on their way, both looking much more like their old selves than they had in days.

“Despite all the worry and uncertainty that sent us to Boston Children’s, once we arrived we had a wonderful experience,” Nancy says. “They knew Kawasaki and how to treat it. But it was more than just that, they also really knew how to care for Jake and his nervous wreck mother. They went out of their way to explain everything to us and engaged us in every part of the recovery process. It turned a terrible situation into a great one.”

Because Kawasaki disease can sometimes be difficult to diagnose—especially in patients over age 5—the disease may not be immediately recognizable to many health care providers. But timely diagnosis and treatment of the condition are the best defense against Kawasaki disease and lowering the risk of dangerous heart complications. To speak with one of our Kawasaki experts, please visit the Kawasaki Disease Program website.

  • Aleisha Poole

    Thank you for sharing this. My son had Kawasaki disease at 8 years old and his symptoms were much like what was described her! Our case when almost exactly the same way!

  • Dara Agan Cassidy

    Our son was also ten when he was diagnosed with Kawasaki’s. We went to the doctor on Friday and he thought he had hives and sent us home with instructions to call them on Saturday morning. He appeared to be better but by Sunday, he felt much worse with vomiting and what felt like a high fever although when I would take his temp, it was only 99-100 degrees. Luckily, our pediatrician had Sunday hours so we took him right away. After drawing blood and seeing it was all out of whack, they sent us to a nearby hospital. Thankfully, between the pediatrician and a dermatologist, they had him diagnosed by early Monday afternoon and started him right away on the IVIG. We had one follow-up EKG instead of the recommended one every six months. He saw a cardiologist about 2 years ago and was told his heart was stronger than most his age. I am so thankful our drs. were on the ball with his diagnosis! He will soon be 21 and is as healthy as a horse!