Teen Proves Kawasaki Disease is Not One Size Fits All

Audrey is a teen with Kawasaki disease. Here, she is pictured with her cello before the condition caused a large aneurysm to form in her heart. 

Whether she’s performing in her school’s jazz band, teaching cello, painting or working as a YMCA counselor, Audrey Wright, 16, seems to do it all. She especially doesn’t let an aneurysm in her heart that developed as a result of Kawasaki disease get in the way of being a busy teenager and her dreams of majoring in studio art.

Audrey was diagnosed with Kawasaki disease, a serious illness that causes inflammation of blood vessels throughout the body, after coming down with what she and everyone else thought was a really bad viral infection. Despite visiting her pediatrician four times in six days, it wasn’t until her blood pressure dipped dangerously low that she was transported to Seattle Children’s. There, doctors in the Pediatric Intensive Care Unit began to unravel the mystery of what was causing her illness.

“Once they got the report back, all of the pediatricians she had seen before coming to the hospital couldn’t believe it was Kawasaki disease,” Karen Wright, Audrey’s mom, said. “They were pretty shocked because she’s not the typical patient.”

Addressing Kawasaki disease misconceptions

Kawasaki disease affects about 7,000 children in the U.S. each year with most cases occurring in children under age 5. Since Audrey was 11 at the time of her illness, Kawasaki disease wasn’t considered until later when other tests were inconclusive.

“Commonly held misconceptions about Kawasaki disease can delay a crucial early diagnosis,” said Dr. Michael Portman, a cardiologist in Seattle Children’s Kawasaki Disease Clinic and researcher at Seattle Children’s Research Institute. “One mistakenly held belief is that the disease only occurs in children under age 5. This just isn’t true.”

According to Portman, another misconception is that Kawasaki disease exclusively affects Asian or Asian-American populations. At Seattle Children’s, which provides ongoing care for 600 children with Kawasaki disease, only about 30% of patients come from an Asian background.

Kawasaki disease and quality of life

When treated early, most children who are ill as a result of their Kawasaki disease can fully recover in a matter of days. Kawasaki disease that goes untreated for 10 or more days or does not respond to standard treatment can cause serious complications, including lifelong aneurysms, stent placement, heart transplant, skin disorders and childhood anxiety.

A recent study published by Portman in the Journal of Pediatrics found that children with Kawasaki disease suffer from greater health-related quality of life burdens than children newly diagnosed with cancer. Portman and his coauthors report that treatment in the hospital causes the most stress and anxiety in patients and families. However, it remains unclear the long-term impacts on children, like Audrey, who develop aneurysms or other acquired heart disease and need ongoing care.

“Audrey’s Kawasaki disease is really advanced,” Portman said. “Her aneurysm is a setup for developing a clot, so she’ll need to take blood thinners for the rest of her life. That alone restricts the type of activities she can take part in.”

Fortunately, Audrey has always preferred a paintbrush or musical instrument to what she calls “rough and tumble sports.” And although the diagnosis was chaotic at first, she has since adjusted to a new normal.

“Coming home from something like that was crazy,” Audrey said. “Eventually everything returned to normal. I really don’t let having a heart condition affect my life. I have so much other stuff going on that I hardly think about it.”

In addition to taking blood thinners, Audrey comes to Seattle Children’s one to two times a year to check for any changes in the size of her aneurysm. At same time, Portman also checks her overall heart function and the arteries around her heart.

Research to find a better treatment for Kawasaki disease

A talented musician and budding artist, Audrey, now 16, doesn’t let having a heart condition get in the way of the activities she loves.

Audrey also had the opportunity to participate in research Portman is leading to improve treatment of Kawasaki disease.

One clinical trial involves adding another drug to the primary treatment for Kawasaki disease, intravenous gamma immunoglobulin (IVIG). IVIG is effective in reducing fever in 70% to 80% of Kawasaki disease cases. However, children who do not respond to IVIG need another therapy to prevent serious damage to the heart. Portman’s team is analyzing the results of the multicenter trial, which recently completed enrollment of over 200 patients with Kawasaki disease.

Another factor driving treatment response to IVIG is the patient’s genetic background. Findings from the clinical trial above and other ongoing research have started to show differences in treatment response based on genetic traits.

“In the future, personalized medicine will help direct Kawasaki disease treatment,” Portman said. “Instead of treating all Kawasaki disease patients the same, we can look at the patient’s genetic background and determine the best therapy for that person – whether that’s IVIG or IVIG plus another drug.”

Perspective after a terrifying ordeal

Now five years after learning about her Kawasaki disease, it’s full steam ahead for Audrey.

“There’s no precedent for Audrey,” Wright said. “There was no page in the book for a child as old as her with as large of an aneurysm. Once we met with Dr. Portman, we felt really hopeful that it wasn’t going to get any worse. ”

Audrey’s aneurysm has remained stable, allowing her to keep up with all the activities she loves. She believes having gone through this experience and being a teen with Kawasaki disease has made her stronger too.

“It sounds cliché, but having Kawasaki disease put a lot of things into perspective,” Audrey said. “When you’re 11 years old, you’re focused on things like going to school and packing your lunch. Then I got sick and all those little things just melted away. I can see the bigger picture better because of my situation.”

You can help fund Kawasaki disease research at Seattle Children’s by donating to the KD-KIDS Guild at Seattle Children’s.

Families of children with Kawasaki disease can contact the Kawasaki Disease Clinic at 206-987-2015 for a referral, a second opinion or more information. They can also contact the Kawasaki Disease Research Program at [email protected] or 206-987-5153 for research information or participation.