Kawasaki. Not the Motorcycle

Ann Hutchins’ grandson Garrett Stevens

Everywhere I go I run into someone who tells me a story that catches my interest. Ann Hutchins, for instance. We were seated together at the Go Red For Women Luncheon last month when she told me about her grandson Garrett. He’d been very, very sick recently with Kawasaki Disease.”What on Earth is Kawasaki Disease?” you ask. Precisely what I said to Ann.

I found out that Kawasaki Disease is a fairly uncommon illness that causes inflammation in the blood vessels. Every year in this country, it’s diagnosed in somewhere between 9 and 19 per 100,000 children under the age of five. Ann’s grandson recovered, but it was a scary situation. She put me in touch with her daughter Caitlin Stevens, who filled me in on the details.

“I had never even heard of Kawasaki Disease,” Caitlin told me, “until Garrett got it.” Their story began on a Friday night about three months ago, when Garrett was just nine months old. “He wasn’t acting himself,” described Caitlin. “He wouldn’t sleep and he was crabby. On Saturday he had a real high fever, so we took him to the ER. They did blood work, a chest x-ray, and a couple of other tests. They checked him for a urinary tract infection and they said they thought he just had an ear infection and gave us some antibiotics and sent us home. He was just miserable, and the next morning we noticed a rash and we took his temperature. It was 104, so we decided to bring him back to the ER.”

Back in the ER at York Hospital, the doctors ran more tests and tried unsuccessfully to get his fever down. They decided to admit him. “From there on, he did nothing but get worse. The rash just exploded. He got red as a cooked lobster and had a 105 temperature. Nothing they did could get it down and he wouldn’t eat or drink anything. ”

Garrett’s pediatrician consulted with pediatricians at Maine Medical Center. Some of his symptoms pointed to Kawasaki Disease.

Signs and Symptoms of Kawasaki Disease

• Fever, which often is higher than 101.3 F and lasts five or more days
• Extremely red eyes (conjunctivitis) with no drainage
• A rash that is often worse in the genital area
• Red, dry, cracked lips and an extremely red, swollen tongue (“strawberry” tongue)
• Swollen, red skin on the palms of the hands and the soles of the feet
• Swollen lymph nodes, especially in the neck
• Irritability

The decision was made to transfer Garrett to The Barbara Bush Children’s Hospital, where he would be assessed by a team of specialists. On the ambulance ride to Portland, Caitlin observed that he was getting worse. “His lips and tongue were very, very red and dry. The rash spread to his whole body and it was raised. One thing after another just kept happening.”

Among the team of doctors was a pediatric cardiologist. I contacted Dr. Adrian Moran, a pediatric cardiologist with Congenital Heart, to ask him about the connection between Kawasaki Disease and the heart. He confirmed that Kawasaki Disease is a condition that causes inflammation in the walls of the arteries throughout the body. If it isn’t treated, it can permanently damage the coronary arteries, which deliver blood to the heart. “Ultimately it is a cardiac condition,” he explained, “but it is an acquired condition of childhood, not a congenital condition that you are born with.”

While Garrett had a growing number of signs and symptoms related to Kawasaki Disease, the team wanted to make sure of the diagnosis. Because there is no specific diagnostic test available, a variety of factors had to be taken into consideration. “The diagnosis is a clinical one,” outlined Dr. Moran. “Lab testing is supportive, but not diagnostic. Other common infections can mimic it, as can certain rheumatologic conditions. Close cardiac follow up is critical with serial echocardiograms — to ensure that the coronary arteries and/or aorta do not dilate.” (Rheumatologic conditions include arthritis and other diseases of the joints, muscles and bones.)

Other infections and conditions were ruled out and an echocardiogram showed that Garrett’s coronary arteries were enlarged. On Wednesday evening, five days after his first symptoms, the doctors agreed that it was Kawasaki Disease and began treating him with high-dose gamma globulin (IVIG) and aspirin.

The next morning Garrett’s fever was down to 101 and continued to go down throughout the day. The rash started going away within a few hours. “It was pink instead of red and didn’t cover his whole body,” reported Caitlin. “Within a few days the rash was gone. It took a couple weeks for the swelling in the lymph node to go away totally. He started eating and drinking again Thursday afternoon.” Garrett had to stay in the hospital a few more days to make sure his fever didn’t spike again and that he didn’t need a second treatment. It didn’t and he didn’t.

He’s had two follow-up echocardiograms show his coronary arteries are back to normal. Today, you’d never know little Garrett had been so sick, says his grateful mother, noticing that, “It’s amazing how fast life goes back to normal. You remember it and everything, but looking back on it, it’s like, oh my goodness that was so scary. How did we make it through?”

If he hadn’t been treated, Garrett probably would have gotten better on his own in about 12 days, but with possible damage to his heart. According to the American Heart Association, cardiac complications develop in 15 to 25 percent of children who are not treated within ten days of the onset of the illness. With treatment, there is still a 1 to 5 percent risk.

No one knows what causes Kawasaki Disease. Dr. Moran says there have been many theories over the years, including a link to carpet cleaning. “In reality, the cause is likely a combination of genetic susceptibility triggered by an environmental insult, such as a viral infection.” If you’d like to learn more about Kawasaki Disease, you’ll find a lot of information on the Kawasaki Disease Foundation Website.

We all love happy endings and so, I give you one for Garrett’s story. He turns one on Sunday, April 8. What a celebration that will be! Happy Birthday Garrett!