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Condition that leads to heart disease in kids often undiagnosed

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Chicago Tribune


CHICAGO - For many frantic parents, typing "fever, rash, swelling and red eyes" into an Internet search engine is the first clue they have that their child may have Kawasaki disease, a little-known disorder that is the leading cause of acquired heart disease in children.

"That's how I found out about it," said Lisa Morin, 42, of Chicago. "There was one line that said if it looks like scarlet fever, but isn't, then it's probably Kawasaki disease."

The disease is far from rare, yet it lingers below the radar screen of both parents and many doctors because its outward symptoms are similar to many other common childhood maladies. Unlike these other diseases, Kawasaki does not have a blood test that can identify it.

About 2 1/2 years ago, Morin's son Clark, then just over 2 years old, came down with a lingering illness that her regular doctors could not diagnose.

"I didn't like being told, We don't know what he has. It's just a rash and fever,'" she said. "Even when I was telling them that I thought it could be Kawasaki disease, they were saying,Oh no. He doesn't have Kawasaki disease. We haven't seen that in 10 years.'"

His disease was correctly diagnosed only after the skin on Clark's fingers and toes started peeling six days into his illness and Morin called Children's Memorial Hospital. She was told to rush him to the emergency room, and intravenous gamma globulin therapy was started.

Doctors at Children's diagnose 40 to 60 Kawasaki cases a year, said Dr. Anne Rowley, a pediatric infectious disease expert, and in about 25 percent of the cases, parents are the first to suspect Kawasaki based on an Internet search.

"That disturbs me a lot," said Rowley, also a professor at Northwestern University's Feinberg School of Medicine. "Pediatricians should be the ones diagnosing this disease."

Kawasaki disease is a reasonably common infectious disease; an estimated 4,000 children develop the illness in the United States each year, said Dr. Ermias Belay, a medical epidemiologist at the Centers for Disease Control and Prevention, who tracks the disease.

Still, parents often are unaware of it. "Pretty much every parent knows about SARS or Lyme disease, and yet the chance of their child developing Kawasaki disease is incredibly higher than either of those diagnoses," Rowley said.

First diagnosed by Dr. Tomisaku Kawasaki of Japan more than 30 years ago, the disease that bears his name is a mystery. The disease is thought to be caused by a common infectious agent that affects genetically susceptible children, usually under age 5.

Besides such outward symptoms as rash and fever, the disease also causes inflamed arteries in about one of four children. Inflammation in coronary arteries can cause aneurysms when weakened artery walls balloon out.

"Without treatment, more than 20 percent of children will develop inflamed arteries, but even with treatment, 10 percent of children will have affected arteries," Belay said.

Fortunately, the vast majority of these cases recover with no complications, Belay said. The death rate from ruptured aneurysms or blood clots blocking coronary arteries is less than 1 percent, but some researchers believe that damage to coronary arteries from inflammation can lead to heart disease later in life.

"It's the leading cause of acquired heart disease among children in the U.S.," Belay said. "If a child acquires a cardiac problem after birth, chances are that patient had Kawasaki disease."

Surveys show that Asian children, primarily of Japanese heritage, have the highest risk of contracting the disease, followed by African-Americans and whites.

Between 30 and 40 cases each year are referred to Advocate Lutheran General Hospital in Park Ridge for diagnosis, said Dr. Julie Kim Stamos, chief of pediatric infectious diseases.

"It's real hard for us to ask parents to be able to recognize it because even doctors have a hard time recognizing it," she said.

Stamos said one child died of a ruptured aneurysm several years ago and another had to have bypass surgery. "These were patients who were referred to us very late," she said. Therapy should start within 10 days from the onset of symptoms.

Patients are treated with gamma globulin, an extract from donated blood that contains antibodies against a wide variety of infectious agents. Gamma globulin may help subdue a runaway immune response thought to underlie Kawasaki. Other medicines are used to control high fevers.

How gamma globulin works is not known, but it is believed to provide passive immunity against a virus or bacteria that may be causing inflammation in the arteries, said Rowley of Children's Memorial.

Rowley is trying to identify an infectious agent in the lung and artery tissue of patients who have died so a vaccine may be developed to prevent Kawasaki disease.

"I think their immune response is trying really hard to get rid of the infectious agent," she said. "They're sending all kinds of inflammatory cells - white blood cells - to their coronary arteries to help fight off the infection.

"The problem is that the coronary arteries are pretty delicate and become swollen. The children can develop clots in the swollen arteries and have heart attacks. Or a swollen coronary artery can burst."

Researchers believe that the infectious agent that triggers the immune attack in Kawasaki disease probably infects almost everyone, but some children have a genetic predisposition to overreact.

A smaller group of children have a further genetic susceptibility allowing the infection to spread to their coronary arteries, said Dr. Jane Burns, director of the Kawasaki disease research program at the University of California at San Diego.

"I think it's likely that it's going to be one or a small number of agents that trigger Kawasaki disease," she said. "We're hot on the trail of two of them, and there are going to be a handful more that will be important to identify through future studies."


(c) 2004, Chicago Tribune. Distributed by Knight Ridder/Tribune News Service.

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