Sun May 20, 2012
A Windborne Clue To A Mysterious Childhood Disease
Originally published on Mon May 21, 2012 3:30 pm
At first, Deborah Kogan of New York says, she thought it would pass. Her 4-year-old son's fever had been on the rise for hours, and he was looking puffy. Kogan took Leo to the pediatrician, who thought it might be strep throat. It wasn't.
A few days later, Leo "woke up, and he looked as if he was one of the characters in The Nutty Professor. His face ballooned about twice its normal size." She posted a photo of Leo on Facebook. That's when the crowdsourced diagnosis took shape.
On Facebook, a cousin wrote, "Look, I really think you should check and make sure it's not Kawasaki disease." A pediatrician from the Bay Area posted, "I hope the doctors have ruled out Kawasaki disease." And so it continued.
Kogan had never heard of the disease but didn't need more warnings. She took him to the nearest hospital, where, indeed, he was diagnosed with Kawasaki disease.
Kawasaki disease was discovered by Japanese pediatrician Tomisaku Kawasaki in 1967, but it wasn't until the 1970s that the Japanese Ministry of Health made a further inquiry. That's when researchers realized that children were dying — not from the disease, but from the heart attacks that followed, says Dr. Jane Burns of the University of California, San Diego.
Burns is the foremost expert on Kawasaki disease, but even so, not a lot is understood about it. It's a condition that causes arterial inflammation and also affects lymph nodes and mucous membranes. Symptoms include swelling, like Leo's face, fever and even peeling skin.
It's a rare disease that mainly affects children younger than 4. It's estimated that more than 4,000 children get the disease each year in the U.S., but it's far more common in Asia, particularly in Japan, where 1 out of 150 children is infected. Scientists say it's unclear how the disease spreads.
But a few years ago, Burns started to notice a pattern: The disease appeared to be seasonal. "That meant that in January, February, March we were going to be very busy, and then in September, October, that was a good time to take our vacations," she says.
Japanese researchers noticed a similar pattern. Together, they worked with a group of climate scientists who started to analyze atmospheric data.
What the climate researchers found was an association with the direction of wind circulating in the troposphere at heights of 3,000 meters, Burns says. When those wind currents blew in one direction, across Japan, then across the Pacific, to the West Coast and Hawaii, the number of U.S. cases increased. When the wind blew in the opposite direction, the number of cases fell.
The data were so overwhelming that Burns and her team thought it couldn't be coincidental. If this theory about Kawasaki disease is true, it could open up a whole new understanding of how other human pathogens are spread. No human disease has ever been shown to cross an ocean by wind and still remain infectious.
Meanwhile, Leo Kogan recovered fairly quickly after being pumped with a blood product made up of antibodies, a process that his mother says was terrifying.
"I don't think I have been traumatized by anything as much as I was traumatized by having a child with Kawasaki disease," she says. "What is extremely difficult about having a kid with Kawasaki disease, before the diagnosis, is just the not knowing."
Leo is now a healthy 5-year-old. While Kawasaki disease is still rare, the number of cases both in the U.S. and Japan have been rising steadily each year.
GUY RAZ, HOST:
To another story about scientific research also inconclusive and very much theoretical but if proved true could revolutionize our understanding of how human pathogens are spread. First, to the mysterious disease in question.
DEBORAH KOGAN: So Mother's Day last year, my son, who was 4 at the time, woke up with kind of a rash and a fever.
RAZ: At first, Deborah Kogan figured it would pass. But as the hours ticked by, her son, Leo's, fever became worse. So she took him to the pediatrician who figured it was strep.
KOGAN: The next day, he woke up, and he was puffy. Not really puffy but slightly puffy, and it sort of looked like he had the mumps.
RAZ: The strep test result was negative. But by Tuesday morning...
KOGAN: He looked as if he were one of the characters on the "Nutty Professor." His face had ballooned to about twice its normal size.
RAZ: Deborah called up her doctor, made an appointment. But in the meantime, she posted photos of Leo onto her Facebook page, and a crowd-sourced diagnosis began to take shape.
KOGAN: Within 10 minutes of posting that photo, a friend of mine called me on the phone. She said, look...
RAZ: She told Deborah to take Leo to the hospital immediately, and then the flood of Facebook posts began. Her cousin Emily wrote...
KOGAN: Look, I really think you should check and make sure it's not Kawasaki disease. I hope the doctors have ruled out Kawasaki disease.
RAZ: On and on it went.
KOGAN: Within an hour, I was hearing the word Kawasaki disease. I had never heard of it before.
RAZ: Deborah never bothered going back to the doctor's office.
KOGAN: I picked up my kid, I ran to the hospital nearest us. We live in Harlem, so I went up to 165th to the Columbia Presbyterian Children's Hospital with an excellent rheumatology department.
RAZ: Kawasaki disease was first identified by a Japanese pediatrician Tomisaku Kawasaki in 1967. It's not contagious, and it's still not clear how it spreads. It mainly affects children under the age of 4.
DR. JANE BURNS: It wasn't until 1970 when the Japanese Ministry of Health did an inquiry.
RAZ: That's Jane Burns. She's a foremost expert on Kawasaki disease based at the University of California at San Diego.
They realized that there were children dying following this disease, and they were dying from heart attacks.
Which, in children, is extremely rare. In the U.S., about 4,500 children are infected with the disease each year. And a few years ago, Jane Burns started to notice a pattern, a seasonality to the cases she was diagnosing.
BURNS: That meant that in January, February, March, we were going to be very busy, and then in September and October, we knew that that was a good time to take our vacation.
RAZ: Japanese researchers noticed a similar pattern. So together, they worked with a group of climate scientists who started to analyze atmospheric data.
What the climate scientists found when they began sifting through all of the satellite data and the atmospheric data - these are things like temperature, precipitation, surface ocean temperature - what leapt out of the computer at them was an association with the direction of the wind circulating in the troposphere, so these are heights of 3,000 meters, and that these large-scale wind currents blew in one direction across Japan, then across the Pacific Ocean to Hawaii and the West Coast of the U.S. when the cases were increased, and it blew in the opposite direction when there was a fall in the number of cases.
The data was so overwhelming that Burns and her team thought it couldn't be coincidental. Kawasaki is far more common in Asia than it is in the U.S. In Japan, for example, one out of every 150 kids is infected. So with this new data, Burns published a controversial theory last November in the journal Scientific Reports.
She suggested that the disease may be carried by wind currents across the ocean inside tiny dust particles. If that theory is true, it could open up a whole new understanding into how other human pathogens are spread. No human disease has ever been shown to cross an ocean by wind and still remain infectious.
In Deborah Kogan's case, the doctor examining her son, Leo, wasn't sure whether it was indeed Kawasaki disease.
KOGAN: She just said: Look, we're going to treat him for Kawasaki disease because he's really sick. And hopefully, by tomorrow, he'll be better.
RAZ: Leo did recover fairly quickly after being pumped with a blood product made up of antibodies, but it was terrifying.
KOGAN: I don't think I've been traumatized by anything as much as I was traumatized by having a child with Kawasaki disease, the big question mark hanging in the air for so long, the hours and hours it took to figure out what was wrong with him. Kawasaki disease is so rare that most doctors have never seen it. I think that's what is extremely difficult about having a kid with Kawasaki disease before the diagnosis is just the not knowing.
RAZ: Leo is now a healthy 5-year-old. And while Kawasaki disease is still rare, the number of cases, both in the U.S. and in Japan, have been rising steadily each year.
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