Medical Mysteries

A Fever That Was Frightening

Toddler Fell Ill Every Three Weeks

By Sandra G. Boodman
Special to the Washington Post
Tuesday, June 24, 2008; Page HE01

Nighttime, Greg Licameli remembers, was always the worst.

Those were the hours when whatever was wrong with his baby daughter Claire seemed most terrifying, when alternating doses of Motrin and Tylenol did nothing to quell the fever that would spike as high as 106 degrees and when the surgeon and his wife, a nurse, would tersely confer about whether to pack their toddler in ice to try to stave off another seizure that would require a sprint to the emergency room.

For months, these nights occurred every three weeks or so like clockwork, preceded by 24 hours of dread, waiting for the onset of the mysterious fever that lasted four or five days. The prelude was always the same: A day before the fever, Claire would turn lethargic, and the small lymph nodes in her neck would become enlarged. Once when an episode was imminent, the family arrived at the Atlanta airport to discover that their flight home to Boston had been canceled.

"It was definitely frightening," Licameli said of the episodes that baffled at least half a dozen eminent pediatric specialists. "This was certainly outside of my scope of training."

Licameli did not know it at the time, but it was his training as an ear, nose and throat surgeon that ultimately helped him figure out how to help his daughter, now 6, and other children whose little-known malady has only recently been identified. Earlier this year Licameli, an attending physician at Boston's Children's Hospital, and his colleagues published a study in the Archives of Otolaryngology detailing the results of the treatment Claire and 26 other children underwent that cured all but one of them.

"I think it's very ironic that the cure is something I do frequently," Licameli said.

Before the mysterious cyclical fevers began, Claire, a healthy baby and the second of the couple's two children, had experienced the usual illnesses. When she suffered the initial episode, shortly after her first birthday, her pediatrician said a bad virus had probably caused her temperature to escalate from 101 to 105 degrees in less than an hour and had made her throat so sore it caused her to drool. After five long days, Claire recovered.

But every three weeks, it seemed, brought a repeat of the first episode. It was clear this was no ordinary virus -- or maybe not a virus at all, said Licameli, an assistant professor of otolaryngology at Harvard Medical School. Doctors prescribed antibiotics, thinking the problem might be bacterial, but they had no effect.

"She saw everyone I knew," her father recalled, in an attempt to figure out what was wrong. After Claire's fever got so high she had a seizure and was rushed to the ER, doctors performed an MRI to rule out a brain tumor or other lesion. They found nothing.

An infectious disease specialist thought the problem might be cyclic neutropenia, a rare immune disorder in which the white blood cell count drops dramatically every 21 days or so; testing ruled that out.

A pediatric rheumatologist suspected juvenile rheumatoid arthritis, another dead end. Licameli consulted experts at the National Institutes of Health to see whether his daughter might have a genetic fever syndrome. She didn't.

"One of the things that struck us was the periodicity of the fever," Licameli said. The only thing that seemed to reduce it was a dose of prednisone, which eliminated the fever in a number of hours, for reasons that remain unclear. But the corticosteroid, which reduces inflammation, had another, less desirable effect: It caused the fever to return in 11 days, rather than the usual 22.

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