A Fever That Was Frightening

No one seemed to know what was wrong.

One night, Licameli recalled, he sat down at his home computer and launched a thorough search to see what he could find. He unearthed 15 articles written more than a dozen years ago describing a syndrome called PFAPA, an acronym for its hallmark symptoms: periodic fever, aphthous (mouth) ulcers, pharyngitis (sore throat) and adenitis, or enlarged and often infected lymph nodes. The condition was also known as Marshall syndrome, named after the doctor who first described it in 1987.

Licameli said he was immediately struck by how similar the descriptions were to whatever was ailing Claire: The children in the articles were younger than 5, had abruptly developed high fevers that lasted four or five days every four to six weeks or so, along with a sore throat and enlarged lymph nodes in their necks. Some developed small ulcers in their mouths as well. Over-the-counter pain relievers had been useless in reducing the fever, and the children seemed normal between episodes and had no underlying illnesses that would account for the fevers.

One Italian study involved six children, four of whom had been cured of the recurrent fevers after their tonsils and in some cases adenoids were removed -- although the tissue showed no signs of infection.

"That's my bread and butter," said Licameli of tonsillectomy, one of the most common surgical procedures performed on children. Intrigued, he proposed performing the operation on selected patients who had been referred to Children's for recurrent fevers.

The hospital's institutional review board, a panel that monitors the safety of research studies, agreed, and Licamelis decided that Claire should be among the study's patients.

"I felt a little desperate at that point, and I had no idea whether she would be cured," said her father, whose colleague operated on Claire.

At 19 months, she was the youngest patient in the study, which involved 27 children. In 26 cases, including Claire's, the fevers stopped and have not recurred.

Although surgery appears to be a cure for many, no one knows why. The prevailing theory, according to Licameli, is that children who develop recurrent fevers may have a hypersensitive immune system and that the fevers represent an exaggerated response to an immunological trigger, possibly a chronic and as yet undetectable infection of the tonsils.

Licameli has treated 60 children with the disorder, some from as far away as California, nearly all of whom have been cured. He said he thinks the disorder may be more common than believed, because many physicians may see only one or two cases in their careers. In the past few years, teams at other hospitals across the country have reported success treating PFAPA with tonsillectomy.

"I think the syndrome is pretty well accepted," Licameli said, "but the treatment is not yet mainstream because people don't know about it."

"I used to think, 'Oh, geez, why my kid?' " he recalled. "Now I think things happen for a reason."

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