Medical Mysteries:

Changing hospitals

On Jan. 2, an ear, nose and throat specialist pulled her aside. " 'If it were me, I'd get her out of here,' " he advised, adding that her daughter's illness "has exceeded the capacity of this hospital."

Aud, who had reached the same conclusion, wanted Shannon to go to either the University of Virginia Medical Center in Charlottesville, reasonably close to their home, or Georgetown University Medical Center, not far from her office.

But when she broached the idea to the doctor in charge of Shannon's case, he bristled. "If you want to transfer her," Aud recalls him saying, "you'll have to find a bed and a doctor who'll take her."

Unnerved, Aud picked up the phone and called her uncle, a plastic surgeon in central Illinois. An hour later, the arrangements had been made: Shannon was headed for Georgetown, where she was admitted to the pediatric intensive care unit.

Doctors took a detailed history and began running tests, and the now familiar specialists appeared. Several hours later, Susan Aud recalls, one of the junior doctors arrived with news of a tentative diagnosis.

Infectious-disease specialist Charlotte Barbey-Morel strongly suspected she had Lemierre disease, also known as Lemierre syndrome, an extremely rare complication of a throat infection that tends to affect otherwise healthy teenagers and young adults. Dubbed the "forgotten disease" -- it was once common and killed 90 percent of its victims -- Lemierre's was virtually eradicated by penicillin.

The disease, named after French physician Andre Lemierre, who described 20 cases in 1936, is caused by Fusobacterium necrophorum, which thrives without oxygen. It develops after a severe sore throat typically caused by strep creates a peritonsillar abscess, a crater near the tonsils that fills with bacteria and pus. The bacteria then invade the jugular vein in the neck, causing an infected clot to form. The clot can break up and travel through the bloodstream, causing pneumonia, widespread infection, even death.

Barbey-Morel said she suspected Shannon had Lemierre's because of two telltale symptoms: a jugular infection and a blood clot. Blood cultures and CT scans confirmed her suspicion. There was another, more personal reason, she later told Susan Aud: When her son was 16, he had a similar infection, which was caught and treated earlier.

A resurgence of interest

Barbey-Morel said she is not surprised that other doctors were stumped. Most have never seen a case and may not even have heard of the disease because it is so rare: The incidence is estimated to be one in one million. After decades in which the disease never appeared at Georgetown, she said, a case surfaced about 10 years ago. Now doctors there see at least one annually.

Georgetown's experience is not unique, and there appears to be a resurgence of interest in the disease. Twenty studies have been published in medical journals this year, among them a report by doctors at Howard University Hospital of a 9-year-old boy who developed Lemierre's after an ear infection. In August, researchers in England suggested that the "apparent increase . . . may be due to antibiotic resistance or changes in antibiotic prescription patterns."

Because Shannon's infection was too entrenched to be treated solely by drugs, she underwent surgery to remove her tonsils and a salivary gland and to clean out dead tissue in her jaw. She also required treatment for pneumonia, the result of tiny clots that had settled in her lungs.

"Shannon was very sick, but she was very brave," Barbey-Morel recalled.

She was discharged in mid-January, weighing 95 pounds, 20 pounds less than normal, and remained on IV antibiotics for weeks and a blood thinner for months. It was clear that returning to college would have to wait.

For two months, she was too weak to climb stairs. "I did not look like myself for a long time," she said.

The illness changed her. "I used to be really carefree," she said. "Being in the hospital, there was a feeling of despair I'd never felt before." She returned to Virginia Tech in August.

Barbey-Morel said that as rare as Lemierre's is, recurrences are rarer still. "But I suspect it won't take much for doctors to treat her sore throat immediately for the first year or two," she said.

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