Fighting for his life : Rexburg teen contracts little-known disease

By PHIL DAVIDSON
14 sept 2008--Justin Smith, 18, smiles at a joke while recovering from Lemierre's syndrome at Eastern Idaho Regional Medical Center on Friday. Smith went to see a doctor a couple of weeks ago because of a sore throat when it was discovered he had the extremely rare and sometimes fatal disease. He is expected to make a full recovery - Amanda Smith / asmith@postregister.com
Justin Smith felt a tingle in his throat a few weeks ago, but was sure the discomfort wouldn't disrupt his move to Portland, Ore.
The 2008 Madison High School graduate felt destined to uproot to the Beaver State metropolis. A sore throat wasn't going to delay his dream.
But that inflammation wouldn't go away, and Smith's health gradually deteriorated. His mother, a nurse, decided he should see the family doctor.
Things worsened from there, and on Aug. 22, Smith was taken to the emergency room at Eastern Idaho Regional Medical Center.
Doctors discovered he was suffering from an extremely rare disease, Lemierre's syndrome. He was cooped up at the hospital, near death, for more than two weeks.
Smith was just released Saturday.
Portland's on the back-burner for right now, but Smith is recuperating and expected to make a full recovery.
The aspiring novelist and his mother, Elaina, want to share their story in the hopes of educating the public about Lemierre's, which the medical field has dubbed the "forgotten disease."
"I just feel very compelled to educate people about this," Elaina Smith said. "My son is one of the lucky ones who survived."
Justin Smith is sure that if he contracted Lemierre's 100 years ago, he'd be dead.
"I don't know why I'm still alive," he said.
Forgotten disease
Save for a broken arm, Smith's had a relatively clean bill of health growing up in Rexburg. The 18-year-old ran cross-country and wrestled for a period in high school.
And Lemierre's is extremely rare. A Danish study reported an annual occurrence of 3.6 cases per million.
Named after Dr. Andre Lemierre in 1936, the syndrome is caused by Fusobacterium necrophorum, a bacterium doctors say is commonly found in people's mouths.
Knowledge of Lemierre's is still limited. Some people develop severe infections from the bacteria while others don't.
Smith is in that rare, former group. And the bacteria took a mighty toll on him.
Within a week of first noticing that throat tingle, Smith developed kidney failure, bone marrow failure, congestive heart failure, liver impairment and pneumonia in both lungs.
Elaina Smith said doctors diagnosed her son with pneumonia at first, but she credited Dr. Jeffrey Stieglitz, an emergency room physician, for realizing the ailments were much worse.
"I've never seen (Lemierre's) before, but I've seen what it's not," Stieglitz said. "You know it's something else."
Smith had a golf ball-sized abscess on his neck and clotting in his jugular vein; Stieglitz knew his symptoms were that of an unusual disease.
Stieglitz was happy to hear Smith is expected to make a full recovery, but he's still perplexed as to why Smith contracted Lemierre's.
"I don't think we know why some people get such severe reactions to common bacteria," he said. "That's just a puzzle to modern medicine."
Stieglitz didn't diagnose Smith; Dr. Richard Nathan of Idaho Falls Infectious Diseases drew that task.
Nathan, who could not be reached Monday for this story, knew of five other Lemierre's cases in Pocatello in the past six months, Elaina Smith said.
Efforts to confirm those figures with the Southeastern District Health Department were unsuccessful, but Smith believes those numbers indicate the disease may not be as uncommon as some people think.
"This is real," she said. "The public needs to be educated about this terrible infection that kills young people in one to two weeks' time."
Research
Lemierre's isn't currently a U.S. Centers for Disease Control and Prevention-reportable disease, but Dr. Robert Centor, a professor of internal medicine at the University of Alabama-Birmingham and a renowned expert on sore throats, hopes to change that.
Centor is trying to get articles published on the rare disease in medical journals and maintains a regularly updated blog featuring related threads at www.medrants.com.
"Nobody's paying attention to (Lemierre's)," he said. "I've become a champion for this disease."
With Lemierre's, which was a relatively common illness in the early 20th century, Centor said the infection inside the jugular classically metastasizes to brain and lung infections.
His research found that the disease typically presents itself in adolescents and young adults.
Extrapolating data from the Denmark study, he figures there are between 500 and 1,000 cases of Lemierre's annually in the United States, 10 percent of which are fatal. It strikes approximately one in 70,000 adolescents, he surmises.
But the thing about Lemierre's, which Centor said many doctors know nothing about, is that it's treatable if detected early.
Smith's elixir, as his mother put it, was "good old-fashioned" penicillin.
Centor believes the disease can be controlled if patients can be diagnosed much earlier.
"It's a rare disease that kills adolescents that shouldn't kill adolescents," he said. "That's what makes my blood boil."
Portland
Smith, who was bed-ridden for 12 days and a mute for 10 (thanks to intubation tubes), is expected to make a complete recovery, his mother said.
He's still plans on attending Portland State University to study creative writing once he moves there and establishes residency for a year. But he's uncertain when he'll take off; he still has at least four weeks of daily antibiotics.
If there's a silver lining to his harrowing past few weeks, it's that he had the immediate support of his mother and family instead of being alone in Portland with a potentially fatal disease.
The well-read teen, who sports horn-rimmed glasses and sideburns akin to Neil Young's, also said the experience has taught him how fragile life is.
"I realized how much I took for granted before," he said.
Reporter Phil Davidson can be reached at 542-6750.
Did you know?
Dr. Robert Centor, a professor of internal medicine researching Lemierre's syndrome and an expert on pharyngitis, said parents shouldn't be alarmed if all of a sudden their children have a sore throat.
However, he said adolescents with sore throats should be better in three to five days. If they develop a fever, neck swelling or other complications after that time period, Centor recommends seeking medical care.