OGDEN — There's a chance some patients who had brain or spinal surgery at McKay-Dee Hospital were exposed to a rare, fatal brain disease. But the risk of "catching" Creutzfeldt-Jakob disease from surgical instruments is extremely remote, state and national health experts say.
Still, the hospital sent a letter outlining that risk to about 155 of the patients who had neurosurgery at the hospital last year after it was confirmed that a patient who underwent brain surgery in April died with CJD in November. He had no symptoms of the disease at the time of the brain surgery, said Dr. Richard C. Arbogast, medical director of the hospital.
The hospital first learned he had symptoms that might be CJD, including progressive confusion and dementia in October, said Arbogast. But the disease can only be confirmed by a brain biopsy after death. After his death, the sample was sent to the national prion center at Case Western in Cleveland, which confirmed CJD, although the subtype is not known.
CJD is an extremely rare human prion disease affecting one in 1 million people. Utah did not have a case in 2003 or 2004, according to Susan Mottice, an epidemiologist with the Utah Department of Health. She said that 85 percent of CJD cases are "sporadic" with no known risk factors. The other 15 percent are "familial," with a genetic predisposition. CJD is always fatal.
The man died with the classic, sporadic form of CJD, not a "variant" form like the so-called "mad cow" disease, the best-known of the protein prion diseases. Other well-known prion diseases include scrapie in sheep, bovine spongiform encephalopathy in cows and chronic wasting disease in deer.
There are about 250 to 300 cases of sporadic CJD every year in the United States, according to the Centers for Disease Control and Prevention. Most CJD patients die within a year of diagnosis.
Symptoms may start with mood swings and depression but rapidly progress to dementia, behavioral changes, poor coordination and vision problems, then coma and death.
Once they received confirmation that the patient did die of CJD, hospital officials debated whether to notify patients. The CDC and the state health department both told McKay-Dee officials they were not required to inform patients because the risk is so low, said Clark Jensen, hospital spokesman. The hospital said there is "absolutely no risk of exposure" for patients who had any other types of surgery or treatment.
"You could make a case that by telling them about something they are so unlikely to get, you're needlessly frightening them. But we could never get it down to a zero chance of transmission, so we decided the right thing to do was to send out the notice," Arbogast said, adding that about seven U.S. hospitals have faced the notify/don't notify dilemma in recent memory. "Half told, half didn't."
There have been a few cases of transmission in surgical procedures, according to the Creutzfeldt-Jakob Disease Foundation. But it typically involves tissue, not instruments. In 25 years, there have been no known cases of transmission of CJD by surgical instrument anywhere in the world, according to the letter the patients received. "And they were much more casual about sterilization 25 years ago than what we are today," Arbogast said.
The letters patients received Tuesday included a hotline number where they can call to talk to hospital staff knowledgeable about CJD and its symptoms. As of Thursday morning, only nine people had called and "most of them have been people saying just let me know what the signs or symptoms are," said Jensen. "There's been absolutely no panic."
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