Mass. hospital also used surgical equipment exposed to brain disease

MANCHESTER, N.H.Eight people who may have been exposed to the fatal brain infection Creutzfeldt-Jakob Disease this summer at a New Hampshire hospital have been contacted personally by the hospital's president, who says they aren't panicking.

Dr. Joseph Pepe of Catholic Medical Center called the patients Thursday a day after health officials announced they may have been exposed to Creutzfeldt-Jakob (CJD), an incurable brain disease characterized by rapidly progressive dementia which can cause death within months after symptoms first appear.

One expressed more concern the hospital or its surgeons would be harmed by the publicity over the incident, he said.

"They are all fine at this point, but I let them know that they can not only call my chief medical officer and the patient advocate ... but also myself, and we will stay with them as long as they need us," he said, adding that he apologized for causing them any anxiety.

Officials believe the extremely rare disease caused the August death of a patient who had brain surgery at the hospital in May, although the cause of death won't be certain until more tests are completed. If that patient had CJD, there's a small chance it was transmitted to other brain surgery patients because the abnormal proteins that cause the disease, called prions, can survive standard sterilization practices.

In addition to the eight Catholic Medical Center patients, health officials in Massachusetts said five patients there may have also been exposed because a specialized instrument used on the New Hampshire patient had been rented and reused at Cape Cod Hospital in Hyannis.

The Massachusetts patients have also been notified.

"The CJD risk to the Massachusetts patients is extremely low, as those patients underwent spinal surgery and not brain surgery," the state health department said in a statement.

About one in one million people worldwide develop CJD each year, according to the National Institute of Neurological Diseases and Stroke (NINDS), with about 200 U.S. cases reported annually.

The vast majority of infections occur spontaneously -- what's known as sporadic CJD -- when normal prions transform into disease-causing, abnormal proteins.

In fewer than 1 percent of cases, the disease is transmitted by exposure to brain or nervous system tissue, and there have been only four reported cases of transmission via surgical instruments. None of those were in the U.S., and the most recent case was in 1976, Pepe said.

CJD is often associated with another prion disease found only in animals, Bovine spongiform encephalopathy (BSE), or "mad cow disease."

If a human eats contaminated meat, he or she can contract variant Creutzfeldt-Jakob disease (vCJD), which also rapidly decreases a person's mental health and movement ability.

Some hospitals might opt not to tell patients because of the low risk involved and the anxiety it could create for them, Pepe said, but it was important to keep them informed.

"We felt the risk of that anxiety did not outweigh the ethical principle of letting them know and also preventing them from possibly contaminating or exposing others should they have another brain operation," Pepe said.

The only definitive way to diagnose the disease is through a brain biopsy or autopsy. There are no screening tests, and tests that would point toward a diagnosis of the disease are only effective once symptoms such as memory loss and impaired coordination appear, Pepe said.

The hospital will arrange counseling sessions if any of the patients request them, he said.

"Some may get angry later on, they may have anxiety, and then there are others who do not think anything of it," he said. "One person said, 'You know, I have really many other things more concerning than this.'"