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NH Hospital Warns Patients of Possible Exposure to Fatal Brain Disease
09/05/2013   Reported By: Patty B. Wight

Thirteen people in multiple states may have been exposed to a rare, degenerative brain disease that was found at a New Hampshire hospital. Catholic Medical Center in Manchester announced yesterday that a patient treated at the hospital was later diagnosed with what's likely a case of Creutzfeldt -Jakob Disease, or CJD. Patty Wight tells us more about the disease, how patients were exposed, and the level of risk they face.

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In May, doctors at Catholic Medical Center performed brain surgery on an elderly patient to remove a brain cyst. The surgery was successful. But in August, the patient returned to the hospital with rapidly progressing dementia. Doctors performed tests and discovered the patient likely had Creutzfeldt-Jakob Disease, or CJD.

The chances of developing CJD, says the hosptial's CEO Dr. Joseph Pepe, are literally one in a million. "Once symptoms appear, however, the average time to death is about four months," he says. "There is no treatment. There is no cure. And there is no screening test."

The patient died soon after the test results came back. Doctors believe the patient had sporadic CJD, which means it developed spontaneously, for no known reason. This accounts for about 85 percent of CJD cases. Most others are hereditary. Less than one percent OF CASES are acquired, and of that, a tiny number, says Dr. Pepe, are from surgical instruments that come into contact with brain tissue or spinal fluid contaminated with with CJD that then touch another person's brain tissue or spinal fluid.

That's exactly what happened at Catholic Medical Center. After the initial patient's brain surgery, those same instruments were used on eight other hosptial patients.

"When you think about how rare the disease is, and the fact that this patient had a brain surgery, and we didn't know that this person had CJD - when you look at those odds, it is phenomenal," Dr. Pepe says.

The hospital has notified the exposed patients, and sent a tissue sample from the initial patient to confirm they indeed had CJD. But there's another wrinkle to the situation, says New Hampshire's Director of Public Health Dr. Jose Montero.

"And it's that in this particular surgery, a piece of equipment that was loaned by a private company was used," Dr. Montero asys. "And after that, that piece of equipment was sent outside New Hampshire and used in other states and different hospitals."

Montero says up to five patients in other states have also been exposed to CJD, but declined to specify which states because the investigation is ongoing. Both the loaned equipment and hospital equipment are in quarantine, and will be disposed of if test results are positive for CJD.

The hospital followed standard sterilization practices, which is a multi-step process that includes using ultrasound, detergents and enzymes, and high pressure steam. But the problem is, CJD particles can withstand that rigorous process.

The only way to get rid of it is to use strong chemicals like bleach, which, Dr. Pepe says, ruins the instruments. "And then they wouldn't be able to be used on other patients. So it's such a rarity to go through that, it's just not practical," he says.

Dr. Pepe says CJD is so rare, that even if it turns out that the elderly patient did have the disease, the chances that other exposed patients will develop CJD is close to zero.

"There have only been four cases in the world literature of this type of transfer - four cases with surgical instruments," he says. "And those four cases have been all before 1976. All four of those cases were outside of the United States."

But the chance, no matter how slight, still exists. And the incubation period - the time from when someone contracts the disease to the time they exhibit symptoms, like tremors, dementia, or low cognitive function, can be from one year to several decades.

Doctors say the exposed patients, who range from the mid-30s to the 80s, have handled the news relatively well, and each has a dedicated hospital navigator for support. Final results on whether this is a confirmed case of Creutzfeldt -Jakob Disease are expected in 4 to 5 weeks.

We had reporting help with this story from Todd Bookman, of New Hampshire Public Radio.

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