CJD Creutzfeldt-Jakob Disease – Mayo Clinic – See Video
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N.H. patients possibly exposed to fatal brain disease
By Kay Lazar and Chelsea Conaboy Globe Staff September 05, 2013
Eight patients at a New Hampshire hospital may have been exposed to a rare, fatal brain disease from surgery equipment that was used on a patient who likely had the incurable disease, state health officials said Wednesday.
The patients underwent brain surgery at Catholic Medical Center in Manchester between May and August and have been notified of their potential exposure, the officials said during a news conference.
Other equipment used in the surgery on the patient who likely had the disease, which was rented from Minneapolis-based Medtronic, may also have been used on five patients in unnamed other states before health officials realized the instruments were possibly contaminated with tissue from the initial patient. That person had undergone surgery in May, but only last month was it discovered that the patient had symptoms of Creutzfeldt-Jakob disease.
Standard methods for sterilizing surgical equipment before every operation do not protect against the rare disease, specialists said.
Health officials said the risk to the potentially exposed patients is believed to be extremely low.
Video: 8 possibly exposed to disease
“But after extensive expert discussion, we could not conclude that there was no risk, so we are taking the step of notifying the patients and providing them with as much information as we can,” said Dr. José Montero, New Hampshire’s director of public health. “Our sympathies are with all of the patients and their families, as this may be a confusing and difficult situation.”
These patients could be living with a gnawing uncertainty for a long time because the disease, which eats away the brain, can take years to develop and there is no way to know whether they will get sick.
‘Very small residues can even survive being passed through incinerators.’
The eight patients range in age from their mid-30s to mid-80s, officials said, but they declined to provide any other identifying information, citing patient privacy laws.
Dr. Joseph Pepe, Catholic Medical Center’s chief executive, said the patients took the news “very well. I don’t believe there were people who were angry or extremely emotionally upset.” He said patients would be provided counseling and other support.
A similar incident happened in 2004 at Emory University Hospital in Atlanta, where staffers notified 98 brain and spine surgery patients that they may have been exposed to the rare disease after one surgery patient was diagnosed with Creutzfeldt-Jakob.
New Hampshire health officials said there have been only a handful of Creutzfeldt-Jakob cases around the world thought to be transmitted to other patients through contaminated surgical instruments, and none documented in medical journals since 1976.
Creutzfeldt-Jakob disease is unlike the vast majority of infectious diseases because it is not caused by bacteria, a virus, or a fungus, but by abnormal proteins called prions. About 200 cases a year are reported in the United States. (Mad cow disease is another illness caused by prions.)
Byron Caughey, who researches prion diseases as a senior investigator at the National Institutes of Health, said the disease is “really impervious to typical attempts to destroy it.”
Detergents and other sterilization methods, such as using an autoclave, a device that uses high pressure steam, “just aren’t enough to completely decontaminate this material or remove it, especially from stainless steel instrumentation,” he said. “Very small residues can even survive being passed through incinerators.”
Bleaching for long periods of time can work, he said, but that can damage sensitive instruments, and other effective chemicals can leave dangerous residue or otherwise harm the tools.
Because the disease is “such a rarity, it’s just not practical” to take the extra steps to eradicate prions from surgical equipment, Pepe said. “No hospital throws out their instruments after each and every surgery,” he said.
The hospital’s standard method for cleaning surgical equipment is a five-step process that includes ultrasound, washing with detergents and enzymes, and sterilization in an autoclave.
Tests are being conducted on tissue samples taken after the patient died last month. Those tests are not due back for about a month. Creutzfeldt-Jakob disease can be definitively diagnosed only after death.
While the patient was alive, the hospital did test the spinal fluid, and results came back suggesting it was Creutzfeldt-Jakob disease. Those results, combined with the rapid onset of dementia symptoms, led hospital leaders to conclude with “90 to 95 percent certainty” that the patient had the disease, officials said.
But analyzing spinal fluid of the other eight patients for the abnormal protein would not be conclusive, said Dr. Raef Fahmy, Catholic Medical Center’s chief medical officer.
“You would have to have advanced disease, showing symptoms, for that spinal fluid to be positive,” he said.
Creutzfeldt-Jakob disease is marked by rapid mental deterioration that resembles dementia. Most people eventually lapse into a coma.
The incubation period for the disease, the time between when patients were exposed and when they start experiencing symptoms, can be anywhere from a year to several decades, health officials said.
“Once symptoms appear, the average time to death is about four months,” said Pepe, the hospital’s chief executive. “There is no treatment, there is no cure.”
Health officials said there is no risk to the families of the patients who may have been exposed, nor to other patients at the hospital. The disease cannot be transmitted through the air or through touching or most other forms of casual contact, according to the National Institutes of Health.
Medtronic declined to make executives available to comment but issued a statement saying it had recently been notified by the hospital about the case.
“Upon notification that our instruments had been used in this case, we followed procedures to quickly track that specific set of instruments and confirmed they were used in five additional cases,” it said. “We are assisting the hospitals and the appropriate state health authorities as they manage this situation. We have also notified the US Food and Drug Administration.”
The company declined to say where the five other patients lived.
Kay Lazar can be reached at firstname.lastname@example.org. Follow her on Twitter @GlobeKayLazar.
Correction: Because of incomplete information provided to the Globe, an earlier version of this story imprecisely described how eight patients at Catholic Medical Center in Manchester, N.H., were potentially exposed to a rare brain disease. A hospital-owned instrument previously used on a patient who likely had Creutzfeldt-Jacob disease was used in the eight patients’ surgeries, not a device rented from Medtronic.