Washington Regional postpones Fayetteville surgeries following rare brain disorder discovery

File photo/NWA Democrat-Gazette Washington Regional Medical Center in a 2015 file photo.
File photo/NWA Democrat-Gazette Washington Regional Medical Center in a 2015 file photo.

FAYETTEVILLE -- Washington Regional Medical Center didn't use its operating rooms Wednesday morning after officials learned a patient was preliminarily diagnosed with a rare degenerative brain disease.

Hospital officials learned Tuesday a patient undergoing a diagnostic lumbar puncture Feb. 15 was diagnosed with Creutzfeldt-Jakob Disease by a national laboratory in Chantilly, Va.

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob Disease is a human prion disease. It is rapidly progressive and always fatal neurodegenerative disorder. Infection with this disease leads to death usually within 1 year of onset of illness.

The disease is classified as a transmissible spongiform encephalopathy along with other prion diseases that occur in humans and animals. The most common form of classic Creutzfeldt-Jakob Disease is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions.

This sporadic disease occurs worldwide, including the United States, at a rate of roughly 1 to 1.5 cases per 1 million population per year, although rates of up to two cases per million are not unusual. The risk of the disease increases with age, and in persons over 50 years old, the annual rate is approximately 3.4 cases per million.

Source: U.S. Centers for Disease Control and Prevention

A news release from the Fayetteville hospital said surgical cases resumed Wednesday afternoon after workers thoroughly sterilized and replaced certain surgical instruments, in adherence with recommendations of the World Health Organization.

The disease occurs at the rate of about one person per million each year worldwide and about 300 cases in the United States each year, according to the National Institute of Health. Dr. Gary Wheeler, branch chief of infectious diseases at the Arkansas Department of Health, said there isn't a full explanation for what causes the disorder, it can be traced to abnormal proteins in the brain called prions.

He said there hasn't been a case of Creutzfeldt-Jakob Disease being transmitted in a hospital since 1976, before much was known about the disorder and modern surgical sterilization techniques were adopted by hospitals.

"There is never a zero chance, but the chances of transmitting this disease are really, really tiny," Wheeler said.

Dr. James Newton, Washington Regional infectious disease specialist, also stressed in the release there's an extreme unlikelihood of any other patients being affected and patients, workers and the public are at no risk. He said Washington Regional adopted the recommended sterilization techniques for all operations 40 years ago.

"We continue to seek the best available guidance and information from the CDC, the Arkansas Department of Health and the medical literature," Newton stated. "The extreme rarity of this disease and the nature of the potential exposure, which is so small it cannot be quantified, make it a challenge to find published guidelines that are applicable to our situation. Nevertheless, we are committed to finding and sharing with our patients the best available advice."

Wheeler said the surgical instruments used on the infected patients likely had to undergo more stringent soaking using stronger agents such as undiluted chlorine bleach and additional time in an autoclave.

The hospital is collaborating with the Centers for Disease Control and Prevention and the Arkansas Department of Health to identify and advise patients who received hospital care since the Feb. 15 procedure. Less than 1 percent of cases nationwide are classified as acquired, meaning the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures.

The disease usually appears later in life and progresses rapidly, according to the National Institute of Health. Symptoms typically start around age 60 and about 90 percent of people die within a year.

Early stages of the disease cause failing memory, behavioral changes, lack of coordination and visual disturbances. As the disease progresses, mental deterioration becomes pronounced and involuntary movement, blindness, weakness of the extremities and coma may occur.

Wheeler said testing for Creutzfeldt-Jakob Disease is advancing, and there are some labs that can test spinal fluid. The best way to diagnosis the disease is by a brain biopsy, he said.

Washington Regional stated in its news release the disease wasn't a variant condition known as bovine spongiform encephalopathy, or "mad cow" disease.

Wheeler said he also heard people worried the Washington Regional case could be tied to chronic wasting disease, a similar brain disorder found in deer and elk. He said the disorder has never been transmitted from an animal to a person.

"Hopefully it will stay that way," he said. "Fear of the unknown is what people have to deal with."

NW News on 03/17/2016

Upcoming Events