Prion Contamination Unstoppable

Forsyth Medical Center in Winston-Salem said Monday that up to 18 patients may have been exposed to Creutzfeldt-Jakob disease, a rare but fatal degenerative brain disorder. The center said a neurosurgical procedure was performed on a patient Jan. 18, who was later confirmed to have the disease. Another 18 patients, all neurosurgical patients, were exposed between Jan. 18 and Thursday, said Dr. Jim Lederer, a Novant Health Inc. infectious disease expert.

This CJD exposure in North Carolina this week highlights many of the points made in our book about the mismanagement of all prion diseases, including Alzheimer’s disease and CJD. This should never happen. We have warned against this risk several times. This hospital and the others that have had similar cases of mismanagement are still contaminated. Prions are unstoppable. Plus, North Carolina has other troubling signs as it relates to prion diseases among people and wildlife. We will update and expand upon previous posts on those topics.

The exposure occurred because the specialized surgical equipment used on the patient with the disease was cleaned through a typical sterilization procedure, but did not receive the enhanced sterilization procedures required for Creutzfeldt-Jakob disease.

The disease is caused by a deadly form of protein that can adhere to surgical equipment and withstand standard sterilization. Lederer said patients were identified through tracing when the surgical equipment was used during the 19-day period. Although Lederer said the exposure risk to the other 18 patients “is very low,” he added the hospital “realizes this is devastating news” to have to provide to those patients and their families.

Forsyth spokeswoman Jeanne Mayer said privacy laws “prevent us from talking about a patient’s specific treatment,” including how hospital officials determined that the patient had the disease.

Lederer said there were “reasons to suspect” that the patient had the disease or another brain disease at the time of the surgery. “The extra cautions should have been taken, but were not,” he said.

Jeff Lindsay, the hospital’s president, said “any exposure is simply unacceptable.”

Lederer said the hospital wanted to notify patients and their families first, but it still has not reached all of them as of Monday afternoon. Lederer said another factor is that there are just a handful of U.S. laboratories that test for the disease.

The disease supposedly affects one in 1 million patients worldwide annually, or about 300 Americans a year, and has no known cause or treatment. Symptoms in some instances may not appear for years, if not decades.

However, death typically occurs within a few weeks to four months of symptoms arising, according to Novant and the Creutzfeldt-Jakob Disease Foundation Inc.

Less than 1 percent of cases of Creutzfeldt-Jakob Disease are acquired through iatrogenic and variant exposure.

Among the iatrogenic risks are contaminated surgical instruments, dura mater transplant, corneal transplant and human growth hormone. The variant risks can come from eating contaminated beef or being exposed to contaminated blood or a blood plasma transfusion.

Lederer stressed the patients were not exposed to “Mad Cow disease,” which only occurs in cows.

The last confirmed case of a transmission though surgical instruments occurred in 1976. Novant said that there have been only four confirmed cases of such transmission in the world.

The sporadic version of the disease accounts for 85 percent of the annual cases, including the Forsyth patient who has been identified with the disease. A hereditary version accounts for about 14 percent.

Although Florence Kranitz, president of the Creutzfeldt-Jakob Disease Foundation, said the chances of the 18 exposed patients contracting the disease is low, “it is unsettling when this kind of exposure occurs because it should not happen.”

“The incubation period is typically long, and hopefully none of the other patients will have anything to worry about,” Kranitz said.

A flier on the foundation’s website warns that “confirmation of a case of variant Creutzfeldt-Jakob Disease originating in the U.S. would likely lead to a chain reaction of panic-driven decisions and policymaking.”

Lindsay said Novant is making “all the necessary changes to make sure this does not ever happen again.” That includes all surgical equipment used in brain surgery receiving the enhanced sterilization treatment across all Novant facilities.

The N.C. Department of Health and Human Services said it has been made aware of the patient exposure by the hospital.

“Our primary concern is the health, safety and welfare of patients, and we will continue to closely monitor the situation.” DHHS spokesman Kevin Howell said.

According to DHHS data, there were 22 North Carolinians diagnosed with the disease in 2013, up considerably from an average of 12 cases annually from 2008 to 2012.

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Gary Chandler is a prion expert. He is the CEO of Crossbow Communications, author of several books and producer of documentaries about health and environmental issues around the world. Chandler is connecting the dots to the global surge in neurodegenerative disease, including Alzheimer’s disease, Parkinson’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and other forms of prion disease. The scientific name for prion disease is transmissible spongiform encephalopathy.