Experts on Creutzfeldt-Jakob disease, a rare but fatal brain disease, say there hasnt been a documented case of transmission from contaminated surgical instruments since the 1970s, when sterilization techniques were more primitive.
But there is still a risk. And thats what Novant Health officials are now explaining to 18 patients and their families after apologizing Monday for exposing those patients to surgical instruments that had been used on a CJD patient at Forsyth Medical Center in Winston-Salem.
Theres no excuse for this. It should never have happened, said Florence Kranitz, president of the Creutzfeldt-Jakob Disease Foundation in New York. I know the hospital is apologizing. People are always tragically sorry for what happens.
But Kranitz said the same thing happened three months ago at a New Hampshire hospital. And 19 other similar incidents have been reported to the Centers for Disease Control and Prevention since 1998.
Theyre doing brain surgery on very sick people, Kranitz said. It stands to reason that you would keep those instruments isolated until you know what youre dealing with.
In a statement Monday, Forsyth President Jeff Lindsay said Novant has taken appropriate steps to prevent any future occurrence. Forsyth followed guidelines from the Association for the Advancement of Medical Instrumentation, the statement said. But an immediate change has been made so that at all Novant hospitals, all instruments used in brain surgery cases (will) go through an enhanced sterilization process.
Dr. Jim Lederer, Novants infectious disease specialist, was not available for comment Tuesday. Jeanne Mayer, spokeswoman for Novants Winston-Salem market, said he was speaking in person and on the telephone to affected patients and families to make sure they have the correct information and understand what happened.
On Monday, Lindsay and Lederer explained that a patient who had brain surgery Jan. 18 was later diagnosed with CJD, which affects about 1 in 1 million people each year worldwide. Although the instruments used in that operation were sterilized by the typical method, they were not subjected to enhanced sterilization procedures that should be used after treating a patient with CJD.
In the past three weeks, 18 patients who had brain surgery at Forsyth were exposed to the instruments that had been used on the patient with CJD.
No treatment or cure for CJD
The majority of CJD cases are sporadic, meaning the cause is unknown. About 10 percent are inherited. Only 1 percent to 2 percent of CJD cases are acquired through contaminated instruments or other exposure to brain tissue.
CJD is often confused with mad cow disease, which occurs only in cows. But there is a type of the disease, called variant CJD, that occurs in humans who consume contaminated beef.
Classic CJD can be similar in symptoms to other progressive neurological diseases, such as Alzheimers disease or Huntingtons disease. But CJD causes unique changes in brain tissue that can be seen at autopsy. There is no treatment or cure.
At Carolinas HealthCare System, if there is a suspicion that a patient may have CJD, no matter how small, instruments used in brain surgery are quarantined until the diagnosis is confirmed or ruled out, said Katie Passaretti, medical director of infection prevention. If its positive, then we throw the instruments out. They would get incinerated, typically.
She said CHS is developing a surgical checklist that would include a concern about CJD that should be discussed and checked off before every brain surgery.
Low risk, long incubation
Dr. Zack Moore, medical epidemiologist for the North Carolina Department of Health and Human Services, said Novant officials notified the state about the CJD exposure Friday night after they had figured out they had a problem. Weve been in pretty regular contact with them. Theyve taken a very proactive stance.
Moore said the likelihood of CJD transmission from surgical instruments is minimal.
Theres never been a case of CJD transmission due to reuse of neurosurgical instruments in this country, ever. It has happened elsewhere in the world, but only four cases, and those were in the 1970s.
Im not trying to minimize how worrisome this must be for the patients, but the risk, from what we know, appears to be small, Moore said.
He plans to use this as a teachable moment, to re-emphasize to North Carolina hospitals and doctors the importance of following CDC guidelines.
If youre doing a neurosurgical procedure where the diagnosis is unknown, you should either quarantine those instruments until you have a diagnosis or treat them as if they were contaminated with CJD, Moore said.
Kranitz, whose husband died of CJD 13 years ago, said the news about Novants patients made her sad and angry. Im sure the hospital feels pretty much the same way.
What makes the exposure so tragic is that its a long incubation period, Kranitz said. The patients will live with uncertainty for years. She said she has two colleagues in Australia who, for different reasons, received hormone injections that they later learned were contaminated with CJD. Thirty years later, neither has symptoms.
Its just a tiny sliver of hope. Not everyone thats exposed gets the disease, Kranitz said. I just hope that these people who were operated on (in Winston-Salem) will live long and healthy lives.
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