Q&A about Possible CJD Exposure at Forsyth Medical Center

February 14th, 2014

Since posting the story about how 18 patients at Forsyth Medical Center in Winston-Salem, NC, were possibly exposed to the rare but deadly Creutzfeldt-Jakob Disease (CJD), we’ve had folks ask for more information. While there has been no additional information provided by the hospital, we’ve put together this list of answers to common questions:

I’ve never heard of Creutzfeld-Jakob Disease—what is it?

CJD is an incurable brain disease that causes rapidly progressive dementia, leading to death within months. It is rare, but deadly.

According to the Mayo Clinic, initial signs and symptoms of CJD include personality changes, depression, memory loss, impaired thinking, blurred vision, insomnia, difficulty speaking and swallowing, and having sudden, jerky movements. The symptoms all get worse as the disease progresses. Most people eventually lapse into a coma.

It can take years for the symptoms to develop once the disease is present, but death usually occurs within a year of diagnosis. The only definitive test for CJD is examination of brain tissue, which requires brain surgery. If CJD is suspected, doctors may use magnetic resonance imaging (MRI) to view pictures of the brain, electroencephalograms (EEG) to measure the brain’s electrical activity, or spinal fluid tests (the so-called spinal tap) to look for a particular protein marker.

How do you get CJD?

CJD is caused when mutated prions spread throughout a person’s brain. Sometimes the abnormal prions come from the outside (such as if you eat beef from a cow infected with Mad Cow disease), and sometimes it happens when normal prion proteins spontaneously mutate and spread within the brain.

What are prions?

Prions are proteins that are commonly found in living things—including people. Problems arise when one of these proteins mutates into a form that causes a disease. One mutated prion wouldn’t cause disease, but unfortunately, the new, mutated prion acts as a kind of template encouraging other proteins to become mutated. That’s how the disease “spreads.”


Is this the same as Mad Cow Disease?

It’s not the same, but it is similar. Mad Cow and CJD are both caused by mutant prions, and they cause the same symptoms. A person can get the human form of Mad Cow disease by eating infected beef. The person at Forsyth Medical Center had a form of CJD that started in his own body, not by eating infected meat.

How do you kill a prion?

Prions aren’t “alive” the way viruses or bacteria are alive. They are pure protein. So to get rid of the risk from mutated prions, you don’t “kill” them; you “inactivate” them.

Prions are very, very difficult to inactivate. If you simply bury mutated prions (such as burying the carcass of a cow with mad cow disease), the prions will live on in the soil and could possibly leach into the groundwater. What about burning? Experiments have shown that prions can remain active even after being exposed to temperatures of 1100 to 1800 degrees Fahrenheit. Radiation doesn’t inactivate them. Freeze them in ice, and they will still be active when unfrozen.

The proper way to sterilize surgical instruments used on patients with CJD is very complex (see information from the Centers for Disease Control and Prevention (CDC) here: http://1.usa.gov/1nzhwnD). It involved immersing the instruments in a pan of chemicals, then heating them in a gravity displacement autoclave before continuing with additional sterilization.

How were patients at Forsyth Medical Center exposed to CJD?

According to the announcement by the hospital, doctors operated on a patient who was suspected (and later confirmed) to have CJD. After the surgery, the medical instruments used went through standard sterilization. If CJD is suspected, the Centers for Disease Control and Prevention (CDC) recommends that medical instruments either get disposed of (and incinerated), or go through a more extensive sterilization that inactivates prions.

Because the medical instruments only went through standard sterilization, any remaining prions would be capable of infecting anyone who received surgery using those same instruments. According to the hospital, 18 patients had neurosurgery with the improperly sterilized medical instruments, so they were potentially exposed to CJD prions.

Has this ever happened in North Carolina before?

As far as we know, this exact problem hasn’t occurred before. However, in 2004, Duke University Medical Centers improperly sterilized surgical instruments, exposing hundreds of patients to disease and toxic fluid. In that case, instead of standard sterilization with detergent, the detergent was negligently replaced with elevator hydraulic fluid that had been drained from a parking deck elevator during maintenance. HensonFuerst Attorneys in Raleigh represented dozens of those patients who had physical problems resulting from that sterilization problem.