CJD nightmare’ leads to routine high sterilization

A staggering 3,600 patients notified

The aftermath of one undiagnosed case of Cruztfeld-Jakob disease (CJD) was so traumatizing across the board that a hospital decided to adopt extremely rigorous sterilization procedures on a permanent basis.

"Potential CJD exposures after an unsuspected case can have an enormous emotional and financial impact," said Carlene Muto, MD, hospital epidemiologist at the University of Pittsburgh Medical Center. "Routine use of extended processing can be implemented to [prevent] unintentional CJD exposures. Other large institutions, I think, in Europe especially, might consider the extended processing in light of increasing cases of prion diseases."

The unusual decision was reported recently in Washington, DC, at the annual meeting of the Society for Healthcare Epidemiology of America (SHEA).1 CJD is an extremely rare, progressive degenerative brain disorder caused by a prion protein. Most cases are sporadic or genetic. However, direct inoculation can cause disease and 260 nosocomial transmissions have been reported worldwide. The vast majority of those have been linked to contaminated hormones, dura mater, and corneal grafts, but transmission has occurred on surgical instruments used on successive patients.

"[CJD] is really resistant to traditional methods of chemical and physical decontamination, so what we have to do is extend the [sterilization] cycles," she said.

Potentially exposed patients face considerable anxiety because there is no testing to determine exposure, the incubation period can be decades, and the disease is universally fatal. Yet somehow, 3,600 patients had to be told they may have been exposed to a fatal disease on surgical instruments at the hospital.

The "nightmare" began in March 2002, when an autopsy was performed on a patient who had undergone a craniectomy 11 months earlier. Operating room instruments used in the latter procedure had been processed in typical fashion, steam sterilization at 270° F for four minutes. Autopsy pathology showed evidence of widespread spongiform changes, and CJD was confirmed on testing.

"I really felt strongly the risk of transmission was minimal," Muto told SHEA attendees. "The patient’s brain was not penetrated during the surgery. The transmission of CJD via surgery after the case, therefore, was extremely unlikely. But there was a lot of [exposed patients]."

All patients potentially exposed to the same surgical instruments used on the patient were notified via letters.

Full disclosure was made to the media, and the hospital set up a hotline to answer patient questions and decrease anxiety. To date, there has been no documented transmission related to the case, but as noted earlier, the incubation period for CJD can be considerable. As a result of the case, the decision was made to routinely process all OR instruments at 274° F for 18 minutes to routinely kill CJD on instruments.

"The reason we are doing this is to preempt another unrecognized exposure, [which] was very costly," she said. "It is not that much more costly for us to sterilize at the extended cycle. You have to make sure you have enough instruments because it is 18 minutes vs. four [minutes]. But there are no increases in labor costs or anything like that to do this extended [sterilization]. We have been doing it for almost a year now, and it is very well supported."


1. Pokrywka MF, Andro R, Bingham P, et al. Creutzfeldt-Jakob disease (CJD): Impact of an unsuspected case. Abstract 245. Presented at the annual meeting of the Society for Healthcare Epidemiology of America. Washington, DC; April 2003.