Emory Report
October 4, 2004
Volume 57, Number 7


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October 4 , 2004
Steps taken for patient safety following rare brain diagnosis

BY ron sauder

Emory officials believe Creutzfeldt-Jakob disease (CJD), an extremely rare degenerative disease, is the probable diagnosis of an Emory Hospital patient following a brain biopsy, and are taking steps on the basis of that assumption. Given the rarity of this disease, the definitive test is done by a national laboratory in Cleveland, Ohio. Due to time requirements and the complexity of analysis, the results are not expected for several weeks.

Nevertheless, late last week the hospital was in the process of notifying 98 brain and spine surgery patients of the remote possibility they may have been exposed to the protein that causes CJD, a very rare disease that occurs at the rate of only one person per million per year worldwide.

Emory physicians said potential exposures might have occurred following the Sept. 10 brain biopsy of a patient who later received a preliminary diagnosis of CJD.

After the biopsy, the surgical equipment used was sterilized according to the hospital’s normal procedures, which call for instruments to be cleaned in a solution and heated to 270 degrees Fahrenheit for four minutes in a prevacuum surgical autoclave.

There have been no known cases of CJD transmitted by surgical instruments in the past 28 years since the routine use of these sterilization techniques. However, bodies such as the World Health Organization (WHO) recommend certain enhanced sterilization measures specifically for instruments used in cases of CJD.

As a first precaution, on Sept. 15, following receipt of preliminary biopsy results, all neurosurgical equipment was resterilized according to WHO guidelines for CJD. On Monday, Sept. 27, all hospital surgical equipment was resterilized according to the same enhanced guidelines as a further precaution.

“Although we believe the chances of an exposure are extremely small, we cannot guarantee they are zero. That is why Emory is taking every possible step to deal with this matter,” said Allan Levey, professor and chair of neurology.

William Bornstein, chief quality officer for Emory Healthcare, said, “All sterilized surgical equipment in the hospital has been resterilized using the enhanced procedures recommended for this rare disease. We have also instituted a new policy that exceeds hospital norms and calls for treating every brain biopsy as a potential case of CJD and sterilizing the instruments using the enhanced process, no matter how unlikely CJD may appear at the time.”

Hospital officials have written to 418 non-neurosurgical patients who were operated on in the hospital between Sept. 10–27, describing the events to them and explaining that any risks to non-neurosurgical patients are even lower, if any. There are only six known cases of CJD transmitted by surgical equipment, and all six of these patients had brain procedures. All of those cases occurred in the 1970s—before current standards of sterilization, such as those used by Emory Hospital, were adopted.

CJD is a progressive degenerative disease of the brain, typically presenting as premature dementia and gradual loss of muscular coordination. It is believed to be caused by the accumulation of an abnormal protein, called a prion, found in nerve cells. Sporadic CJD, which has no identified underlying cause, afflicts one case per million people per year worldwide. It is not the disease known popularly as “mad cow disease.”

“It is Emory’s policy to notify patients when we become aware of these types of issues,” Bornstein said. “It is also true, unfortunately, that there is no diagnostic test to determine whether they have been exposed, nor are there any known means of prevention or treatment. We believe the risk is extremely low, but we also believe we have an obligation to share our initial findings with our patients.”

“The well-being of our patients is always our first concern,” said John Fox, president and CEO of Emory Healthcare. “We want to assure our patients we are taking every step possible to ensure quality patient care.”

The Emory Health Connection (404-778-7777) is available for patients who would like more information. Emory also is making physicians available to patients and their families.