News release: Colorado - Denver/Boulder

March 29, 2001

For more information, call:
Steve Krizman
Kaiser Permanente
Phone: (303) 344-7932
E-mail: Steve.A.Krizman@kp.org

Answers to questions about Creutzfeldt-Jakob disease

Denver, CO – Media reports this week concerning two Kaiser Permanente patients who died of Creutzfeldt-Jakob disease have raised many questions and concerns. The following points address the most common issues raised:

Creutzfeldt-Jakob disease fact sheet

The physicians and staff of Exempla Saint Joseph Hospital and Kaiser Permanente extend their sympathies to the families of our patients who experienced a loss associated with Creutzfeldt-Jakob disease (CJD). CJD strikes suddenly and without warning, and is truly tragic.

This should not be referred to as "mad cow" disease. The U.S. Department of Agriculture says not a single case of mad cow disease has been reported in the United States. Mad cow disease is the name given to bovine spongiform encephalopathy, which has been linked to a variant of CJD in Britain.

CJD is a rare disease, occurring in one in a million people. Most cases of CJD are referred to as sporadic, meaning that cause of the disease is unknown. Approximately 10 to 15 percent of cases are inherited, and a much smaller percentage may occur as an unintended consequence of a surgical procedure.

Kaiser Permanente's two CJD cases this year are not related. They are sporadic cases, with no known cause. They died of the classic version of the disease, not the variant that has gained so much attention in Great Britain and Europe. Colorado reports two to four CJD deaths a year.

Between November 20 and December 11, there is a remote possibility that six patients may have been exposed to CJD during brain surgery at Exempla Saint Joseph Hospital.

Exempla Saint Joseph Hospital and Kaiser Permanente have contacted six patients who had brain surgery between November 20 and December 11, notifying them that they may have been exposed to the prion protein of CJD. Instruments used in a brain biopsy of a patient who later was diagnosed with CJD were used in these patients' operations. These instruments underwent stringent sterilization after each operation—as with all reusable surgical equipment. But it is not clear whether that sterilization is adequate to destroy the prion protein that can spread CJD.

The possible exposure of the six patients to CJD is extremely unfortunate and something that the physicians and staff of Saint Joseph Hospital and Kaiser Permanente deeply regret. However, we are confident in saying that the possibility of exposure is limited to these specific patients. Other patients who had surgery during this period were not exposed.

The Centers for Disease Control have no reports of any cases where CJD was transmitted through surgical instruments in the past 20 years. Recorded cases occurred prior to 1970, when hospital sterilization procedures were less stringent than today. Saint Joseph Hospital's sterilization procedures are state-of-the-art. We hand-wash the instruments, then soak them in a highly alkaline solution, and then sterilize them under high heat. There has been no scientific study to confirm that this procedure is effective in destroying the prion protein that is believed to cause CJD, but it's similar to the procedures that were proven effective in earlier studies.

The chance of transmitting the disease through brain surgery is minute. Traveling Denver's busy streets is a much greater risk to one's life. We told the patients of this potential exposure so they can evaluate the information for themselves.

Other than the six patients who were contacted, no other patient was potentially exposed. Once CJD was diagnosed, the instruments were immediately quarantined.

We will continue to monitor the affected patients' health. There currently is no laboratory test to conclusively determine whether they have been exposed. It can be years before symptoms develop.

Exempla Saint Joseph Hospital and Kaiser Permanente acted quickly once CJD was suspected. A biopsy of a lesion in the patient's brain was conducted November 20 by a Kaiser Permanente neurosurgeon, who initially did not suspect CJD. The tissue was sent to the Mayo Clinic for analysis. An initial diagnosis of Creutzfeldt-Jakob disease was communicated to the hospital on December 11. Instruments that may have been used in the brain biopsy were immediately put under quarantine and the Colorado Department of Public Health and Environment was notified. In early January, the nation's foremost expert on the disease at Case Western Reserve University confirmed the diagnosis.

Meanwhile, the hospital compiled a list of patients who may have come in contact with the surgical instruments between November 20 and December 11. The Centers for Disease Control was consulted, and we worked closely with the Colorado Department of Public Health and Environment. We researched the medical literature to determine what, if any, chance exists that CJD could have been transmitted through the instruments. Physicians and medical ethicists discussed the benefits and risks of notifying the patients of the extremely small chance of disease transmission.

The patients' neurosurgeons met individually with the patients to discuss the potential exposure. They will continue to talk with them about this and their other health issues along with the patients' primary care physicians.

This was an unusual set of circumstances. The neurosurgeon did not suspect the patient might have CJD when he operated. In fact, the diagnosis wasn't confirmed for several weeks after the operation.

Saint Joseph Hospital has written new operating room guidelines for handling similar surgical cases where the CJD diagnosis is suspected prior to surgery. These new guidelines would not have mattered in this case, however, because CJD was not suspected.