Hospital Reports Rare Case of Creutzfeldt-Jakob Disease


LITTLETON, Colo.Tissue samples from a patient who had brain surgery on Feb. 13, 2006 at Littleton Adventist Hospital have tested positive for a prion disease associated with Creutzfeldt-Jakob disease (CJD), a rare but fatal neurological disorder that affects approximately one in a million people worldwide.

Initial symptoms were not consistent with CJD, and therefore, CJD was not first suspected.  As the patients condition worsened post-surgery, CJD was considered on March 14. The patient died from the disease on March 23, and after an independent autopsy was performed, brain tissue was submitted to Case Western Reserve University in Cleveland, the only prion testing facility in the United States. On May 9, Littleton Adventist Hospital received laboratory results from Case Western Reserve University confirming a diagnosis of CJD. 

Six patients had neurosurgery between Feb. 13 and March 14 involving the brain and/or dura mater, the translucent membrane that covers the brain and spinal cord.  While there is an extremely remote possibility they may have been exposed to the prion protein associated with CJD, the hospital believes that they have a right to know of the possibility of exposure. Physicians have met with each of the six patients individually to discuss the potential exposure. Other than the six patients who were contacted, no other patients or associates at Littleton Adventist Hospital could have been exposed.

Littleton Adventist Hospitals sterilization procedures are state-of the-art; however, these sterilization methods may not have effectively sterilized against the prion that causes CJD, and research has demonstrated a remote possibility that surgical instruments can transmit CJD during a neurosurgical procedure.  The actual chance the disease was transferred to the six patients through surgery is extremely minute.  In fact, there is no documented case in the United States of anyone contracting CJD from surgical instruments.

Once CJD was diagnosed, LittletonAdventistHospital acted quickly and appropriately.  Authorities at the Centers for Disease Control and Prevention (CDC), the Colorado Department of Public Health and Environment (CDPHE) and the Joint Commission on Accreditation of Healthcare Organizations (JCAHO) were notified. The surgical instruments were disinfected using current standards, have been quarantined and are no longer in use at the hospital.  According to the CDC, there have only been four confirmed cases worldwide where CJD was transmitted through surgical instrumentsthe last case occurred in the 1970s, when hospital sterilization processes were significantly less stringent than today.

 The possibility of contracting the disease during surgery is extraordinarily remote.  While the CDC did not feel it was necessary for us to inform the six patients, we feel it is our ethical responsibility to inform them and their family members so that they are aware of the situation, said Lawrence Wood, MD, chief medical officer of Littleton Adventist Hospital. We encourage our patients to take an active role in their health care to help them make more informed medical decisions. Patients trust us as caregivers and deserve to know everything pertaining to their medical care.Unfortunately, no medical test exists to determine whether or not an individual is carrying the disease.

Symptoms associated with the initial stages of the disease can be subtle, with ambiguous symptoms including insomnia, depression, confusion, personality and behavioral changes, strange physical senses and problems with coordination and sight. CJD is difficult to diagnoseoften, the diagnosis happens through a process of elimination of other diseases, including Alzheimers disease. There currently is no laboratory test to conclusively determine whether the patients have been exposed. Symptoms of surgically transmitted CJD typically develop within three years. A definitive diagnosis of CJD only can be confirmed through a biopsy of brain tissue.

We understand this information may be concerning, and were working with the patients and their families, said David Crane, president and chief executive officer of Littleton Adventist Hospital. Were committed to our patients well-being and we have offered to monitor the health of each patient for the long-term.

Since the recent case, Littleton Adventist Hospital has reviewed and updated its infection control policy for handling similar surgical cases when a CJD diagnosis is suspected prior to surgery. The updated policy provides a comprehensive process for screening patients prior to scheduling surgery and a process to ensure that all instruments used in neurological procedures will be marked and tracked.

Source: Littleton Adventist Hospital

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