Exposure to Creutzfeldt-Jakob Disease

Within the last year, the Joint Commission has received reports of two separate incidents at accredited hospitals where patients--a total of 14--may have been exposed to Creutzfeldt-Jakob Disease through instruments used during brain surgeries. Although CJD is rare--only one in one million people worldwide have been diagnosed with CJD--and iatrogenic transmission of CJD by exposure to central nervous system tissue or spinal fluid of a diseased person is even rarer, these new incidents signal the need for renewed awareness of preventive measures in health care organizations in regards to infection control (Comprehensive Accreditation Manual for Hospitals, IC.1-IC.6.2). This is particularly important since regular sterilization techniques are not yet proven to be effective against the CJD organism.

A transmissible spongiform encephalopathy (TSE), a prion disease, CJD is a degenerative, always fatal brain disorder that typically occurs in older people. A new variant of CJD (vCJD), caused by mad cow disease, occurs in younger people; most reported cases are in Great Britain. This issue of Sentinel Event Alert focuses on classic CJD. CJD is insidious, taking up to 20 or more years for symptoms to appear, with death taking place within five to 14 months after symptoms present.

Lessons learned from atypical patient

Exempla Saint Joseph Hospital in Denver encountered exposure to CJD in 2000 and is encouraging JCAHO to share with other health care organizations information gained from its root cause analysis. A patient who did not present with CJD symptoms underwent brain biopsy to rule out vasculitis. The pathology report confirmed CJD three weeks following the surgery. In the meantime, six patients underwent brain biopsy using the same instruments. The hospital had in place a manual tracking system that helped identify patients operated on with those specific instruments. The hospital then did what it considered the ethical and the right thing to do: it informed the six patients about their possible exposure.

The hospital's root cause analysis encompassed communication, competency of staff, sterilization and use of instruments, and interval between biopsy and pathology report. From its root cause analysis, the hospital came away with three important lessons learned that they want to share with other health care organizations:

• A CJD or prion disease patient does not always present with symptoms of CJD.
• The time interval between biopsy and pathology report should be monitored and reviewed to assure shortest time from biopsy to results.
• Instruments used in brain biopsy procedures should not be reused when the patient's diagnosis is uncertain at the time of the procedure.

Exempla St. Joseph revised its policies and procedures to 1) follow-up on pathology reports within one week, and (2) quarantine instruments used in neurosurgery--including brain biopsies--with unknown diagnoses or uncertain diagnoses until a confirmation can be determined. As the basis for these policies and procedures, the hospital uses the core curriculum from the Association of Professionals in Infection Control and the Centers for Disease Control and Prevention's Issues in Health Care Settings.

Risk factors

The World Health Organization's Infection Control Guidelines for Transmissible Spongiform Encephalopathies recommends a high index of suspicion for patients with confirmed or suspected transmissible spongiform encepalopathies to avoid iatrogenic transmission of CJD or vCJD. While less than 1 percent of identified TSE patients developed the disease from iatrogenic transmission, procedures in which such transmission is known to occur include administration of human cadaver-derived growth or pituitary hormones, cornea transplants, dura mater grafts, or other neurosurgery. At this time, there is no evidence that CJD or vCJD can be transmitted through blood (although research is being conducted) or that CJD can be transmitted by eating infected beef.

Risk reduction strategies

"These are remarkably hardy transmissible agents. Usual disinfection methods—rapid disinfection, boiling, alcohol—are not effective. Health care organizations need to establish a policy for disinfecting or throwing away instruments used in neurological surgery on suspected or confirmed CJD patients."  -- Richard T. Johnson, M.D., consultant to NIH on TSE's and professor of neurology, microbiology and neuroscience, Johns Hopkins University

CJD prions appear to be particularly resilient to regular disinfection techniques. "These are remarkably hardy transmissible agents," says Richard T. Johnson, M.D., consultant to NIH on TSEs and professor of neurology, microbiology and neuroscience at Johns Hopkins University. "Usual disinfection methods--rapid disinfection, boiling, alcohol--are not effective. Health care organizations need to establish a policy for disinfecting or throwing away instruments used in neurological surgery on suspected or confirmed CJD patients." Dr. Johnson recommends high-pressure disinfection in sodium hydroxide for one hour. "Some instruments, like stereotactic electrodes, that won't take the sodium hydroxide will have to be thrown out," says Paul Brown, M.D., senior investigator at the National Institutes of Health in Bethesda, MD, and a career scientist in the field of transmissible spongiform encephalopathies.

Exempla Saint Joseph's risk reduction strategies include the use of disposable instruments in undiagnosed brain biopsy procedures and known CJD cases as much as possible, thereby minimizing the use of reusable instruments in these cases; quarantining instruments used in neurosurgery until the diagnosis can be confirmed; and incinerating instruments used in neurosurgery when CJD is confirmed. The World Health Organization's Infection Control Guidelines for Transmissible Spongiform Encephalopathies also recommend the use of single-use surgical instruments and the destruction of reusable instruments that come into contact with high infectivity tissues.

Recommendations

Joint Commission recommends that organizations establish policies for 1) the disinfection or disposal of instruments used in neurosurgery in general and when CJD is suspected or confirmed, and 2) the quarantine of such surgical instruments until an unclear diagnosis or biopsy is clarified.

Published for Joint Commission accredited organizations and interested health care professionals, Sentinel Event Alert identifies the most frequently occurring sentinel events, describes their common underlying causes, and suggests steps to prevent occurrences in the future.

During the on-site survey of accredited organizations, Joint Commission surveyors assess the organization's familiarity with and use of Sentinel Event Alert information. Organizations are expected to (1) review each Sentinel Event Alert, (2) consider the suggestions, as appropriate to the organization's services, and (3) implement the suggestions, or reasonable alternatives, or provide a reasonable explanation for not implementing relevant changes.

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