1. Creutzfeldt-Jacob Disease: A Sentinel Event

2. Presented by SPSmedical Largest sterilizer testing Lab in North America with over 50 sterilizers Develop and market sterility assurance products that offer advanced technologies Provide full day sterilization Seminars and on-site Facility audits for compliance with best practices Corporate member: CSA and AAMI, serving on numerous sterilization working groups

3. Association for the Advancement of Medical Instrumentation Meets in Washington, DC throughout each year and establishes guidelines for sterility assurance which become our American National standards. Membership includes: Health care facilities Health care organizations Government agencies Medical device manufacturers Testing Labs and Consultants AAMI standards are available in text and on CD-ROM.

4. Association for the Advancement of Medical Instrumentation Information on processing CJD-contaminated patient care equipment and environmental surfaces is discussed in Annex C of AAMI ST79:2006, which is our American national standard for Steam sterilization. The purpose of this Annex is to provide general guidance to hospital CS departments reprocessing devices that have been exposed to patients with known or suspected CJD.

5. Objectives After viewing this program, participants will be able to: describe what CJD is and what causes it, identify the four main types of CJD and how this disease is transmitted, understand what recent studies have shown regarding deactivation of prions, list the AAMI recommendations for processing CJD contaminated patient care equipment and environmental surfaces.

6. What is CJD? Creutzfeldt-Jakob disease is a rare, degenerative, invariably fatal brain disorder. It affects about 1 in 1 million people each year worldwide. In the United States, there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90% of patients die within 1 year. In the early stages of the disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

7. What causes CJD? The leading scientific theory is that CJD is caused by a type of protein called a prion. Prions are microscopic particles similar to a virus, but lack nucleic acid. Prions are not living organisms and cannot be "killed" any more than table sugar or rubbing alcohol can be. Although many people use the term "killed" in relation to prions, a more technically correct way to describe their destruction would be to say the prions were "denatured" or "inactivated".

8. Examples of Prion Diseases HumansCJD (Creutzfeldt-Jakob disease) GSS (Gerstmann Sträussler Syndrome) Cattle BSE (Bovine Spongiforme Encephalopathy) SheepScrapies Deer/ElkCWD (Chronic Wasting Disease)

9. Four main types of CJD 1)Sporadic CJD – (spCJD) appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD and accounts for at least 85% of cases. 2)Variant CJD – (vCJD) is also known as “human BSE” or mad cow disease. It was first identified in 1996 and the first case developed symptoms in 1994.

10. Four main types of CJD 3)Genetic CJD – (gCJD) is inherited rather than contracted, so there is usually a family history of the disorder. This form of CJD is very rare. 4)Iatragenic CJD – (iCJD) is acquired through a medical accident. While CJD has been inadvertently transmitted by medical and surgical treatments, these cases are rare with only about 250 reported worldwide.

11. How is CJD transmitted? Variants of CJD may be acquired through: tissue implants, administration of hormones contracted from contaminated human organs, contact with contaminated instruments.

12. How is CJD transmitted? The vast majority of healthcare associated transmission cases have resulted from the use of contaminated tissues or grafts. Iatrogenic CJD (acquired CJD) has occurred during the following circumstances: a. after patients received extracted pituitary hormones (130 cases); b. after patients received an implant of contaminated grafts from humans (cornea - 3 cases, dura mater (fibrous membrane covering the brain and spinal cord -110+ cases);

13. How is CJD transmitted? c. after the use of contaminated medical equipment and surgical instruments (2 confirmed, 4 unconfirmed cases). It is important to point out, that all six cases associated with neurosurgical instruments occurred in Europe over 25 years ago and the details of the reprocessing procedures are incomplete.

14. How is CJD transmitted? All known instances of acquired CJD resulted from exposure to infectious brain, pituitary, or eye tissue. Studies have confirmed the highest prion concentration are brain and dura mater. Transmissibility is directly related to the concentration of prions in tissues. Until recently, there have been no known episodes of CJD associated with blood transfusion.

15. How is CJD transmitted? Recent discovery in UK of 3 individuals infected with CJD from contaminated blood transfusions American Red Cross currently gathering blood samples from CJD patients and family members – building repository of blood for research to check for presence of pre-clinical diagnostic markers Developing methods to detect prions in blood products – key to protecting nation’s blood supply

16. What do the studies show? Several studies have been published showing inactivation of prions by disinfection and sterilization processes; however, these studies have not reflected the reprocessing procedures in a clinical setting. In disinfection studies reviewed by Rutala and Weber, chlorine provided the most consistent prion inactivation results; however, the corrosive nature of chlorine makes it unsuitable for many devices, i.e. surgical instruments and endoscopes.

17. What do the studies show? In steam sterilization studies reviewed by Rutala and Weber, the recommendation of 134°C for 18 minutes (prevacuum) and 132°C for 60 minutes gravity, have shown to provide significant (but not complete) reduction of infectivity under worst case conditions. However, a 2003 report in the UK published by the Advisory Committee on Dangerous Pathogens and Spongiform Encephalopathy, indicated there are no known sterilization procedures completely effective against prions or prion-related diseases.

18. Sentinel Event Alert June 2001, Issue 20 JCAHO recommends that organizations develop and establish policies and strategies for: (1) the decontamination and/or disposal of instruments used in neurosurgery, particularly when CJD is suspected or confirmed; and (2) quarantining neurosurgical instruments until a diagnosis of CJD can be confirmed or ruled out.

19. A suspect or diagnosed case of CJD is a “Sentinel Event” - report to JCAHO Any time a sentinel event occurs, the health care organization is expected to complete a thorough and credible root cause analysis, implement improvements to reduce risk, and monitor the effectiveness of those improvements. The root cause analysis is expected to drill down to underlying organization systems and processes that can be altered to reduce the likelihood of error in the future and to protect patients from harm when error does occur.

20. AAMI ST79:2006 Recommends the following for devices and equipment contaminated with high-risk tissues (defined as brain, spinal cord and eye tissue) from high risk patients known or suspected of CJD: 1) Devices that are constructed so that cleaning procedures result in effective tissue removal (e.g. surgical instruments) can be cleaned and then steam sterilized at 134°C for greater than or equal to 18 minutes in a pre-vacuum sterilizer or at 121°C to 132°C for 1 hour in a gravity displacement sterilizer.

21. 2) Devices that are impossible or difficult to clean can be discarded. Alternatively, the contaminated device can be placed in a container filled with a liquid (e.g. saline, water or phenolic solution) to retard adherence of material to the medical devices, then initially decontaminated by steam sterilizing it at 134°C for 18 minutes in a pre-vacuum sterilizer (liquids must be removed before device is sterilized) or at 121°C to 132°C for 1 hour in a gravity displacement sterilizer or by soaking in 1N NaOH for 1 hour. The device is then cleaned, wrapped, and terminally sterilized by conventional means. AAMI ST79:2006

22. 3) To minimize drying of tissues and body fluids on the object, devices should be kept moist until cleaned and decontaminated. 4) Flash sterilization should not be used for reprocessing these devices. 5) Contaminated items that have been in contact with high risk tissue and have not been processed according to these recommendations (e.g. medical devices used for brain biopsy prior to diagnosis) should be recalled and appropriately reprocessed. AAMI ST79:2006

23. 6) A tracking system should be in place that permits recall of devices used on high risk tissue and high risk patients. This tracking system should permit identification of the patient on which the devices were used, the date they were used, the procedure performed, and the surgeon’s name. Facilities that do not have a commercially available or automated system should create a manual system. A simply system can be created using a steam sterilizable two part card, with an external CI that is affixed to the outside of instrument trays. When the tray is used, the bottom part of the card is removed and affixed to the patient’s chart to identify all items used on the patient.

24. AAMI ST79:2006 To ensure accurate tracking of sets and devices, all items should be given a unique number. For example, if the facility has four craniotomy trays, they should be numbered #1, #2, #3 and #4 to identify the specific tray used on the patient.

25. AAMI ST79:2006 7) Environmental surfaces (noncritical) contaminated with high risk tissues (e.g. laboratory surfaces in contact with the brain tissue of a person infected with CJD) should be cleaned with a detergent and then spot decontaminated with 5,000 ppm sodium hypochlorite. The concentration usually results from a 1/10 dilution of household bleach. However, the label should be checked for the amount of sodium hypochlorite present; concentrations in U.S. products can range from 3% to more than 6% sodium hypochlorite.

26. 8) Noncritical equipment contaminated with high risk tissue should be cleaned and then disinfected with 5,000 ppm hypochlorite or 1 N NaOH, depending on material compatibility. All contaminated surfaces must be exposed to the disinfectant. 9) Equipment that requires special prion reprocessing should be tagged after use. Clinicians and reprocessing technicians should be thoroughly trained on the proper tagging of equipment and on special prion reprocessing protocols. 10) Use of power drills or saws that are likely to contact high risk tissue should be avoided. Power drills and saws by their very nature and design are difficult to clean and too expensive to discard.

27. AAMI recommends the following for devices and equipment contaminated with low-risk tissues (defined as cerebrospinal fluid, kidney, liver, spleen, lung, and lymph node tissue) from high risk patients: 1)Devices can be cleaned and disinfected or sterilized using conventional protocols of high level disinfection, thermal sterilization, or chemical sterilization. 2)Environmental surfaces contaminated with low risk tissues require only standard disinfection using disinfectants recommended by OSHA for decontaminating blood contaminated surfaces (e.g. 500 to 5,000 ppm sodium hypochlorite).

28. AAMI recommends the following for devices and equipment contaminated with no-risk tissues (defined as peripheral nerve tissue, intestinal tissue, bone marrow, blood, leukocytes, serum, thyroid gland tissue, adrenal gland tissue, heart tissue, skeletal muscle, adipose tissue, gingiva, prostate tissue, testicular tissue, placental tissue, tears, nasal mucus, saliva, sputum, urine, feces, semen, vaginal secretions, milk) from high risk patients: 1)Devices can be cleaned and disinfected or sterilized using conventional protocols of high level disinfection, thermal sterilization, or chemical sterilization.

29. 2)Endoscopes (except neurosurgical endoscopes) are likely to be contaminated only with no risk materials; hence, standard cleaning and high level disinfection protocols are adequate for reprocessing. 3)Environmental surfaces contaminated with no risk tissues or fluids require only standard disinfection suing disinfectants recommended by OSHA for decontaminating blood contaminated surfaces (e.g. 500 to 5,000 ppm sodium hypochlorite). ANSI/AAMI ST79:2006 Comprehensive guide to steam sterilization in health care facilities. Pp 127-131.

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31. May 2001 The Hotel-Dieu Hospital (Windsor, Ontario Canada) temporarily closed it’s operating rooms fearing a possible outbreak as two months earlier neurosurgeons had operated on a patient who was determined to likely have the disease. The same set of instruments may have been used on several other patients. Recent Prion Events in Canada

32. November 2006 London Health Services in Toronto, Canada cancelled all surgeries for 3 days (73 elective surgeries) over concerns surgical instruments may have been contaminated with CJD. All surgical instruments were recalled as they were unable to identify which subsequent patients instruments were used on. Recent Prion Events in Canada Terry Robb, Manager Sterile Processing presentation at 2008 IAHCSMM conference

33. Recent Prion Events in Canada August 2006, another mad cow diseased animal identified in Alberta, Canada –Dairy cow between 8 & 10 years of age –Exposure likely occurred before or during introduction of new feed regulations that were supposed to stop the spread of the disease Y-T-D 8 confirmed cases of vCJD in cattle

34. USA Cattle Industry $27 billion annually Beef - largest single item traded in the world First reported case of Mad Cow was in Washington state 12/03 (originated in Canada) -Trade borders closed to Canada -Japan closed trade borders to USA beef 2 nd reported case in Texas 6/05 3 rd reported case in Alabama 3/06

35. October 2000 Tulane University Hospital (New Orleans, LA) reported that eight (8) patients might have been Infected with CJD. Once again, they reused some of the same instruments on those 8 patients that had been used previously on a CJD patient from 7 months earlier. Recent Prion Events in USA

36. St. Joseph Hospital (Denver, CO) announced that six (6) patients might have been infected with CJD. The instruments used on them were the same that were used in November 2000 on a patient who died a few weeks later, and was CJD diagnosed with a postmortem brain biopsy. Recent Prion Events in USA

37. April 22, 2002 The Florida Dept. of Health and the CDC announced they are investigating a likely case of a 22-year-old citizen of the United Kingdom living in Florida. Later confirmed, this is the first case of vCJD reported in a USA resident. The CDC said of the 125 vCJD patients worldwide, almost all had multiple-year exposures in the U.K. between 1980 -1996 during the occurrence of a large outbreak among cattle there. Recent Prion Events in USA

38. June, 2003 The NJ Department of Health and Senior Services (NJDHSS) and CDC were notified of a suspected cluster of deaths caused by CJD in persons reportedly linked to Garden State Racetrack in Cherry Hill, NJ. Concerns were raised that these deaths might have resulted from consumption of meat contaminated with the agent causing bovine spongiform encephalopathy (BSE, commonly called "mad cow disease") served at racetrack restaurants during 1988-1992. Recent Prion Events in USA

39. December, 2003 The U.S. Department of Agriculture (USDA) made a preliminary diagnosis of BSE in a dairy cow in WA state. This diagnosis was confirmed by a reference lab in England. A second BSE case was documented in mid-2005 and again in 2006. The occurrence of BSE in the USA reinforces the need for physicians to be aware of the clinical features of vCJD and to arrange for brain autopsies in all decedents with suspected or probable CJD to assess the neuropathology of these patients. Recent Prion Events in USA

40. CJD Reportable States 36 states require reporting of suspect CJD New York is a reportable state Not reportable in Montana, California, Nevada, New Mexico, Iowa, Illinois, Indiana, Kansas, Kentucky, Louisiana, Alabama, West Virginia, Vermont, Delaware.

41. April 27, 2005 ALBANY, NY - The New York State Department of Environmental Conservation (DEC) announced it has received a preliminary positive result for chronic wasting disease (CWD) in a wild deer sampled in Oneida County. This deer, along with another deer nearby was confirmed and are the first known occurrence of CWD in the wild in New York State. www.dec.ny.gov Recent Prion Events in USA

42. March 2006 A woman filed a lawsuit against Emory Healthcare in Atlanta, because she had surgery there with instruments that had been exposed to CJD. Emory University denies the allegations. The woman does not know if she has the disease but worries she will develop it. In October 2004, Emory University Hospital sent warning letters to 500+ surgical patients after an unnamed patient had a brain biopsy, and preliminary testing revealed that person had CJD. Emory alerted 98 patients who had brain and spine surgery and another 418 surgical patients who had other kinds of procedures. Emory University officials said they are aware of no CJD cases among the alerted patients.

43. Recent Prion Events in USA April 2008 - 22 yr old woman in Virginia with vCJD February 2008 - 42 yr old woman in Illinois August 2007 - 57 yr old woman in Illinois July 2007 - 70 yr old woman in New York May 2007 - 50 yr old woman in Illinois June 2006 - 60 yr old woman in Florida

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46. References & Resources Association for the Advancement of Medical Instrumentation 1110 North Glebe Road, Suite 220, Arlington, VA 22201-4795 703-525-4890 Fax: 703-276-0793 www.aami.org Association of periOperative Registered Nurses 2170 South Parker Road, Suite 300 Denver, CO 80231-5711 800-755-2676 www.aorn.org Canadian Standards Association 5060 Spectrum Way Mississauga, Ontario L4W 5N6 CANADA 800-463-6727 Fax: (416) 747-2510 www.csa.ca Certification Board for Sterile Processing & Distribution 2 Industrial Park, Suite 3 Alpha, NJ 08865 908-454-9555 www.sterileprocessing.org International Assoc. of Healthcare Central Service Materiel Management 213 W. Institute Place, Suite 307 Chicago, IL 60610 312-440-0078 Fax: 312-440-9474 www.iahcsmm.org