Creutzfeldt-Jakob disease (CJD) is one of many neurological diseases known as transmissible spongiform encephalopathies (TSE) caused by a unique class of infectious proteins. When infected with TSE-causing prions, the brain develops tiny holes much like a sponge and the result is fatal. According to the Centers for Disease Control and Prevention (CDC), the incidence of CJD in the United States is approximately 1 case per million population per year.
Prions are highly resistant to conventional physical and chemical cleaning and sterilization. All prion diseases are infectious; however, the risk of infection is not the same for all tissue. Based on experimental transmission in rodents, the risk of infection from tissue types are classified as high, low, or no risk. High risk tissue includes the posterior eye retina or optic nerve, brain, pituitary gland, and spinal cord. Low risk tissue includes the liver, lung, spleen, kidney, and lymph nodes, body fluids, blood, and urine.
Devices exposed to CJD require special precautions and protocols to inactivate the prions. Single-use instruments should be used on high-risk tissue on patients at high risk for prion disease. CJD is ultimately diagnosed by brain biopsy. Single-use brain biopsy sets are commercially available. As an alternative to purchasing disposable instruments, a facility could assemble a set using instruments at the end of their useful life and then discard them after use.
Instruments that cannot be cleaned or require sterilization using low-temperature technologies should not be used or should be discarded if they are exposed to high-risk tissue on patients at high risk for prion disease.
If reusable instruments are used and have come in contact with high-risk tissue from patients known or suspected to have a prion disease they should be treated according to the most current infection prevention guidelines. The 2010 Society for Healthcare Epidemiology of America (SHEA) Guideline for Disinfection and Sterilization of Prion-Contaminated Medical Instruments, recommends cleaning chemicals to be used on instruments that have evidence of prionicidal activity and that are compatible with the instrumentation.
According to SHEA, instruments exposed to prions should be kept moist until they are cleaned and decontaminated. Instruments should be decontaminated in a mechanical washer as soon as possible after use.
After decontamination, one of the following three methods recommended by SHEA should be used to steam sterilize instruments exposed to high-risk tissue:
• Prevacuum sterilizer: 134° C (273° F) for 18 minutes,
• Gravity displacement sterilizer: 132° C (270° F) for 60 minutes, or
• Immerse in 1 N NaOH* for 60 minutes, then remove, rinse in water, and sterilize using one of the cycles noted above. (*1 N NaOH is a solution of 40 grams NaOH in 1 L water)
Healthcare facilities should establish, document, and implement evidence-based policies and procedures to decrease the risk of prion disease transmission. Policies and procedures should be developed by a multidisciplinary team that includes infection preventionists, perioperative RNs, sterile processing personnel, surgeons, representatives from the clinical pathology laboratory, and other involved stakeholders and based on:
• the patient’s risk of having a prion disease,
• the level of infectivity of the tissue involved, as defined by the World Health Organization, and
• the intended use of the medical device.
The references below provide very detailed information that can help guide a healthcare facility in creating an evidence based policy and procedure on processing CJD contaminated patient care instrumentation, equipment and the environment.
• Recommended practices for Cleaning and Care of Surgical Instruments and Powered Equipment, In: Standards, Recommended Practices, and Guidelines. Denver, CO: AORN, Inc; 2014:541-560.
• Recommended Practices for Environmental Cleaning. In: Perioperative Standards and Recommended Practices. Denver, CO: AORN, Inc; 2014:255-276.
• Recommended Practices for Prevention of Transmissible Infections in the Perioperative Practice Setting: In: Perioperative Standards and Recommended Practices. Denver, CO: AORN, Inc; 2014:385-417.
• Annex C Processing CJD-contaminated patient care equipment and environmental surfaces, In: Comprehensive guide to steam sterilization and sterility assurance in health care facilities, ANSI/AAMI ST79:2010 & A1:2010 & A2:2011 & A3:2012 & A4:2013:163-167.
• McDonnell G, Dehen C, Perrin A, et al. Cleaning, disinfection and sterilization of surface prion contamination. J Hosp Infect. 2013;85(4): 268-273. doi:10.1016/j.jhin.2013.08.003; 10.1016/j.jhin.2013.08.003. [IIA]
• Rutala WA, Weber DJ. Guideline for disinfection and sterilization of prion-contaminated medical instruments. Infect Control Hosp Epidemiol. 2010;31(2): 107-117. doi:10.1086/650197. [IVA]Ru)
• Rutala WA, Weber DJ. Creutzfeldt-Jakob disease: recommendations for disinfection and sterilization. Clinical Infectious Diseases. 2001;32(9): 1348-1356