INFECTION CONTROL POLICIES & PROCEDURES Title: CREUTZFELDT-JAKOB DISEASE- PRECAUTIONS

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Section: Infection Control Policies & Procedures Effective Date: 9/04 Revision Date: 10/12 Approved by: Health System-Wide Infection Control Committee Department-specific
PURPOSE

Review Date: 10/12 Non-Clinical Clinical

To provide guidance to conduct a Creutzfeldt-Jakob Disease (CJD) risk assessment, identify patients who should be suspected of having CJD* and determine recommended measures to prevent the transmission of CJD. *Patients suspected of having CJD include: those with undiagnosed, unusual, progressive neurological disease consistent with CJD (sporadic, familial and iatrogenic including Gerstmann-Straussler-Scheinker Disease [GSS] and Fatal familial insomnia [FFI]) members of families with familial CJD, GSS and FFI, with symptoms of neurological disease consistent with CJD recipients of human dura mater grafts, corneal grafts, human pituitary hormones(growth hormones) or who have undergone neurosurgical procedures prior to 1980 or in countries not applying appropriate control measures those with rapidly progressing dementia BACKGROUND

Creutzfeld-Jacob Disease (CJD), Gerstmann-Straussler-Scheinker syndrome, Kuru, and diseases caused by prions are rare (incidence estimated 1: 1,000,000 population per year) fatal illnesses characterized by rapid, progressive dementia, sensory disturbances, myoclonus, and an often typical electronencephalogram (EEG). The causative agent(s) is/are thought to be prions, which are resistant to a number of standard sterilization and disinfection procedures. The exact mode of transmission in humans is not known. Definitive diagnosis involves histological examination of the affected brain tissue. Iatrogenic transmission of CJD has been associated with percutaneous exposure to medical instruments contaminated with prior CNS (Central Nervous System) tissue residues, with transplantation of CNS and corneal tissues and treatment with human growth hormone and gonadotropin. Transmission of CJD has not been associated with environmental contamination or person-to-person spread via skin contact. The incubation period can vary from months to decades but once symptoms develop, the disorder is usually fatal within one year. There are no effective vaccines, no completely reliable and validated laboratory test for detecting infection and no specific therapy for prion diseases.

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Although most episodes of CJD are sporadic, a small number (~10%) are familial and may be related to genetic predisposition. Eighty percent of sporadic cases are diagnosed in patients between the ages of 50-70 years. A previously unrecognized form of CJD has been identified in Great Britain and has raised the possibility that this variant (vCJD) form may somehow be related to the increase of bovine spongiform encephalopathy (BSE) in that country. Although isolated episodes of CJD have occurred in healthcare workers, the incidence of CJD in this group does not exceed what would be expected by chance alone. Prions in general are resistant to a number of standard disinfection and sterilization procedures. Based on the epidemiology of iatrogenic or occupationally acquired episodes of CJD, the only exposures in patient care settings that have resulted in infection are those instances involving devices which cannot be cleaned and which are contaminated with high infectivity tissue.
DEFINITIONS OF INFECTIVITY Risk of CJD Transmission in persons with CJD LEVEL OF INFECTIVITY/Risk of Infection TISSUES, SECRETIONS, and EXCRETIONS High Infectivity Brain, spinal cord, eye* (including optic nerve, retina, sclera, and cornea), dura mater*, pituitary hormone* Low Infectivity CSF**, kidney, liver, lung, lymph nodes, spleen, No demonstrable Infectivity Adipose tissue, adrenal gland, gingival tissue, heart muscle, intestine, peripheral nerve, prostate, skeletal muscle, testis, thyroid gland, feces, milk, nasal mucous, saliva, semen, vaginal secretions, serous exudate, sweat, tears, urine, blood, bone marrow, placenta

Note: Assignment of different organs and tissues to categories of high and low infectivity is chiefly based upon the frequency with which infectivity has been demonstrable, rather than upon quantitative assays of the level of infectivity, for which data are incomplete. Experimental data include primates inoculated with tissues from human cases of CJD, but have been supplemented in some categories by data obtained from naturally occurring animal transmissible spongiform encephalopathy (TSEs). Actual infectivity titers in the various human tissues other than the brain are extremely limited, but data from experimentally infected animals generally corroborate the grouping shown in the table.

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*Believed to be of lower risk based on transmission data since implementation of appropriate control measures in 1980 ** Although CSF is classified as low infectivity and is less infectious than high infectivity tissues it is recommended that instruments contaminated by CSF should be handled in the same manner as those in contact high infectivity tissues in high risk patients.
EVALUATING CJD TRANSMISSION RISK IN HEALTHCARE ENVIRONMENTS

Determining the level of risk for transmission of CJD and the appropriate measures to prevent transmission from patients to other individuals is based on at least three considerations: The probability that an individual has or will develop CJD The level of infectivity in tissues or fluids of these individuals The nature or route/device of the potential or actual exposure to these tissues Based on these considerations a level of risk (high, medium, low) should be determined. Appropriate staff education should be reinforced and applicable infection control measures implemented i.e. Standard Precautions vs. Standard Precautions with additional special precautions e.g. special signage or disposal processes.
Patient risk level Tissue Infectivity Device risk level Disinfection/sterilization

High risk patient High risk patient Low risk patient High risk patient

High risk tissue Low/no risk tissue High risk tissue High risk tissue

Critical/semi-critical Critical/semi-critical Critical/semi-critical Non-critical

Special prion reprocessing See Section 3.5.9 Conventional disinfection and sterilization Conventional disinfection and sterilization Conventional disinfection and sterilization

POLICY Before admission to the hospital, the attending surgeon/physician will determine the level of risk for CJD transmission and notify Nursing Administration, Neurosurgery Manager, Infectious Disease Fellow and Infection Prevention and Clinical Epidemiology unit that a patient with a confirmed diagnosis or suspected of having CJD * is being admitted to the hospital. This notification and determination of level of transmission risk must be made to assure that the appropriate actions are taken to decrease the likelihood of inadvertent transmission of CJD. Surgical procedures involving high infectivity tissues of patients who are diagnosed with or suspected* of having CJD should only be done after a thorough transmission risk assessment and must be carefully planned. This includes determining the

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process of the procedure, instrument handling, storage, cleaning and decontamination or disposal. All laboratory specimens (i.e. lumbar punctures, surgical pathology, etc.) from suspect cases should be clearly labeled as such and laboratory notification should be provided to assure appropriate handling and processing. Consider using disposable surgical instruments on patients known or suspected of having CJD. Reusable devices/instruments contaminated with high infectivity tissues from patients known or suspected of having CJD, during all brain, spinal cord, eye and surgical procedures will be quarantined until a diagnosis is confirmed. Prior to an autopsy: Morgue and mortuary personnel must be notified and provided with appropriate training and equipment.

PROCEDURE 1. Use Standard Precautions for providing care to patients diagnosed with or suspected to have CJD for all normal social and clinical contacts and for noninvasive clinical investigations (see Supportive Data). 2. Special precautions are not required for patient care items (e.g. feeding tubes, suction tubes) and eating utensils. 3. A private room is not required. 4. Routine environmental surface cleaning is adequate in the normal patient care areas. 5. Linen should be handled according to routine linen policies unless visibly contaminated with high infectivity tissues i.e. OR linen. Visibly contaminated linen should be contained, clearly labeled and incinerated. 6. Additional environmental surface cleaning is needed when high infectivity tissue is visibly present: 6.1 Remove visible residues of high infectivity tissue with a disposable cloth soaked in undiluted chlorine bleach prior to cleaning. 6.2 Used cloths will be bagged, clearly labeled and incinerated 7. Collecting/Handling Specimens 7.1 Use Standard Precautions for collecting, handling and processing of blood, urine, body fluids/tissues and fecal specimens. 7.2 Health care workers should wear single-use personal protective equipment (PPE) when collecting and handling high infectivity tissues. 7.3 Bag, label and incinerate any clothing contaminated with high infectivity tissue through the waste hauler vendor. 7.4 Collect high infectivity tissues in a sealable, disposable container and clearly labeled as high risk for CJD. A sealed container and closed centrifuge bucket must be used when mixing or centrifuging is to be done. Specimen and container disposal will be by incineration through the waste hauler vendor .

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8. Use single-use disposable instruments when performing intracranial monitoring. After use, disposable instruments should be collected in a sealable container, clearly labeled and incinerated through the waste hauler vendor. MEDICAL PROCEDURES Although endoscopy is a low-risk procedure it should be avoided whenever possible on patients diagnosed with or highly suspected of having CJD. If endoscopy is necessary patients diagnosed with or highly suspected of having CJD should be scheduled at the end of the procedure day to allow for environmental cleaning and standard instrument pre-cleaning and high-level disinfection. Disposable internal EEG and intra-cerebral electrodes should be used on a patient diagnosed with or suspected of having CJD. Such electrodes should be discarded and disposed of by incineration through the waste hauler vendor (Do not use needle
electrodes). Non contact air or puff tonometers that totally avoid contact with the cornea should be used whenever possible. Tonometers, suction cups used in laser procedures, and other

instruments that come into direct contact with corneas of patients diagnosed with or highly suspected of having CJD should be discarded and incinerated through the waste hauler vendor . Childbirth should be managed using Standard Precautions. However, the placenta and other products of conception from a patient diagnosed with CJD should be disposed of as pathological waste and not used for any other purpose. Standard/Universal precautions and current Peri-Operative policies and procedures should be followed for all surgical and diagnostic (i.e. lumbar punctures) procedures involving tissues with low infectivity or no detectable infectivity on patients with a diagnosis of or suspected of having CJD.
BRAIN BIOPSY PROCEDURES FOR PATIENTS KNOWN OR HIGHLY SUSPECTED OF CJD

1. All Neurology/neurosurgery/brain biopsy procedures: 1.1 Limit supplies and equipment to only what is necessary for the procedure. 1.2 Contain all devices required for brain biopsy procedures in one clearly marked tray i.e. "Brain Biopsy Procedure". 2. Sterile processing will identify instruments and instrument trays by the documentation of the date, sterilizer number, and load number of the exposure to steam sterilization process. This information will be logged on a daily Steam Load Sheet kept in SPD. .

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3. At the completion of the procedure, Operating Room (OR) personnel will place patient identification labels on the plastic bag, rigid container and used instrument container identified below. These labels will also contain the identification number of the Brain Biopsy Procedure tray or other Neurosurgical instrument trays used in that procedure. 4. Operating Room personnel will segregate contaminated devices as follows: 4.1 All disposable devices contaminated with high infectivity tissue will be placed in a plastic autoclave bag marked Brain Biopsy Quarantine, disposable devices. 4.2 All disposable sharp devices contaminated with high infectivity tissue will be placed in a rigid container marked "Brain Biopsy /Neurosurgery Quarantine, disposable sharps". 4.3 All non-disposable devices contaminated with high infectivity tissue will be placed in a used instrument container labeled "Brain Biopsy /neurosurgery Quarantine, used instruments". 4.4 All disposable devices contaminated with low/no detectable infectivity tissues will be disposed of, or contained for transport to the Sterile Processing Department using standard procedures. 5. On receipt of brain biopsy devices, Sterile Processing personnel will: 5.1 Place the plastic autoclave bag marked "Brain Biopsy /neurosurgery Quarantine, disposable devices." and the rigid container marked "Brain Biopsy Quarantine, disposable sharps" in the designated secured quarantine area. 5.2 Fill the used instrument container marked "Brain Biopsy Quarantine, used instruments" with enzymatic solution, secure the lid and place it in the designated secured quarantine area. 6. The Pathology Department will send a copy of the biopsy report to the Infection Control Coordinator and Sterile Processing Manager for appropriate follow-up. 6.1 The identification number of the Brain Biopsy Procedure Tray indicated on the patient identification labels on the quarantined containers will be transferred to this report. 6.2 Copies will be retained within the Sterile Processing and Infection Control departments. 7. For terminal care of instruments: 7.1 if the biopsy results rule out CJD, the quarantined devices will be disposed of or reprocessed in the standard manner. 7.2 If the biopsy results are positive or suggestive of CJD, all devices in quarantine will be incinerated through the waste hauler vendor. 7.2.1 When incineration is not possible, Sterile Processing will do the following: Those devices constructed so that cleaning procedures result in effective tissue removal can be cleaned in the normal manner and then sterilized by autoclaving at: o a. 134 C for >18 minutes in a prevacuum sterilizer, or o o b. 121 C 132 C for 1 hour in a gravity displacement sterilizer

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7.2.2 Those devices that are difficult or impossible to clean may be discarded by incineration. Alternatively, devices should be placed in a container filled with saline, water or phenolic solution to retard adherence of material to the medical device. Devices should then be decontaminated by one of the methods listed below, INITIAL DECONTAMINATION METHODS: o a. 134 C for >18 minutes in a prevacuum sterilizer, or o o b. 121 C-132 C for 1 hour in a gravity displacement sterilizer, or c. soaking in 1N NaOH for one hour Clean, wrap and sterilize decontaminated items by conventional means.
PROCEDURES INVOLVING HIGH INFECTIVITY TISSUES ON PATIENTS KNOWN OR SUSPECTED OF HAVING CJD

1. Surgical Procedures: 1.1 Limit supplies and equipment to only what is necessary for the procedure. 1.2 Operating Room personnel will segregate contaminated devices as follows: 1.2.1 All disposable devices contaminated with high infectivity tissue will be placed in a plastic autoclave bag marked CJD, disposable devices Incinerate Only". 1.2.2 All disposable sharp devices contaminated with high infectivity tissue will be placed in a rigid container marked "CJD, disposable sharps - Incinerate Only". 1.2.3 All non-disposable devices contaminated with high infectivity tissue will be placed in a used instrument container labeled "CJD non-disposable devices, special procedures required" 1.2.4 All disposable devices contaminated with low/no detectable infectivity tissues will be disposed of, or contained for transport to the Sterile Processing Department using standard procedures. 2. Sterile Processing will assure that all disposable devices and sharps, contaminated with high infectivity tissues, are incinerated upon receipt in accordance with Medical Center policy. 3. On receipt of CJD non-disposable devices, Sterile Processing personnel will: 3.1 Those devices constructed so that cleaning procedures result in effective tissue removal can be cleaned and then sterilized by autoclaving at: o a. 134 C for >18 minutes in a prevacuum sterilizer, or o o b. 121 C-132 C for 1 hour in a gravity displacement sterilizer 3.2 Those devices that are difficult or impossible to clean may be discarded by incineration through the waste hauler vendor. Alternatively, devices should be placed in a container filled with saline, water or phenolic solution to retard adherence of material to the medical device. Devices should then be decontaminated by one of the methods listed below,

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INITIAL DECONTAMINATION METHODS: o d. 134 C for >18 minutes in a prevacuum sterilizer, or o o e. 121 C-132 C for 1 hour in a gravity displacement sterilizer, or f. soaking in 1N NaOH for one hour 3.3 Clean, wrap and sterilize decontaminated items by conventional means. 4. Ophthalmology Procedures: 4.1 Tonometers will be incinerated through the waste hauler vendor or sterilized as outlined in Section 3above. 4.2 All instruments used in eye operations on known or suspect CJD patients will be considered contaminated and either incinerated or sterilized according to Section 3.5.9 above. 4.3 Patients with suspected or known CJD will not be used as donors for corneal grafting. 5. Post-mortem Care 5.1 Standard/Universal Precautions will be used when handling and wrapping the body. Wear appropriate PPE in order to avoid contact with blood or body fluids 5.2 Nursing will document CJD or suspected CJD on the Release to Mortuary Form. 5.3 The Department Manager/designee will notify Descendent Affairs and Pathology of the patients death, suspect or known CJD diagnosis. Medical Records will notify the individuals assigned to transport the deceased to the mortuary.
EMPLOYEE HEALTH ISSUES

1. All exposures to high infectivity tissues of patients diagnosed with or suspected of having CJD should be reported to COEM. 1.1 When there is accidental contamination of unbroken skin, wash the skin with detergent and copious quantities of warm water avoid vigorous scrubbing, rinse, and dry. 1.2 For an accidental needle stick injury or laceration with equipment or an instrument, wash the wound with copious quantities of warm soapy water, avoid vigorous scrubbing, rinse, dry and cover with a waterproof dressing. Further treatment (e.g., sutures) should be appropriate to the type of injury. 1.3 If splashing into the eye or mouth occurs, immediately irrigate with either saline (eye) or tap water (mouth or eye).
SPECIAL CONSIDERATIONS

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Do not allow tissues/body fluids to dry on instruments (e.g. keep instruments in liquid) Some decontamination procedures (e.g. aldehydes) fix protein and this may impede effectiveness of disinfection and sterilization processes. Equipment parts, which become contaminated with high infectivity tissues from a patient diagnosed with or suspected to have CJD, need to be incinerated whenever possible. Contaminated devices that must be incinerated will be sealed in an appropriate container and clearly labeled. The EVS Director and safety officer will be contacted for information regarding transportation and incinerating facility information.

The transmission of CJD infections in patient care settings involves the use of prioncontaminated devices or fomites. Transmission is dependent on the concentration of the agent on the device or in and on the fomite and the presence of a mechanism to introduce the agent into a patients body in an efficient manner. Reported cases of iatrogenic and occupationally acquired episodes are tissueassociated, and transmission is linked with unintentional exposure to high infectivity tissues. The only reported instances of patient-to-patient transmission involve devices such as depth electrodes exposed to high infectivity brain tissue, which are difficult to clean, and were soaked in alcohol and formalin, which is not a sterilization process. When a potentially contaminated device can be cleaned, and the prion/tissue load decreased or removed physically, the probability of infection transmission is significantly reduced.
ATTACHMENTS: A. Generator Certification of Pre-Treatment Form ADDENDUM: CJD Waste Pre-Incineration Treatment Protocol

REFERENCES:

Bolton, D.C. Prions and proteins: distinguishing between conformations, The Lancet, Vol. 358 July 21, 2001, 164-165. Bruce, M.E., McConnell, I., Will, R.G., Ironside, J.W., Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues, The Lancet, Vol. 358, July 21, 2001, 208-209.

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Ramasamy I, Law M, Collins S, Brooke F. Organ distribution of prion proteins in variant Creutzfeldt-Jakob disease.Lancet Infect Dis. 2003 Apr;3(4):214-22.
Rutala, WA and Weber, DJ Creutzfeldt-Jakob disease: Recommendations for Disinfection and Sterilization, CID 2001;32:1348-56 Draft Infection Control Guidelines for Creutzfeldt-Jakob disease (CJD) in Canada, Division of Nosocomial and Occupational Infections, Bureau of Infectious Diseases, Laboratory Centre for Disease Control Draft 21, April 25, 2001 Rutala, WA, Prion Disease: Lessons from Europe, Disinfection and Sterilization for Prion Diseases A US Perspective. SHEA Conference, April 2002. WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies, Geneva, Switzerland, 23-26 March 1999 CDC unpublished data. APIC Text (Association for Professionals in Infection Control and Epidemiology) 2009. Rutala, WA Weber DJ Guideline for Disinfection and Sterilization of PrionContaminated Medical Instruments Infection Control and Hospital Epidemiology Feb 2010, Vol. 31, No. 2