Trust Policy Policy Title: The Prevention of Transmissible Spongiform Encephalopathies (Prion diseases including CJD) in the Healthcare Setting

Transcription

1 Trust Policy Policy Title: The Prevention of Transmissible Spongiform Encephalopathies (Prion diseases including CJD) in the Healthcare Setting Purpose Date Version August This guidance provides advice on safe working practices with the aim of preventing the transmission of Transmissible Spongiform Encephalopathies (TSEs), including Creutzfeldt-Jakob Disease (CJD) and variant (v)cjd, in hospital and community healthcare settings. Who should read this document? These guidelines are applicable to all staff, to include Ministry of Defence (MOD) personnel; contractors, those employed on a fixed term contract; honorary contract; agency or locum staff, and students affiliated to educational establishments and volunteers. Key messages Transmissible Spongiform Encephalopathies (TSEs) are rare neurological diseases characterised by a progressive and universally fatal course. The group of illnesses affect both humans and animals and in certain circumstances can cross from one species to another. TSEs are unique as they are inheritable, infectious and able to arise spontaneously. The infectious agent is believed to be an abnormal form of a naturally occurring protein that is not readily degraded by enzymes, chemicals or heat. Standard steam sterilisation cycles and other conventional methods of decontamination are ineffective against these agents. The incubation period of a TSE may be years or decades. The prevention of healthcare transmission of TSEs depends on the identification of infected individuals with these diseases and those at risk of incubating disease. Accountabilities Production Review and approval Ratification Dissemination Dr Peter Jenks, Director of Infection Prevention & Control Infection Control Committee 21 st August 2013 Greg Dix, Director of Nursing Trust-wide Compliance NHSLA & CQC Essential Standards of Quality & Safety The Hygiene Code

2 Links to other policies and procedures Infection Control Manual PHNT/Staff Net/Trust Documents Version History V5.1 August 2013 Update of Version 4 of the guidelines for The Prevention of Transmissible Spongiform Encephalopathies (Prion diseases including CJD) in the Healthcare Setting V5.2 October 2013 Minor change made to V5.1 by Peter Jenks following further information received from the Department of Health. Last Approval Due for Review August 2013 August 2018 The Trust is committed to creating a fully inclusive and accessible service. By making equality and diversity an integral part of the business, it will enable us to enhance the services we deliver and better meet the needs of patients and staff. We will treat people with dignity and respect, promote equality and diversity and eliminate all forms of discrimination, regardless of (but not limited to) age, disability, gender reassignment, race, religion or belief, sex, sexual orientation, marriage/civil partnership and pregnancy/maternity. An electronic version of this document is available on the Trust Documents Network Share Folder (G:\TrustDocuments). Larger text, Braille and Audio versions can be made available upon request.

3 Section Description Page 1 Summary 4 2 Introduction 5 3 Identification and reporting of individuals with TSE 6 4 General prevention of transmission of TSE in hospital 13 5 General infection control procedures for operative and 16 endoscopic procedures on all patients at risk of TSE or at risk of incubating disease 6 Endoscopy in patients with vcjd and CJD uncertain 24 7 Checklist on what to do on identifying an individual at risk of, or 26 those with known or suspected TSE, including those with a neurological disease 8 NICE Interventional Procedure Guidance Bibliography 27 Appendix A Human TSEs and their means of transmission 29 Appendix B Categorisation of patients by risk 30 Appendix C Tissue infectivity and Handling of instruments 31 Appendix D Algorithm chart for precautions for reusable instruments for 33 surgical procedures on patients with, or at increased risk of, CJD, vcjd and other human prion diseases Appendix E Highly Transfused vcjd Risk Assessment Form 34 Appendix F Information for patients undergoing surgery or neuro-endoscopy 40 on high risk tissues Appendix G Quarantining of Surgical Instruments 42 Appendix H Protocol for Management of Instruments and Tissues from 46 Brain Biopsy Procedures Appendix I Consultation List 48 Appendix J Dissemination Plan 49 Appendix K Review and approval check list 50 Appendix L Equalities and Human Rights Impact Assessment 51

4 1 Summary Prevention of healthcare transmission of Transmissible Spongiform Encephalopathies (TSEs), including Creutzfeldt-Jakob Disease (CJD) and variant (v)cjd, depends on the identification of infected individuals with these diseases and those at risk of incubating disease. A categorisation of patients by risk is given in Appendix B. The Infection Prevention and Control Team (IPCT) should be informed as soon as such patients are identified. Individuals who are at risk of or with known or suspected TSE may be identified by the following means: o Those already notified to the National CJD Surveillance Registry for known and suspected cases. This will be documented in the notes o Patients known to be at risk of vcjd will have a Patient Alert on their electronic and paper patient records. o Performing a risk assessment (see Appendices D and E). Individuals with TSEs pose little risk to other patients and members of staff, providing Universal Precautions are used. The greatest risk to others occurs when surgical or endoscopy instruments used on affected individuals are reused. If individuals at risk of or those with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered, have invasive procedures the surgical instruments used may need to be destroyed. It remains the responsibility of the admitting clinician to identify individuals with these conditions and to inform the relevant parties. It remains the responsibility of the operating clinician to ensure all operative instruments including endoscopes used on such individuals are dealt with as outlined in this policy. The IPCT and Sterilisation and Disinfection Unit (SDU) manager are available to assist in the identification and management of individuals with TSE. No patient at risk of or those with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered, should undergo an invasive procedure or endoscopy without first discussing the individual with the IPCT and SDU Manager. If an inadvertent or emergency surgical or endoscopic procedure is performed on an individual at risk of, or with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered, the used surgical

5 instruments or endoscope should be placed in a secure site and the IPCT and SDU manager should be contacted immediately. If an inadvertent or emergency surgical or endoscopic procedure is performed on High Risk tissue of an individual of unknown TSE status (for example because the patient is unconscious), the used surgical instruments or endoscope should be placed in a secure site until the patient s status is clarified. The IPCT and SDU manager are available for further advice if required. Members of staff must undertake the appropriate precautions outlined in this document when performing invasive surgical procedures or endoscopy. Instruments used to biopsy non-focal Central Nervous System (CNS) lesions must either be single use or be quarantined (see Appendix G) until a diagnosis other than TSE is made. Due to logistical reasons the former option is preferred. If after the procedure TSE cannot be excluded and remains a possible diagnosis, the individual should be considered as a possible case of TSE and appropriate precautions followed. 2 Introduction 2.1 Description of Transmissible Spongiform Encephalopathies Transmissible Spongiform Encephalopathies (TSEs) are rare neurological diseases characterised by a progressive and universally fatal course. The group of illnesses affect both humans and animals and in certain circumstances can cross from one species to another. TSEs are unique as they are inheritable, infectious and able to arise spontaneously. The infectious agent is believed to be an abnormal form of a naturally occurring protein that is not readily degraded by enzymes, chemicals or heat. Standard steam sterilisation cycles are ineffective against these agents. The incubation period of a TSE may be years or decades. 2.2 Types of mammalian Transmissible Spongiform Encephalopathies Human TSEs Idiopathic: Sporadic Creutzfeldt-Jakob Disease (CJD) and Sporadic Fatal Insomnia Inherited: Familial CJD, Fatal Familial Insomnia and Gerstmann-Straussler-Scheinker Syndrome Acquired: Iatrogenic CJD, Variant (v) CJD and Kuru Examples of Animal TSEs Bovine Spongiform Encephalopathy Scrapie Cattle Sheep, Goats and Moufflon

6 Transmissible Mink Encephalopathy Feline Spongiform Encephalopathy Mink Domestic and captive cats 2.3 Routes of transmission Details of the human TSEs are given in Appendix A and either: are inherited from known and well defined familial lineages are acquired from reused contaminated surgical instruments are acquired from TSE contaminated tissues or pharmaceutical agents arise sporadically. The overall incidence of TSE in the UK is one per million per year. Most sporadic disease occurs in the elderly with an incidence in this age group of 5 per million per year. Currently the majority of human clinical disease is due to sporadic CJD. The ingestion of foodstuffs contaminated with prions associated with Bovine Spongiform Encephalopathy (BSE) has resulted in the emergence of a new human clinical variant of CJD (vcjd). The estimated prevalence of vcjd in the UK population has previously been estimated as approximately between 1 in 2000 and 1 in 4000 and although the infective risk of these preclinical cases is uncertain, there is the potential for ongoing iatrogenic transmission. The different forms of human TSE and the means of transmission are outlined in Appendix A. When considering measures to prevent transmission, it is useful to make a distinction between symptomatic patients (those who fulfil the diagnostic criteria for definite, probable or possible TSE) and asymptomatic patients (those with no clinical symptoms, but who are potentially at risk of developing one of these diseases due to a medical or family history). The classification of the risk status of symptomatic and asymptomatic patients is outlined in Appendix B. 3 Identification and reporting of individuals with TSE 3.1 Why it is important to identify individuals with TSE It is important to identify individuals with TSEs because: prion proteins are transmissible on surgical instruments conventional methods of decontamination are ineffective at removing prion proteins individuals incubating disease may be infectious whilst being asymptomatic tissues other than the brain and spinal cord can be infectious TSEs can be transmitted on surgical instruments used on High Risk or Medium Risk tissues of symptomatic patients as well as asymptomatic individuals incubating disease (Appendix C). Tissue of the brain, spinal cord and posterior eye are classified as High Risk and that of the olfactory epithelium as Medium Risk (Appendix C). In vcjd, there is extensive pre-symptomatic prion replication in peripheral lymphoid tissues, including tonsils, lymph nodes and gut-associated lymphoid

7 tissue. These tissues represent potential sources of transmission of disease and are classified as Medium Risk. Surgical instruments used on tissue potentially containing prion proteins must be destroyed by incineration. Clinical differentiation of the various clinical forms of TSE can be difficult so throughout this policy all TSEs are considered to have an equivalent potential to spread. It is important that individuals at risk of, or those with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered and those having biopsy of non-focal lesions of the CNS are identified pre-operatively to prevent the reuse of contaminated surgical instruments. 3.2 Actions to be taken on the initial diagnosis and on subsequent hospitalisations of an individual with known or suspected TSE When individuals with TSEs are identified, the IPCT and SDU Manager must be informed. Although ante-mortem diagnostic techniques exist, the diagnosis is frequently made by post mortem histological examination of the brain. No individuals with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered, should have any form of surgical procedure or endoscopic examination without first discussing it with the IPCT and SDU Manager. If an inadvertent or emergency procedure is performed, place the used instruments and/or endoscope at a secure site and contact the IPCT. All surgical instruments (including endoscopes) used on High Risk or Medium Risk tissues (Appendix C) of individuals with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered, must be destroyed. Instruments must be placed in combustible, sealed containers and taken directly to the incinerator (contact IPCT/SDU for further details). Instruments used on procedures involving only low risk tissues can be processed in the SDU as normal. If a diagnostic test for a TSE is requested for a patient, any surgical instruments used in that patient on High Risk or Medium Risk tissue must be quarantined pending the test result (See Appendix G) and the IPCT informed. Actions to be taken 1. Report suspected or confirmed cases to IPCT and the National CJD Surveillance Scheme (details from IPCT). Notification and diagnosis will usually be by a Consultant Neurologist or Neurosurgeon. If in doubt contact the IPCT. 2. Report all notified cases to the IPCT on all subsequent hospitalisations. 3. The IPCT must be informed during the planning stages for any invasive procedure (including endoscopies). If the suspicion is raised intra-operatively or in the event of an emergency procedure being required the IPCT or SDU manager must be informed as a matter of urgency. This will allow used instruments to be dealt with accordingly. 3.3 Identification of patients with TSE: Assessment of all surgical and endoscopic procedures There is currently no pre-mortem test to identify individuals at risk of TSE. It is important to identify these individuals, since they may pose a risk of subsequent transmission, even if they are asymptomatic. All patients about to undergo any elective or emergency surgical or endoscopic procedure should be asked the question:

8 Have you ever been notified that you are at increased risk 1 of CJD or vcjd for public health purposes? The actions to take following the patient s response to the above question are given in Table 1: Table 1. Action in response to assessment of increase risk of CJD or vcjd Patient s response No Yes Action Surgery or endoscopy should proceed using normal infection control procedures unless the procedure is likely to lead to contact with high risk tissue. Please ask the patient to explain further the reason they were notified. Special infection control precautions should be taken for all surgery or endoscopy involving contact with medium or high infectivity tissues (see Appendix C) and the IPCT should be consulted for advice. Section 5.5 provides advice on the precautions to be taken during the treatment of patients with or at increased risk of CJD or vcjd, and Section 5.7 provides information on endoscopic procedures. Unable to respond The patient s response should be recorded in their medical notes for future reference. Surgery or endoscopy should proceed using normal infection control procedures unless the procedure is likely to lead to contact with high risk tissue. If this is the case, please refer to the additional recommendations for high risk procedures outlined below. 3.4 Identification of patients with TSE: Assessment of procedures involving High Risk Tissue For patients undergoing invasive procedures on High Risk tissues (neurosurgery, neuro-endoscopy, or surgery involving intradural, posterior ophthalmic or pituitary gland procedures) a risk assessment should be performed. With regards to endoscopy, these additional recommendations are only applicable to those assessing patients for intradural neuro-endoscopic procedures. It is the responsibility of the pre-operative assessment clinic to perform this for elective admissions and the admitting clinician for emergency admissions. Patients should be asked the questions in Table 2 prior to elective or emergency surgical or neuroendoscopic procedures likely to involve contact with tissues of potentially high infectivity. 1 When someone is notified that they are at increased risk of CJD or vcjd, they are asked to take certain precautions to reduc e the risk of spreading the infection to others. These include: o o o Not donating blood, tissue or organs; Informing healthcare staff if they need to undergo an invasive surgical, medical or dental procedure; Informing a family member or someone close to them, in case they need emergency surgery or endoscopy in the future The individual s GP is asked to record the patient s CJD risk status in their primary care records. The GP should also includ e this information in any referral letter should the patient require invasive surgical, medical or dental procedures.

9 Table 2. Additional pre-assessment of patients undergoing procedures on high risk tissue Question to Patient 1 Have you a history of CJD or other prion disease in your family? If yes, please specify. 2 Have you ever received growth hormone or gonadotrophin treatment? If yes, please specify: i) whether the hormone was derived from human pituitary glands ii) the year of treatment iii) whether the treatment was received in the UK or in another country 3 Have you ever had surgery on your brain or spinal cord? 4 Since 1990, have you ever had any transfusions of blood or blood components (red cells, plasma or platelets) Notes to clinician Patients should be considered to be at risk from genetic forms of CJD if they have or have had: i) Genetic testing, which has indicated that they are at significant risk of developing CJD or other prion disease; ii) A blood relative known to have a genetic mutation indicative of genetic CJD or other prion disease; iii) 2 or more blood relatives affected by CJD or other prion disease Recipients of hormone derived from human pituitary glands, e.g. growth hormone or gonadotrophin, have been identified as at increased risk of sporadic CJD. In the UK, the use of human-derived growth hormone was discontinued in 1985 but human-derived products may have continued to be used in other countries. In the UK, the use of human-derived gonadotrophin was discontinued in 1973 but may have continued in other countries after this time. (a) Individuals who underwent intradural brain or intradural spinal surgery before August 1992 who received (or might have received) a graft of human-derived dura mater are at increased risk of transmission of sporadic CJD (unless evidence can be provided that human-derived dura mater was not used). (b) NICE guidance emphasises the need for a separate pool of new neuroendoscopes and reusable surgical instruments for high risk procedures on children born since 1st January 1997 and who have not previously undergone high risk procedures. These instruments and neuroendoscopes should not be used for patients born before 1st January 1997 or those who underwent high risk procedures using reusable instruments before the implementation of this guidance. (a) Individuals who have been identified as having received blood or blood components from 300 or more donors since January 1990 are at increased risk of vcjd. (b) Individuals who have been treated with certain implicated UK sourced plasma products between 1990 and 2001 are at increased risk of vcjd.

10 If the answer to any of questions 1-4 is 'Yes', the IPCT should be contacted for an assessment of risk of CJD/vCJD. The IPCT will mark the patient record and liaise with the Public Health England, as well as the surgical team and theatres for patients confirmed to be at risk. If the answer to question 4 is 'Yes', the Highly Transfused vcjd risk Assessment Form (Appendix E) should be completed electronically and sent by to the transfusion department (plhtr.transfusionteam@nhs.net) with a copy to the IPCT (plh-tr.ipct-admin@nhs.net). It is essential that the NHS number of the patient is recorded to allow the transfusion service to determine the patient's transfusion history. They will then send that on to the IPCT for assessment of risk of CJD/vCJD. The IPCT will mark the patient record and liaise with Public Health England, as well as the surgical team and theatres for patients confirmed to be at risk. Appendix F is an information sheet for pre-surgical patients undergoing surgery or neuro-endoscopy on high risk tissues about the questions they will be asked. The actions to be taken following the patient s response to the above questions are given in Table 3. Table 3. Action in response to pre-operative assessment Patient s Action response No to all Surgery or neuro-endoscopy can proceed using normal infection control questions procedures. Yes to Further investigation into the nature of the patient s CJD risk should be any of undertaken, and the patient s CJD risk assessed. This assessment of CJD risk questions should be recorded in the patient s medical notes for future reference. 1, 2, 3 or 4 If the patient is found to be at increased risk of CJD or vcjd following investigation, or the risk status is unknown at the time of the procedure, special infection control precautions should be taken for the patient s procedure including quarantining of instruments (Appendix G), and the IPCT should be consulted for advice. Section 5.5 provides advice for the precautions to be taken during the treatment of patients with or at increased risk of CJD or vcjd, and Section 5.7 provides information on neuro-endoscopic procedures. If the patient is found to be at increased risk of CJD or vcjd they should also be referred to their GP, who will need to inform them of their increased risk of CJD or vcjd and provide them with further information and advice. This is available from Public Health England: Patients who are at increased risk of genetic forms of CJD should be offered the opportunity of referral to the National Prion Clinic, based at the National Hospital for Neurology and Neurosurgery, Queen Square, London: Unable to respond Patients who are at increased risk of sporadic CJD due to receipt of humanderived growth hormone or gonadotrophin should be offered the opportunity of referral to the UCL Institute of Child Health, London. Contact: L.Davidson@ich.ucl.ac.uk, See below for advice.

11 3.5 Emergency surgery or neuro-endoscopy which may involve contact with high risk tissue In the event that a patient about to have emergency surgery or neuro-endoscopy is physically or otherwise unable to answer any questions, a family member, or someone close to the patient (in the case of a child, a person with parental responsibility), should be asked the CJD risk questions prior to the surgery or neuroendoscopy. If the family member, or someone close to the patient, is not able to provide a definitive answer to the CJD risk questions, the surgery or neuro-endoscopy should proceed but all instruments should be quarantined following the procedure outlined in Appendix G. The patient s GP should be contacted after the surgery or neuro-endoscopy, and enquiries made as to whether the patient is at increased risk of CJD/vCJD. The actions to be taken following the GP s response are given in Table 4: Table 4. Action in response to assessment by GP GP s Action response No to all The instruments can be returned to routine use after undergoing normal questions decontamination processes. Yes to Further investigation into the nature of the patient s CJD risk should be undertaken, any of and the patient s CJD risk confirmed or rejected. Confirmation or rejection of CJD questions risk should be recorded in the patient s medical notes for future reference. 1, 2, 3 or 4 If the patient is found to be at increased risk of CJD or vcjd following investigation then the quarantined instruments should be destroyed. The patient s GP should inform the patient that they are at increased risk of CJD or vcjd and provide them with further information and advice. This is available from Public Health England: Patients who are at increased risk of genetic forms of CJD may benefit from discussions with the National Prion Clinic, based at the National Hospital for Neurology and Neurosurgery, Queen Square, London: Uncertain about any of questions 1, 2, 3 or 4 Patients who are at increased risk of sporadic CJD due to receipt of human derived growth hormone or gonadotrophin may benefit from discussions with the UCL Institute of Child Health, London. Contact: L.Davidson@ich.ucl.ac.uk, The instruments should be kept in quarantine. The IPCT and SDU Manager will carry out a risk assessment, and may wish to involve the local Control of Communicable Disease Consultant in this process. The outcome of the risk assessment should determine whether or not to return the instruments to routine use

12 3.6 Additional actions to be taken during pre-surgery assessment for CJD risk In addition to asking the patient CJD/vCJD risk questions, the following actions should also be carried out before any surgical or endoscopic procedure involving contact with high risk tissue. The clinician undertaking the pre-surgery assessment should: Check the patient s medical notes and/ or referral letter for any mention of CJD or vcjd status Consider whether there is a risk that the patient may be showing the early signs of CJD or vcjd, i.e. consider whether the patient may have an undiagnosed neurological disease involving cognitive impairment or ataxia These actions, in conjunction with the CJD/vCJD risk questions, will minimise the chance of a CJD incident occurring and therefore reduce the risk of transmission of CJD or vcjd to subsequent patients. 3.7 Action to be taken on identification of at risk individuals No individuals at risk of TSEs should have any form of surgical procedure or endoscopic examination without first discussing it with the IPCT and SDU Manager. If an inadvertent or emergency procedure is performed, place the used instruments and/or endoscope at a secure site and contact the IPCT. All surgical instruments (including endoscopes) used on High Risk or Medium Risk tissues (Appendix C) of individuals at risk of TSEs may need to be destroyed. Instruments must be placed in combustible, sealed containers and taken directly to the incinerator (contact IPCT/SDU for further details). Instruments used on procedures involving only low risk tissues can be processed in the SDU as normal. If a diagnostic test for a TSE is requested for a patient, any surgical instruments used in that patient on High Risk or Medium Risk tissue must be quarantined pending the test result and the IPCT informed (see Appendix G). Actions to be taken 1. Report at risk cases to IPCT and the National CJD Surveillance Scheme (details from IPCT). 2. Report all notified cases to the IPCT on all subsequent hospitalisations. 3. The IPCT must be informed during the planning stages for any invasive procedure (including endoscopies). If the suspicion is raised intra-operatively or in the event of an emergency procedure being required the IPCT or SDU manager must be informed as a matter of urgency. This will allow used instruments to be dealt with accordingly. 3.8 Actions to be taken prior to performing a surgical or endoscopic procedure on a patient with neurological disease of unknown aetiology where the diagnosis of TSE is being considered. No surgical procedure should be performed on a patient with unexplained dementia, or other clinical features consistent with a TSE, until an opinion as to whether the patient may be suffering from a TSE is sought from a suitably qualified Specialist, such as a Consultant Neurologist or Neurosurgeon. If a diagnosis of TSE is being considered, instruments used in surgical and endoscopic procedures involving High Risk or Medium Risk tissues (Appendix C) should be carefully risk assessed. If the

13 diagnosis of TSE is considered likely enough to perform a TSE diagnostic test then until a diagnosis other than TSE can be made, the patient should be considered as a possible case. Surgical instruments and endoscopes in contact with tissue potential contaminated with the TSE agent should be placed in quarantine (see Appendix G). Similarly, if an inadvertent or emergency procedure is performed the surgical instruments and endoscope should be placed in quarantine and the IPCT contacted. If a definite diagnosis of a disorder other than CJD is made (and there is no other evidence to suggest any form of CJD), the instruments can be reprocessed and reused (see Appendix G). If a definite diagnosis of CJD is made, the neurosurgical instruments should be destroyed by incineration. If the diagnosis remains uncertain, the instruments should remain in quarantine until a definite diagnosis is made, or the patient dies. If the patient dies without a diagnosis, consent should be sought for postmortem examination of the brain. Iif consent for post-mortem examination is not given or if the diagnosis is still uncertain after post-mortem examination of the brain, the instruments used for biopsy should be destroyed. 4 General prevention of transmission of TSE in hospital TSEs have rarely been transmitted from patient to patient. When it has occurred, transmission has been exclusively due to the reuse of neurosurgical instruments or the use of contaminated tissue or organs. 4.1 Instrument sterilisation and tracking As the full burden of TSE in the community is not known all surgical instruments should be thoroughly cleaned and decontaminated, preferably in automated machines in the SDU. Thorough cleaning of instruments prior to decontamination is particularly important in reducing the risk of transmission of prion-associated disease. Failure to use automated methods must be supported with an adequate risk assessment, written Standard Operating Procedure (SOP) and regular audit of procedure. Anyone who manually cleans an instrument by hand must be suitably trained, wear appropriate personal protective equipment (PPE) and be vaccinated against hepatitis B. Effective tracking of re-usable instruments should be in place, so that instruments can be related to use on a particular patient. 4.2 General precautions There is no reason to believe that horizontal transmission occurs outside of the specific conditions noted previously and basic infection control practices are generally sufficient when managing potentially infected patients. With the exception of precautions relating to procedures outlined in Section 5, individuals at risk of incubating disease should be treated as for any other hospitalised patient. The following precautions relate to those suspected or confirmed as having a TSE:

14 (a) There is no need to isolate the individual for reasons other than those applicable to all patients (b) The most infectious tissues such as cerebrospinal fluid (CSF) must be handled with care. Personal protective equipment, including gloves, apron and eye protection, should be worn for all procedures, including lumbar puncture (LP) and venepuncture. Only trained and experienced staff should perform LPs and single use disposable equipment must be used (c) Body secretions other than CSF are unlikely to be infectious for TSE and standard precautions should be used at all times (d) Fouled bed linen should be handled as for all patients, as detailed in the Linen Services Policy (e) Spillage of any bodily fluid should be mopped up by a suitably trained member of staff wearing appropriate PPE with an absorbent substance and the surface disinfected with bleach (10,000 ppm available chlorine). All waste, including mop heads used to clean the spill area, towels and used PPE, should be disposed of as normal clinical waste in orange bags (f) The spillage of a High Risk or Medium risk secretion or tissue (Appendix C) is likely to be an exceptional event. Initial cleaning should be with an absorbent material which should be disposed of by incineration. The contaminated surface should be disinfected with a bleach solution containing 20,000ppm available chlorine. This is a toxic and corrosive solution and must be used with great care. In this situation the IPCT should be informed and further advice sought (g) Waste known or suspected to be contaminated with TSE agents, including high- or mediumrisk tissues, from patients with, or at increased risk of, CJD/vCJD, must be disposed of by incineration in suitable authorised facilities. Low-risk tissues and body fluids from patients with, or at increased risk of, CJD/vCJD, should be disposed of as normal clinical waste in orange bags (h) Any inoculation injury or exposure of a mucous membrane to a secretion that may transmit a TSE (e.g. CSF) from a suspected, known or at risk individual should be dealt with as for any other inoculation injury (see Management of Inoculation Injuries Policy). The potentially infectious nature of the secretion should be noted on the incident form and sent to Occupational Health to ensure the exposure is formally documented. 4.3 Pregnancy In the event of a woman with known or suspected TSE becoming pregnant the delivery should be managed using standard infection control procedures. The placenta and other by-products of the birth should be considered infectious and disposed of as clinical waste unless required for investigations (see section 6). Instruments used during delivery can be reprocessed in the normal manner providing they have not been used on High Risk or Medium risk tissues (see Appendix C). 4.4 Procedures after death of individuals at risk of or with known or suspected TSE As with any deceased patient where there is a residual infectious risk the body should be placed in a body bag, labelled as High-Risk and the infection control notification she should be completed (see also Policy for Handling Cadavers).

15 For advice regarding precautions to be taken by undertakers and embalmers please see the Policy for Handling Cadavers or contact the Consultant in Communicable Disease Control (number available from IPCT). In general viewing and hygienic preparation can be performed, but embalming is not recommended. There is no reason why patients with confirmed TSE cannot be buried as opposed to cremated. Post mortem examination of the brain is currently the only way of confirming the diagnosis of TSE and further advice regarding where and how the post mortem will be performed is available from the Department of Histopathology. 4.5 Occupational Health & Wellbeing notification of individuals potentially exposed to TSE Individuals with TSEs pose little risk to other patients and members of staff providing standard precautions used with all patients are applied and appropriate precautions are taken when performing invasive surgical procedures. The Department of Health have advised that a note should be made in the Occupational Health & Wellbeing files of staff who fall into one of the following categories and that the nature and risks of the exposure are discussed with them by the Occupational Health & Wellbeing Health Department: Any member of staff performing invasive clinical procedures on patients known or suspected to be suffering from a TSE, particularly where there is a risk of exposure to high and medium risk tissues (Appendix C) Laboratory staff handling tissue specimens from patients with known or suspected TSE Staff undertaking post-mortem examinations of patients who have died with a TSE Any inoculation injury should be managed according to the Management of Inoculation Injuries Policy. It is important that the Occupational Health & Wellbeing Department is informed of the nature of the injury. Responsibility for notifying Occupational Health & Wellbeing of individuals falling into these risk categories lies with the Consultant treating the patient for medical staff, the Senior Nurse of the Directorate treating the patient for nursing staff and the respective Heads of Departments for laboratory staff. Notification should occur for every contact and should use the standard notification form available from Occupational Heath and Staff Wellbeing. The relevant manager should: 1. perform a local risk assessment (COSHH) 2. make a list of employees with significant exposure 3. send the list to the occupational health team 4. explain the nature and risks of exposure to the employee, as well as the appropriate safety procedures. The Occupational Health & Wellbeing team should keep the list of significantly exposed employees for at least 40 years, and the relevant contents of which will be available to the individual themselves and their Health and Safety Manager.

16 5 General infection control procedures for operative and endoscopic procedures on all patients at risk of TSE or at risk of incubating disease 5.1 Avoiding the reuse of TSE-contaminated instruments Prevention of healthcare transmission of TSEs depends on the identification of infected individuals with these diseases and those at risk of incubating disease. See Section 3 for the identification of these individuals and process for dealing with potentially contaminated instruments, including endoscopes. In certain situations contaminated instruments may not be incinerated but donated for Prion research. This will be discussed on a case-by-case basis with the National CJD Surveillance Unit. 5.2 General planning All surgical procedures should be carefully planned to allow all appropriate precautions and instrument selection to be made and to minimise the number of instruments requiring incineration. Practicalities surrounding instrument cleaning and disposal need careful consideration. It is ultimately the responsibility of the admitting clinician to identify individuals at risk of TSE and the operating clinician to ensure the correct procedures as detailed in this document are followed. The IPCT and SDU manager are available to assist with this and should be involved at an early planning stage. Non-invasive procedures do not require specific precautions over and above those needed for any other patient. 5.3 Anaesthetic equipment Single-use anaesthetic equipment should be used wherever possible when this is as reliable and safe as the re-usable alternatives. Any re-usable equipment used, for example in the maintenance of an airway in individuals at risk of, or those with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered, should be destroyed or quarantined pending discussion with the IPCT (see Appendix G). It is vital that fibreoptic equipment is avoided unless one is clear that the exposure is such that destruction of the equipment is not warranted. All airway devices used during tonsillectomy and adenoidectomy should be discarded after use. 5.4 Instrument disposal All instruments for incineration must be transported from the theatre to the incinerator in a combustible sealed container. On the rare occasions that quarantining instruments is considered, the protocol in Appendix G should be followed. All other instruments may be reprocessed as per normal protocol. It remains the responsibility of the operating clinician to ensure that the instruments are delivered to the appropriate site (i.e. incinerator or SDU). A named member of the Theatre Team should be responsible for transportation of the instruments. 5.5 Precautions during invasive procedures For all patients with, or at increased risk of, CJD or vcjd, the following precautions should be taken for surgical procedures: Wherever appropriate and possible, the intervention should be performed in an operating theatre;

17 Where possible, procedures should be performed at the end of the list, to allow normal cleaning of theatre surfaces before the next session; Only the minimum number of healthcare personnel required should be involved; Protective clothing should be worn, i.e. liquid repellent operating gown, over a plastic apron, gloves, mask and goggles, or full-face visor; for symptomatic patients, this protective clothing should be single use and disposed of in line with local policies; for patients at increased risk of CJD/vCJD, this protective clothing need not be single use and may be reprocessed; Single-use disposable surgical instruments and equipment should be used where possible, and subsequently destroyed by incineration or sent to the instrument store; Effective tracking of re-usable instruments should be in place, so that instruments can be related to use on a particular patient; Instruments may be streamed into those for incineration/quarantine or those that may be reprocessed. Instruments that have been in direct contact with the cut surface of potentially infected tissues should be regarded as potentially contaminated with the TSE agent. If possible, they should be separated from other instruments that have only come into contact with the external surface of such tissues. Such instruments should either be incinerated after use or quarantined for re-use exclusively on the same patient. Instruments that have not been in direct contact with the cut surface of medium infectivity tissues may be reprocessed; For high cost equipment (e.g. some complex retractors), where it is feasible, efforts should be taken to protect the retractor by using physical barriers, for example sheaths and impervious drapes. In cases where only part of the instrument has come into contact with the cut surface of medium infectivity tissue, where possible, that specific instrument component should be discarded or quarantined for reuse exclusively on the same patient. 5.6 Neurosurgical biopsy of non-focal CNS lesions and biopsy in patients with dementia of unknown cause Brain biopsies are often performed for diagnostic purposes on patients with non-focal CNS lesions and dementia of unknown cause or neurological disease of unknown aetiology where the diagnosis of TSE is being considered (See also Section 3.8). If the diagnosis of TSE is considered likely enough to perform a TSE diagnostic test then until a diagnosis other than TSE can be made, the patient should be considered as a possible case. Surgical instruments used on such patients could potentially be infected with the TSE agent and require incineration and should therefore be placed in quarantine (see Appendix G). Similarly, if an inadvertent or emergency procedure is performed the surgical instruments and endoscope should be placed in quarantine and the IPCT contacted. If a definite diagnosis of a disorder other than CJD is made (and there is no other evidence to suggest any form of CJD), the instruments can be reprocessed and reused (see Appendix G). If a definite diagnosis of CJD is made, the neurosurgical instruments should be destroyed by incineration. If the diagnosis remains uncertain, the instruments should remain in quarantine until a definite diagnosis is made, or the patient dies. If the patient dies without a diagnosis, consent should be sought for postmortem examination of the brain. Iif

18 consent for post-mortem examination is not given or if the diagnosis is still uncertain after post-mortem examination of the brain, the instruments used for biopsy should be destroyed. 5.7 Endoscopy General decontamination and handling of endoscopes. The risk of transmission of prions during endoscopy is low with the exception of endoscopes used on central nervous tissue. Due to more extensive tissue deposition, vcjd poses a greater risk of endoscopic transmission (appendix C). The general procedures set out in the Choice Framework for local Policy and Procedures Decontamination of flexible endoscopes: Policy and Management (CFPP 01-06) ( and the updated BSG Guidelines for Decontamination of Equipment for Gastrointestinal Endoscopy (2008, to be updated 2013) should be followed. This risk can be further reduced for all endoscopic procedures by: using single-patient-use channel cleaning brushes using single-patient-use (disposable) biopsy forceps and other accessories that pass through the endoscope if channel cleaning brushes and, if biopsy forceps or other accessories have been passed, the rubber valve on the endoscope biopsy/instrument channel port should be disposed of as clinical waste after each use. systems that enable endoscopes, together with all their detachable components and any re-used accessories, to be traced to the patients on whom they have been used. Following use in patients at risk of vcjd endoscopic accessories (including normally reusable devices such as heater probes) and cleaning aids such as brushes should be disposed of by incineration. When decontaminating the endoscope cleaning equipment, the Endoscope Washer Disinfector (EWD) should be put through an empty self-disinfection cycle as per recommended routine. Provided that the cleaning equipment is decontaminated as indicated, there is no known risk of transmission of TSE agents via this route. As defined below, endoscopes used for endoscopy in the central nervous system and nasal cavity in certain patient groups quarantined pending diagnosis or exclusion of CJD (see Appendix G). The principles and procedures recommended for quarantining are as for surgical instruments, except the endoscope should be fully cleaned and decontaminated immediately after use, before being quarantined (see Appendix G). Endoscopes, other than those used in the central nervous system and nasal cavity, which have been used for invasive procedures in most individuals designated as at increased risk of sporadic or vcjd, can be decontaminated to the standards set out in CFPP and the BSG guidelines and returned to use. The endoscope should be put through all the normal stages of cleaning, and be disinfected separately from other equipment within an automated Endoscope Washer Disinfector. The exception is a small number of asymptomatic individuals at increased risk of vcjd because

19 they have received blood from a donor who later developed vcjd. Endoscopes used to treat these patients should be removed from use or quarantined to be re-used exclusively on the same individual (Appendix G). Further detail is given below Central nervous system endoscopy Introduction of an endoscope into the central nervous system (including the eye) should be avoided in individuals at risk of, or those with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered. Full details of the precautions to be taken if endoscopy is performed are given below Nasal cavity endoscopy The prion protein has been detected in the olfactory epithelium, but not the respiratory epithelium, of sporadic CJD patients. The olfactory epithelium is normally located along the roof of the nasal cavity but its distribution varies between individuals. On the lateral wall it may extend inferiorly onto the superior turbinate and the anterior insertion of the middle turbinate; on the medial wall it may extend onto the uppermost part of the septum. Introduction of an endoscope into the nasal cavity should be avoided wherever possible in individuals with known or suspected TSE, including those with a neurological illness of unknown aetiology in whom a diagnosis of TSE is being considered. The advice of the consultant carrying out the endoscopic procedure in the nasal cavity should be sought to determine whether a risk of contamination of the endoscope with olfactory epithelium can be excluded with confidence. If such contamination cannot be excluded, precautions should be taken appropriate for medium infectivity tissues and full details of the precautions to be taken if endoscopy is performed are given below. If the procedure is performed inadvertently or as an emergency, quarantine the instrument in a secure location and contact the IPCT (see Appendix G) Endoscopy in patients with sporadic and other non-variant CJD This includes sporadic CJD, iatrogenic CJD other than variant CJD, and genetic prion diseases. Please refer to Table 5 below Symptomatic sporadic CJD patients (definite, probable) Neurological endoscopes. Single use or remove from use. Nasendoscopes. Single use or remove from use if contaminated with olfactory epithelium All other endoscopes. Decontaminate according to CFPP and BSG guidelines, with the additional precautions for flexible endoscopes as set out above Symptomatic patients (possible sporadic, or diagnosis unclear but variant CJD is NOT being considered) Neurological endoscopes. Single use or quarantine with option of re-use exclusively in same individual patient (see Appendix G). If further clarification of the diagnosis is not possible, the endoscope should be removed from use. Nasendoscopes. If there is a risk that the endoscope could become contaminated with olfactory epithelium, a single use endoscope should be used where possible. If this is not appropriate, the endoscope should be decontaminated singly, then quarantined pending a more definitive diagnosis. The quarantined endoscope may be re-used