Date Completed 13 November 2016 Final Document Approval Committee Policy Approval Group Date Approved 23 November 2015 Final Document Ratification

Transcription

1 Creutzfeldt-Jakob Disease (CJD) and other Transmissible Spongiform Encephalopathy Policy HH(1)/IC/659/15 Previous document(s) being replaced Location Policy No Policy Name HHFT HH(1)/IC/659/13 Creutzfeldt-Jakob Disease (CJD) and other Transmissible Spongiform Encephalopathy Document Summary This policy describes the steps which must be taken to manage patient treatment in order to minimise risk and is based on national guidelines for preventing the transmission of any form of Transmissible Spongiform Encephalopathy (TSE) namely Creutzfeldt-Jakob Disease (CJD) or Variant Creutzfeldt-Jakob Disease (vcjd). It also provides guidance to staff on the precautions necessary to minimise the risk of occupational exposure to CJD and to prevent transmission of CJD between patients. Ownership Author Hazel Gray Job Title Lead Infection Prevention and Control Nurse Document Type Level Level 1 Trustwide Related Documents Document Details Standard Precautions (incorporating Personal Protective Equipment) Policy Linen Policy Waste Management Policy Sharps Prevention and Management of Needlestick/Sharps Injuries and Exposure to Body Fluids Policy Cleaning, Disinfection and Sterilisation policy Care of Patients at Death Policy Relevant Standards CQC Regulations Regulation 12 Equality Impact Completed by Steve Mullen Assessment Date Completed 13 November 2016 Final Document Approval Committee Policy Approval Group Date Approved 23 November 2015 Final Document Committee Executive Committee Ratification Date Ratified 26 November 2015 Authorisation Authoriser Mary Edwards Job Title Chief Executive Signature Date Authorised 1 December 2015 Dissemination Target Audience All Trust Staff Dissemination and Implementation Plan Action Owner Due by Publicise detail of new document via Intranet and IPCT and Communication 1 week of publication Midweek message Team Communication to all Senior Managers to advise BNHH Healthcare Library On publication publication of policy The policy will be available on the intranet BNHH Healthcare Library 1 week of authorisation Review date Review date 31 October 2018 Page 1 of 64

2 Document Control Document Amendments Version No. Details Key amendments to note By whom Date 1 Review of BNHFT & WEHCT policies to produce harmonised HHFT policy Kordo Saeed Hazel Gray June Updated with new guidance 3 Updated with new guidance New guidance regarding patients who have had blood transfusions before 1999 Guidance and procedures for: Endoscopy Linen Waste New human prion Variably Protease-Sensitive Prionopathy (VPSPr) Pregnancy Ophthalmology Laboratory samples Information and guidance tables updated Hazel Gray October 2014 Hazel Gray August 2015 Page 2 of 64

3 Contents 2. Purpose Scope Explanation of Terms Duties Human Transmissible Spongiform Encephalopathy (TSE) Diagnosis and Treatment Categorisation of Patients by Risk Infection Control Measures Post Mortem Organ donation and transplantation Health4Work Records Surgical Procedures Identification of a case of known or suspected TSE in a patient who has previously undergone invasive/surgical procedures Re-usable Instruments Theatre Procedure Pregnancy Specific Guidance related to Ophthalmology Specific Guidelines for Pathology Laboratories for the handling of tissues from TSE at risk or confirmed sources Stakeholders Engaged During Consultation Training Monitoring Compliance with the Document References Associated Documentation Contributors Appendix A Equality Analysis Form Appendix B Assessment to be carried out before surgery and endoscopy to identify patients with, or at risk of, CJD and vcjd Appendix C - Distribution of TSE Infectivity in Human Tissues and Body Fluids Appendix D Endoscopy Page 3 of 64

4 Appendix E Common flexible endoscopic procedures classified as invasive or noninvasive Appendix F Handling High/Medium Risk Tissue Appendix G Surgical Procedures on patients known, suspect or at risk of CJD Appendix H Algorithm chart for precautions for surgical procedures on known, suspect or at risk patients; CJD other than variant CJD Appendix I Algorithm chart for precautions for surgical procedures on known, suspected of at risk patients: variant CJD Appendix K - Known CJD case sign Appendix L - CJD Log Book Appendix M - Theatre CJD Procedure Appendix N - Theatre Spillage of Bodily Fluid Procedure Page 4 of 64

5 1. Introduction/Background Creutzfeldt-Jakob disease (CJD) is a human form of a transmissible spongiform encephalopathy (TSE). It is a rare degenerative disease of the nervous system. It has a long incubation period which makes identification and prevention difficult. TSEs are believed to be caused by the cellular prion protein (PrP) which is found mainly in the brain and spinal cord, although lower levels may be found in some lymphoid tissues such as the spleen and tonsils. Creutzfeldt Jacob Disease (CJD) is classified according to whether it is sporadic, inherited, or acquired: Sporadic CJD - this is the most common affecting approximately 60 people in the UK each year. It occurs worldwide in all populations and the incidence is 1 per million per annum; the patients are usually over the age of 50. The change in protein structure occurs spontaneously as a chance event with no known cause. Variably Protease-Sensitive Prionopathy (VPSPr) is a recently described human prion disease exhibiting features similar to Sporadic CJD. Inherited or familial (genetic) this is very rare and results from a genetic mutation in the prion gene (the gene responsible for the production of the prion protein). For most people symptoms develop between the ages of 30 and 50 years. There are three forms of the disease recognised: Inherited CJD, Gerstmann-Straussler- Scheinker Disease and Fatal Familial Insomnia. Acquired - prion disease has been transmitted to people in a few very specific ways: o o o Iatrogenic. All cases have involved use of or contamination with high-risk tissue as a result of a surgical or medical procedure e.g. cornea or dura mater grafts from infected donors. Other iatrogenic routes have included the use of inadequately sterilised neurosurgical instruments, and the use of human derived pituitary gonadotropin and growth hormones. Variant (v-cjd). This was first identified in 1996 and is associated with the consumption of BSE infected cattle. Most of the cases have been in people under 30 years of age. It differs from other forms of the disease in that the atypical protein has been found in lymphoid tissue such as the appendix and tonsils. To date vcjd has never been transmitted through surgery, but it has been transmitted through blood transfusion in the UK. Kuru. First identified in 1950s in Papua New Guinea. Transmitted from infected bodies as a result of the practice of ritualistic cannibalism. To date there have been no documented cases of occupational transmission. The clinical presentation of prion disease includes dementia, personality disorders and neuromuscular symptoms e.g. unsteadiness, involuntary muscular jerking. Diagnosis is difficult and can only be confirmed by histological examination of the brain following brain Page 5 of 64

6 biopsy or after death. There is currently no non-invasive test which can diagnose CJD during the incubation period and no effective treatment. The use of the term CJD in this guidance encompasses sporadic CJD, sporadic fatal insomnia, variable protease-sensitive prionopathy (VPSPr), vcjd, iatrogenic CJD, genetic CJD, Fatal Familial Insomnia (FFI) and Gerstmann-Straussler-Scheinker Disease (GSS), in order to assist readability. It is essential that infection control is seen as an organisational responsibility and priority, that adequate isolation facilities and resources are provided, and that appropriate infection control staff and support services are available. 2. Purpose The purpose of this policy is to: provide guidance on the precautions necessary to minimise the risk of occupational exposure to CJD; provide guidance on the precautions necessary to prevent transmission of CJD between patients; describe safe working practices to prevent the transmission of CJD and related disorders; protect staff and patients from any infection risk from instruments used on patients known, suspect or at risk of possible CJD. 3. Scope This policy and procedure will be applied fairly and consistently to all employees and service users regardless of their protected characteristics as defined by the Equality Act 2010 namely, age, disability, gender reassignment, race, religion or belief, gender, sexual orientation, marriage or civil partnership, pregnancy and maternity. For employees this policy also applies irrespective of length of service, whether full or part-time or employed under a permanent or a fixed-term contract, irrespective of job role or seniority within the organisation. Where an employee or service user has difficulty in communicating, whether verbally or in writing, arrangements will be put in place as necessary to ensure that the processes to be followed are understood and that the individual is not disadvantaged during the application of this policy. The application of this policy is completely clinically based and ensuring prompt testing/ treatment would be the priority, however the Trust would endeavour to continue to meet patients individual needs as far as is practicable. In line with the Equality Act 2010, the Trust will make reasonable adjustments to the processes to be followed where not doing so would disadvantage an individual with a disability during the application of this policy. Page 6 of 64

7 This policy complements professional and ethical guidelines and the Nursing and Midwifery Council (NMC) Code of Professional Conduct (NMC 2015). 4. Explanation of Terms Transmissible Spongiform Encephalopathy (TSE) - a group of rare and fatal degenerative conditions of the central nervous system, which are transmissible. TSEs are currently thought to be caused by infectious proteins known as prions. TSEs occur in both man and certain animal species. There is currently no known treatment vaccine or prophylaxis for TSEs. Creutzfeldt-Jakob Disease (CJD) - a rare and ultimately fatal degenerative brain disease first classified in the 1920s. Variant Creutzfeldt-Jakob Disease (vcjd) - a variant first identified in 1996 strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE). Variably Protease-Sensitive Prionopathy (VPSPr) - VPSPr is a recently described human prion disease, which appears to be a rare sporadic disorder affecting patients in an age range similar to those affected by sporadic CJD. Prion - a protein particle that is believed to be the cause of brain diseases such as BSE, scrapie, and CJD. Prions are not visible microscopically, contain no nucleic acid, and are highly resistant to destruction. 5. Duties Postholders The Chief Executive Officer (CEO) has overall responsibility for the strategic and operational management of the Trust ensuring there are appropriate strategies and policies in place to ensure the Trust continues to work to best practice and complies with all relevant legislation in regard to the CJD Policy The Director of Infection Prevention and Control (DIPC) is the Trust Director responsible to the board for the delivery of IPC standards. The Director of Nursing will ensure that the Divisional Directors take clinical ownership of the policy. The Divisional Operational Directors will ensure that all healthcare workers comply with this policy and that all healthcare workers attend mandatory infection prevention and control training. They are responsible for ensuring adequate facilities and resources are available to adhere to this policy. Page 7 of 64

8 The Clinical Service Managers/Leads will ensure that the current version of this policy is available in all of their areas. They will ensure that all healthcare workers comply with this policy and that all healthcare workers attend mandatory infection prevention and control training. Theatre Manager/Pre Assessment Manager ensures that ALL patients have had a pre assessment for HCAI and specifically CJD risk factors. Staff who may be required to use endoscopic equipment or perform procedures must ensure that patients are risk assessed at pre assessment or pre procedure. All Trust employees will comply with this policy and inform the Infection Prevention and Control Team about any issues or concerns relating to the policy. All staff will attend mandatory Infection Prevention and Control training annually. Infection control is the responsibility of ALL staff associated with patient care. A high standard of infection control is required on ALL wards and units, although the level of risk may vary. It is an important part of total patient care. Groups/Committees The Infection Prevention and Control Team (IPCT) will act as a resource for information and support. They will provide education in relation to this policy which includes mandatory training. They will monitor the implementation of this policy via audit within clinical areas and be responsible for regularly reviewing and updating it. The Health4Work department will act as a resource for information, and support and consult with managers, the Infection Prevention and Control Team and healthcare workers regarding the use of personal protective equipment. The Health and Safety Team will act as a resource for information, and support and consult with managers, the Infection Prevention and Control Team and healthcare workers regarding the use of personal protective equipment. 6. Human Transmissible Spongiform Encephalopathy (TSE) Classic (sporadic) CJD occurs worldwide with a frequency of approximately one per million population per annum. vcjd is thought to have resulted from oral exposure to the bovine spongiform encephalopathy (BSE) agent and is currently rare with approximately 176 cases in the UK up to July The abnormal prion proteins are present in certain tissues before patients show any symptoms and some carriers may remain asymptomatic. Consequently there is a risk of contamination of instruments used during invasive procedures such as surgery and endoscopy. Prions are highly resistant to standard methods of disinfection and sterilisation, and therefore a special approach must be adopted in the care of patients, disposal of clinical waste and handling of surgical instruments and other medical devices. Since 1997, when the theoretical risk of vcjd transmission through blood was first considered, the UK blood services have taken a number of precautionary measures to Page 8 of 64

9 protect the blood supply and associated plasma products. These precautionary measures to reduce the risk include: Blood components, plasma products or tissues obtained from any individual who later develops vcjd are withdrawn/recalled to prevent their use; Plasma for the manufacture of plasma products, such as clotting factors, has been obtained from non-uk sources since 1998; Synthetic (recombinant) clotting factor for treatment of haemophilia has been provided to those aged under 16 since 1998, and for all patients in whom it is suitable since 2005; Since 1999 white blood cells (which may carry a significant risk of transmitting vcjd) have been reduced in all blood used for transfusion, a process known as leucodepletion; Since 2002, fresh frozen plasma for treating babies and young children born on or after 1 January 1996 has been obtained from the USA. In 2005 its use was extended to all children up to the age of 16; Since 2004, individuals who have received a transfusion of blood components since January 1980, or are unsure if they have had a blood transfusion, are excluded from donating blood or platelets; Since 2009, cryoprecipitate, a special cold-treated plasma preparation, has been imported from the USA for children up to the age of 16. As non CJD diseases are extremely rare this guidance will refer to CJD specifically. However the principles and guidance will be the same for other prion diseases. 7. Diagnosis and Treatment Diagnosis is usually made clinically; there are currently no widely available laboratory tests for human TSEs. (CSF) samples can be tested at the National Reference Laboratory but the definitive diagnosis can only be made by examination of brain tissue after death. Brain biopsy may be used in investigating cases of suspected TSE but may not be definitive in establishing a diagnosis. Reaching a clinical diagnosis of TSE may take a period of months as some patients may have an atypical presentation and this may not have been considered during the initial investigation. There are no proven specific treatments available. 8. Categorisation of Patients by Risk When considering measures to prevent transmission to patients or staff in the healthcare setting, it is useful to make a distinction between: symptomatic patients, i.e. those who fulfil the diagnostic criteria for definite, probable or possible CJD (see Appendix B for full diagnostic criteria), and; Page 9 of 64

10 patients at increased risk i.e. those with no clinical symptoms, but who are at increased risk of developing CJD, because of their family or medical history. For this group of patients, the infection prevention and control advice differs in some circumstances for: o o o patients at increased risk of genetic CJD patients at increased risk because they have received blood from an individual who later developed variant CJD other patients at increased risk of iatrogenic CJD (see table below) details the classification of the risk status of symptomatic patients and patients at increased risk Patients at increased risk of CJD A number of patients have been identified as at increased risk due to a medical or family history which places them at increased risk of developing CJD. These patient groups are outlined in table below. In most routine clinical contact, no additional precautions are needed for the care of patients in the at increased risk patient groups. However, when certain invasive interventions are performed, there is the potential for exposure to the agents of TSEs. In these situations it is essential that control measures are in place to prevent iatrogenic CJD transmission. All people who are at increased risk of CJD are asked to help prevent any further possible transmission to other patients by following this advice: Don t donate blood. No-one who is at increased risk of CJD, or who has received blood donated in the United Kingdom since 1980, should donate blood; Don t donate organs or tissues, including bone marrow, sperm, eggs or breast milk; If you are going to have any medical, dental or surgical procedures, tell whoever is treating you beforehand so they can make special arrangements for the instruments used to treat you if you need certain types of surgery or investigation; You are advised to tell your family about your increased risk. Your family can tell the people who are treating you about your increased risk of CJD if you need medical or surgical procedures in the future and you are unable to tell them yourself. Taken and adapted from Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection Department of Health (June 2003 Amended February 2015). Patients should be categorised as follows, in descending order of risk: Patient Category Symptomatic patients Patient Group Patients who fulfill the diagnostic criteria for definitive, probable or possible CJD or vcjd Patients with neurological disease of unknown aetiology, Page 10 of 64

11 Patients at increased risk from genetic forms of CJD who do not fit the criteria for possible CJD or vcjd but where the diagnosis of CJD is being actively considered Individuals who have been shown by specific genetic testing to be at significant risk of developing CJD Individuals who have a blood relative known to have a genetic mutation indicative of genetic CJD Patients identified as at increased risk of vcjd through receipt of blood from a donor who later developed vcjd Patients identified as at increased risk of CJD/vCJD through iatrogenic exposures Individuals who have or have had two or more blood relatives affected by CJD or other prion disease Individuals who have received labile blood components (whole blood, red cells, white cells or platelets) from a donor who later went on to develop vcjd Recipients of hormone derived from human pituitary glands, e.g. growth hormone, gonadotrophin are at increased risk of transmission of sporadic CJD. In the UK the use of human-derived gonadotrophin was discontinued in 1973 and use of cadaver-derived human growth hormone was banned in However use of humanderived products may have continued in other countries after these dates. 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 at at increased risk of transmission of sporadic CJD (unless evidence can be provided that human-derived dura mater was not used). Individuals who have had surgery using instruments that had been used on someone who went on to develop CJD/vCJD or was at increased risk of CJD/vCJD Individuals who have received an organ or tissue from a donor infected with CJD/vCJD or at increased risk of CJD/vCJD Individuals who have been identified as having received blood or blood components from 300 or more donors before January 1990 Individuals who have given blood to someone who went on to develop vcjd Individuals who have received blood from someone who Page 11 of 64

12 has also given blood to a patient who went on to develop vcjd Individuals who have been treated with certain implicated UK sourced plasma products between 1990 and Recipients of ocular transplants, including corneal transplants, are not considered to be at increased risk of CJD/vCJD. GPs are asked to record their patient s CJD risk status in their primary care records. The GP should also include this information in any referral letter should the patient require surgical, medical or dental procedures. If any patient is assessed to fall within any of the above categories, the IPCT must be informed as soon as possible, prior to any clinical procedures being carried out. If an invasive procedure is planned for a patient who is known to have received a blood product or plasma derivative that may have been contaminated by a TSE agent the IPCT must be informed. Responsibility for investigating, assessing and managing CJD incidents (and where appropriate notifying patients) rests with local trusts, health boards and CCG s in the same way as most other incidents that place patients at infection risk. Novel issues that arise with respect to CJD risk management and infection control, or difficulties with interpretation of current guidance, can be referred to the Public Health England CJD Section based in Colindale. If necessary, advice may be sought from the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Risk Management Sub-Group. Identification and management of patients at risk of Transmissible Spongiform Encephalopathy All patients undergoing elective and emergency surgery or endoscopy must be asked whether they have been notified that they are at increased risk of vcjd or CJD (see Appendix B for details) for public health purposes. Record patient s/relative s responses in the medical notes. Follow flow chart below: Page 12 of 64

13 Ask the patient if they have been notified that they are at increased risk of CJD or vcjd? Yes No / unable to respond Clarify specific details with patient Consult with Infection Prevention and Control Team Special infection control precautions are indicated for all surgery / endoscopy if in contract with medium / high risk infectivity tissues. 1 Contact with medium / high risk infectivity tissues? 1. Yes No Further risk assess CJD / vcjd infection using questions in Appendix B 2. Seek further guidance from the infection prevention and control team Proceed using standard infection control measures 1. See Appendix B for further information 2. If patients are unable to answer the questions in Appendix B then consult a family member to risk assess for CJD / vcjd. If this is not feasible then proceed with surgery / endoscopy and quarantine the instruments and seek advice from the infection control team. Page 13 of 64

14 9. Infection Control Measures Normal social or routine clinical contact with a patient with CJD or related disease, does not present a risk to healthcare workers, relatives or the community (DH, 2003). Isolation of patients with such diseases is therefore not necessary. They can be nursed on the open ward or at home. Personal Protective Equipment (PPE) As for all other patients, healthcare staff must wear PPE when in contact with body fluids, i.e. gloves and aprons, plus mask and eye protection when there is a risk of splashing to the face. This should be single use wherever possible. See Standard Precautions (incorporating Personal Protective Equipment) policy. Spillages of Blood and Body Spillages See Appendix M for use by Theatres. When a spillage of any fluid (including blood and CSF) from a patient with, or at increased risk of, CJD occurs in a healthcare setting, the main defence is efficient removal of the contaminating material and thorough cleaning of the surface. All spillages should be cleaned up promptly to minimize the risk of transmission of bloodborne viruses and other pathogens. If there is any delay in dealing with a spillage, e.g. staff with appropriate training are not readily available, the spillage must be safely cordoned off and/or the room locked. If in a public area, it must not be left unattended by staff. Spillage of Blood and Blood stained Body Fluid on impervious flooring Wear protective clothing (gloves, apron, goggles, footwear) Cover spillage with Actichlor add concentrate 10,000 ppm Leave for 5 minutes (prepare bucket with hot water and detergent solution) Scoop up the spillage with paper towels and discard as clinical waste into yellow bag Clean area with hot water and detergent using disposable cloths, rinse and dry Clean bucket in fresh water and detergent, rinse and dry Dispose of protective clothing and cloths/mop heads as clinical waste and wash hands NB: If a spill contains glass or other sharps, these should be picked up with forceps and disposed of carefully into a sharps bin. Use single use forceps wherever possible and dispose of in the clinical waste. (See Appendices L and M for use by Theatres.) Spillage of Low-risk Body Fluids, e.g. urine, vomits, onto any flooring or Blood Spillage on carpet Wear protective clothing (gloves, apron, goggles and footwear) Use paper towels to absorb as much of the spillage as possible Page 14 of 64

15 Clean area thoroughly using hot water and detergent and disposable cloths, rinse and dry Impervious flooring: wipe over the area with chlorine solution, e.g. Actichlor tablets made up to 10,000 ppm strength, and paper towels Dispose of all materials/mop heads as clinical waste Clean the bucket in fresh water and detergent, rinse and dry Dispose of protective clothing as clinical waste and wash hands Carpet: arrange for the carpet to be steam cleaned Disposal of Clinical Waste All clinical waste will be double bagged according to Trust policy in yellow bags and sent for destruction by incineration see table below. All sharps will be placed in a new sharps bin, which will be sealed and removed for incineration along with the other clinical waste. (See Waste Management policy). Disposal of Clinical Waste from patients with or at increased risk of CJD/vCJD Diagnosis of CJD High or medium risk tissue Low risk tissue and body fluids * Definite Incinerate Normal clinical waste disposal Probable Incinerate Normal clinical waste disposal At Increased Risk Incinerate Normal clinical waste disposal * Tissues and materials deemed to be low risk include body fluids such as urine, saliva, sputum, blood and faeces. Blood from vcjd patients is considered to be low risk except when transfused in large volumes. Accidental Inoculation Injuries For any accident involving sharps, or contamination of abrasions with blood or body fluid(s), wounds should be gently encouraged to bleed, gently washed (avoid scrubbing) with warm soapy water, rinsed, dried and covered with a waterproof dressing, or further treatment given appropriate to the type of injury. Splashes into the eyes or mouth should be dealt with by thorough irrigation. The accident should be reported via the Datix incident reporting system and responded to in line with the Sharps Prevention and Management of Needlestick/Sharps Injuries and Exposure to Body Fluids policy. Ward Guidelines A patient with CJD does not routinely require isolation as CJD is not transmissible from person to person by 'normal social contact or routine clinical contact. However, if heavy environmental contamination with blood is likely patients must be nursed in a side-room. No particular precautions, beyond routine infection control as used for all other patients i.e. standard precautions, are necessary, unless there is bleeding from the mouth, in which case disposable cutlery should be provided. There is no evidence of infectivity in saliva, body secretions or excreta. Sample-taking and other invasive medical procedures When taking samples or performing other invasive procedures the possible infectivity of the tissue(s) involved must be considered, and if necessary suitable precautions taken. Information on tissue infectivity for CJD is included in Appendix C of this guidance. It is Page 15 of 64

16 important to ensure that only trained staff, who are aware of the hazards, carry out invasive procedures that may lead to contact with medium or high risk tissue. Body secretions, body fluids (including saliva, blood, cerebrospinal fluid [CSF] and excreta) are all low risk for CJD. It is therefore likely that the majority of samples taken or procedures performed will be low risk. Contact with small volumes of blood (including inoculation injury) is considered low risk, though it is known that transfusion of large volumes of blood and blood components may lead to vcjd transmission. Blood and body fluid samples from patients with, or at increased risk of, CJD should be treated as potentially infectious for blood-borne viruses and handled with standard infection prevention and control precautions as for any other patient i.e.: use of disposable gloves and eye protection where splashing may occur avoidance of sharps injuries and other forms of parenteral exposure safe disposal of sharps and contaminated waste in line with locally approved arrangements single-use disposable equipment should be used wherever practicable When taking biopsy specimens of medium or high risk tissue, for example tonsil biopsy in a patient with suspected vcjd, or intestinal biopsy in a patient at increased risk of vcjd, every effort should be taken to minimise the risk of infecting the operator or contaminating the environment. Therefore these procedures should be undertaken in a theatre setting or a clinical room which can be deep cleaned after. Samples from patients with, or at increased risk of, CJD should be marked with a Biohazard label (Danger of Infection), and it is advisable to inform the laboratory in advance that a sample is being sent. Linen and Laundry All items of theatre linen including clothes and shoes will be treated as clinical waste. Single use linen should be used wherever possible. (See Appendices L and M for use by Theatres.) Patients clothes and linen can be washed as normal. Foul linen should be washed separately and be sent to the linen room in a RED alginate bag before placing into a CLEAR plastic bag for linen. Any re-usable linen must also be disposed of as clinical waste following a procedure involving any category of CJD patient. 10. Last Offices On the death of a patient the removal of the body from the ward to the mortuary should be carried out using normal infection control measures. It is recommended that the deceased patient be placed in a body bag prior to transportation to the mortuary, in line with normal procedures for bodies where there is a known infection risk. The outside of the body bag should be labeled with a danger of infection sticker. See Care of Patients at Death policy. Page 16 of 64

17 11. Post Mortem A post mortem is usually essential in order to confirm clinical diagnosis and the cause of death in patients with suspected CJD or vcjd. HHFT mortuaries currently do not perform examination of patients suspected of having a TSE. The responsible histopathologist will determine the need for further investigations and where they should be undertaken based on risk assessment above. For Post Mortem precautions follow the protocol for infectious cases. 12. Organ donation and transplantation Blood donation, organs or tissue for transplantation must not be taken from definite, probable, possible or at risk CJD or vcjd patients. In addition, members of ophthalmic teams must make specific enquiries to exclude patients with definite, probable, possible or at risk of CJD or vcjd before taking corneas from demented or psychiatric patients, or those who die from obscure undiagnosed neurological disease. 13. Health4Work Records It is recommended that healthcare workers who perform invasive procedures on, or handle potentially infectious material from any category of TSE patient have this recorded in their confidential occupational health clinical record. Exposure to handling contaminated or potentially contaminated medical devices or equipment must be minimized and all equipment used should be quarantined or contained for incineration by theatre staff, pathology staff or mortuary staff immediately after use. This can be done by management discussing the process with the healthcare worker and then writing to Health4Work giving the name, job title and date of birth of each healthcare worker. For example such record keeping is needed in the following situations (for healthcare workers who perform invasive procedures on, or handle potentially infectious material from any category of TSE patient see (Appendix J and K): All surgical procedures Processing of tissue specimens Post mortem If a healthcare worker needs occupational health advice regarding TSE they should contact Health4Work directly. In the above situations the manager of the department involved must: Page 17 of 64

18 Send to Health4Work a list of all staff directly involved, along with the nature of the procedure and each staff member s involvement. Inform each member of staff that they may attend Health4Work for advice if needed. 14. Surgical Procedures For Surgical Procedures see Appendix F For algorithm chart for precautions for surgical procedure on known, suspect or at risk patients: CJD other than variant CJD see Appendix G For algorithm chart for precautions for surgical procedures on known, suspect or at risk patients: variant CJD See Appendix H 15. Identification of a case of known or suspected TSE in a patient who has previously undergone invasive/surgical procedures In the event of the above happening the IPCT should be informed immediately. It is essential that sets of instruments and any additional items that were used are identified and held immediately in quarantine until a decision is made on how to proceed. Known patients Patients with a definitive diagnosis will need to have any equipment used for their procedure to be destroyed by incineration. Surgery should be avoided wherever possible for this category of patients. In the eventuality of such a patient needing surgery; see Appendices L and M for use by Theatres. Suspect patients Surgery on suspect patients should be avoided apart from life threatening situations. In the eventuality of such a patient needing surgery; see Appendices L and M for use by Theatres. At risk patients Sterile Services must not under any circumstances process any equipment that has been used on a known or suspected CJD case. 16. Re-usable Instruments This refers to instruments and equipment not designated as single-use or disposable. Where single-use instruments are not available reusable instruments may have to be used. These cannot just be decontaminated and reused in the usual way: they must be either destroyed or quarantined pending classification of the diagnosis, if there is any risk they may have come into contact with infectious tissue. The following table defines the action to be taken. Incineration of instruments The instruments should already be in a combustible sealed container. This should then be disposed of via the clinical waste stream, ensuring that this results in incineration. Page 18 of 64

19 Complex instruments Some expensive items of equipment, such as drills and operating microscopes, may be prevented from being contaminated by using shields, guards or coverings, so that the entire item does not need to be destroyed. In this case, the drill bit, other parts in contact with high or medium risk tissues, and the protective coverings, then need to be incinerated. However, in practice, it may be difficult to ensure effective protective covering, and advice should be sought from neurosurgical staff and the manufacturer to determine practicality. Handling of instruments patients with, or at increased risk of, CJD (other than vcjd) Status of Patient Tissue Infectivity Definitive or probable Possible At increased risk High* Brain Spinal cord Cranial nerves, specifically the entire optic nerve and the intracranial components of the other cranial nerves Cranial ganglia Posterior eye, specifically the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid and optic nerve Single use or Destroy or Quarantine for re-use exclusively on the same patient Single use or Destroy or Quarantine for re-use exclusively on the same patient pending diagnosis Single use or Destroy or Quarantine for re-use exclusively on the same patient Pituitary gland Medium Spinal ganglia Olfactory epithelium Single use or Destroy or Quarantine for re-use exclusively on the same patient Single use or Destroy or Quarantine for re-use exclusively on the same patient pending diagnosis Low No special precautions No special precautions Single use or Destroy or Quarantine for re-use exclusively on the same patient No special precautions Page 19 of 64

20 Handling of instruments patients with, or at increased risk of vcjd Status of Patient Tissue Infectivity Definitive or probable Possible At increased risk High* Brain Spinal cord Cranial nerves, specifically the entire optic nerve and the intracranial components of the other cranial nerves Cranial ganglia Posterior eye, specifically the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid and optic nerve Single use or Destroy or Quarantine for re-use exclusively on the same patient Single use or Destroy or Quarantine for re-use exclusively on the same patient pending diagnosis Single use or Destroy or Quarantine for re-use exclusively on the same patient Pituitary gland Medium Spinal ganglia Olfactory epithelium Tonsil Appendix Spleen Single use or Destroy or Quarantine for re-use exclusively on the same patient Single use or Destroy or Quarantine for re-use exclusively on the same patient pending diagnosis Single use or Destroy or Quarantine for re-use exclusively on the same patient Thymus Adrenal gland Lymph nodes and gut Associated lymphoid tissues Low No special precautions No special precautions No special precautions Page 20 of 64

21 *Although dura mater is designated low infectivity tissue, procedures conducted on intradural tissues (i.e. brain, spinal cord and intracranial sections of cranial nerves) or procedures in which human dura mater has been implanted in a patient prior to 1992, are high risk and instruments should be handled as such. NOTE: IT IS IMPERATIVE THAT INSTRUMENTS KNOWN TO BE OR POTENTIALLY BE CONTAMINATED BY CJD ARE NOT RETURNED TO TSSU. See Appendices L and M for use by Theatres. Forward planning by the medical team of any procedure and adherence to this policy is required to ensure that staff and patient safety is maintained: Equipping levels in the theatre to be kept to a minimum safe level with all unnecessary items removed prior to the procedure commencing Staffing levels in the theatre to be kept to a minimum level appropriate to the procedure TSSU staff to be notified Any sharps injury or personal contamination must be reported according to the Prevention and Management of Needlestick/Sharps injuries and Exposure to Body Fluids policy. 17. Theatre Procedure This section identifies the procedure for handling cases of known or suspected CJD cases within the operating theatres and has been formulated with the sole intention of managing the risks associated to exposure to known or suspected contaminants. Standard precautions regarding protective equipment apply to each risk group (no individual theatre is designated to carry out known suspect or at risk procedures): No Risk Possible risk Low Risk Tissue Possible Risk High/Medium Risk Tissue Instrumentation Follow routine procedure for the disposal of waste and reusable instrumentation via the Sterile Services department Follow routine procedure for the disposal of waste and reusable instrumentation via the Sterile Services department a. Wherever possible disposable instruments and medical devices should be used b. All non-disposable instruments must be handled as normal except that they must not be sent to SSD for reprocessing. Similarly no attempt must be made to reprocess or decontaminate any instrument(s) within the theatre environment c. All used instruments must be wrapped in their original packing drape and tray and then placed into two appropriately sized yellow clinical waste bags (double bag) Page 21 of 64

22 18. Pregnancy d. The container s lid must be fully secured and be labelled correctly with a Biohazard label Once contained and sealed the container will be quarantined in an appropriate area. See Appendices L and M for use by Theatres. In the event that a patient with, or at increased risk of, CJD becomes pregnant, it is important to ensure that patient confidentiality is properly maintained, and that any action taken to protect public health does not prejudice individual patient care. In the event that a patient with, or at increased risk of, CJD becomes pregnant, no additional infection prevention and control precautions need to be taken during the pregnancy. If a home delivery is decided upon, it is the responsibility of the midwife to ensure that any contaminated material is removed and disposed of in line with the procedures described above. 19. Specific Guidance related to Ophthalmology The risk of iatrogenic transmission of CJD during a surgical or diagnostic procedure is dependent on the risk of tissue infectivity and the nature of the procedure itself: Any posterior segment eye surgery or procedure is considered high risk Any anterior segment eye surgery or procedure is considered low risk Instruments that have been used in procedures involving tissues designated as high or medium infectivity (see above), on patients with known, suspected or at risk of CJD/vCJD should be disposed of by incineration or quarantined pending a confirmed diagnosis. Further guidance is contained in Annex L: Managing CJD/vCJD Risk in Ophthalmology, Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection 20. Specific Guidelines for Pathology Laboratories for the handling of tissues from TSE at risk or confirmed sources Specific national guidance for the handling of these tissues within the pathology laboratory setting is contained in the documents produced by the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Working Group, Transmissible spongiform encephalopathy agents: Safe working and the prevention of Infection: Annex K; nnex_k_-_guidelines_for_pathologist_and_pathology_laboratories.pdf These guidelines detail the relevant precautions that should be taken when handling tissues where possible CJD infectivity levels are deemed to be medium or high risk (see Appendix VI). This includes: Page 22 of 64

23 How to identify a potential case of CJD or vcjd prior to handling tissues from a living patient, or performing an autopsy on a patient with a history of dementia or a progressive neurodegenerative disorder The procedures for handling tissues of high or medium levels of infectivity from patients with, or at risk from, CJD or vcjd What to do in the event that a routinely handled tissue sample is subsequently found to be from a patient with, or at risk of, CJD or vcjd An algorithm is included in Appendix I of this policy to enable easy decision making on the treatment of tissue samples from patients with or at risk of CJD or vcjd, based on patient diagnosis and tissue infectivity risk. If the patient is symptomatic, or asymptomatic but at risk, then the appropriate staff must be informed prior to any procedures being carried out. The samples should be handled only by fully trained staff who are aware of the relevant laboratory handling and health and safety guidelines. Blood is classified as a low risk tissue and does not require special precautions for biochemical or cytological investigations. CSF is classified as a low risk tissue and does not require special precautions for biochemical or cytological investigations. Special precautions are required for trimming and fixing tissue and for microtomy work. It is advised that no frozen section work should be done on high risk tissues for patients with, or at risk of, CJD or vcjd. The situation may arise whereby a patient is not suspected of having CJD, but is subsequently diagnosed following a biopsy or autopsy. Local procedures should be enacted in consultation with the Infection Prevention and Control Team. This may require the identification and disposal of samples. Refer to the above National Guidance and the Infection Prevention and Management Team for further advice and support. 21. Stakeholders Engaged During Consultation Stakeholder Date of Consultation Infection Prevention and Control (Lead Infection Prevention & Control Author Nurse) Health and Safety (Health and Safety Advisor) 25/09/2015 Safeguarding (Trust Safeguarding Lead) 25/09/2015 Information Governance (Information Governance Manager) 25/09/2015 Assistant Risk and Compliance Manager (Risk and Compliance) 25/09/2015 Divisional Directors and Divisional Directors (Operational) 25/09/2015 Equality and Diversity Lead (Equality & Diversity) 25/09/2015 Head of Health4Work 25/09/2015 Infection Prevention and Control Committee 25/09/2015 Page 23 of 64

24 Consultant Microbiologists 25/09/2015 Clinical Service Managers/Leads 25/09/2015 Operational Service Managers 25/09/ Training Individuals in the Trust should receive annual infection prevention and control training to ensure they are aware of their responsibilities. Education and Training will be provided in accordance with the Trust Training Needs Analysis (Learning and Development Policy). 23. Monitoring Compliance with the Document Minimum requirements Effectiveness of policy Requirement Reviewed by Infection Prevention and Control Team Method of Monitoring Observation of practice Frequency of Review Should a case arise Committee where Monitoring is Reported to Decontamination Steering Group 24. References Transmissible Spongiform Encephalopathy agents: safe working and the prevention of infection Department of Health. London June Amended February 2015 Advisory Committee on Dangerous Pathogens, Spongiform Encephalopathy Advisory Committee (1998), Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection, The Stationery Office Department of Health. The decontamination of surgical instruments in the NHS in England update report: A Step Change, June 2005 Department of Health. Assessing the risk of vcjd via surgery: an interim review. Skipton House, London. March 2005 Glatzel M, Ott PM, Lindner T et al. Human prion disease. Epidemiology and integrated risk assessment. Lancet Neurol 2003;2: Department of Health Choice Framework for local Policy and Procedures Decontamination of flexible endoscopes: Policy and management 2/Decontamination_of_flexible_endoscopes.pdf 25. Associated Documentation Standard Precautions (incorporating Personal Protective Equipment) policy Linen policy Waste Management policy Page 24 of 64

25 Sharps Prevention and Management of Needlestick/Sharps Injuries and Exposure to Body Fluids policy Cleaning, Disinfection and Sterilisation policy Care of Patients at Death policy 26. Contributors Contributor Job Title DIPC Contributor Name Dr Kordo Saeed Page 25 of 64

26 Appendix A Equality Analysis Form To be completed by the Policy Author at the development stage of the policy and before consultation. Part 1 should be forwarded to an Equality Analysis Lead (list available on the Document Control Trust Intranet page) for sign off and any comments from them considered and addressed before seeking final approval of the policy. Document Name: Creutzfeldt-Jakob Disease (CJD) and other Transmissible Spongiform Encephalopathy Policy Part 1 Policy Author to complete and forward on to an EA Lead for sign off 1. Could the application of this document have a detrimental equality impact on individuals with any of the following protected characteristics? (See Note 1) Yes/No/ NA a Age No b Disability No c Gender reassignment No d Race No e Religion or belief No f Sex No g Sexual orientation No h Marriage & civil partnership No i Pregnancy and maternity No 2. If Yes to question 1, do you consider the detrimental impact to be valid, justifiable and lawful? If so, please explain your reasoning. Summarise the equality and diversity related elements within the policy 3. Specify with which, if any, individuals and groups you have consulted in reaching your decision. N/A Part 2 Equality Analysis Lead to complete and forward back to the Policy Author Provide a brief summary of the potential impact of the policy and whether sufficient consideration has been given to the Equality Duty. 1. Is this document recommended for publication? Yes If yes go to question 3 if No complete number 2 below. 2. This document is not recommended for publication because: Page 26 of 64