TRUST POLICY AND PROCEDURES FOR TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY (CREUTZFELDT-JAKOB DISEASE)

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1 TRUST POLICY AND PROCEDURES FOR TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY (CREUTZFELDT-JAKOB DISEASE) Reference Number CL-RM Version / Amendment History Version 3.2 Status Final Author: Dr Gnanarajah Helen Forrest Job Title Version Date Author Reason 1 Sept 2005 Infection Control Team Consultant microbiologist / Infection Control Doctor. Lead Nurse Infection Prevention and Control iginal Policy 2 April 2009 J Fletcher Review and reformat 3 January September February 2016 Dr Gnanarajah J Fletcher Dr Gnanarajah H. Forrest Dr Gnanarajah J. Halliwell Update and reformat Update Updated following review of new DH guidelines Intended Recipients: All medical and clinical staff, Associate Directors, Service Managers, Heads of Nursing, Clinical Governance Facilitators, and Matrons. Training and Dissemination: Dissemination via the Trust Intranet. Targeted training To be read in conjunction with: Trust Policy for the Decontamination of Reusable Medical Devices; Trust Policy and Procedures for Cleaning and Disinfection; Trust Policy for Standard Infection Control Precautions; Trust Policy and Procedures for the Care of the Cadaver; Trust Policy for the Packaging, Handling and Delivery of Laboratory Specimens. Inoculation Incident policy, Safe Sharps Policy, Hand Hygiene Policy, 1

2 In consultation with and Date: Infection Control Operational Group, Medical Advisory Committee, Theatre Risk Group, Decontamination Operational Group, Microbiology Laboratory, Infection Control Committee EIRA stage one Completed Yes Stage two Completed N/A Procedural Documentation Review Group Assurance and Date Approving Body and Date Approved Date of Issue Review Date and Frequency Contact for Review Executive Lead Signature Lead Nurse Infection Prevention and Control Director of Patient Experience & Chief Nurse Approving Executive Signature Director of Patient Experience & Chief Nurse Contents 2

3 Section 1 Introduction 2 Purpose and Outcomes 3 Definitions 4 Key Responsibilities/Duties 5 Managing the Policy and Procedures for of CJD/vCJD 5.1 Types and Methods of Transmission of of CJD/vCJD 5.2 Classification of Risk for CJD/vCJD 5.3 Handling of Instruments / Endoscopes 5.4 Identifying patients at risk of CJD/vCJD 5.5 Diagnosis and Treatment 5.6 Care of Symptomatic Patients or at Increased Risk of CJD/vCJD 5.7 Occupational Exposure 5.8 Surgery and Invasive Procedure 5.9 Special Consideration for Eye Surgery 5.10 Flexible Endoscopes 5.11 Death of a Patient 5.12 Laboratories 5.13 Dentistry 5.14 Look Back Exercise in the Event of a Newly Diagnosed Case of CJD/vCJD 6 Monitoring Compliance and Effectiveness 7 References 3

4 Appendices Appendix 1 Appendix 2 Appendix 2 Appendix 3 Appendix 4 Diagnostic Criteria Additional CJD risk questions for patients about to undergo elective or emergency procedures likely to involve contact with tissues of potentially high level infectivity Quarantining of Surgical Instruments and Endoscopes Distribution of TSE infectivity in human tissue & body fluid Common flexible endoscopic procedures classified as invasive or non-invasive 4

5 1. Introduction Transmissible Spongiform Encephalopathies (TSE s) are a group of rare, uniformly fatal degenerative disease affecting the central nervous system (CNS). Diseases caused by unconventional infectious agents called prions (infectious proteins). Once clinical signs appear there is no known treatment or prophylaxis. The only way to confirm diagnosis is by brain biopsy or post mortem. The human TSE s have a pre clinical phase which may last for many years (i.e. they do not show any symptoms of the disease). Creutzfeldt - Jakob disease (CJD) belongs to a group of diseases called TSE s. CJD accounts for 95% of all TSE s. For almost all routine clinical contacts no special precautions are required for the care of patients with or identified as being at risk of developing CJD/vCJD. TSE s including CJD/vCJD, exhibit unusual and extreme resistants to conventional decontamination methods such as heat (e.g. autoclaving) or chemical disinfection. Therefore, transmission may occur if a surgical instrument or flexible endoscope which has been in contact with infected tissue is subsequently reused on another patient. The level of infectivity of various tissues is outlined in appendix 4. All patients undergoing a surgical or endoscopic procedure involving medium or high risk tissue must be assessed for their risk of having CJD/vCJD, or of increased risk of developing CJD/vCJD, prior to their procedure being undertaken, in order to prevent such transmission taking place. Any patient identified as confirmed, probable or possible CJD/vCJD must undergo further assessment by the Infection Control Doctor/Consultant Microbiologist or Infection Prevention and Control Team regarding the level of tissue infectivity involved and the procedure to be undertaken. The Infection Control Doctor/Consultant Microbiologist or Infection Prevention and Control Team will provide advice on Infection Control Measures and identify which procedures require instruments or devices to be quarantined or disposed of. Patients who are known or suspected of having CJD/vCJD or other TSE infections should not undergo invasive procedures without prior discussion with the Infection Control Doctor/Consultant Microbiologist. Unless in an emergency. To prevent transmission of TSEs it is necessary to take a two stage risk based approach. First patients affected with or at risk of developing a TSE must be reliably identified, and second measures must be taken to ensure that any invasive device used on such patients are not used on other patients. 2. Purpose and Outcomes The purpose of this policy is to ensure that effective measures are in place to prevent patients, staff and others being put at risk of contracting transmissible spongiform encephalopathies, as a consequence of healthcare delivered by Derby Hospitals NHS Foundation Trust. 5

6 3. Definitions TSE Prions CJD vcjd Iatrogenic CJD Collective name for a group of degenerative diseases affecting the central nervous system Infectious proteins capable of transmission, which do not share the normal properties of viruses or bacteria and are resistant to conventional chemical and physical decontamination methods. A human form of transmissible spongiform encephalopathy (TSE) which causes a variety of neurological symptoms including dementia and personality changes. The outcome is fatal. A form of human CJD first identified in 1996, thought to have been linked to ingesting meat form cattle infected with Bovine Spongiform Encephalopathy (BSE). A form of CJD which occurs when CJD is accidently transmitted during medical or surgical procedures Table 1 4. Key Responsibilities/Duties Director of Infection Prevention and Control (DIPAC) Responsible for ensuring the policy is implemented within the organisation Responsible for notifying the Trust Board of any incidents Convene, Coordinate & lead incidence meetings as required Infection Control Doctor/Consultant Microbiologist Will provide advice on individual cases identified as confirmed, probable or possible CJD/vCJD and appropriate instrument / device management. Will refer complex queries to Public Health England Infection Prevention and Control Team Will assist clinical teams in taking appropriate infection control measures Identify which procedures require instruments or devices to be quarantined or disposed of. Ensure advice is documented within the patient medical records. Inform the consultant and theatre/endoscopy manager via Will maintain a CJD database and produce reports as required. Trust Decontamination Lead The nominated Decontamination Lead reports directly to the Executive via the Director of Patient Experience and Chief Nurse in exceptional circumstances, 6

7 normally reporting through the Infection Prevention and Control Committee, and is knowledgeable enough about decontamination matters to provide advice at that level. The Decontamination Lead is organisationally responsible for the effective, and technically compliant, provision of decontamination services within and for the Trust (as a contracted service). The Decontamination Lead is responsible for the operational policy for decontamination, its implementation and monitoring. The decontamination Lead will work in co-ordination with the Infection Prevention and Control team to ensure all relevant legislative standards are met and maintained. Infection Control Committee Will endorse the TSE (CJD) Policy and review at the agreed intervals Will provide the Board with assurances that effective control measures are in place throughout the Trust to minimise the risk and ensure patient safety Admitting Clinician / Consultant Will risk assess the possibility of CJD/vCJD in all patients undergoing surgery or endoscopy involving high or medium risk tissue and check medical notes / referral letter for any mention of CJD/vCJD status. Managers/Clinical Leads/ Matrons / Senior Sisters/ Charge Nurses Will ensure their own practice complies with this policy and advocate others to do so. As part of the routine surgical / endoscopic assessment procedure, will ensure all patients are assessed for risk of CJD / vcjd, at the earliest opportunity. Will ensure that the policy is implemented and complied with in their areas of responsibility. Will liaise with Infection Prevention and Control when an at risk patient has been identified. Theatre / Endoscopy Staff Will liaise with Infection Prevention and Control when an at risk patient has been identified. Will ensure that instrument identification labels are placed in the patients records following all surgical procedures. Will document in patient s records if single use instruments have been used or reusable instruments have been incinerated if a patient who is either known, suspected or at increased risk for public health purposes of having CJD/vCJD, is operated on. Ensure that all surgical instruments/endoscopes are tracked in line with the Decontamination policy and that instruments do not migrate between sets, especially if sets are used for procedures on the posterior eye and spinal surgery. Will ensure their practice complies with this policy. Individual Employees Are responsible for ensuring their own practice complies with this policy and encouraging others to do so. 7

8 Are responsible for ensuring infection prevention and control recommendation are implemented and adhered to. 5. Managing the Policy and Procedures for CJD 5.1 Types and Methods of Transmission of CJD There are four types of CJD, not all types have the same disease characteristics and are not necessarily transmitted in the same way. In addition, their infectivity varies at different phases of the disease. The risk of transmission is dependant on the type of procedure undertaken and the type of tissue involved. Classic or sporadic CJD It is the most common type and its cause is unknown. It is not thought to be found outside the brain. Subsequent transmission of the prion is thought to be instruments which have been contaminated by brain tissue or by the transplant of tissue itself e.g. dura mater. Familial CJD This type is very rare and is caused by an inherited abnormality in the gene that produces normal protein. In the majority of cases this is known within the family because of the family history. Subsequent transmission is the same as for sporadic CJD. Iatrogenic CJD Caused by medical procedures or treatments. It has been shown to be transmitted by procedures such as injections with human pituitary hormones, dura mater grafts, neurosurgical instruments and blood / blood products. Variant CJD (vcjd) - this was first recognised in 1996 and generally affects young adults. It is associated with the same transmissible agent that causes Bovine Spongioform Encephalopathy (BSE) in cattle. Primary infection is thought to be caused by consuming BSE contaminated food products. This prion is found throughout the body in the lymphatic tissue and central nervous system. Transmission may occur via instruments contaminated with infective tissue and following transfusion of contaminated blood or blood products. 5.2 Classification of Risk for CJD/vCJD A distinction should be made between symptomatic patients i.e. those fulfilling the diagnostic criteria for definite, probable or possible CJD/vCJD and patients potentially at increased risk of developing CJD or vcjd because of their family or medical history. The following table 2 details the classification of risk status in descending order of risk. 8

9 Patient groups Symptomatic patients (this could be definite, probable or possible Patients at increased risk from genetic forms of CJD Categories of patient Patients who fulfil the diagnostic criteria for definite, probably or possible CJD or vcjd Patients with neurological disease of unknown aetiology 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/other prion disease. Individuals who have a blood relative known to have a genetic mutation indicative of familial CJD Individuals who have or have had two or more first generation blood relatives (e.g. parents, siblings, grandparents) affected by CJD or other prion disease Patients identified as at increased risk of vcjd through receipt of blood from a donor who later develops vcjd Patients identified as at increased risk of CJD/vCJD through iatrogenic procedures Individuals who have received labile blood components (whole blood, red blood cells, white blood cells or platelets), from a donor who later went onto 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 use of human derived gonadotrophin was discontinued in 1973, and use of cadaverderived human growth hormone was banned in However, use of human derived products may have continued in other countries after these dates Individuals who underwent intradural neurosurgical or intradural spinal procedures 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). Patients 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 since January Individuals who have received blood from someone who went on to develop vcjd Individuals who have given blood to someone who went on to 9

10 develop vcjd Individuals who have received blood from someone who has also given blood to a patient who went on to develop vcjd Individuals who have been treated with certain implicated UK source plasma products between 1990 and 2001 Table 2 Recipients of ocular transplants, including corneal transplants, are not considered to be at increased risk of CJD/vCJD. All people identified at increased risk of developing CJD/vCJD are asked to help prevent any further possible transmission by: - Not donating blood - Not donating organs or tissues, including bone marrow, sperm, eggs or breast milk - Informing whoever is treating them if they are to undergo any medical, surgical or dental procedure - Informing family members so that they can pass the information on if required and the person is unable to do so. G.P.s are asked to record their patient s CJD/vCJD risk status in their primary care records. The GP should also include this information in any referral letter should the patient require any medical, surgical and dental procedures. 5.3 Handling of Instruments / Endoscopes Patients with or at increased risk: of CJD (other than vcjd) Status of patient Tissue Infectivity Definite / probable Possible At increased risk HIGH Brain Spinal cord Dura mater Cranial nerves, specifically the entire optic nerve and the intracranial components of other cranial nerves Cranial ganglia Posterior eye, specifically the posterior hyaloids face, retina, retinal pigment epithelium, choroids, sub retinal fluid and optic nerve Pituitary gland Single use Destroy Quarantine (Discuss with Infection Prevention and Control Team) Single use Quarantine pending diagnosis Single use Destroy Quarantine (Discuss with Infection Prevention and Control Team) MEDIUM Spinal Ganglia Single use Single use Single use 10

11 Olfactory epithelium Destroy Quarantine pending diagnosis Destroy Quarantine Quarantine (Discuss with Infection Prevention and Control Team) (Discuss with Infection Prevention and Control Team) LOW No special precautions No special precautions No special precautions Table 3 For the purposes of this policy lymphoid tissue refers to the spleen, thymus, tonsils and adenoids, lymph nodes, the appendix and the gastro-intestinal tract sub-mucosa (see appendix 4) Patients with, or at increased risk of vcjd Status of patient Tissue Infectivity Definite /probable Possible At increased risk HIGH Brain Single use Single use Single use Spinal cord Dura mater Cranial nerves, specifically the entire optic nerve and the intracranial components of other cranial nerves Cranial ganglia Destroy Quarantine (Discuss with Infection Prevention and Control Team) Quarantine pending diagnosis Destroy Quarantine (Discuss with Infection Prevention and Control Team) Posterior eye, specifically the posterior hyaloids face, retina, retinal pigment epithelium, choroids, sub retinal fluid and optic nerve Pituitary gland MEDIUM Spinal Ganglia Single use Single use Single use 11

12 Olfactory epithelium Tonsil Appendix Destroy Quarantine pending diagnosis Destroy Spleen Quarantine Quarantine Thymus Adrenal gland (Discuss with Infection Prevention and Control Team) (Discuss with Infection Prevention and Control Team) Lymph nodes and other organised lymphoid tissues containing follicular structures. Gut associated lymphoid tissue LOW Table 4. No special precautions No special precautions No special precautions 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. 5.4 Identifying patients at risk of CJD/vCJD See appendix 2 for further information In order to prevent the transmission of CJD / vcjd in the healthcare setting it is of the utmost importance that all patients are assessed for risk factors according to Department of Health criteria. All patients about to undergo any elective or emergency surgical or endoscopic procedure (excluding arthroscopy) should be asked the following questions at the earliest opportunity: - Have you ever been notified that you are at increased risk of CJD or vcjd for public health purposes? - Have you received multiple (300 or more donors) transfusions of blood or blood components (red cells, cryoprecipitate or platelets) or plasma (150 or more donors) since January 1990 Patients who are to undergo ophthalmic surgery to the posterior eye and spinal surgery should be asked - Have you ever been notified that you are at increased risk of CJD or vcjd for public health purposes? 12

13 - Have you received multiple (300 or more donors) transfusions of blood or blood components (red cells, cryoprecipitate or platelets) or plasma (150 or more donors) since January Have you ever received growth hormone or gonadotrophic treatment? If yes please specify: o o o Whether the hormone was derived from human pituitary glands The year of treatment Whether the treatment was received in the UK or in another country - Have you had surgery to your brain or spinal cord before August 1992? - Do you have a family history of CJD or other prion disease? Note: This does not include autologous transfusion or plasma products such as IVIG, albumin, coagulation factors and anti-d. If the patient answers yes to any of the above questions, the consultant in charge of the patient and the Infection Control Doctor/Consultant Microbiologist or Infection Prevention and Control Team must be informed, prior to the procedure taking place, in order to provide further assessment of the procedure to be undertaken, and guidance on surgical instrument / endoscope management. Infection Prevention & Control team can be contacted Monday Friday (8.30 5pm) extension or page Out of hours, weekends & bank holidays contact the on-call microbiologist via switch board. These questions must be asked at the earliest possible opportunity, e.g. when the decision for admission / procedure is made, in order to enable correct and timely management of any risks identified. Identified risks must be documented in the medical records. In the event that a patient is unable to answer any questions a family member or someone close to the patient should be asked the CJD risk questions prior to surgery / endoscopy. If the family member, or someone close to the patient, is not able to provide a definitive answer to the CJD risk questions, the patients GP should be contacted and enquiries made to the patients risk identified at the earliest opportunity. If surgery / endoscopy must proceed before this information is gained all instruments / endoscopes should be quarantined until risk factors for CJD/vCJD are identified. 5.5 Diagnosis and Treatment For symptomatic cases there are internationally accepted diagnostic criteria for definite, probable and possible CJD/vCJD. The diagnosis of CJD/vCJD is beyond the scope of this policy. Patients suspected of having CJD or vcjd must be referred to a neurologist. 13

14 All diagnostic specimens must be labelled as high risk and the suspected condition identified. Specific laboratory investigations for the diagnosis of CJD must be discussed with a Consultant Microbiologist and the NCJDSU before any specimens are taken. Contact details for the NCJDSU are: The National Creutzfeldt-Jakob Disease Surveillance Unit Western General Hospital Crewe Road Edinburgh EH4 2XU Telephone Clinical Office Pathology Care of Symptomatic Patients or at Increased Risk of CJD/vCJD General Considerations No additional precautions are needed for most routine clinical contacts. Epidemiological evidence does not suggest that normal social or routine clinical contact with a CJD / vcjd patient represents any risk to healthcare workers, relatives and others in the community. However, when certain invasive procedures are performed there is the potential for exposure. Although there is no evidence of any transmission it is essential that control measures are in place to prevent iatrogenic transmission, through good standard infection control practices, with the control of sharps and protective equipment to prevent splashing and droplet contamination of mucosal surfaces Communication If any healthcare worker becomes aware of a patient falling into any of the risk categories, who was previously unknown, they should urgently inform the Infection Control Doctor/Consultant Microbiologist or Infection Prevention and Control Team. Surgical or endoscopic procedures should not be undertaken until advice has been obtained and appropriate measures have been put into place. In emergencies, where delay is not possible, all instruments and endoscopes must be quarantined until advice on processing or disposal is obtained Body Fluids There is no evidence of infectivity in saliva, body secretions or excreta. Any potential exposure to these body fluids should be handled in line with the standard infection control precautions policy Specimens for Pathology Due to the unusual resistance of CJD/vCJD, single use disposable equipment should be used wherever practicable when obtaining specimens and all other equipment contaminated whilst collecting specimens should be destroyed by incineration. Discussion with infection prevention and control prior to any invasive procedure, e.g. obtaining a biopsy, is required. 14

15 Biopsy and lumbar puncture samples should only be taken by trained personnel who are aware of the hazards involved. The collection of blood samples should involve the same precautions normally used for venepuncture. Particular care should be taken while obtaining and handling CSF and lymphoid tissue specimens. Eye protection should be worn for procedures where splashing may occur. The agents of CJD/vCJD are classified as Hazard Group 3 pathogens by the Advisory Committee on Dangerous Pathogens. For this reason all specimens from known or at risk patients must be labelled with a risk of infection sticker. The laboratory should be informed in advance that the sample is being sent. For CJD CSF s The Microbiology laboratory will freeze the sample ready for collection by Edinburgh. Once Edinburgh has received the paperwork from the Clinical Team they telephone the named contact on the form to arrange for collection using their own courier. The laboratory will also do a cell count on a portion of the CFS beforehand as Edinburgh need this information for their investigations. The laboratory is also responsible for correct disposal of the sample Spillages The infectious agents associated with CJD/vCJD are usually resistant to inactivation techniques. Dilution is the most important element in cleaning up spillages on a hospital ward. Exposure to high concentration (20,000ppm available chlorine) sodium hypochlorite for one hour is known to be effective. Its use is not practical in a ward setting as it is corrosive to many surfaces; however it can be used in exceptional circumstances. Advice should be sought from the Infection Prevention and Control Team. Normal standard infection control precautions should be followed to clean up spillages, including spillages of blood and cerebrospinal fluid. Potentially infectious material should be removed using absorbent material and any waste, including cleaning tools such as mop heads, disposed of as infectious waste for incineration. Disposable gloves and apron should be worn when removing spillages, eye protection should be worn for procedures where splashing may occur Childbirth Childbirth should be managed using standard infection control precautions. The placenta and other associated material and fluids should be treated as if infected and disposed of as infectious waste unless they are needed for investigation Waste Management Waste generated whilst caring for patients with or at risk of CJD/vCJD is unlikely to contain high risk material. It should be disposed of as normal offensive waste Bed Linen and Crockery No special precautions are needed. Linen and crockery can be handled in line with normal policies Room Cleaning 15

16 There are no additional requirements above normal room cleaning or terminal disinfection. 5.7 Occupational Exposure Although cases of CJD/vCJD have been reported in healthcare workers there have been no confirmed cases linked to occupational exposure. It is, however, prudent to take a precautionary approach. The highest potential risk in the context of occupational exposure is from exposure to high infectivity tissues through direct inoculation (e.g. as a result of an inoculation injury) and exposure of the mucous membranes. Compliance with standard infection control precautions is sufficient. Clinical or laboratory personnel who work with patients with known of suspected CJD/vCJD or with potentially infected tissues should be informed about the nature of the risk and the relevant safety procedures. The inoculation incident policy must be followed for any accidents involving sharps, or contamination of abrasions with blood or body fluid(s). 5.8 Surgery and Invasive Procedures General Measures Communication All staff directly involved in invasive procedures on patients in the risk groups for CJD/vCJD, or in the subsequent re-processing or disposal of potentially contaminated items, should be made aware of the specific precautions required. These staff should also be made aware of any clinical intervention in sufficient time to allow the necessary preparations for the procedure. This will allow time to obtain the most suitable instruments and equipment. Single use disposable items should always be used wherever possible Instrument Tracking All reusable instruments and invasive devices (including endoscopes) must be subject to tracking procedures. Tracking provides a permanent record to indicate which instrument or device is used on which patient. Tracking also links each device to a validated decontamination cycle prior to use. The tracking system must be capable of identification of instruments used on a particular patient and also the patients on whom the same instruments had subsequently been used. In the case of surgical instruments tracking may be a record of individually marked instruments or alternatively of instruments used and processed together. Sets of instruments should be managed to minimise migration of instruments between sets. Instrument migration in ophthalmology sets used on the posterior eye should be reduced to zero. NICE guidance emphasises the need for a separate pool of instruments for high risk procedures on children born since January 1997 and who have not previously undergone high risk procedures Specific Precautions for Symptomatic Patients and Asymptomatic Patients Potentially at Risk of CJD 16

17 The measures to be taken when performing invasive surgery depend on how likely the patient is to be carrying the infectious agent and how likely it is that infection could be transmitted by the procedure being carried out Precautionary Measures for Surgical Procedures Theatre Management For all symptomatic patients and for asymptomatic patients at risk from familial or iatrogenic CJD/vCJD, the following precautions should be taken when high or medium risk tissues are encountered during the procedure, or as advised by the Infection Control Doctor/Consultant Microbiologist or Infection Prevention and Control Team: Wherever appropriate and possible, the intervention should be performed in an operating theatre Where procedures are performed at the bedside e.g. lumbar puncture, care should be taken to ensure the environment may be readily cleaned should a spillage occur. The protective equipment described below should be worn by healthcare personnel performing diagnostic procedures. Where possible procedures should be performed at the end of the list, to allow normal cleaning and drying of theatre surfaces before the next session. Only the minimum number of healthcare personnel required should be involved. The following single use protective equipment should be worn: - liquid repellent single use operating gown, over a plastic apron - gloves - mask and goggles or full face visor use single use disposable surgical instruments and equipment where possible destroy all single use instruments by incineration the principles for reducing the risks from percutaneous exposure to blood borne viruses apply equally to CJD Storage of Instruments for Research Purposes Instruments that may have been in contact with CJD may be required for research purposes. Contact the following for advice: Surgical Instrument Storage Facility Centre for Preparedness and Emergency Response Public Health England Porton Down Salisbury Wiltshire SP4 OJG Tel:

18 This facility will send appropriate packaging and despatch instructions to the hospital to facilitate their transport to them. If the above facility does not require the instruments, they should be disposed of by incineration Complex Instruments and Medical Devices Some expensive items of equipment may be prevented from being contaminated by using shields, guards or coverings, so the entire item does not need to be destroyed. The parts in contact with high risk tissue and the protective covering would need to be incinerated. However, in practice it may be difficult to ensure effective protective covering and advice should be sought from specialist staff, including the national CJD incident panel and the manufacturer to determine practicality Use of Laser for Tonsillectomy: Smoke Plumes Laser techniques are sometimes used as an alternative to conventional surgery for tonsillectomy. There is no evidence of the transmission of CJD/vCJD by the respiratory route. Any risk to surgeons from smoke flumes is thought to be very low, but there is not data for vcjd. 5.9 Special Considerations for Eye Surgery Posterior segment eye surgery or procedure is defined as any surgery or procedure that involves potential contact with the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid and optic nerve. Due to the high incidence of subretinal fluid drainage performed either intentionally or inadvertently during scleral buckling surgery, this form of surgery is considered as posterior segment surgery.tissues in the posterior eye are considered high risk for CJD/vCJD. Any posterior segment eye surgery or procedure is considered high risk. All patients who have procedures on the posterior eye and other high risk tissues must have the extended risk assessment questions asked prior to surgery. Precautions must be taken to ensure that migration between sets of instruments used on high risk tissue is zero. Where possible individually identified instruments should be used and consideration given to using single use instruments if practicable. Anterior segment surgery or procedure is defined as any surgery or procedure involving ocular tissues other than those stated above, including: o Ocular adnexal tissue including eyelids, periorbital tissue and lacrimal system o Conjunctiva o Cornea and limbus o Iris o Crystalline lens o Anterior vitreous (excluding the posterior hyaloid face) o Anterior vitrectomy performed via the cornea o Extra-ocular muscle surgery o Ciliary body 18

19 o o Sclera (but not if allogeneic sclera used) Tissues of the orbit except optic nerve Any anterior segment eye surgery or procedure is considered low risk 5.10 Flexible Endoscopes General Considerations There is currently no evidence that vcjd has been transmitted from one patient to another via an endoscopic procedure. However, because lymphoid tissue of the gastro-intestinal tract sub-mucosa is considered a medium infectivity tissue, investigation of known or suspected patients has implication for all patients requiring endoscopy. Flexible endoscopes contaminated with prion containing material cannot be completely reliably decontaminated using current methods. The risks of transmission are not confined to the endoscope itself but also accessories such as biopsy forceps which have an equal or even greater risk as they are less easily cleaned. As a consequence, disposable accessory equipment is now advised wherever possible Decontamination of endoscopes In order to decrease the risk of transmission of CJD/vCJD through endoscopic procedures, additional precautions for the decontamination of flexible endoscopes used in all patients known or suspected of having CJD/vCJD, and in those identified as at risk of developing CJD/vCJD, are recommended and general precautions are reinforced : a) Channel cleaning brushes and, if a biopsy has been taken, the valve on the biopsy/instrument channel port used with flexible endoscopes should be disposed of as infectious waste after each use. Single use, disposable biopsy forceps should be used routinely in all patients. Accessories should be single-use wherever possible, but where this is not possible, they should be kept together with the endoscope, forming a unique set, until the accessories are disposed of. It is essential to have systems in place that enable endoscopes, together with all re-usable accessories, to be traced to the patients on whom they have been used. b) Endoscopes used for certain procedures in individuals with possible CJD/ vcjd, or in whom the diagnosis is unclear, should be removed from use or quarantined pending diagnosis or exclusion of CJD/vCJD. c) Disinfectants with fixative properties should not be used on flexible endoscopes used for any procedure Precautionary measures for endoscopic procedures Gastrointestinal endoscopy on symptomatic or asymptomatic patients with, or at risk of, TSE other than vcjd will not involve high or medium risk tissues. Therefore no special precautions are necessary during or after the procedure, and the endoscopes should be cleaned and disinfected in the normal thorough way. 19

20 Prion protein has been detected in the olfactory epithelium of sporadic CJD patients. Endoscopic procedures involving the nasal cavity may come into contact with olfactory epithelium. If nasal endoscopy is performed on patients in table 2, the infection Prevention & Control Team should be contacted for advice. Following consultation with the person carrying out the endoscopic procedure, the risk of contamination of the endoscope with olfactory epithelium can be assessed. If contamination cannot be excluded precautions appropriate for medium infectivity tissues must be taken, a single use endoscope or destruction of the endoscope following the procedure. As lymphoid follicles and germinal centres are widely distributed in the gastrointestinal tract and other mucosal surfaces, endoscopy on patients may involve medium risk tissues, particularly if biopsy forceps are used. Patients with definite or probable vcjd should only undergo flexible endoscopy if this is deemed a clinical necessity. It may be possible to loan an instrument from the National CJD Surveillance Unit the Infection Control Doctor/Consultant Microbiologist or Infection Prevention and Control Team will advise. If a tissue biopsy or other invasive procedure (e.g. ERCP or diathermy) is performed on a patient with or at risk of vcjd the endoscope must be assumed to be potentially contaminated with lymphoid tissues. This applies to upper and lower gastrointestinal endoscopy, bronchoscopy and any other procedure where a flexible endoscope is used to obtain a tissue biopsy or another invasive procedure is performed. Therefore, endoscopic biopsies should only be performed if the results are deemed to be sufficiently important to warrant quarantine of the endoscope. Taking random biopsies in a patient with or at risk of vcjd is unacceptable clinical practice. However, the fundamental principle that patient care must remain unaffected is paramount. If a patient with suspected vcjd is inadvertently endoscoped, the IPCT must be called urgently, and the instrument quarantined. If a patient, who has been previously endoscoped, subsequently develops vcjd, the Infection Control Doctor/Consultant Microbiologist or Infection Prevention and Control Team must be called urgently and the instrument quarantined. If endoscopy was performed without biopsy (and without contact with nasal tissues or central nervous system tissues) this can be regarded as a low risk procedure. All endoscopes should have a unique identifier, and this must be recorded in the notes of the patient undergoing endoscopy with that instrument. This will enable a look back exercise to be performed if necessary Death of patient National guidelines recommend that patients with CJD/vCJD or at risk of CJD/vCJD, need to be placed in a cadaver bag and to have labels attached to both the deceased and the outside of the bag stating Risk of Infection. Viewing of patients can take place Mortuary Post-mortem examinations are required in order to confirm a clinical diagnosis and the cause of death in patients with suspected CJD/vCJD. However, such procedures have the potential to expose pathologists and mortuary staff to tissues containing high levels of infectivity. 20

21 If a post mortem is required for anyone who falls into the risk groups arrangements should be made for the body to be removed to a regional neuropathology centre where specialist expertise exists. Liaison between local pathologists and the National CJD Surveillance Unit is preferable Laboratories General Considerations All TSE agents, except scrapie, are all classified as Hazard Group (HG) 3, as listed in the Health and Safety commission s Approved List of Biological Agents. In general the Control of Substances Hazardous to Health (COSHH) Regulations 2002 require that when working with an agent the laboratory containment level must match the hazard group of the agent. Most specimens examined from patients diagnosed with, or at risk of, a TSE are likely to carry a very low risk of infection for laboratory or other Trust staff. For this reason most specimens can be processed using standard operating procedures under normal containment for the specimen being examined Specimens and Request forms All specimens from patients, who are diagnosed with, or at risk of, a TSE, must be labelled as risk of infection, with details of diagnosis and examination required. The relevant laboratory must be contacted in advance for advice before submitting tissue specimens and CSF for examination Dentistry The risks of transmission of infection from dental instruments are thought to be very low provided optimal standards of infection control and decontamination are maintained. Dental instruments used on patients can be handled in the same way as those used in any other low risk surgery, i.e. taking a precautionary approach, these instruments can be reprocessed according to best practice and returned to use. Optimal reprocessing standards must be observed Look Back Exercise in the Event of Newly Diagnosed Cases of CJD / vcjd Where there is a possibility that a patient could have been exposed to CJD/vCJD through contaminated surgical instruments that were previously used on a patient with or at increased risk of CJD/vCJD the infection prevention and control team must be informed. A look-back exercise may be required. Advice will be given by the Consultant Microbiologist and the local Public Health England Tracing of Instruments To enable a look back to be effective every individual instrument set must be uniquely numbered to enable it to be withdrawn from a cohort of sets of the same name. It is essential the tracing system is efficiently managed and easily interrogated; records should be retained for at least 11 years. 21

22 6 Monitoring Compliance and Effectiveness Monitoring Requirement : Incidents where non-compliance with this policy is noted should be reported via the incident reporting system. Incidents pertaining to Infection Prevention and Control are monitored at the Infection Control Committee. Any non-compliance issues will be reported to the division Matron or the site manager as appropriate. Annual cross Trust process compliance assurance audits. Monitoring Method: Report Prepared by: Monitoring Report presented to: Frequency of Report Annual cross Trust process compliance assurance audits will be undertaken by the Infection Prevention & Control Team Infection Prevention & Control Team Infection Control Committee Annually 22

23 7 References Guidance Minimise transmission risk of CJD and vcjd in healthcare settings Department of Health (2006). Creutzfeldt Jakob Disease (CJD) DoH, London National Institute for Health and Clinical Excellence (2008) Patient Safety and reduction of risk of transmission of CJD via interventional procedures NICE CJD guidance Creutzfeldt-Jakob Disease: Guidance for Healthcare Workers is available at ntrolv3.0.pdf Transmissible Spongiform Encephalopathy Agents: Safe working and the prevention of infection, Department of Health. June 2006, updated December

24 Diagnostic Criteria Appendix 1 1. Symptomatic Patients Those who fulfil internationally accepted diagnostic criteria set out below for definite, probable and possible CJD/vCJD. Neuropathological / immunocytochemical confirmation is required for a diagnosis of definite sporadic CJD. 2. Sporadic CJD Probable sporadic CJD patients will have rapidly progressive dementia, and at least two of the following four symptoms: a) Myoclonus b) Visual or cerebellar problems c) Pyramidal or extra pyramidal feature d) Akinetic mutism Plus typical electroencephalogram (EEG) or clinical criteria for possible sporadic CJD (see below) and a positive assay in the cerebrospinal fluid (CSF). Possible sporadic CJD patients will have rapidly progressive dementia, two of the abovesymptoms (a-d) and duration of less than two years. 3. Iatrogenic CJD Iatrogenic CJD patients display progressive cerebellar syndrome in a pituitary hormone recipient or sporadic CJD with a recognised exposure risk (e.g. dura mater transplant). A definitive diagnosis of iatrogenic CJD still requires a neuropathological examination. 4. Familial CJD Patients with familial CJD will have definite or probable CJD (see definitions above), plus definite or probable CJD in a first degree relative (i.e. a parent, child or sibling) or a neuropsychiatric disorder plus a disease specific mutation in the prion protein gene. 5. Definite vcjd Patients Will have a progressive neuropsychiatric disorder and neuropathological confirmation of the disease, showing spongiform change and extensive PrPC deposition with florid plaques throughout the cerebrum and cerebellum. 6. Probable vcjd Patients Can be classified under two sets of criteria: a) They will have progressive neuropsychiatric disorder of duration greater than six months where routine investigations do not suggest an alternative diagnosis. They will also have at least four of the following five symptoms: 1. Early psychiatric symptoms (depression, anxiety, apathy withdrawal, delusions). 2. Persistent painful sensory symptoms (frank pain and/or unpleasant dysaethesia. 3. Ataxia 4. Monoclonus, chorea or dystonia 5. Dementia 6. These patients would have had no history of potential iatrogenic exposure b) Alternatively, a probable vcjd patient will have had a progressive neuropsychiatric disorder for a period of longer than six months, where routine investigations do not support an alternative diagnosis, and where there is no history of potential of iatrogenic exposure, plus a positive tonsil biopsy which is positive for PrP-res. Possible vcjd patients will have progressive neuropsychiatric disorder of duration greater than six months where routine investigations do not suggest an alternative diagnosis, and no history of potential iatrogenic exposure. They will also have at least four out of five of the symptoms listed above and an EEG does not show the typical appearance of sporadic CJD or no EEG has been performed. 24

25 Appendix 2 additional CJD risk questions for patients about to undergo elective or emergency procedures likely to involve contact with tissues of potentially high level infectivity 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? Notes to clinician Patients should be considered to be at risk from genetic forms of CJD if they have or have had: 1. Genetic testing, which has indicated that they are at significant risk of developing CJD or other prion disease; 2. A blood relative known to have a genetic mutation indicative of genetic CJD or other prion disease; 3. 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 humanderived growth hormone was discontinued in 1985 but humanderived 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) Patients who underwent intradural neurosurgical or spinal procedures before August 1992 may have received a graft of human-derived dura mater and should be treated as at increased risk, unless evidence can be provided that humanderived dura mater was not used. Patients who received a graft of human-derived dura mater before 1992 are at increased risk of transmission of sporadic CJD, but not vcjd. 25

26 Appendix 3 Quarantining of Surgical Instruments and Endoscopes Instruments that have been used in procedures involving tissues designated as high or medium infectivity, on a possible CJD/vCJD patient, can be quarantined pending a confirmed diagnosis. This appendix provides guidance on the procedures which should be followed when quarantining surgical instruments or endoscopes. After completion of a surgical procedure on a possible CJD/vCJD patient, single-use instruments should be separated and disposed of by incineration. Re-usable instruments should be washed to remove gross soil, using detergent as standard. Care should be taken to avoid splashing and generating aerosols, by holding instruments below the surface of the water in a sink into which water is running and draining out continuously. Instruments should not be held directly under a flowing tap, as this is likely to generate splashes. Operatives should wear protective gloves and either a visor or goggles, and care must be taken to avoid penetrating injuries. After washing, instruments should be placed on a disposable instrument tray and allowed to air-dry. They should then be placed in an impervious rigid plastic container with a closefitting lid. The lid should be sealed with heavy duty tape (e.g. autoclave tape) and labelled with the patient s identification details (i.e. name, date of birth and hospital number). The label should also state the surgical procedure in which the instruments were used and the name of the responsible person. The instrument tray should be disposed of by incineration. The sealed box can be stored indefinitely until the outcomes of any further investigations are known. If the patient is confirmed as suffering from CJD/vCJD, the box and its contents should be incinerated without any further examination. Endoscopes other than those used in the CNS and nasal cavity, which have been used for invasive procedures in individuals designated as at risk of vcjd should be removed from use or quarantined to be re-used exclusively on the same individual patient if required. The principles behind the procedures recommended for quarantining of surgical instruments should be followed except the endoscope should be fully cleaned and decontaminated immediately after use, before being quarantined. The endoscope should be decontaminated alone using an Automatic Endoscope Reprocessor (AER). The AER should then be run empty for a cycle. If an alternative diagnosis is confirmed, the instruments or endoscopes may be removed from the box by the responsible person (or a named deputy) and reprocessed according to best practice and returned to use. Additional decontamination procedures are not required. Records must be kept of all decisions, and the Sterile Service Department (SSD) must be informed. 26