Portsmouth Hospitals Procedural Document Template

CREUTZFELDT JAKOB DISEASE (CJD) POLICY MANAGEMENT OF PATIENTS WITH OR SUSPECTED OF HAVING

Version 3

Name of responsible (ratifying) committee Infection Prevention Management Committee

Date ratified 28 January 2015 Consultant Infection Prevention & Control, Document Manager (job title) Decontamination Manager Date issued 03 February 2015

Review date 01 January 2017

Electronic location Infection Control Policies Decontamination of reusable medical devices, Related Procedural Documents Infection Control, Standard Precautions Transmissible spongiform encephalopathies (TSE’s), Creutzfeldt-Jakob Disease (CJD), variant CJD (vCJD), Key Words (to aid with searching) Safe Working and Prevention of Infection, Distribution of infectivity in tissues, principles of decontamination and quarantining of medical devices.

Version Tracking Version Date Ratified Brief Summary of Changes Author 3 28/01/2015 Amended following updates to guidance D. Carter / C. Mitchell

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 1 of 23 CONTENTS

QUICK REFERENCE GUIDE...... 3 1. INTRODUCTION/BACKGROUND...... 4 2. PURPOSE...... 4 3. SCOPE...... 4 4. DEFINITIONS...... 5 5. DUTIES AND RESPONSIBILITIES...... 5 6. PROCESS...... 6 7. TRAINING REQUIREMENTS...... 11 8. REFERENCES AND ASSOCIATED GUIDANCE...... 11 9. EQUALITY IMPACT STATEMENT...... 12 10. MONITORING COMPLIANCE WITH PROCEDURAL DOCUMENTS...... 13

APPENDIX I: Diagnostic criteria for CJD...... 14 APPENDIX II: Risk categorization of patients having CJD / vCJD...... 16 APPENDIX III: CJD risk assessment questionnaire for patients about to undergo elective or emergency surgical or neuro-endoscopic procedures which are likely to involve contact with tissues of potential high level infectivity for CJD...... 17 APPENDIX IV: Distribution of Tissue Infectivity – please note recent updates...... 18 APPENDIX V: Algorithm for precautions for reusable instruments for surgical procedures on patients with, or 'at increased risk' of, CJD, vCJD and other human prion diseases...... 19 APPENDIX VI: Identification of a CJD surgical incident and procedure lookback periods...... 20 APPENDIX VII: Algorithm for the processing of tissue………………………………………………..21

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 2 of 23 QUICK REFERENCE GUIDE

For quick reference the guide below is a summary of actions required. This does not negate the need for the document author and others involved in the process to be aware of and follow the detail of this policy.

1. This Policy provides guidance on safe working practices with the aim of preventing the transmission of sporadic CJD, genetic CJD, Fatal Familial Insomnia (FFI), Gerstmann- Straussler-Scheinker Disease (GSS), variant CJD (vCJD) and Variably Protease-Sensitive Prionopathy (VPSPr).

2. Immediately inform the Infection Prevention Team of any patient admitted who fulfills the possible, definite/probable and “at increased risk” category of CJD/vCJD/TSE disease.

3. All patients about to undergo surgery, endoscopy or any other invasive clinical procedure must be assessed as to their risk of CJD or vCJD. See Appendix II and III of this policy.

4. Specific precautions must be adopted for patients fulfilling the possible, definite/probable, and “at increased risk” category of CJD/vCJD/TSE disease who are about to undergo surgery, endoscopy or any other invasive clinical procedure (see Section 6.3 of this policy). HSDU must be informed of any invasive reusable medical devices used on such patient groups.

5. There is no evidence that normal social or routine clinical contact of a CJD/vCJD patient presents a risk to others. Isolation of a CJD/vCJD patient is not necessary and they can be nursed in an open ward using standard infection prevention and control precautions.

6. Blood and body fluids from patients with, or at increased risk of, CJD/vCJD should be treated the same as potentially infectious for blood-borne viruses and handled with standard infection prevention and control precautions.

7. Specific Laboratory Control Measures must be adopted when working with tissues potentially containing TSE agents. Pathology staff must refer to this guidance when undertaking work with samples possibly containing TSE agents (see section 6.10).

8. Specific guidance must be followed with the management of the deceased patient (see section 6.8).

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 3 of 23 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: - 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 gonadotrophin 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.

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 biopsy or after death. There is currently no non-invasive test which can diagnose CJD during the incubation period and no effective treatment.

2. PURPOSE This Policy defines the actions that should be taken by the Trust to reduce the risk of transmission of TSEs and to ensure the clinical needs of patients are met. This Policy should be read in conjunction with the CJD guidance documents listed in the references and associated documentation section

3. SCOPE This Policy applies to all PHT health and non-healthcare staff including agency, bank and locum staff.

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 4 of 23 ‘In the event of an infection outbreak, flu pandemic or major incident, the Trust recognises that it may not be possible to adhere to all aspects of this document. In such circumstances, staff should take advice from their manager and all possible action must be taken to maintain ongoing patient and staff safety’

4. DEFINITIONS

Decontamination: A term used for the removal and destruction of microorganisms which then renders an item (medical device) ready for reuse and safe for staff to handle (NHS Estates, 2000). Endoscopy: A procedure in which a lighted viewing instrument (endoscope) is used to look inside a body cavity or organ to diagnose or treat disorders Iatrogenic: Induced inadvertently by the medical treatment or procedures or activity of a physician or surgeon e.g. infections acquired by the patient during the course of treatment. Incubation Period The time interval between the initial infection with an infectious agent and the appearance of the first symptom or sign of disease. Mutation: This is an abnormality or fault found in genes which produces an altered code, which results in the production of abnormal proteins. Risk assessment: The evaluation of an individual's personal and family history, often by using questionnaires to estimate the degree to which that person is at risk for developing certain diseases. Single use items: This is a device designated for ‘single-use’ and must not be reused. It should only be used on an individual patient during a single procedure and then discarded. It is not intended to be reprocessed and used again, even on the same patient

Standard precautions: Standard (previously known as universal) precautions are the practices adopted by all healthcare workers when potentially coming into contact with any patient’s blood or body fluids. They are a set of principles designed to minimise exposure to and transmission of a wide variety of micro-organisms. Since every patient is a potential infection risk, it is essential that standard precautions are applied to all patients at all times. Such precautions involve the use of safe work practices, protective barriers, and the safe disposal of blood, body fluids and sharps.

5. DUTIES AND RESPONSIBILITIES  Matrons, Clinical Directors, Clinical leads and Heads of Specialties have a duty of care to ensure that staff receive education on all aspects of infection control and adhere to the policies and guidelines of the Trust including the contents of this document  All staff have a duty of care to the patients and themselves to ensure they deliver high standards of infection control practice at all times. All staff are required to undertake their duties in accordance the Trust policies including the procedures and guidelines contained in this document.  The consultant/clinician in charge of clinically suspected CJD has a responsibility to inform the local Consultant, the Infection Prevention Team, and the local Health Protection / Public Health Unit  The Infection Prevention Team will give advice regarding the associated risk of exposure to TSE agents, containment and control measures and the management of patients, environment and equipment.

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 5 of 23 6. PROCESS 6.1 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 II for details) for public health purposes.

Record patient’s / relative’s responses in the medical notes.

Ask the patient if they have been notified that they are at increased risk of CJD or vCJD?

No / unable to Yes respond

Contact with medium / high Clarify specific details with patient risk infectivity tissues?1. 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

Yes No

Further risk assess CJD / Proceed using vCJD infection using questions standard infection in Appendix III2. control measures Seek further guidance from the infection prevention and control team

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 6 of 23 1. See Appendix IV for further information 2. If patients are unable to answer the questions in Appendix III 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.

6.2 Management of patients known, suspected or at risk of CJD/vCJD  There is no evidence that TSEs have been spread from person to person by close contact or through occupational exposure.  There is no need to isolate the patient provided standard precautions are followed  Infection Prevention and Control Team (IPCT) must be informed if the patient is going to be or has been admitted  All cases of clinically suspected CJD of any type should be reported by the clinician caring for the patient to the local Consultant, Health Protection Unit and the National CJD Surveillance Unit, Director, National CJD Surveillance Unit, Western General Hospital, Crewe Road,

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 7 of 23 Edinburgh EH4 2XUT, Tel: 0131 332 2117, Fax: 0131 343 1404

6.3 Precautionary measures for surgical procedures on patients known, suspected or at risk of CJD 6.3.1 Theatre management:  IPCT must be informed before any surgical procedure is carried out  Whenever possible the procedures should be carried out in an acute hospital theatre at the end of the list to ensure thorough cleaning of all surfaces before the next session  Only the minimum number of healthcare personnel should be in the theatre  Single use protective clothing should be used - a liquid repellent gown over a plastic apron - gloves - Masks and goggles or a full face visor  Single use disposable surgical instruments and equipment must be used where possible and incinerated after use  Where practical, expensive reusable equipment e.g. drills should be protected from contamination by using shields, guards or similar protective covering which should be destroyed by incineration at the end of the operation. However in practice effective protective covering may not be feasible and therefore advice should be sought from the manufacturer.  Drapes contaminated with CSF or other neural tissue from patients in the high and medium risk category should be incinerated  Advice should be sought from HSDU manager and Infection Control on the management of the surgical instruments- refer to CJD Policy produced by the Sterile Services Unit and Algorithm for management of surgical instruments in Appendix IV

6.3.2 Surface decontamination and the management of spillages:  Surfaces in contact with high or medium risk material from definite, probable or high risk cases should be thoroughly cleaned and then (eg. 10,000ppm chlorine-releasing agent) should be used to decontaminate the surface.  Standard infection control precautions should be followed for any spillages, which should be cleared up as quickly as possible, keeping contamination to a minimum. Disposable gloves and an apron should be worn when removing such spillages.  For spillages of large volumes of liquid, absorbent material should be used to absorb the spillage.  Standard disinfection for spillages (eg. 10,000ppm chlorine-releasing agent) should be used to decontaminate the surface after the spillage has been removed. A full risk assessment may be required.  All materials should be incinerated

6.3 Precautionary measures for surgical procedures on patients known, suspected or at risk of CJD (continued) 6.3.3 Management of surgical instruments (refer to Appendix V)  Before any procedure a risk assessment must be carried out – see section 6.1  Instruments that have been used in procedures involving tissues designated as high or medium infectivity (see Appendix IV), on patients with known, suspected or at risk of CJD/vCJD should be disposed of by incineration or quarantined pending a confirmed diagnosis.  The options available should be discussed either with the HSDU manager or Infection control.  Wherever possible single use instruments should be used Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 8 of 23  Instruments which are to be destroyed should be discarded directly into autoclave bins which must be sealed and sent for incineration at the end of the operation –see Appendix V  Re-usable instruments used on patients with possible CJD can be quarantined until a diagnosis has been made (see appendix IV). Discuss with HSDU manager before performing the procedure.  If the procedures involve low risk tissues (see appendix IV) single use instruments should be used wherever feasible. Reusable instruments should be reprocessed within HSDU observing current best practice. When reusable instruments have to be used the HSDU manager must be informed before the operation.  For guidance on the use of flexible endoscopes discuss either with the HSDU manager or Infection control. Reference to Annex F of the UK Guidance on decontamination of flexible endoscopes for TSE infection control (Dept of Health website) must be followed (https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/270734/An nex_F_Endoscopy.pdf). .

6.3.4 Collection of blood, biopsy and CSF sample  If these procedures are carried out in the ward area then every effort must be made to ensure the environment is easy to clean  Blood specimens should be collected using standard precautions as for any patient  All lumbar punctures should be carried out wearing disposable gloves and aprons using single-use disposable instruments  The laboratory must be informed in advance these samples are being sent  Linen contaminated with CSF or other neural tissue from patients in the high and medium risk category should be incinerated

6.3.5 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 Appendix IV), 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” document (see references)

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 9 of 23 6.4 Action to be taken following a newly diagnosed or suspected case of CJD or a person at increased risk of CJD  Specific Action needs to be initiated following a report of: - a newly diagnosed or suspected case of CJD - a person at increased risk of CJD - a surgical procedure carried out on a patient with CJD or at increased risk of CJD where TSE infection control guidelines were not followed  Outline of actions to take - Inform the Infection Prevention Team of the incident - Follow TSE infection control guidelines for future procedures with a risk of CJD transmission (refer to Section 6.3) - Perform procedure lookback and risk assessment to identify whether a surgical incident has occurred (refer to Appendix VI) - Remove from general use any surgical instruments or endoscopes that pose an onward transmission risk (obtain guidance on this issue through the Infection Prevention Team and the HSDU Manager)  The decision to Identify and inform patients who may have an increased risk of CJD following an incident will need consideration. Discuss this issue with the Infection Prevention Team.

6.5 Clinical waste Tissues and contaminated materials such as dressings and sharps, from patients with, or “at increased risk” of, CJD/vCJD, should be disposed of by incineration if they are contaminated by high or medium risk tissue (see Appendix IV) or in the normal clinical waste stream if they are low risk tissue or body fluids (see Appendix IV)

6.6 Maternity care Childbirth should be managed using standard infection control procedures and single use instruments. The placenta and all other associated fluids and materials should be considered as infected clinical waste and incinerated

6.7 Dental Care This should not be compromised. DH guidance is that endodontic reamers and files and other fine instruments which are difficult to clean are single use. This was endorsed by the Chief Dental Officer for England in April 2007

6.8 Management of the deceased patient If the patient is known or suspected of having a TSE the mortuary must be informed. On the death of a patient defined in Appendix I of this policy, the removal of the deceased from the ward, community setting or hospice, to the mortuary, should be carried out using normal infection control measures. It is recommended that the deceased is placed in a body bag, which should be labelled as High-Risk or Danger of Infection prior to transportation to the mortuary, in line with normal procedures for deceased patients where there is a known infection risk. For further information refer to the National Guidance from the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Working Group, Transmissible spongiform encephalopathy agents: Safe working and the prevention of Infection: Annex H After Death; (https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/209766/Ann ex_H_-_After_death.pdf)

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 10 of 23 6.9 Caring for the patient in the community No special measures over and above standard infection control precautions are necessary

6.10 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; (https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/209769/Ann ex_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: - 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 Annex V 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.

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 11 of 23 Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 12 of 23 7. TRAINING REQUIREMENTS

Staff required to use the risk assessment form should be trained to ask in a sympathetic manner

8. REFERENCES AND ASSOCIATED GUIDANCE Guidance from the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Working Group, Transmissible spongiform encephalopathy agents: Safe working and the prevention of Infection, The Stationery Office (Also available on line at https://www.gov.uk/government/publications/guidance-from-the-acdp-tse-risk- management-subgroup-formerly-tse-working-group) (2003) Updated January 2014

National Institute for Health and Clinical Excellence Patient safety and reduction of risk of transmission of Creutzfeldt – Jakob disease (CJD) via interventional procedures November 2006

The vCJD Working Party of the Standing Advisory Committee on Transfusion Transmitted Infections. Creutzfeldt- Jakob Disease Policy statement 5th June 2006

Department of Health Spongiform Encephalopathy Advisory Committee (SEAC): Position statement of vCJD and endodontic dentistry 2006.

Department of Health: Potential vCJD transmission risk via dentistry: an interim review Dec 2007

CFPP 01-01 – Management and Decontamination of surgical instruments used in acute care, Department of Health, Estates & Facilities Policy Division, 2012

Public Health Action following a report of a new case of CJD or a person at increased risk of CJD, CJD Section, Public Health England, Version 1.3 (July 2013)

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 13 of 23 9. EQUALITY IMPACT STATEMENT

Portsmouth Hospitals NHS Trust is committed to ensuring that, as far as is reasonably practicable, the way we provide services to the public and the way we treat our staff reflects their individual needs and does not discriminate against individuals or groups on any grounds.

This policy has been assessed accordingly

All policies must include this standard equality impact statement. However, when sending for ratification and publication, this must be accompanied by the full equality screening assessment tool. The assessment tool can be found on the Trust Intranet -> Policies -> Policy Documentation

Our values are the core of what Portsmouth Hospitals NHS Trust is and what we cherish. They are beliefs that manifest in the behaviours our employees display in the workplace. Our Values were developed after listening to our staff. They bring the Trust closer to its vision to be the best hospital, providing the best care by the best people and ensure that our patients are at the centre of all we do. We are committed to promoting a culture founded on these values which form the ‘heart’ of our Trust:

Respect and dignity Quality of care Working together No waste

This policy should be read and implemented with the Trust Values in mind at all times.

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 14 of 23 10. MONITORING COMPLIANCE WITH PROCEDURAL DOCUMENTS

This document will be monitored to ensure it is effective and to provide assurance of compliance.

Minimum requirement Lead Tool Frequency of Reporting arrangements Lead(s) for acting on to be monitored Report of Recommendations Compliance Infection Prevention and Caroline Infection Prevention Annually Infection Prevention Management CSC leads control protocols Mitchell and Control report Committee

Incidents reported, Caroline Data collection Quarterly Infection Prevention Management CSC leads documented and Mitchell Committee escalated appropriately Appropriate use of Darren Carter Decontamination Annually Infection Prevention Management CSC leads medical devices and Audits Committee adequate tracking and tracing

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 15 of 23 APPENDIX I: Diagnostic criteria for CJD

Cases should be classified by a neurologist from the National CJD Surveillance Unit on an on-going basis. It is recorded at four key stages  At notification  When the patient is first seen by the neurologist  The highest classification on the sole basis of clinical information – not including neuropathological information  When the Surveillance Unit review is completed

Sporadic CJD Definite Neuropathological/ immunocytochemical confirmation needed Probable Rapidly progressive dementia with at least two of the following symptoms  Myoclonus  Visual or cerebellar problems  Pyramidal or extrapyramidal features  Akinetic mutism Plus typical EEG with generalised triphasic periodic complexes at approx 1 per second Or clinical criteria for possible sporadic CJD and a positive assay for 14-3-3 protein in the CSF Possible Rapid progressive dementia with two of the above symptoms and a duration of less than 2 years

Variant CJD Definite Progressive neuropsychiatric disorder and neuropathological confirmation of the disease showing spongiform change and extensive PrPc deposition with florid plaques throughout the cerebrum and cellebellum Probable Can be classified under two sets of criteria:

1) Progressive neuropsychiatric disorder of longer than 6 months where routine investigations do not suggest an alternative diagnosis. Must have at least four of the following symptoms  Early psychiatric symptoms – depression, anxiety, apathy, withdrawal, delusions  Persistent painful sensory symptoms (frank pain and / or dysaesthesia)  Ataxia  Myoclonus or chorea or dystonia  Dementia EEG does not show the typical appearance of sporadic CJD AND there is a symmetrical high signal in the posterior thalamus on a MRI brain scan. There is no history of potential iatrogenic exposure

2) Progressive neuropsychiatric disorder of longer than 6 months where routine investigations do not suggest an alternative diagnosis. There is no history of potential iatrogenic exposure A tonsil biopsy is positive for PrP-tse

Possible Progressive neuropsychiatric disorder of longer than 6 months – routine investigations do not suggest an alternative diagnosis. Must have at least four of the following symptoms

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 16 of 23  Early psychiatric symptoms – depression, anxiety, apathy, withdrawal, delusions  Persistent painful sensory symptoms  Ataxia  Myoclonus or chorea or dystonia  Dementia EEG does not show the typical appearance of sporadic CJD

The CJD unit have three additional categories for patients who have been referred to the unit but do not meet the criteria for possible CJD

Diagnosis unclear The diagnostic criteria are not met BUT there is no reasonable alternative diagnosis therefore CJD remains a possibility

CJD thought unlikely Patient has atypical disease features/ atypical course/atypical clinical investigation results and/or a reasonable alternative diagnosis is made but not confirmed

Definitely not CJD CJD is not the diagnosis and there is alternative definite diagnosis proven on the basis of clinical examination or investigation

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 17 of 23 APPENDIX II: Risk categorization of patients having CJD / vCJD

Patient Groups Symptomatic patients  Patients who fulfill the diagnostic criteria for definite, probable or possible CJD or vCJD (see APPENDIX 1 for diagnostic criteria)  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 Patients “at increased risk”  Individuals who have been shown by specific genetic testing to be from genetic forms of CJD at significant risk of developing CJD.  Individuals who have a blood relative known to have a genetic mutation indicative of genetic CJD;  Individuals who have or have had two or more blood relatives affected by CJD or other prion disease Asymptomatic Patients  Individuals who have received labile blood components (whole identified as “at increased blood, red cells, white cells or platelets) from a donor who later risk” of vCJD through went on to develop vCJD receipt of blood from a donor who later developed vCJD Asymptomatic Patients  Recipients of hormone derived from human pituitary glands, e.g. identified as “at increased growth hormone, gonadotrophin, are “at increased risk” of risk” of CJD/vCJD through transmission of sporadic CJD. In the UK the use of human- iatrogenic exposures derived gonadotrophin was discontinued in 1973, and use of cadaver-derived human growth hormone was banned in 1985. However, use of human-derived products may have continued in other countries after these dates.

 Individuals who underwent intradural brain or intradural spinalsurgery 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).

 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 since January 1990;

 Individuals who have given blood to someone who went on to 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 sourced plasma products between 1990 and 2001

Recipients of ocular transplants, including corneal transplants, are not considered to be “at increased risk” of CJD/vCJD. Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 18 of 23 APPENDIX III: CJD risk assessment questionnaire for patients about to undergo elective or emergency surgical or neuro-endoscopic procedures which are likely to involve contact with tissues of potential high level infectivity for CJD

1 Have you any history of CJD or other Notes to clinician prion disease in your family? If yes, please Patients should be considered to be at risk from specify genetic forms of CJD if they have or have had 1. Genetic testing, which has indicated 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. Two or more blood relatives affected by CJD or other prion disease

2. Have you ever received growth hormone Notes to clinician or gonadotrophic treatment? If yes, please Recipients of hormone derived from human pituitary specify; glands e.g. growth hormone or gonadtotrophin have i) Whether the hormone was derived from been identified as potentially at risk of CJD. In the human pituitary glands UK, the use of human growth hormone was stopped ii) The year of the treatment in 1985 but human-derived products may have been iii) Whether the treatment was received in continued to be used in other countries. In the UK, the UK or in another country the use of human-derived gonadotrophin was discontinued in 1973 but may have been continued in other countries after this time.

3. Have you ever had surgery on your brain Notes to clinician or spinal cord? Individuals who underwent intradural brain or intradural spinal surgery before August 1992 who received (or might have received) a graft of human- derived dura mater are “at increased risk” of transmission of sporadic CJD (unless evidence can be provided that human-derived dura mater was not used). 4 Since 1980 have you had any Notes to clinician transfusions of blood or blood components Patients who have received blood from more than (red cells, plasma, cryoprecipitate or 80 donors have been identified as at increased risk platelets). NB This does not include of CJD. Further information on this is available from autologous transfusion, plasma products the HPA such as IVIG, albumin, coagulation factors (http://www.hpa.org.uk/vCJDpresurgicalassessment) and anti-D If yes, have you either i) received more than 50 units of blood or blood components?, or ii) received blood or blood components on more than 20 occasions? Where possible please provide the names of all the hospitals where you have received blood products

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 19 of 23 APPENDIX IV: Distribution of Tissue Infectivity – please note recent updates

Tissue Infectivity Levels High Medium Low Brain and spinal cord Spinal ganglia Anterior eye and cornea Cranial nerves, Olfactory epithelium Peripheral nerve specifically the entire Tonsil (only vCJD) Dental pulp optic nerve, only the Appendix (only vCJD) Gingival tissue intracranial components Spleen and thymus (only Blood and bone marrow of other cranial nerves vCJD) CSF* Cranial ganglia Other lymphoid tissues Placenta Posterior eye specifically (only vCJD) Urine the posterior hyaloid Skeletal muscle face, retina, retinal Other tissues pigment epithelium, Tonsil (only CJD) choroid, subretinal fluid Dura mater and optic nerve Lymph nodes (CJD only) Pituitary gland Appendix

* As yet the atypical prion has not been detected in either sporadic or variant CJD – experimental transmission of infectivity has been achieved at a low rate in sporadic CJD

Taken and adapted from “Transmissible spongiform encephalopathy agents, safe working and the prevention of infection” Department of Health June 2003, updated 10/1/2012

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 20 of 23 APPENDIX V: Algorithm for precautions for reusable instruments for surgical procedures on patients with, or 'at increased risk' of, CJD, vCJD and other human prion diseases

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 21 of 23 APPENDIX VI: Identification of a CJD surgical incident and procedure lookback periods

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 22 of 23 APPENDIX V II:

Policy for the Management of Patients with or Suspected of Having Creutzfeldt Jakob Disease (CJD) Version: 3 Issue Date: 03 February 2015 Review date: 01 January 2017 (unless requirements change) Page 23 of 23