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Responsible Directorate:

Public Health

Responsible Director:

Dr Judith Hooper

Date Approved:

10 March 2010


Governance Committee



Revision date:

March 2013

NICE GUIDANCE Once NICE guidance is published, health professionals are expected to take it fully into account when exercising their clinical judgment. However, NICE guidance does not override the individual responsibility of health professionals to make appropriate decisions according to the circumstances of the individual patient in consultation with the patient and/or their guardian or carer.

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Section 1. 2. 3. 4. 5. 6. 6.1. 6.2. 6.3. 6.4. 6.5

Introduction Associated policies and procedures Aims and objectives Scope of the policy Accountabilities and responsibilities What is CJD? Types of CJD Method of transmission Identification of patients with CJD /vCJD Notification and further advice General management of a symptomatic CJD patient in their own home/ward 6.6 Dentistry 6.7 Invasive medical procedures including podiatry 6.8 Accidental inoculation injuries 6.9 Occupational health records 6.10 Blood or body fluid spillage in the ward environment 6.11 Blood or body fluid spillage in the home environment 6.12 Relatives and voluntary carers 6.13 Other care services 6.14 Labelling of specimens 6.15 Management of laundry 6.16 Management after death 7. Equality impact assessment 8. Training needs analysis 9. Monitoring compliance with this policy 10. References Appendices A B C D

Definitions Key stakeholders consulted/involved in the development of the policy/procedure Equality impact assessment tool Sign off sheet regarding dissemination of procedural documents

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Page 3 3 3 3 4 4 4 5 5 6 6 7 8 8 9 9 9 9 10 10 10 10 10 10 10 11 12 13 14 15

Policy Statement The purpose of the policy is to provide information to staff on the precautions necessary to minimise the risk of occupational exposure to Creutzfeldt -Jakob disease (CJD) and to prevent transmission of CJD between patients


Introduction This policy will give details of the precautions required to minimise the risk of CJD to patients and staff in accordance with national guidelines produced by the CJD working party and Department of Health.


Associated policies & procedures This policy should be read in accordance with the following Trust policies, procedures and guidance: • • • • • • • • •


Aims and objectives • • • •


Hand decontamination policy Universal precautions policy Decontamination policy Waste management guidelines Health and Safety policy COSHH policy Incident reporting policy Management of clinical sharps policy Antiseptics and Disinfectants

To describe safe working practices to prevent the transmission of CJD and related disorders. To protect staff and patients from any infection risk from instruments used on patients with definite, probable or possible CJD. To ensure that single use items are not re- processed for use To ensure compliance with the Health and Social care Act 2008: The Code of Practice for the NHS on the Prevention and Control of Health Care Associated Infections and related guidance.

Scope of the policy This policy must be followed by all NHS Kirklees employees who are developing policy and procedural documents or developing guidance for colleagues. It must be followed by Page 3 of 16

all staff who work for NHS Kirklees, including those on temporary or honorary contracts, bank staff and students. Breaches of this policy may lead to disciplinary action being taken against the individual. Independent contractors are responsible for the development and management of their own procedural documents and for ensuring compliance with relevant legislation and best practice guidelines. Independent Contractors are encouraged to seek advice and support as required. 5.

Accountabilities and Responsibilities The Chief Executive is accountable for ensuring that effective arrangements for Infection Prevention and Control are in place within NHS Kirklees.The Director of Infection Prevention and Control has responsibility to provide assurance to the Board that infection prevention and control policies are in place and their compliance audited. The Infection Prevention and Control team will ensure the policy is reviewed as required and work with Heads of Service to implement changes to practice.


What is CJD? Creutzfeldt - Jakob disease (CJD) is a rare and fatal neurological condition that affects the nervous system. It is one of a group of transmissible diseases known as the prion diseases or transmissible spongiform encephalopathies (TSE). All types of CJD are associated with a change in a protein called the ‘prion protein’. The abnormal form of this protein accumulates in the brain in these disorders and results in death of nerve cells. CJD has a long incubation period and disease may not be manifested for many years. There is no simple non invasive test to identify CJD before the onset of symptoms and diagnosis is made on neurological criteria and by brain biopsy (following death). Prion proteins are resistant to normal methods of decontamination.


Types of CJD There are several types of CJD: Sporadic: This is to date the commonest form and occurs as autosomal dominant disease – caused by a dominant mutant gene or an autosome. The usual age of onset is late middle age. Most patients present with rapidly progressive dementia with focal neurological signs including ataxia, myoclonus, visual disturbances and rigidity. Death usually occurs within 4-6 months of clinical onset. Iatrogenic: Transmitted by medical and surgical procedures including injections with pituitary hormones, dura mata grafts and rarely by neurosurgical instruments. The incubation period can range from 1-2 years for neurological routes of transmission and up to 30 years in some pituitary hormone recipients Variant CJD (vCJD): Variant CJD is associated with the same transmissible agent that is responsible for Bovine Spongiform Encephalopathy (BSE). Variant CJD is thought to have resulted from the consumption of contaminated bovine food products. Whilst rare Page 4 of 16

there has been a gradual increase in the numbers of people being diagnosed. Variant CJD tends to affect young adults with the clinical illness lasting an average of 14 months. The symptoms may include both psychiatric and sensory abnormalities, which are followed by ataxia, myoclonas and other movement disorders and dementia. 6.2

Method of transmission There is no evidence to date that CJD or vCJD are spread from person to person by close contact but it is known that transmission of sporadic CJD can be associated with medical intervention, e.g. administration of hormones prepared from human pituitary glands dura mater preparations, corneal grafts and recently, from blood transfusions. Individuals who have had a blood transfusion since 1980 are excluded from donating whole blood/platelet apheresis; this will also include individuals who are unsure if they have been transfused. Transmission of CJD / vCJD has also been reported following neurosurgical procedures with inadequately decontaminated instruments.


Identification of patients with CJD /vCJD It is the responsibility of the clinician to ensure that an assessment to determine risk is undertaken, using Table 1 as guidance. Table 1 Categorisation of patients by risk 1.



Symptomatic patients

Asymptomatic patients at risk from familial forms of CJD linked to genetic mutations

Asymptomatic patients identified as potentially at risk from iatrogenic exposure


Patients who fulfil the diagnostic criteria for definite, probable 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 or have had two or more blood relatives affected by CJD or other prion disease, or a relative known to have a genetic mutation indicative of familial CJD.


Individuals who have been shown by specific genetic testing to be at significant risk of developing CJD or other prion disease.


Recipients of hormone derived from human pituitary glands e.g. growth hormone, gonadotrophin.


Individuals who have received a graft of dura mater (people who underwent neurosurgical procedures or operations for a tumour or cyst of the spine before August 1992 may have received a dura mater graft and should be treated as at risk unless evidence can be provided that dura mater was not used).


Patients who have been contacted as potentially at Page 5 of 16

risk for public health

It is the responsibility of the referring doctor to notify clinicians if any patients are considered to fall into any of the above categories. If any patient is assessed to fall within any of the above categories, the Infection Prevention and Control Team must be informed as soon as possible, prior to any clinical procedures being carried out. The case clinician must also notify the local Consultant in Communicable Disease (CCDC) of each new case within their geographical area. 6.4

Notification and further advice All cases where CJD of any type is a possible diagnosis should be reported to the National CJD Surveillance Unit, so that any necessary advice and action can be followed. Contact address: National CJD Surveillance Unit Western General Hospital Crewe Road Edinburgh EH4 2XUT Telephone no: 0131 537 2128 There is a Prion Disease Clinical Nurse Specialist based at the National Hospital for Neurosurgery and Neurology, London. Telephone number 020 7061 9896


General management of a symptomatic CJD patient in their own home/ ward Normal social or routine clinical contact with a patient with CJD or related disease does NOT present a risk to healthcare workers, relatives or the community (DH, 2003). Isolation of patients with such diseases is therefore not necessary. They can be cared for on the open ward or at home. As for all other patients, health care staff must wear protective clothing when in contact with body fluids, i.e. gloves and aprons, plus mask and eye protection when there is a risk of splashing to the face. Carers must also be provided with appropriate protective equipment. All sufferers of vCJD are allocated a Key Case Worker by the National CJD Surveillance Unit in Edinburgh. For other forms of TSE a Key Case Worker should be identified by the PCT to oversee the care of the patient in the community and the support of their family. Prior to discharge from hospital, the responsible hospital Clinicians should ensure that the Key Case Worker is given adequate information and that discharge to the community is thoroughly planned across all disciplines and agencies involved. The same infection prevention control measures are required for the care of symptomatic CJD patients in the community as in hospital. Although cases of CJD/vCJD have been reported in healthcare workers, there have been no confirmed cases linked to occupational exposure. The highest potential risk in Page 6 of 16

the context of occupational exposure is from exposure to high infectivity tissues through direct inoculation (e.g. as a result of sharps injuries, puncture wounds or contamination of broken skin), and exposure of the mucous membranes such procedures involving these risks should also be avoided If possible. Compliance with standard infection prevention and control precautions will minimise risks from occupational exposure. Healthcare workers, who work with symptomatic patients with definite, probable or possible CJD or vCJD, or with potentially infected tissues, should be appropriately informed about the nature of the risk and relevant safety procedures. The disease often presents after a long incubation period, where the individual is often asymptomatic, but potentially infectious. Until further information is available, it is difficult to predict the number of humans who may be incubating vCJD. Thus, adequate decontamination of all instruments used in all invasive procedures is essential. Decontamination of instruments used in invasive procedures cannot be achieved by autoclaving, as this does not inactivate TSE agents. Thorough cleaning and physical removal of organic matter from instruments is essential. Washer disinfectors must conform to, and be validated against HTM 2030 and HTM 01-05. 6.6

Dentistry Dental care of patients defined as being at risk of CJD should not be compromised in any way. As for all patients, satisfactory standards of decontamination are required. Recent research has indicated that a low level of prion contamination may theoretically be present on some instruments following contact with dental tissues. This applies if these instruments have previously been used in the care of a prion disease carrier who may exhibit no symptoms and may not go on to develop the disease. For those tissues where evidence suggests this risk is most pronounced, the Chief Dental Officer for England has published requirements for endodontic files and reamers to be single-use instruments in all cases. Other instruments or device types for which a reliable cleaning regime is not available should also be considered for replacement by single-use types or by the single use of reprocessible instruments. Information regarding risk in this important area is contained in ‘Potential vCJD transmission risks via dentistry: an interim review’ (December 2007). Where patients indicate that they are in a high-risk group, guidance provided by the Advisory Committee on Dangerous Pathogens – Transmissible Spongiform Encephalopathy Working Group (ACDP-TSE WG) should be followed ( acdp/tseguidance/index.htm). The guidance suggests that special precautions beyond full instrument decontamination will not be necessary. However, specific advice is available by contact with the committee secretariat. For all other instruments used in dentistry, the risk of prion transmission will be usefully reduced by compliance with the decontamination procedures Health Technical Memorandum (HTM 01-05). The HTM statement is based on various studies (conducted on behalf of Department of Health) that examined the effect of steam sterilisation on prion infectivity. These studies showed that the steam sterilisation methods provide a useful level of deactivation of prion infectivity. While this would not be significant in highrisk tissue surgery, the effect is large enough to be of significance in dentistry (excluding Page 7 of 16

endodontic procedures). In addition, there is a risk of prion transmission through protein contamination of instruments.

Endodontic reamers and files are treated as single use. In view of Spongiform Encephalopathy Advisory Committee (SEAC) advice, emerging research findings and of the microbiological evidence which shows that endodontic reamers and files cannot be reliably decontaminated it is advised that these instruments be treated as single use and disposed of appropriately after each patient. This must be done whether or not the instruments are labelled as single use. Satisfactory standards of decontamination are observed for all dental instruments Emerging research findings reinforce the need for the highest standards of decontamination and cleaning to be carried out routinely in dental practice, whether reprocessing is carried out using local decontamination facilities or a sterile services department. For local decontamination it is recommended that ultrasonic baths, washer disinfectors and benchtop steam sterilisers are used in a separate room from patient activity (DH 2008) Manufacturers’ decontamination instructions are followed for all instruments. The Medical Devices Directive requires manufacturers of dental instruments to provide validated reprocessing instructions. These instructions should include information on appropriate cleaning, disinfecting, and packaging and where appropriate re-sterilisation to allow re-use of the device. They must always be followed. Where instruments are difficult to clean, single use instruments should be used if available. Should dental treatment progress to head and neck surgery, special precautions may need to be taken to reduce any possible transmission of CJD. Therefore the patient will need to be referred to an acute hospital setting. Information about the patient’s risk status must be included in any referrals for surgery and also recorded in the patient’s dental records. There is no reason why any CJD patients, or their relatives, should be refused routine dental treatment. 6.7

Invasive medical procedures (including Podiatry) Because of the unusual resistance of the CJD prion, which may not be destroyed by autoclaving at 134ºC, single use disposable equipment must be used for invasive procedures on patients defined as being at risk. The collection of blood specimens from patients defined as being at risk should involve the same precautions used for venepuncture for any patient, i.e. avoidance of sharps injury and the safe disposal of sharps and the use of Personal Protective Equipment (PPE). Although podiatry is classed as low risk, decontamination must be compliant with the national decontamination strategy and legislation.


Accidental inoculation injuries Normal safe practice should be followed during drug administration by injection and for venepuncture, i.e. avoidance of sharps injuries. Use of the appropriate protective clothing should also prevent any mucocutaneous exposure (splashing of blood onto mucous membranes). However, if such an exposure should occur, the post-exposure Page 8 of 16

prophylaxis policy must be followed. (See management of clinical sharps injuries and exposure to blood and body fluids policy.)


Occupational health records COSHH requires employers to keep a list of employees who work with TSE agents, e.g. laboratory staff working on TSE agents or staff performing invasive procedures on highly infective tissues, e.g. central nervous or eye tissue. This will not apply in NHS Kirklees, but in cases of unintentional exposure, a list should be kept where there is a likelihood of exposure to TSE and not simply when there has been a known incident or accident. The information recorded needs to include the type of work done and any specific exposure. The list must be kept for 40 years after the last exposure (DH, 2003).


Blood or body fluid spillage in the ward environment Spillage of potentially CJD infectious material i.e. blood must be removed using absorbent material and the surface wetted with Sodium Hypochlorite solution of 10,000 ppm for one hour. Due to the large amount of chlorine released in this concentration it must performed in a well ventilated area away from patients/staff with a known chest condition. The Infection Prevention and Control Team (IPCT) must be informed in the event of any spillage of body fluids from a known, suspected or at risk patient. The appropriate concentration of sodium hypochlorite will be held in pharmacy and must be ordered and available prior to admission of a patient with known CJD. Staff must wear disposable gloves and apron when dealing with spills and waste material and protective clothing must be disposed of as waste guidelines. Hand washing must always follow the removal of protective clothing.


Blood or body fluid spillage in the home environment When dealing with blood / bodily fluids staff and carers must wear protective clothing ie, disposable gloves and apron and eye protection if risk of splashing. All waste must be disposed of as infectious waste in accordance with NHS Kirklees Waste Management Guideline.

Absorb as much of the blood as possible using paper towels (e.g. kitchen roll) and discard into an orange clinical waste bag

• •

Clean the area with hot soapy water, rinse and allow to dry.

• 6.12

Use disposable cloths and discard after use. If a re-usable cloth is used then wash thoroughly after use. (e.g. washing machine cycle) Dispose of gloves, apron and used disposable equipment in an orange clinical waste bag.

Relatives and voluntary carers The Key Case Worker should advise relatives and carers of any risks to themselves in caring for the patient and advise on the necessary infection prevention control measures. Page 9 of 16

Friends and visitors should be encouraged to visit as normal. NHS Kirklees must provide any necessary equipment e.g. gloves for the relatives and carers providing care.


Other care services e.g. private agencies, social services The Key Case Worker must advise these services of the necessary information needed to safely carry out their work. As employers, these organisations must provide their staff with the necessary personal protective equipment to perform their work safely.


Labelling of specimens All pathology specimens from patients with a definite, probable or possible diagnosis of CJD must be labelled as “infection risk” in accordance with NHS Kirklees policy prior to transfer to the laboratory.


Management of laundry Patient’s clothes and bed linen should be laundered as normal although it is preferable to launder fouled linen separately at a temperature of 60º C. Laundry can be sent to commercial laundries, this may be of great assistance to carers. Laundry in the ward area must be placed in a water soluble bag for relatives to take home or to send to the hospital laundry if available.


Management after death On the death of a known, suspect or at risk patient, the removal of the body from the ward or community setting must be carried out using standard infection control measures. It is recommended that the deceased person’s body is placed in a cadaver bag prior to transportation to the mortuary. Advice can be sought from the Infection Prevention and Control team.


Equality Impact Assessment This policy has been assessed for the potential adverse impact as set out in Appendix C.


Training Needs Analysis All induction programmes and Infection Prevention and Control annual mandatory training will include information on standard precautions and decontamination of reusable medical devices.


Monitoring Compliance with this policy All reported incidents will be monitored and a Route Cause Analysis form (RCA) completed if necessary which will include lessons learned and will be supported by the Assurance Framework. Page 10 of 16

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Advisory Committee on Dangerous Pathogens and the Spongiform Encephalopathy Advisory Committee. Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection. Guidance from the Advisory Committee on Dangerous Pathogens and the Spongiform Encephalopathy Advisory Committee. London: Department of Health2003 Department of Health (2000). Creutzfeldtz-Jacob Disease: Guidance for Health care Workers. London. Department of Health. Department of Health (2007) Clarification and Policy summary – Decontamination of re-Usable Medical Devices in the Primary, Secondary and Tertiary Care Sectors.London.DH Department of Health (2008) HTM 01-05: Decontamination in Primary care and Dental Practices. London: DH Department of Health (2006) HTM 07-01: Environment and sustainability: safe management of healthcare waste Department of Health (2007) Potential vCJD transmission risks via dentistry: An interim review. London: DH Department of Health (2003) Transmissible spongiform encephalopathy agents: safe working and the prevention of infection – Guidance from the Advisory Committee on Dangerous Pathogens and The Spongiform Encephalopathy Advisory Committee.London.DH Guidance on local reporting by clinicians of Creutzfeldtz-Jacob Disease and local act by Consultant in Communicable Disease Control. November 2003. HSC 2000/032: Decontamination of Medical Devices, Department of Health, 2000 National Institute for Clinical Excellence. (2006). Patient Safety and reduction of risk of Transmission of Creutzfeldt –Jacob disease (CJD) via interventional procedures

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Appendix A - Definitions

CJD Creutzfeldt- Jacob Disease This is a neurological condition that affects the nervous system and caused by prion disease


Personal Protective Equipment Equipment to provide protection from blood and body fluids i.e. gloves, aprons, eye protection and masks.

IPCT Infection Prevention and Control team The team provides the following advice:● ● ● ● ● ● ●

Provision of information education and advice on infection prevention and control. Monitoring and reviewing infections in the community e.g. MRSA and C. difficile. Development of relevant policies and procedures for health care staff. Advise on measures to tackle healthcare associated infections. Support for staff/patients/service users and carers in the prevention, management and control of infection. Monitoring of infection control standards and practices. Changing behaviours, promoting a culture of cleanliness.

SEAC Spongiform Encephalopathy Advisory Committee The role of SEAC is to provide independent expert scientific advice to the Government on transmissible spongiform encephalopathies such as bovine spongiform encephalopathy (BSE) and Creutzfeld-Jacob disease. SEAC's remit is wide-ranging, and covers public health, food safety and animal health issues.


Transmissible Spongiform Encephalopathy Transmissible Spongiform Encephalopathies (TSEs), also known as prion diseases are a group of progressive conditions that affect the brain and nervous system of many animals, including humans. They are transmitted by prions. The disorders cause impairment of brain function, including memory changes, personality changes and problems with movement that worsen over time. Prion diseases of humans include classic Creutzfeldt-Jakob disease, new variant Creutzfeldt-Jakob disease (a human disorder related to mad cow disease).

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Appendix B Key stakeholders consulted/involved in the development of the policy / procedure Key Participant Yes/No Yes

Feedback requested Yes/No Yes

Feedback accepted Yes/No Yes

KCHS Community Dental Services



Not received

KCHS Podiatry Services



Not received

HVMH Maple Ward and Day Surgery Unit



Not received

Stakeholders name and designation Kirklees Infection Control Committee

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Appendix C Equality Impact Assessment Tool Creutzfeldt- Jacob Disease Policy Yes/No 1.



4. 5. 6. 7.

Does the policy/guidance affect one group less or more favourably than another on the basis of: • Race • Ethnic origins (including gypsies and travellers) • Nationality • Gender • Culture • Religion or belief • Sexual orientation including lesbian, gay and bisexual people • Age • Disability - learning disabilities, physical disability, sensory impairment and mental health problems Is there any evidence that some groups are affected differently?

If you have identified potential discrimination, are any exceptions Valid, legal and/or justifiable? Is the impact of the policy/guidance likely to be negative? If so can the impact be avoided? What alternatives are there to achieving the policy/guidance without the impact? Can we reduce the impact by taking different action?


No No No No No No No No No



No -

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Those associated with familial CJD, who would be identified by the National CJD surveillance unit and followed up by their support worker

Appendix D Sign Off Sheet regarding Dissemination of Procedural Documents

Title of Document:

Creutzfeldt-Jakob Disease (CJD) Policy

Lead Director:

Dr Judith Hooper

Date Approved: Where approved: Dissemination Lead: Placed on Website: Review Date:

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