IPC 3.2 Creutzfeldt-Jakob Disease or any Transmissible Spongiform Encephalopathy
Contents
1.0 Introduction
2.0 Mode of transmission
2.1 Signs and symptoms - clinical indications
3.0 Antimicrobial treatment guidance
4.0 Infection prevention and control principles
4.1 Isolation
4.2 Hand hygiene
4.3 Respiratory / Cough hygiene
4.4 Personal protective equipment
4.5 Equipment
4.6 Environmental cleaning
4.7 Linen
4.8 Body fluids
4.9 Waste standard
4.10 Occupational exposure
5.0 Surveillance
6.0 References/Source documents
Appendix 1
Record of changes
1.0 Introduction
Organism
Transmissible Spongiform Encephalopathies (TSEs) sometimes known as prion diseases are fatal degenerative brain diseases, which occur in humans and animals. TSE’s are thought to be caused by the build-up of an abnormal form of the naturally occurring ‘prion’ protein. The TSE’s which affect humans are classified according to whether they are sporadic, inherited or acquired.
Incubation period
This is unclear but is thought to be from 10 years - 50 years or longer.
Creutzfeldt-Jakob disease - NHS (www.nhs.uk)
Individuals at risk
Can occur sporadically.
May be familial or inherited (genetic CJD, Gerstmann-Strauussler Scheinker Disease and Fatal Familial Insomnia.)
Variant CJD - the disease is acquired by having had contact with infective agents from an affected individual either directly or indirectly, e.g. blood components, high-risk tissue or consumption of infected meat products. Examples of disease which fall into this category are latrogenic CJD or variant CJD which are the most common forms of TSE’s seen
Iatrogenic - the disease is acquired through medical or surgical treatment which can occur from, for example recipients of hormone derived from human pituitary glands e.g. growth hormone gonadotrophin.
Notifiable disease
Yes
All cases where CJD of any type is a possible diagnosis should be reported to:
National Prion Clinic, Institute of Prion Diseases, Courtauld Building, 33 Cleveland Street, London, W1W 7FF uclh.prion.help@nhs.net.
The National CJD Surveillance Unit, so that any necessary action can be taken:
The National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Bryan Matthews Building, Western General Hospital, Crewe Road, Edinburgh EH4 2XU
Telephone: Main office +44 (0)131 537 1980/2128/3103
Neuropathology Laboratory +44 (0)131 537 3084
Informing IPC team
Yes
2.0 Mode of transmission
CJD is not highly contagious. There have been no reports of occupational transmission.
2.1 Signs and symptoms - clinical indications
- Symptoms of CJD include
- Loss of intellect and memory
- Changes in personality
- Loss of balance and co-ordination
- Slurred speech
- Vision problems and blindness
- Abnormal jerking movements
- Progressive loss of brain function and mobility
- Most people will die within a year of the start of symptoms.
3.0 Antimicrobial treatment guidance
Requires specialist management for symptoms, no antimicrobial treatments available.
4.0 Infection prevention and control principles
4.1 Isolation
Not required - There is no evidence social or clinical contact poses a risk.
4.2 Hand hygiene
NHS England » Chapter 1: Standard infection control precautions (SICPs)
4.3 Respiratory / Cough hygiene
NHS England » Chapter 1: Standard infection control precautions (SICPs)
4.4 Personal protective equipment
NHS England » Chapter 1: Standard infection control precautions (SICPs)
4.5 Equipment
Procedures carried out in NHCFT settings, including Dentistry, do not involve contact with tissues deemed to be medium or high infectivity for vCJD (DH 2015; NICE 2020) , Dental instruments that are used on patients/ Service Users with or at increased risk of CJD or vCJD can be handled in the same way as those used in any other low risk surgery. The risks of transmission of infection from dental instruments are thought to be very low provided satisfactory standards of infection prevention and control and decontamination are maintained.
These instruments can be reprocessed according to best practice and returned to use. The DH guidance HTM01-05 Dental decontamination (DH 2013) provides information regarding emerging evidence around the effectiveness of decontamination in primary care dental practices, and the possibility of prion transmission through protein contamination of dental instruments.
TSE agents are not destroyed by the autoclaving process. Some autoclaves may include a prion cycle; however these have been found to be ineffective and should not be relied upon to render equipment prion free. Effective automated cleaning is the most efficient method of eliminating these agents. There is research evidence that shows that endodontic reamers and files cannot be reliably decontaminated therefore it is advised that these instruments should be single use and be disposed of after use.
The risk of transmission of infection from minor surgical/dental/podiatry instruments are thought to be very low provided optimal standards of infection control and decontamination are maintained. This low risk will be the same for other minor invasive procedures undertaken by any other healthcare workers.
4.6 Environmental cleaning
NHS England » Chapter 1: Standard infection control precautions (SICPs)
4.7 Linen
NHS England » Chapter 1: Standard infection control precautions (SICPs)
4.8 Body fluids
The potential source of infection is from high-risk tissues, especially brain, spinal cord and posterior eye. Body secretions, body fluids (including saliva, blood, CSF, and excreta) are all low risk for CJD, so are standard precautions.
Samples from patients with a definite, probable, or possible diagnosis of CJD, must be labelled as ‘infection risk’ CJD should be identified as a “high risk” sample on the request and telephoned through. Sherwood Forest Hospital laboratory will require telephoning to inform in advance of the risk prior to the sample being sent. Nottingham University Hospitals and Bassetlaw laboratories do not require contacting prior to sending.
NHS England » Chapter 1: Standard infection control precautions (SICPs)
4.9 Waste standard
NHS England » Chapter 1: Standard infection control precautions (SICPs)
4.10 Occupational exposure
CJD is not highly contagious. There have been no reports of occupational transmission. No special measures over and above standard infection control are generally required as it is unlikely that procedures undertaken will result in contact with high or medium risk tissues.
5.11 Care of deceased
A cadaver bag is required for transportation to the mortuary/funeral directors. This should be labelled as High Risk or Danger of Infection prior to transportation to the mortuary, in line with normal procedures for deceased patients with a known infection (DH 2010 ANNEX H).
There is no evidence that CJD can be passed from one person to another by contact with the skin or hair. Therefore, the body bag can be opened to allow relatives to view the body, and, if they wish, have contact with the deceased.
NHS England » Chapter 1: Standard infection control precautions (SICPs)
Appendix 12: Transmission based precautions for deceased patients with infection
5.0 Surveillance
The National CJD Surveillance Unit monitors cases.
6.0 References/Source documents
Department of Health (2013) Minimise transmission risk of CJD and vCJD in healthcare settings. updated version. Department of Health. London. Minimise transmission risk of CJD and vCJD in healthcare settings - GOV.UK (www.gov.uk) (accessed 15/02/2024)
Department of Health (2013) HTM 0105:Dental decontamination in primary care dental practices. Department of Health. London. NHS England » Health Technical Memorandum 01-05: decontamination in primary care dental practices (accessed 15/02/2024)
Department of Health (2015) Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection v.4. Department of Health. London
Department of Health (2010) Guidance from the ACDP TSE Working Group ANNEXE H AFTER DEATH. London. HMSO.
Department of Health (2013) Environment and sustainability Health Technical
Creutzfeldt-Jakob disease - NHS (www.nhs.uk)
Prions and proteopathic seeds: Safe Working and the Prevention of Infection Part 3 Laboratory containment and control measures (accessed 13/03/204)
Appendix 1
Aalzheimers Society - Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob Disease (NHS)
Record of changes
|
Date |
Author |
Policy |
Details of change |
|
May 2017 |
Diane Holmes |
18.12 (Issue 1) |
Review of document and minor alterations throughout the policy |
|
June 2020 |
Jane Finch |
18.12 (Issue 2) |
Multiple revisions and updates throughout the policy on pages 3-9 and 13. |
|
March 2024 |
IPC Team |
7.12 (Issue 3) |
Policy reviewed by the IPC Team, edited and revised into new template format. |
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