Transmissible Spongiform Encephalopathy (TSE),
Creutzfeld-Jacob Disease (CJD) and Variant CJD
Infection Control Folder No: 7
Approved by: Date
Policy and Guideline Ratification Group 17/8/10
Integrated Governance Committee (IGC) 1/9/10
Date of Issue: September 2010
Version No: 1.4
Date of Review: September 2012
Author: Liz McLoughlin, Prevention and Control of Infection Senior Nurse
NHS South Gloucestershire
Document status: Current
Version Date Comments
1.0 25/6/10 Circulated to DIPC, COIC Committee and
Clinical Governance Lead (Provider Arm) for
1.1 28/6/10 Incorporated comments from DIPC
1.2 5/8/10 Revised following PRG comments and Clinical
Governance Lead (Provider Arm) feedback
1.3 17/8/10 Approved by PGRG with small revision
1.4 1/9/10 Approved by Integrated Governance
If you require additional copies of this document please contact Liz McLoughlin, Prevention and
Control of Infection Senior Nurse, 01454 - 418039
NHS South Gloucestershire has made every effort to ensure this policy does not discriminate, directly or
indirectly, against employees, patients, contractors or visitors on grounds of race, colour, age, nationality,
ethnic (or national) origin, sex, sexual orientation, marital status, religious belief or disability. This policy will
apply equally to full and part time employees. All NHS South Gloucestershire policies can be provided in
large print or Braille formats if requested, and language line interpreter services are available to individuals of
different nationalities who require them.
The failure to comply/adhere to this policy may be investigated in line with the ‘Investigating (Employment)
complaints and allegations policy and procedure’ and may result in disciplinary action, up to and including
1. Introduction Page 4, 5
2. Scope Page 5
3. Principles & Purpose Page 5
4. Definitions Page 5
5. Roles and Responsibilities Page 5, 6
6. Consultation Page 6
7. Equality Impact Assessment Page 6
8. Procedures and Precautions Page 6, 14
8.1 UK CJD Surveillance Page 6
8.2 Infection Control Precautions & Risk Management Page 6, 8
8.3 Management of “Risk” Patient Groups Page 8,9
8.3.1 Identification of Possible CJD Diagnosis Page 8,9
8.3.2 Exposure of a Healthcare Worker to CJD Page 9
8.4 Body Fluids Page 9, 10
8.4.1 Body Fluid Spills Page 10
8.4.2 Bed Linen Page 10
8.4.3 Clinical waste Page 10
8.4.4 Invasive Medical Procedures & Samples Page 10, 11, 12
8.4.5 Death Page 12, 13
8.4.6 Reprocessing Instruments Page 13
8.5 Source of Advice Page 14
9. Implementation Page 14
10. Audit Page 14
11. References and Links to PCT documents Page 14, 15, 16
12. Acknowledgements Page 16
Appendix 1 Page 17
Appendix 2 Page 18
Appendix 3 Page 19
Appendix 4 Page 20, 21, 22
Transmissible Spongiform Encephalopathies (TSEs), sometimes known as prion diseases, are rare,
fatal, degenerative brain diseases affecting the central nervous system (CNS) that can occur in
humans and certain other animal species.
There are several recognised TSEs, including
• Creutzfeld-Jakob Disease (CJD) in humans
• Bovine spongiform encephalopathy (BSE) in cattle
• Scrapie in sheep
A common feature of all TSEs is the appearance of microscopic vacuoles (holes) in the grey matter of
the brain, giving a sponge-like appearance from which the conditions derive their names.
All human TSEs are very rare; worldwide the incidence of CJD is approximately 1 per million people
There are 4 types of CJD, (see under section 4 for all definitions):
• Variant CJD (vCJD) – generally affects younger people and early symptoms often include
personality changes and psychological symptoms. It has been associated with exposure to
the prion agent responsible for Bovine Spongiform Encephalopathy (BSE)
(Bovine Spongiform Encephalopathy (BSE) was first recognised in the UK in 1986. Prior to this,
changes occurred in the meat rendering process by removing fats using heat treatment. This was
associated with the concept of adding meat protein to animal feeds. Concerns were expressed that
BSE could be transmitted to humans eating beef and beef products). Much information remains
unknown and research into the disease continues.
Symptoms commonly include the following:
• Personality changes and loss of intellect and memory
• Sensory and motor neurological deficits
• Myclonic jerks, chorea, or dystonia
• Difficulty in speaking, swallowing, moving and incontinence
• Coma and death
The National Institute for Health and Clinical Excellence (NICE) has produced interventional
procedure guidance in 2006. Other sources of up to date information can be found on the Health
Protection Agency (HPA) website. www.hpa.org.uk Research into this debilitating and often fatal
TSE agents exhibit unusual resistance to conventional chemical and physical
decontamination methods, and are not significantly affected by disinfectants and
infectivity persists after autoclaving at conventional timed and temperatures (e.g.134°C
for 3 minutes).
TSE’s are not highly infectious and do not appear to spread from an infected host during
normal contact. There have been documented cases of CJD being transmitted accidentally to
service users via contaminated medical instruments, and effective cleaning of instruments prior
to sterilisation is of the UTMOST IMPORTANCE in reducing this risk as are the processes in place for
the effective tracking of all equipment.
All Infection Prevention and Control policies within the PCT are based on ensuring the requirements
of ‘The Health Act Code of Practice for the Prevention of Health Care Associated Infections,
Department of Health (October 2006), revised January 2008’. The PCT should also have a policy for
staff to manage service users with, or at risk of, CJD, vCJD and other human prion diseases.
When referring to users of health services in the PCT, e.g. patients, clients, service users etc, the
word 'service user' must be used.
Where this document refers to ‘the PCT’, it shall be understood to mean NHS South Gloucestershire.
This policy is applicable to all staff that are directly employed or contracted within NHS South
3. Principles and Purpose
This policy has been written to provide clear guidance for the management and control of TSE agents
in order that staff are able to recognise and reduce the risks of transmission within a healthcare
setting, and ensure prompt recognition of those patients who are at risk. The policy reflects current
legislation. It details the actions required to ensure prompt detection and appropriate management of
CJD. The purpose of this policy is to prevent cross infection of CJD through implementation of a
rigorous risk assessment process prior to surgical procedures and implementation of national
guidance for management of such risks with appropriate decontamination of instruments and
Sporadic CJD (sometimes called ‘Classic’)
Is currently the commonest form of CJD occurring randomly in the community and affecting about 50
people per year. It usually affects people over 45 years of age.
Not thought to be found outside the brain. Transmission of this form of the disease is thought to be
via instruments that have been contaminated by brain tissue or by the transplant of tissue itself e.g.
corneas. Cases of CJD that occur at random throughout the world are categorised as sporadic if they
have no genetic mutation or any known iatrogenic infection, infections are not found outside the brain
Variant CJD [vCJD] (first known as New Variant CJD)
Prion found throughout the body in the lymphatic system and various organs e.g. tonsils, eyes, with
vCJD, latrogenic transmission may occur with greater frequency as instruments contaminated with
lymphatic tissue from other areas of the body are able to transmit the prion.
Familial CJD/ Fatal Familial Insomnia (FFI)
Vary rare (1 person in 10 – 100 million), normally found in clusters that may indicate genetic link
predisposing family members to this form of CJD. Special precautions are required for close blood
relatives of patients diagnosed with FFI.
It is responsible for about 15% of cases and is inherited. It is the caused by mutation in the prion
Iatrogenic CJD is acquired during medical treatments, particularly in the 1970’s, including use of
human pituitary derived growth hormones, through the use of human dura mater grafts and corneas
and through the use of contaminated instruments during surgery.
Gerstmann-Straussler-Scheinker Syndrome Very rare, treat as for FFI above.
Kuru Identified in a New Guinea tribe and thought to be a spread by cannibalism.
5. Roles and Responsibilities
5.1 Line Managers must ensure that:
• All clinical staff understand the risks regarding TSEs and how it is spread in order to apply
correct isolation procedures and infection prevention and control measures
• Staff have access and are aware of the policy
5.2 All staff that have direct contact with service users:
• Must ensure they are aware of the policy and utilise effective infection control procedures
• Attend infection control mandatory training
5.3 Doctors and Dentists
• Should assess all patients for the risk of CJD prior to any invasive procedure. The long pre-
clinical phase is followed by clinical features, which may vary depending upon the type of
CJD. The symptoms are generally progressive and there may also be rapid deterioration.
• CJD is a sensitive subject that has attracted a great deal of media interest, therefore
confidentiality is essential
Dentists and doctors should assess ALL patients for the risk of CJD prior to any invasive
These guidelines have been updated in accordance with current practice.
7. Equalities Impact Assessment
The Trust equality impact assessment template has been completed, and no potential discriminatory
impacts have been found.
8. Procedures and Precautions
8.1 The National CJD Surveillance
The National CJD Surveillance Unit (NCJDSU) is based in Edinburgh and was established in 1990,
primarily to inform the scientific community, policy makers and the general public of changes in the
epidemiology of CJD and vCJD and to identify potential risk factors.
All service users who have a possible diagnosis of a TSE, or in whom TSE is considered amongst the
differential diagnosis must be referred to the NCJDSU.
Notification to NCJDSU is the responsibility of the neurologist, neurosurgeon or other clinician
responsible for the service user, but notifications are also made by other health professionals and via
When notified, a neurologist from the NCJDSU will arrange to review the service user to assess
likelihood of CJD and to collect samples and data.
The NCJDSU will ask the referring team to inform the Consultant in communicable disease control,
based at the Health Protection Agency of all cases of possible, probable or definite CJD. The clinical
team must complete a specific CJD incident reporting form detailing the service user, possible risk
factors and previous surgical interventions, as part of the national formal reporting requirements. This
is required even if the service user has not had surgery or intervention during the current admission.
This form is downloadable and can be found through accessing the below link to the HPA website
then follow via infections a-z, CJD and incidents. This site will also link you to other specialist CJD
8.2 Infection Control Precautions and Risk Management
Social contact with a service user with CJD is not a risk to relatives or the community.
Normal or routine clinical contact with CJD service users does not present a risk to the health care
worker and there have been no confirmed cases of transmission of TSE to humans as a result of
occupation, although TSEs could be transmitted if exposure to infected tissues or materials by direct
inoculation (e.g. puncture wounds, ‘Sharps’ injuries or contamination of broken skin), by splashing
mucous membranes or, exceptionally, by swallowing.
The use of standard precautions will reduce risk and will minimise the exposure of individuals
involved in the health care of service users who have, or may develop TSE, and protect them from
the very remote possibility of infection.
Most of the procedures undertaken within the PCT are usually considered low risk.
There is no need to isolate a service user in a single room because of CJD status.
When considering measures to prevent transmission to service users or staff in the health care
setting, it is useful to make a distinction between:
• Symptomatic service users, i.e. those who fulfil the diagnostic criteria for definite, probable
or possible CJD or vCJD (see Appendix 1 & Annex B for full diagnostic criteria on
Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of
Infection 2003,rev 2010), and
• Service users at “increased risk”, i.e. those with no clinical symptoms, but who are “at
increased risk” of developing CJD or vCJD, because of their family or medical history.
Table 1: Categorisation of patients by risk (taken from TSE Agents: Safe Working and the
Prevention of Infection: Part 4 2003, updated 2010)
1. Symptomatic 1.1 Patients who fulfil the diagnostic criteria for definite, probable or
patients possible CJD or vCJD (Appendix 1)
1.2 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
2. Patients “at 2.1 Individuals who have been shown by specific genetic
increased risk” from testing to be at significant risk of developing CJD/other
genetic forms of CJD prion disease
2.2 Individuals who have a blood relative known to have a
genetic mutation indicative of genetic CJD
3.3 Individuals who have or have had two or more blood relatives
affected by CJD or other prion disease
3. Patients identified 3.1 Recipients of hormone derived from human pituitary
as “at increased risk” glands, e.g. growth hormone, gonadotrophin. In the UK the use of
of CJD/vCJD through human derived gonadotrophin was discontinued in 1973, and use of
iatrogenic exposures cadaver-derived human growth hormone was banned in 1985.
However, use of human-derived products may have continued in
other countries after these dates.
3.2 Individuals who underwent intradural neurosurgical or spinal
procedures before August 1992. These patients may have received a
graft of human-derived dura mater and should be treated as “at
increased risk”, unless evidence can be provided that human-derived
dura mater was not used. Patients who received a graft of human-
derived dura mater before August 1992 are at “increased risk” of
transmission of sporadic CJD
3.3 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
3.4 Individuals who have received an organ or tissue from a donor
infected with CJD/vCJD or “at increased risk” of CJD/vCJD
3.5 Individuals who have been identified prior to high risk surgery as
having received blood or blood components from 80 or more donors
since January 1980
3.6 Individuals who received blood from someone who went on to
3.7 Individuals who have given blood to someone who went on to
3.8 Individuals who have received blood from someone who has also
given blood to a patient who went on to develop vCJD
3.9 Individuals who have been treated with certain implicated UK
sourced plasma products between 1980 and 2001
Recipients of ocular transplants, including corneal transplants, are not considered to be “at increased
risk” of CJD/vCJD.
8.3 Management of “Risk” Patient Groups
Evidence does not suggest that normal social or routine clinical contact with a CJD or vCJD
service user presents a risk to healthcare workers, relatives and others in the community.
Isolation of service users with CJD or vCJD is not necessary, and they can be nursed in an open
ward using standard infection control precautions in line with those used for all other
The following advice is for those involved in the care of service users identified as risk patients in
8.3.1 Identification of Possible CJD Diagnosis after Admission / Treatment
Where it becomes known, after admission, that a patient may have CJD or a related disorder, advice
must be sought immediately from Director of Public Health, the Director of Infection Prevention &
Control (DIPC SG), the local Consultant Microbiologist, the local CCDC and the Infection Control
Nurse. The Director of Public Health must ensure that the DIPC is informed before contacting the
National CJD Incident Panel.
The CJD Incidents Panel provides advice to the local team on what action needs to be taken when a
patient who is diagnosed as having CJD or vCJS, underwent surgery or donated blood, organs or
tissues before CJD / vCJD was identified, will be responsible for identifying contacts who are
potentially at risk.
Any non-disposable surgical instruments/equipment used for such a patient must be identified
immediately and withdrawn from use for other patients pending further guidance.
Having sought advice, there should be agreement of a reasonable probability of diagnosis of CJD,
management of the patient should follow guidance given for “at risk” patients with the addition of the
following EXPRESS INSTRUCTIONS:
• Disposable, single use items should be used unless no such alternative is available.
• Non-disposable instruments must be quarantined pending confirmed diagnosis.
instruments should be washed to remove gross soil. Care must be taken to avoid
splashing. Protective clothing to be worn should include liquid repellent gown with a
plastic apron, gloves, mask, visor or goggles.
• Surfaces where instruments are sorted should be covered with disposable drape or
similar and all surfaces cleaned with hypochlorite using the manufacturers recommended strength
of solution for work surfaces. The most effective reduction or removal, results from the
thoroughness of the physical cleaning action and drying of the surface using disposable materials.
• All disposable drapes and clothing sealed in a yellow clinical waste bag and sent for
• Instruments should then be stored in a rigid plastic container with lid, sealed with
heavy-duty tape and labelled with the patient’s identification number. Stating, “QUARANTINED
INSTRUMENTS DO NOT USE”.
• The rigid plastic container should be stored in a suitably secure area pending
confirmation of diagnosis. If diagnosis of CJD OF ANY TYPE is confirmed, the bin and
contents should be incinerated as “Hazardous Waste” WITHOUT further examination.
• A record of staff involved in the clinical care must be kept.
Look back exercises
If a patient is subsequently identified as being diagnosed with any form of CJD after any
interventional procedure, a ‘look-back’ exercise may be required. Advice from your local Consultant
Microbiologist and Director of Public Health should be obtained in these circumstances.
The National CJD Incident Panel should be contacted for advice.
8.3.2 Exposure of a Healthcare Worker to CJD
In accordance with HSC 1999/178 in circumstances where there us likelihood of exposure
to a case of CJD EITHER because of a definite diagnosis, the presence of clinical symptoms,
or risk factors, a list of staff will need to be kept (in accordance with COSHH) if any of the
• Any staff performing invasive clinical procedures on patients suspected to be suffering
from CJD of any type.
• Laboratory staff handling tissue specimens.
The list must indicate the type of work done and record any specific exposure. Occupational Health
must be informed so that notes can be made and record kept for 40 years. ACDP/SEAC guidance
also recommends including information on; maiden name, NI number, Dob and dates of employment.
This list is not required for those involved in routine clinical care of service users with CJD/vCJD or
Although cases of CJD/vCJD have been reported in healthcare workers, there have been no
confirmed cases linked to occupational exposure. However, it is 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 “sharps” injuries, puncture wounds or
contamination of broken skin), and exposure of the mucous membranes (e.g. conjunctiva) should
also be avoided.
Compliance with standard infection control precautions will help to minimise risks from occupational
Healthcare personnel, who work with 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
8.4 Body Fluids
Body secretions, body fluids (including saliva, blood and cerebrospinal fluid (CSF) and excreta) are
all low risk for CJD/vCJD. Contact with small volumes of blood (including inoculation injury) is
considered low risk, though it is known that transfusions of large volumes of blood and blood
components may lead to vCJD transmission.
Any potential exposure to body fluids should be handled as for any service user, i.e. treated as
potentially infectious in line with standard infection control precautions. Provide relatives with
protective clothing for handling body fluids and information about the importance of hand hygiene and
Most infectivity is likely to be concentrated in the central nervous tissue. In vCJD, infectivity is also
likely to be present in lymphoid tissue, albeit at a lower level. It is important to ensure that only
trained staff, aware of the hazards, carry out invasive procedures involving body fluids that may lead
to contact with infective tissue.
Careful attention to standard infection control precautions will minimise any risks from blood. Drug
administration by injection should involve the same precautions used for all work of this type with any
service user, i.e. avoidance of sharps injuries and other forms of parenteral exposure and the safe
disposal of sharps and contaminated waste.
8.4.1 Body Fluid Spills
The infectious agent associated with TSEs is unusually resistant to inactivation techniques. Standard
infection control precautions should be used to clear up spillages as quickly as possible of all materiel
from service users with or “at increased risk” of, CJD/vCJD in a health care setting.
• In the service users home the area should be cleaned with detergent and water.
• The main defence is efficient removal of the contaminated material, thorough cleaning and
then disinfection using appropriate solution in the correct dilution.
• Dilution is the most important element in cleaning up spillages in clinical areas use 10,000
ppm available chlorine (sodium hypochlorite solution) on hard surfaces.
• The area MUST be well ventilated when using the solution (do not use with urine)
• Potentially infectious materials should be removed using absorbent material, and any
waste (including cleaning tools such as mop-heads) disposed of as clinical waste.
• Disposable gloves and an apron should be worn when removing such spillage(s).
8.4.2 Bed Linen
• Used or fouled bed linen (contaminated with body fluids or excreta), should be removed
from the bed and washed and dried as per normal practice in the health care setting (which is in
accordance with DH HSG (95)18. No further handling or processing
requirements are necessary.
• In the service users home foul linen should be washed separately in the normal way.
• Linen contaminated with CSF or high-risk materiel should be disposed of by incineration.
• Staff must wear gloves and aprons in line with infection control policy when handling soiled linen
8.4.3 Clinical Waste
Table 2 Disposal of clinical waste from patients with, or “at increased risk” of CJD/vCJD
Diagnosis of CJD High or medium risk tissue* Low risk tissue and body
Definite Incinerate Normal clinical waste disposal
Probable Incinerate Normal clinical waste disposal
“At increased risk” Incinerate Normal clinical waste disposal
*See Annex A1 http://www.dh.gov.uk/ab/ACDP/TSEguidance/index.htm
** Tissues and materials deemed to be low risk include body fluids such as urine, saliva, sputum, blood,
and faeces. Blood from vCJD patients is considered to be low risk except when transfused in large
This is unlikely to contain high risk material and should be disposed of in line with current arrangements,
risk assessments should be completed in the normal way. Follow PCT Waste Management Policy.
8.4.4 Invasive Medical Procedures and Samples
Because of the unusual resistance of the TSE agents:
• Single-use disposable equipment should be used wherever practicable
• All other small items of equipment contaminated whilst obtaining specimens should be destroyed by
Blood, biopsy and lumbar puncture samples from service users defined in Table 1 should only be
taken by competently trained personnel who are aware of the potential hazards involved. Under
Health and Safety at work legislation employers have a legal obligation to provide information,
instruction and training for staff involved in potentially hazardous tasks.
• Disposable gloves and eye protection should be worn where splashing may occur.
• Particular care should be taken with lymphoid tissue specimens.
• Standard practice should be to use disposable gloves, aprons and single-use disposable
instruments when performing a lumbar puncture for the collection of cerebrospinal fluid.
HSC 1999/178 specifically states that lumbar punctures should always be carried out
using single-use equipment. It also stresses that devices labelled as “single use” by the
manufacturer should not be re-used under any circumstances.
• Samples should be double bagged and marked with a “Bio-hazard” label, and it is important to
inform the laboratory in advance that a sample is being sent.
Where single-use instruments are not available, the handling of re-usable instruments
depends on a combination of the risk status of the patient, the tissue(s) involved in the
procedure, and the type of CJD. The following matrices (tables 3 & 4) set out the actions to be taken
for CJD and vCJD.
Table 3. Action to be taken with re-usable instruments for all CJD cases
other than vCJD
Status of patient
Tissue infectivity Definite/ Possible At increased risk
HIGH Single use
o Brain Single use Single use
o Spinal cord or
o Dura mater or or
o Cranial nerves, Destroy*
specifically the entire Destroy Quarantine
optic nerve and the pending or
intracranial or diagnosis
components of the Quarantine for re-use
other cranial nerves Quarantine exclusively on the
o Cranial ganglia for re-use same patient
o Posterior eye, exclusively
specifically the on the same
posterior hyaloid patient
face, retina, retinal
fluid and optic nerve
o Pituitary gland
MEDIUM Single use
o Spinal ganglia or
o Olfactory epithelium Destroy
on the same
LOW NO SPECIAL PRECAUTIONS
*In some cases, instruments destined for incineration can be sent for research. Contact Surgical
Instrument Store, HPA, Porton Down on 01980 612583 to discuss whether it would be helpful and
where and how it should be stored.
Table 4. Action to be taken with re-usable instruments for all vCJD cases
Status of patient
Tissue of infectivity Definite/ Possible At increased risk
HIGH Single use Single use Single use
o Spinal cord or or or
o Dura mater
o Cranial nerves, Destroy Quarantine Destroy*
specifically the entire pending
optic nerve and the or diagnosis or
components of the Quarantine Quarantine for re-use
other cranial nerves for re-use exclusively on the
o Cranial ganglia exclusively on same patient
o Posterior eye, the same
specifically the patient
face, retina, retinal
fluid and optic nerve
o Pituitary gland
MEDIUM Single use Single use Single use
o Spinal ganglia
o Olfactory epithelium or or or
o Appendix Destroy Quarantine Destroy*
o Spleen pending
o Thymus or diagnosis or
o Adrenal gland
o Lymph nodes and gut Quarantine Quarantine for re-use
associated lymphoid for re-use exclusively on the
tissues exclusively on same patient
LOW/none detectable NSP NSP
The CJD Incidents Panel may identify individual patients who have been potentially exposed to vCJD
(for example via surgical instruments used on a patient who went onto develop vCJD, or blood
products derived from a donor who went on to develop vCJD). In these circumstances the individuals
will have been informed of the risk by the Panel and advised to inform clinicians in the event of them
Relatives may wish to view or have final contact with the body. This is permitted. When a service
user, who is known, suspected or at risk of CJD or vCJD dies, current evidence suggests removal of
the body from the community setting, care home or hospital using normal standard infection control
measures. The use of a cadaver/body bag prior to transportation to the mortuary should occur in line
with normal precautions where there is known to be an infection risk, Annex H of the TSE Agents:
Safe Working and the Prevention of Infection (May 2010) advises labelling of the cadaver/body bag
as High Risk or Danger of Infection prior to transportation to the mortuary. The undertaker and the
receiving mortuary must be informed of the level of risk, either by the clinical team or the attending
General Practitioner. When the diagnosis of CJD/vCJD is known or suspected it is advisable to avoid
There is Health Services Advisory Committee guidance on “Safe working and prevention of infection
in the mortuary and post mortem room” (2nd ed, 2002 HMSO)
8.4.6 Reprocessing instruments
Single use instruments should be used where possible and provided they do not compromise the
care of the patient.
If reusable surgical instruments are used on any patient in the “at risk” category for other procedures
they MUST be processed following best practice guidance. This will include washing twice in a
validated washer disinfector and then sterilized six times at 134 degrees C for three minutes. The
instruments should then be re-washed and packed, sterilized and returned to use. (Constant research
around this topic remains ongoing).
The following recommendations regarding single use/re-usable instruments are made for
• Disposable Lumbar Puncture sets used on all patients.
• It has been suggested that single use equipment should be used for tonsillectomy,
where the quality of the instruments does not affect outcome. However, current NICE
guidance states that this is not justified by the evidence, and re-usable equipment can
be used except on patients identified in Table 1
• A system implemented to ensure tracing of equipment.
• Use of washer disinfectors for cleaning of equipment and instrument.
• A Risk Assessment should be carried out to determine patients who may be ‘at risk’ of CJD.
• Single use items MUST only be used once and then discarded.
• Any medical devices which come into contact with the surface of the eye e.g.
tonometer heads MUST be restricted to single patient use wherever practicable and
where this does not compromise clinical outcomes.
• Careful specification and quality control of instruments is essential for patient safety.
• All used instruments and protective clothing must be disposed of by INCINERATION.
• Use of enzymatic cleaners in ultrasonic washers is recommended
Quarantining of surgical instruments
Instruments that have been used on a possible CJD or vCJD patient must not be re-used,
but may be quarantined by securely labelling and storing in a rigid, sealed container after use, until
the diagnosis is confirmed.
If the case is confirmed as CJD or vCJD, or if after testing the diagnosis is inconclusive, the
instruments should be disposed of by incineration (or stored safely for use in research- see
Only if a definitive alternative diagnosis is confirmed may the instruments be decontaminated
following the usual routine procedures and returned to use.
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 Instruments Storage Facility.
Centre for Preparedness and Emergency Response.
Health Protection Agency.
Wiltshire. SP4 0JG
Phone 01980 612100
Fax 01980 612622.
This facility will send appropriate packaging and despatch instructions regarding the instruments to
the hospital/area to facilitate their transport to them. If the above facility does not require the
instruments, they should be disposed of by incineration.
8.5 Sources of Advice
The national CJD Surveillance Unit in Edinburgh can provide advice on all clinical and
neuropathological aspects of CJD. They can be contacted at:
The National CJD Surveillance Unit
Western General Hospital
Edinburgh EH4 2XUT
Tel 0131 537 2128
The CJD Support Network (voluntary organisation)
National CJD (Case) Coordinator
Market Drayton, Salop
Helpline tel: 01630 673973
Admin tel/fax: 01630 673993
9. Implementation Plan
• All staff must receive training in infection control as part of their induction programme as per PCT
• Clinical staff must have an annual infection control update this can be face to face training or
through e-learning as per MLE learning tree. Face to face update must occur every 2 years.
• Non-clinical staff must have 2 yearly infection control update, this can be face to face or through e-
learning as per MLE learning tree. Face to face update must occur every 4th year.
• All staff will have access to PCT Infection Prevention & Control Policy and Guidance either via PCT
website or by access to a hard copy.
• Infection control must be discussed at all staff clinical appraisals and is reflected in any personal
development plan and their job description as per Health and Social Care Act (2006, rev 2008).
The policy will be audited via annual peer audit or in the light of new research/guidance from the
appropriate government TSE research committees.
Annual audits of infection control standard precautions by the Prevention & Control of Infection Senior
Nurse in conjunction with the Infection Control Link Practitioners will identify adherence to this policy
in respect of sharps management, use of personal protective clothing, equipment, policy knowledge
and hand hygiene.
NICE Audit Criteria: ‘Interventional Procedure Guidance No. 196;
Patient Safety and Reduction of Risk of Transmission of Creutzfeldt–Jakob Disease (CJD) via
Interventional Procedures’ is also available through www.nice.org.uk
11. References & Links to other PCT documents:
Advisory Committee on Dangerous Pathogens (ACDP) and the Spongiform Encephalopathy Advisory
Group (SEAC) (1998) Guidance on Transmissible Encephalopathy Agents: Safe Working and the
Prevention of Infection HMSO London
Advisory Committee on Dangerous Pathogens (ACDP) and the Spongiform Encephalopathy Advisory
Group (SEAC) (2003, rev 2010) Transmissible Spongiform Encephalopathy Agents: Safe Working
and the Prevention of infection, Infection control of CJD and related disorders in the healthcare
setting Part 4 http://www.doh.gov.uk/cjd/riskassessmentsi.htm
CMO 2001/19 re-introduction of re-usable instruments for tonsil and adenoid surgery.
DH (2007) Decontamination of Re-Usable Medical Devices in the Primary, Secondary and Tertiary
Care Sectors (NHS and Independent providers) Clarification and Policy Summary. DH Gateway No
8199. Available at
DH (2006, rev 2008) The Health & Social Care Act HMSO, London
Health & Safety Executive (1998) The Reporting of Injuries, Diseases and Dangerous Occurrences
Regulations (RIDDOR). Guidance for employers in the healthcare sector. HSF Information Sheet No
1 4/98 HSE Books Sudbury
Health and Safety Executive (1999) Control of Substances Hazardous to Health regulations
HSE Books, Sudbury
Health Services Advisory Committee (2nd ed, 2002 HMSO) Guidance on “Safe working and
prevention of infection in the mortuary and post mortem room”
HSC 1999/178 Variant Creutzfeldt-Jakob Disease (vCJD): Minimising the Risk of
HSC 1999/179 Controls Assurance in Infection Control: decontamination of medical
HSG (95) 18 Health Service Guidance Hospital Laundry Arrangements for Used and Infected Linen
HMSO (1974) The Health & Safety at Work Act
Norwich, The Stationery Office
MDA DA2001 (10) Single use Devices for Tonsil and Adenoid Surgery. Dec 2001.
National Institute for Health and Clinical Excellence (2006) Interventional Procedure
Guidance 196. Patient safety and reduction of risk of transmission of Creutzfeldt-Jakob
disease (CJD) via interventional procedures. The full guidance, which includes a list of
all the surgical procedures covered by the guidance can be found on the NICE website at
PL CMO (2007)2: Decontamination of surgical instruments in light of National Institute for
Health and Clinical Excellence (NICE) Guidance – Patient safety and reduction of risk of
transmission of Creutzfeldt-Jakob disease (CJD) via interventional procedures and advice
from DH Engineering and Science Advisory Committee-Prion Removal (ESAC-Pr)
The decontamination of surgical instruments with special attention to the removal of
proteins and inactivation of any contaminating human prions. 2006 Report from ESAC-Pr
NHS South Gloucestershire Prevention and Control of Infection Policy and Guidelines Manual
incorporating HPA Infection Control Guidelines Community Settings
Hand Hygiene Policy
Wound Management Policy
Management of Medical Devices Equipment and Decontamination Policy
Clinical/Non Clinical Incident Reporting Policy Incorporating Being Open Policy & Procedure
Procedure for the transfer of Service User information (including infection risks)
Health & Safety Policy
Waste Management Policy
Induction Policy and Procedure
Protocol for Removal and Cleaning of Spillage’ in PCT Premises
Thanks to NHS North Somerset, North Bristol NHS Trusts & NHS Bristol Community Health Trust
DIAGNOSTIC CRITERIA FOR DEFINITE, PROBABLE OR POSSIBLE CJD/vCJD
Symptomatic patients are those who fulfil the internationally accepted diagnostic criteria, set out below, for
definite, probable and possible CJD or vCJD.
Neuropathological / immunocytochemical confirmation is required for a diagnosis of definite sporadic CJD.
Probable sporadic CJD patients will have rapidly progressive dementia, and at least two of the following four
(b) visual or cerebellar problems
(c) pyramidal or extrapyramidal features
(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 symptoms listed in
paragraph (a) – (d) above and a duration of less than 2 years.
Iatrogenic CJD patients display progressive cerebellar syndrome in a pituitary hormone recipient or sporadic
CJD with a recognised exposure risk (i.e. dura mater transplant). A definite diagnosis of iatrogenic CJD still
requires a neuropathological examination.
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.
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
Patients can be classified under two sets of criteria:
1. They will have progressive neuropsychiatric disorder of a duration greater than 6 months where routine
investigations do not suggest an alternative diagnosis. They will also have at least four of the following
(a) early psychiatric symptoms (depression, anxiety, apathy withdrawal, delusions)
(b) persistent painful sensory symptoms (inc frank pain and/or unpleasant dysaesthesia)
(d) myoclonus or chorea or dystonia
These patients would have had no history of potential iatrogenic exposure.
2. 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 atrogenic exposure, plus a positive tonsil biopsy, which is
positive for PrP-res.
Possible vCJD patients will have progressive neuropsychiatric disorder of a duration greater than 6 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 in (a) – (e) above and an EEG
does not show the typical appearance of sporadic CJD or no EEG has been performed.
ALGORITHM 1: FOR PRECUATIONS WITH SURGICAL PROCEDURES ON KNOWN, SUSPECT OR AT RISK PATIENTS; CJD OTHER
THAN VARIANT CJD
IN RISK CATEGORY NOT IN RISK
DEFINITE OR PROBABLE POSSIBLE CJD CATEGORY
GENETIC / FAMILIAL AND
PROCEDURE PROCEDURE PROCEDURE PROCEDURE IATROGENIC
INVOLVES INVOLVES INVOLVES INVOLVES
HIGH OR LOW RISK LOW RISK HIGH OR
MEDIUM RISK TISSUES TISSUES MEDIUM RISK
REPROCESS QUARANTINE** HIGH OR LOW RISK
INSTRUMENTS MEDIUM RISK TISSUES
ACCORDING TO TISSUES
AND RETURN TO
DEFINITE OR ACCORDING TO
DISPOSE OF PROBABLE CJD DISPOSE OF INSTRUMENTS BEST
INSTRUMENTS BY CONFIRMED OR REPROCESS BY INCINERATION*** PRACTICE* AND
INCINERATION*** DIAGNOSIS INSTRUMENTS RETURN TO USE
INCONCLUSIVE ACCORDING TO BEST
RETURN TO USE
* Refer to Annex C on Decontamination and Waste Disposal
** Refer to Annex E on Quarantine of Surgical Instruments
*** Instruments destined for disposal by incineration must be retained for research. Contact HPA, Porton Down 01980 61243
ALGORITHM 2: FOR PRECUATIONS WITH SURGICAL PROCEDURES ON KNOWN, SUSPECT OR AT RISK PATIENTS; VARIANT CJD
DEFINITE OR PROBABLE POSSIBLE vCJD IDENTIFIED BY CJD NOT IDENTIFIED BY CJD
vCJD INCIDENT PANEL AS INCIDENTS PANEL
‘AT RISK’ AS ‘AT RISK’
PROCEDURE PROCEDURE PROCEDURE PROCEDURE
INVOLVES INVOLVES INVOLVES INVOLVES
HIGH OR LOW RISK HIGH OR LOW RISK
MEDIUM RISK TISSUES MEDIUM RISK TISSUES PROCEDURE PROCEDURE
TISSUES TISSUES INVOLVES INVOLVES
HIGH OR LOW RISK
MEDIUM RISK TISSUES
INSTRUMENTS QUARANTINE** ACCORDING TO
ACCORDING TO BEST
BEST PRACTICE* PRACTICE* AND
AND RETURN TO RETURN TO USE
DEFINITE OR RETURN TO USE
DISPOSE OF PROBABLE CJD ALTERNATIVE DISPOSE OF
INSTRUMENTS BY CONFIRMED OR DIAGNOSIS INSTRUMENTS BY
INCINERATION*** DIAGNOSIS CONFIRMED INCINCERATION***
* Refer to Annex C on Decontamination and Waste Disposal
** Refer to Annex E on Quarantine of Surgical Instruments
*** Instruments destined for disposal by incineration must be retained for research. Contact HPA, Porton Down 01980 61243
Comprehensive Pre Surgical Assessment
It is essential that clinicians ask CJD risk questions to all patients about to undergo a surgical or endoscopic
procedure that may involve contact with tissues with high or medium level infectivity as part of the pre-
The medical team must
a. assess whether a patient has symptoms which fulfil the diagnostic criteria for definite,
probable or possible CJD or vCJD
b. identify patients with neurological disease of unknown aetiology and who do not fulfil
the criteria for possible CJD or vCJD but where diagnosis is being actively considered.
The medical team should ask the questions detailed over the page for all patients undergoing
elective/emergency surgical procedures likely to involve contact with tissues of potentially high or medium
Tissue Assumed level of infectivity
CJD other than vCJD vCJD
Brain High High
Spinal cord High High
Spinal ganglia High High
Dura mater High High
Cranial nerves High High
Cranial ganglia High High
Posterior eye High High
Anterior eye and cornea Medium Medium
Olfactory epithelium Medium Medium
Tonsil Low Medium
Appendix Low Medium
Spleen and thymus Low Medium
Other lymphoid tissues Low Medium
Table 5 Levels of CJD infectivity in tissue
Clinical questions to be asked prior to surgery
Have you a history of CJD / other prion disease in your family? If yes, please specify
Patients should be considered to be at risk frm familial forms of CJD linked to genetic mutations if they have
or have had:
• Genetic testing, which has indicated that they are at significant risk of developing CJD or other prion
• A blood relative known to have a genetic mutation indicative of familial CJD
• 2 or more blood relatives affected by CJD or other prion disease
Have you ever received growth hormone or gonadotrophin treatment?
If yes, please specify if the hormone was derived from human pituitary glands?
Recipients of hormone derived from human pituitary glands, e.g. growth hormone or gonadotrophin, have
been identified as potentially at risk of CJD. In the UK, the use of human-derived growth hormone was
discontinued in 1985 but human-derived products may continue to have been used in other countries.
Have you had surgery on your brain or spinal cord before August 1992?
People who underwent neurosurgical procedures or operation for a tumour or cyst of the spine before
August 1992 may have received a graft of dura mater, and should be treated as at risk, unless evidence can
be provided that dura mater was not used.
Have you ever been contacted as potentially at-risk of CJD for public health purposes?
The questions above may be re-phrased in order to make them rather more patient friendly, for example;
• Do you or anyone in your family have CJD, Creutzfeldt-Jakob Disease (the human form of mad cow
disease), or anything similar?
• Have you ever had growth hormone / other hormone treatment? (not insulin)
• Have you had brain/spinal surgery (backbone) surgery? Was this before 1992?
• Has anyone ever contacted you to say you have been put at risk of catching CJD, Creutzfeldt-Jakob
Disease, the human form of mad cow disease? This might have been the Blood Transfusion service,
a Public Health Doctor, or your own GP.
In addition to asking the patient CJD risk questions, the following actions should also be carried out before
any surgical procedure involving tissues with high or medium level infectivity to ensure that a comprehensive
pre-surgery assessment is carried out.
The clinician undertaking the pre-surgery assessment should:
• Check the patient’s medical notes and / or referral letter for any mention of CJD status.
Where a patient falls into a risk category, their hospital record should be electronically tagged on the
hospital patient administration system, and their hospital notes marked on the inside of the front
• Consider whether there is a risk that the patient may be showing the early signs of CJD, i.e. consider
whether the patient may have an undiagnosed neurological disease involving cognitive impairment or
These actions, in conjunction with the CJD risk question above, will minimise the chance of a CJD incident
occurring and therefore greatly reduce the risk of transmission of the CJD to subsequent patients.
SHOULD ANY PATIENT, AS A RESULT OF ANY ASSESSMENT, BE DEEMED TO BE AT RISK OF CJD OR vCJD IT IS IMPERATIVE
THAT INFECTION CONTROL ADVICE BE SOUGHT PRIOR TO SURGERY OR ENDOSCOPY
Management of Known or Suspected Patient Groups
Patients who are known or suspected of having CJD or other TSE infections should NOT be admitted
for routine / elective management, without prior discussion with the local Consultant Microbiologist
Precautionary measures for surgical procedures
It is the responsibility of the Consultant Surgeon to ensure risk questions are asked pre-operatively and
appropriate action taken to prevent cross infection.
Effective decontamination is key to reducing the risk of transmission via surgery.
The measures to be taken when performing invasive surgery depend on
• how likely the patient is to be carrying the infectious agent (risk status);
• how likely it is that infection could be transmitted by the procedure being carried out.
Management within the Operating Theatres
For all symptomatic patients (i.e. those who fulfil the criteria for definite, probable or possible CJD or vCJD),
the following precautions should be taken:
• Wherever appropriate, the intervention must be performed in an operating theatre;
• Where procedures are performed at the bedside, e.g. a lumbar puncture, care should be taken to
ensure the environment may be readily cleaned should a spillage occur
• Protective clothing should be worn by healthcare personnel performing diagnostic
o liquid repellent operating gown, over a plastic apron
o mask and goggles, or full–face-visor;
• Where possible, procedures should be performed at the end of the list, to allow normal
cleaning of theatre surfaces before the next session
• Only the minimum number of healthcare personnel required should be involved
• Use single-use disposable surgical instruments and equipment where possible and
destroy all single-use items by incineration.
• For non-disposable instruments refer to guidance and discuss with local microbiologist.
• The principles for reducing the risks from percutaneous exposure to blood-borne viruses apply
equally to CJD and implementation of methods and procedures to reduce such risks, e.g. the use of a
“ neutral zone” for instruments, should be implemented
• For asymptomatic patients at risk from familial or iatrogenic CJD, the same precautions apply,
but the protective clothing may be reprocessed if not designated single-use.