Download Creutzfeldt Jakob Disease (CJD) Policy

POLICY FOR THE MANAGEMENT OF PATIENTS WITH OR SUSPECTED
OF HAVING CREUTZFELDT JAKOB DISEASE (CJD)
Version
4
Name of responsible (ratifying) committee
Infection Prevention Management Committee
Date ratified
17 March 2017
Document Manager (job title)
Consultant Infection Prevention & Control,
Decontamination Manager
Date issued
30 March 2017
Review date
29 March 2020
Electronic location
Infection Control Policies
Related Procedural Documents
Decontamination of reusable medical devices,
Infection Control, Standard Precautions
Key Words (to aid with searching)
Transmissible spongiform encephalopathies (TSE’s),
Creutzfeldt-Jakob Disease (CJD), variant CJD
(vCJD), 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
4
17/03/2017
Updated links to PHE guidance
IPT/HSDU
3
28/01/2015
Amended following updates to guidance
D. Carter / C. Mitchell
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 1 of 22
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 .................................................................................................... 10
8.
REFERENCES AND ASSOCIATED GUIDANCE ....................................................................... 10
9.
EQUALITY IMPACT STATEMENT ............................................................................................ 11
10. MONITORING COMPLIANCE WITH PROCEDURAL DOCUMENTS ........................................ 12
APPENDIX I: Diagnostic criteria for CJD........................................................................................... 13
APPENDIX II: Risk categorization of patients having CJD / vCJD ..................................................... 15
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 ......................................................................... 16
APPENDIX IV: Distribution of Tissue Infectivity – please note recent updates .................................. 17
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 ................... 18
APPENDIX VI: Identification of a CJD surgical incident and procedure lookback periods ................. 19
APPENDIX VII: Algorithm for the processing of tissue from patients with or at risk of CJD including
vCJD .......................................................................................................................................... 20
EQUALITY IMPACT SCREENING TOOL ......................................................................................... 21
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 2 of 22
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), GerstmannStraussler-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 CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 3 of 22
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.
‘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’
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 4 of 22
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 CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 5 of 22
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
respond
Yes



Clarify specific details with patient
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
Contact with medium / high
risk infectivity tissues?1.
Yes


Further risk assess CJD /
vCJD infection using
questions in Appendix III2.
Seek further guidance from
the infection prevention and
control team
No
Proceed using
standard infection
control measures
1.
See Appendix IV for further information
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.
2.
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 6 of 22
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,
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
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 7 of 22
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
 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/A
nnex_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)
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:
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 8 of 22
-


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)
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
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 9 of 22
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.
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-riskmanagement-subgroup-formerly-tse-working-group) (2003) Updated October 2015
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 10 of 22
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)
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
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
Efficiency
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 CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 11 of 22
10. MONITORING COMPLIANCE WITH PROCEDURAL DOCUMENTS
This document will be monitored to ensure it is effective and to ensure compliance
Minimum
requirement to be
monitored
Lead
Tool
Frequency of Report
of Compliance
Reporting arrangements
Lead(s) for acting on
Recommendations
Infection Prevention
and control
protocols
Caroline
Mitchell
Infection
Prevention
and Control
report
Annually
Infection Prevention
Management Committee
CSC leads
Incidents reported,
documented and
escalated
appropriately
Caroline
Mitchell
Data
collection
Quarterly
Infection Prevention
Management Committee
CSC leads
Appropriate use of
medical devices
and adequate
tracking and tracing
Darren
Carter
Decontamin
ation Audits
Annually
Infection Prevention
Management Committee
CSC leads
Policy for the Management of patients with or suspected of having CJD
Version: 4
Issue Date: 30 March 2017
Review Date: 29 March 2020 (unless requirements change)
Page 12 of 22
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
Probable




Possible
Variant CJD
Definite
Neuropathological/ immunocytochemical confirmation needed
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
Rapid progressive dementia with two of the above symptoms and a
duration of less than 2 years
Progressive neuropsychiatric disorder and neuropathological confirmation
of the disease showing spongiform change and extensive PrPc deposition
with florid plaques throughout the cerebrum and cellebellum
Can be classified under two sets of criteria:
Probable





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
 Early psychiatric symptoms – depression, anxiety, apathy, withdrawal,
Policy for the Management of patients with or suspected of having CJD
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Review Date: 29 March 2020 (unless requirements change)
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



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 CJD
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Issue Date: 30 March 2017
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APPENDIX II: Risk categorization of patients having CJD / vCJD
Patient Groups
Symptomatic patients


Patients “at increased risk”
from genetic forms of CJD



Asymptomatic Patients
identified as “at increased
risk” of vCJD through
receipt of blood from a
donor who later developed
vCJD
Asymptomatic Patients
identified as “at increased
risk” of CJD/vCJD through
iatrogenic exposures

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
Individuals who have been shown by specific genetic testing to be
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
Individuals who have received labile blood components (whole
blood, red cells, white cells or platelets) from a donor who later
went on to develop vCJD

Recipients of hormone derived from human pituitary glands, e.g.
growth hormone, gonadotrophin, are “at increased risk” of
transmission of sporadic CJD. In the UK the use of humanderived 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 CJD
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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
prion disease in your family? If yes, please
specify
Notes to clinician
Patients should be considered to be at risk from
genetic forms of CJD if they have or have had
1. Genetic testing, which has indicated 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
or gonadotrophic treatment? If yes, please
specify;
i) Whether the hormone was derived from
human pituitary glands
ii) The year of the treatment
iii) Whether the treatment was received in
the UK or in another country
Notes to clinician
Recipients of hormone derived from human pituitary
glands e.g. growth hormone or gonadtotrophin have
been identified as potentially at risk of CJD. In the
UK, the use of human growth hormone was stopped
in 1985 but human-derived products may have been
continued to be used in other countries. In the UK,
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
or spinal cord?
Notes to clinician
Individuals who underwent intradural brain or
intradural spinal surgery before August 1992 who
received (or might have received) a graft of humanderived dura mater are “at increased risk” of
transmission of sporadic CJD (unless evidence can
be provided that human-derived dura mater
was not used).
Notes to clinician
Patients who have received blood from more than
80 donors have been identified as at increased risk
of CJD. Further information on this is available from
the HPA
(http://www.hpa.org.uk/vCJDpresurgicalassessment)
4 Since 1980 have you had any
transfusions of blood or blood components
(red cells, plasma, cryoprecipitate or
platelets). NB This does not include
autologous transfusion, plasma products
such as IVIG, albumin, coagulation factors
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 CJD
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APPENDIX IV: Distribution of Tissue Infectivity – please note recent updates
Tissue Infectivity Levels
High
Brain and spinal cord
Cranial nerves,
specifically the entire
optic nerve, only the
intracranial components
of other cranial nerves
Cranial ganglia
Posterior eye specifically
the posterior hyaloid
face, retina, retinal
pigment epithelium,
choroid, subretinal fluid
and optic nerve
Pituitary gland
Medium
Spinal ganglia
Olfactory epithelium
Tonsil (only vCJD)
Appendix (only vCJD)
Spleen and thymus (only
vCJD)
Other lymphoid tissues
(only vCJD)
Low
Anterior eye and cornea
Peripheral nerve
Dental pulp
Gingival tissue
Blood and bone marrow
CSF*
Placenta
Urine
Skeletal muscle
Other tissues
Tonsil (only CJD)
Dura mater
Lymph nodes (CJD only)
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 CJD
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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 CJD
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APPENDIX VI: Identification of a CJD surgical incident and procedure lookback
periods
Policy for the Management of patients with or suspected of having CJD
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APPENDIX VII: Algorithm for the processing of tissue from patients with or at risk of CJD including vCJD
Policy for the Management of patients with or suspected of having CJD
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EQUALITY IMPACT SCREENING TOOL
To be completed and attached to any procedural document when submitted to the
appropriate committee for consideration and approval for service and policy
changes/amendments.
Stage 1 - Screening
Title of Procedural Document: Policy for the Management of patients with, or suspected of having,
CJD
Date of Assessment
20/02/2017
Responsible
Department
Infection Prevention
Name of person
completing
assessment
Kathryn Noble
Job Title
Infection
Prevention
Manager/Analyst
Does the policy/function affect one group less or more favourably than another on the basis
of :
Yes/No
 Age
No
 Disability
No
Comments
Learning disability; physical disability; sensory
impairment and/or mental health problems e.g.
dementia
 Ethnic Origin (including gypsies and travellers)
No
 Gender reassignment
No
 Pregnancy or Maternity
No
 Race
No
 Sex
No
 Religion and Belief
No
 Sexual Orientation
No
If the answer to all of the above questions is NO,
the EIA is complete. If YES, a full impact
assessment is required: go on to stage 2, page 2
More Information can be found be following the link
below
www.legislation.gov.uk/ukpga/2010/15/contents
Policy for the Management of patients with or suspected of having CJD
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Stage 2 – Full Impact Assessment
What is the impact
Level of
Impact
Mitigating Actions
(what needs to be done to minimise /
remove the impact)
Responsible
Officer
Monitoring of Actions
The monitoring of actions to mitigate any impact will be undertaken at the appropriate level
Specialty Procedural Document:
Specialty Governance Committee
Clinical Service Centre Procedural Document:
Corporate Procedural Document:
Clinical Service Centre Governance Committee
Relevant Corporate Committee
All actions will be further monitored as part of reporting schedule to the Equality and Diversity
Committee
Policy for the Management of patients with or suspected of having CJD
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