INFECTION CONTROL MANUAL
Chapter 27
Creutzfeldt-Jakob Disease (CJD) and other
Transmissible Spongiform Encephalopathies (TSEs)
Version 8
St Helens & Knowsley Teaching Hospitals NHS Trust- Infection Control Manual - Chapter 27 – CJD Policy– Version 08 (clinical)
Type of document
Policy
Code
STHK 0119
Approved by
Chairman Clinical Performance Council
Policy Sponsor
Medical Director
Lead Executive
Medical Director
Recommended by:
Hospital Infection Prevention Committee
Date recommended :
23 August 2012
Approved by:
Clinical Performance Council
Date approved :
10 September 2012
Author(s):
Director of Infection Prevention & Control
Date issued:
1 October 2012
Review date:
1 October 2015
Target audience:
All clinical staff
Document purpose
This policy describes the infection control precautions for
patients with suspected or confirmed CJD
Training requirements
Refer to induction, mandatory and risk management training
policy – training needs analysis. (2011)
Associated Infection Control Manual chapters:
Associated documents and Chapter 21
Hand decontamination policy
Key References
Chapter 5:
Personal protective equipment
Chapter 9:
Disinfection Policy
Chapter 22
Safe use and disposal of sharps
Chapter 15
Waste disposal
Chapter 4
Care of the isolated patient
Chapter 11D
Operating Theatre Guidelines
Chapter 16
Removal & care of infected bodies
Key words
Issue Date: 1st October 2012
References
Transmissible Spongiform Encephalopathy agents: safe
working and the prevention of infection. Advisory Committee
on Dangerous Pathogens Spongiform Encephalopathy
Advisory Committee, June 2003.
http://www.dh.gov.uk/ab/ACDP/TSEguidance/DH_098253
NICE ICP196: Patient safety and reduction of risk of
transmission of Creutzfeldt–Jakob disease (CJD) via
interventional procedures, 2006.
http://www.nice.org.uk/nicemedia/pdf/ip/IPG196guidance.pdf
Creutzfeldt-Jakob Disease, CJD, Transmissible
Spongiform Encephalopathy, TSE, prions.
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Consultation, Communication
Consultation Required
Analysis of the effects on equality
Low Impact
External Stakeholders
Trust staff consultation via intranet
Authorised By
Date Authorised
Director of Infection 1 October 2012
Prevention & Control
None
Start date: N/A
End date: N/A
Policy Implementation Plan
Describe
implementation Timeframe for RAG
plan for policy
implementation
The Policy will be published on 1 October 2012 G
the intranet. Ward Managers
ensure that paper copies of the
Infection Control Manual are
kept up-to-date. Staff are
informed of Infection Control
Manual at induction
Who is responsible
Director
of
Infection
Prevention & Control
Performance Management of policy Key Performance Indicators (KPIs) (expected
outcomes)
Key Performance How will the Which
Frequency of Lead
Indicators (KPIs)
KPI
be Committee
Review
Monitored?
will
Monitor
this KPI?
None specific to this
policy (see related
Infection Control
Manual chapters)
Issue Date: 1 October 2012
Not applicable
Not applicable
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Not applicable
Not applicable
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Performance Management of minimum NHSLA processes for this policy.
(If policy is not an NHSLA general standard requirement: N/A not applicable)
Minimum
requirement
to be
monitored
Process for Responsible
monitoring individual/
e.g. audit
group/
committee
Frequency
of
monitoring
N/A
N/A
N/A
N/A
Learning from experience
Who is responsible for producing
Action Plans if deficits in KPIs and
associated processes identified?
Performance management of
minimum requirements.
Responsible individual, group or
committee (+ frequency of
review/timescales) for:
Review DevelopMonitorin
of
ment and g of
results update of action
action
plan and
plan
implementation
N/A
N/A
N/A
Which Committee
Will Monitor These
Action Plans?
Frequency of Review
Not applicable
Not applicable
Not applicable
How does learning occur?
Who is responsible Frequency
for disseminating
learning
information
Not applicable
Not applicable
Not applicable
Archiving including retrieval of archived document
By whom will policy be
archived and retrieved
Archiving and retrieval will be in line with Policy on Policies Senior Web Administrator
(Document Control Policy)
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St Helens & Knowsley Teaching Hospitals NHS Trust- Infection Control Manual - Chapter 27 – CJD Policy – Version 08 (clinical)
Document Version History
Date
Version
Summary of key changes
Author
Designation
1
DIPC
2
DIPC
3
DIPC
4
DIPC
5
DIPC
6
DIPC
October 1998
21st December 1999
1st October 2001
1st December 2003
1st December 2006
1st November 2008
7
Format changed.
Page 9: 5.2.1 Linen to be
processed normally.
Waste disposal: Table added
Page 10: “HSDU” replaced by
“Trust Sterile Services”.
Page 11 Specimens for the
laboratory. Procedure amended.
Page 12: Location for
quarantining instruments
changed to: secure cupboard in
the HSDU Dirty Hub (one on
each site).
Page 17 Appendix B: list of at risk
patients updated.
Page 18 Appendix C revised with
new guidelines on risk &
quarantining of instruments.
Appendices D & E deleted.
Page 26 Appendix F: Patient
information updated.
8
Page 2: references updated.
DIPC
Appendices G and H added: risk
assessments for patients
undergoing endoscopy or surgical
procedures.
DIPC
1st October 2011
1 October 2012
1st
October 2015
Issue Date: 1 October 2012
Review
date
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DIPC
St Helens & Knowsley Teaching Hospitals NHS Trust- Infection Control Manual - Chapter 27 – CJD Policy – Version 08 (clinical)
Content
Page
5.1 Introduction
Examples of TSEs
The agents responsible
Definitions of patient risk groups
8
8
8
8
5.2 Isolation precautions
Linen
Waste disposal
Spillages
Sharps injuries
9
9
9
9
9
5.3.Clinical procedures
Phlebotomy
Drug administration by injection
Insertion of intravascular lines
Wound re-dressing
Lumbar puncture
Surgical procedures
Childbirth
Specimens for the Laboratory
Visits to other departments
9
9
9
9
10
10
10
10
11
11
5.4 Reporting of suspected cases
11
5.5 Quarantining of surgical instruments
12
5.6 Staff records
12
5.7 After death of the patient
13
Glossary
13
References
14
Appendices
Appendix A: Classification and diagnostic criteria
Appendix B: Categorisation of patients by risk
Appendix C: Categorisation of tissue infectivity
& handling of instruments
Appendix D: Useful contacts
Appendix E: National CJD reporting form
Appendix F: Patient/carer information
Appendix G: Pre-operative/endoscopy risk assessment
Appendix H: Risk assessment for patients undergoing surgery
on high risk tissues
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15
17
19
22
23
26
27
28
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Executive Summary
Policy aim
This policy aims to reduce the risk of transmission of the prions which cause
Transmissible Spongiform Encephalopathies (TSEs) e.g. Creutzfeldt-Jakob disease
(CJD) to patients or staff within the hospital setting.
.
Policy description
Infection control precautions for patients with confirmed or suspected Creutzfeldt-Jakob
Disease (CJD) and other Transmissible Spongiform Encephalopathies (TSEs).
1. Introduction
Although CJD and other TSEs are rare, they are incurable brain diseases which are
invariably fatal. Therefore it is essential that staff follow the correct infection control
procedures in order to prevent transmission to other patients and to staff.
2. Policy Objectives
The objective of the policy is to provide information to staff on the precautions necessary
to minimise the risk of occupational exposure to TSEs e.g. Creutzfeldt-Jakob disease
(CJD) and to prevent transmission of TSEs between patients.
3. Definitions
Creutzfeldt-Jakob disease (CJD) and the other Transmissible Spongiform
Encephalopathies (TSEs) are rare, degenerative, invariably fatal brain disorders. These
prion diseases are characterised by accumulation of abnormal prion protein (PrP) in the
brain.
4. Duties, accountabilities and responsibilities
For full details of infection control responsibilities see Infection Control Policy, Chapter
28B Infection Control Manual.
4.1. Staff
It is the responsibility of all staff to:
 be aware of the current guidelines.
 put these guidelines into practice.
 bring to the attention of the Unit Manager or Infection Prevention and Control
Team any problems in applying these guidelines.
Breaches of this policy may lead to disciplinary action being taken against the individual.
4.2. Unit managers (person in charge of a ward or department) must ensure that
 The policy is readily accessible to all staff.
 The required facilities and equipment are available to enable compliance with the
policies.
 All staff within their area of responsibility have received training in the appropriate
procedures with respect to infection control.
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5. Process
5.1 Introduction
Transmissible Spongiform Encephalopathies (TSEs), also known as prion diseases, are
fatal degenerative brain diseases.
The name is derived as follows:
Transmissible: the disease can be transmitted from person to person.
Spongiform: holes (vacuoles) appear in the brain, giving it a sponge-like
appearance.
Encephalopathy: abnormality of the brain.
Examples of TSEs
In humans:
In cattle:
In sheep:
Creutzfeldt-Jakob Disease (CJD)
New variant CJD (vCJD)
Kuru
Gerstmann-Straussler-Scheinker syndrome (GSS)
Fatal familial insomnia.
Bovine Spongiform Encephalopathy (BSE)
Scrapie
All human TSEs are very rare. The incidence of CJD is about 1 per million people each
year. The other human TSEs are even more rare.
The agents responsible
TSEs are caused by unconventional infectious agents, currently thought to be infectious
proteins, or prions, which do not have the same properties as bacteria or viruses.
Prions are concentrated in the brain, spinal cord and lymphoreticular tissues of the
affected individual, causing dementia and death.
Prions have exceptional ability to withstand all conventional methods of decontamination
e.g. heat and disinfectants.
Prions are NOT highly contagious and do not spread by normal contact. They spread by
inoculation of infected neural tissue e.g. neurosurgery (on instruments or dura mater
grafts), corneal transplant, injection of hormones (e.g. growth hormone/gonadotrophin)
derived from the pituitaries of cadavers. There is no evidence of transmission to health
careworkers during standard patient care.
Definitions of patient risk group
See Appendix A for diagnostic criteria and Appendix B for categorisation of patients by
risk.
The prion protein is more widespread in the body of vCJD patients than patients with
sporadic CJD. In sporadic CJD prion is restricted to the CNS. In vCJD prions may be
found also in lymphoid tissues including tonsils, spleen, appendix, rectum, thymus and
adrenal gland. Therefore precautions for surgical procedures differ.
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5.2 Isolation precautions
The patient with CJD or other TSE can be nursed on the open ward. No special
precautions are required for excreta, saliva or body secretions. Normal social or routine
clinical contact does not present a risk to healthcare workers or other patients.
5.2.1 Linen: Used or fouled bed linen should be dealt with the same as for other
patients. No further handling or processing is necessary.
5.2.2 Waste disposal:
Disposal of clinical waste from patients with, or ‘at increased risk’ of CJD or vCJD:
Diagnosis of CJD*
Definite
High or medium risk
tissue**
Incinerate
Probable
Incinerate
‘At increased risk’
Incinerate
Low risk tissue and
body fluids**
Normal clinical waste
disposal
Normal clinical waste
disposal
Normal clinical waste
disposal
*See appendix A
**Tissues and materials deemed to be low risk include body fluids such as urine,
saliva, sputum, blood, CSF and faeces. Blood from vCJD patients is considered to
be low risk except when transfused in large volumes. See appendix C for further
details.
5.2.3 Spillages: Disposable gloves and plastic aprons must be worn. Use disinfectant
appropriate to the type of spillage (see Disinfection Policy, Chapter 9, Infection Control
Manual) e.g. 10,000ppm sodium hypochlorite is recommended for blood/CSF spillage.
Dilution is the most important element in cleaning up spillages.
5.2.4 Sharps injury: See Chapter 22, Infection Control Manual.
5.3 Clinical procedures
5.3.1 Phlebotomy/Drug administration by injection/Insertion of intravascular lines
It is not known whether CJD can be transmitted by needle-stick injury. Contact with
small volumes of blood (including inoculation injury) is considered low risk, though it is
known that transfusion of large volumes of blood and blood components may lead to
vCJD transmission.
Standard infection control procedures will minimise any risk. Take care during the
handling and disposal of any sharps.
Phlebotomy: only experienced staff should take blood from these patients.
Drug administration by injection: only experienced staff should give injections to
these patients.
Insertion of intravascular lines: only experienced staff should insert intravascular lines
into these patients.
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5.3.2 Wound re-dressing
Late stage CJD patients may suffer from pressure sores. These lesions should be
dressed regularly, using standard infection control precautions. Contaminated dressings
should be disposed of as clinical waste.
5.3.3 Lumbar puncture
Only experienced staff should carry out this procedure.
Wear gloves, plastic apron. Use eye protection if splashing may occur.
Use only single-use disposable instruments.
Affix Danger of Infection sticker to specimen(s) and request form(s) and inform the
laboratory in advance.
5.3.4 Surgical procedures and endoscopy
The Department of Health recommend that all patients about to undergo any surgery or
endoscopy should undergo an assessment to determine their TSE risk status. Guidance
on performing this risk assessment can be found in Appendix G. It is the responsibility of
the treating consultant to ensure that this risk assessment has been completed prior to
the procedure.
The patient must be last on the list.
Involve only the minimum number of staff required.
Use disposable drapes and dressings.
Protective clothing (in addition to standard operating theatre outfit)
Water repellent gown, over a plastic apron
Gloves
Mask and goggles, or full-face visor
All protective clothing must be destroyed by incineration after use.
Instruments
To determine whether the instruments need to be incinerated/quarantined/processed for
re-use:
1.
Check what category the patient falls into (Appendix A and B).
2.
Determine tissue infectivity (Appendix C). This table tells you how the instruments
should be processed.
Complex instruments
Expensive instruments may be prevented from being contaminated by using shield
guards or coverings so that entire items do not need to be destroyed. Only parts in
contact with high risk tissues and the protective coverings would need to be incinerated.
Laser techniques for tonsillectomy
There is no evidence of transmission of TSEs by the respiratory route. Any risk from
smoke plumes is thought to be very low.
5.3.5 Childbirth
Involve only the minimum number of staff required.
Use disposable drapes and dressings.
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Protective clothing (in addition to standard outfit)
Water repellent gown, over a plastic apron
Gloves
Mask and goggles or full-face visor
All protective clothing must be destroyed by incineration after use.
Instruments
Responsibilities: See Trust Sterile Services/theatre operational procedures.
To determine whether the instruments need to be incinerated/quarantined/processed for
re-use:
1.
Check what category the patient falls into (Appendix A and B).
2.
Determine tissue infectivity (Appendix C). This table tells you how the instruments
should be processed.
Placenta
The placenta, other associated material and fluids should be treated as if infected and
disposed of as infectious clinical waste by incineration, unless they are needed for
investigation.
5.3.6 Specimens for the laboratory
Blood and body fluid samples from patients with, or “at increased risk” of, CJD/vCJD,
should be treated as potentially infectious for blood-borne viruses and handled with
standard infection control precautions as for any other patient, i.e.;
 use of disposable gloves and eye protection where splashing may occur;
 avoidance of sharps injuries and other forms of parenteral exposure;
 safe disposal of sharps and contaminated waste and
 single-use disposable equipment should be used wherever practicable.
Samples from patients with, or “at increased risk” of, CJD/vCJD should be marked with a
‘Biohazard’ label. It is advisable to inform the laboratory in advance that a sample is
being sent.
5.3.7 Visits to other departments
For non-invasive investigation eg X-ray, no specific precautions are required.
5.4 Reporting of suspected cases
From July 2004, a new national reporting system commenced. All new suspect cases of
CJD/vCJD and other prion diseases must be notified to the National Creutzfeldt Jakob
Disease Surveillance Unit (NCJDSU) in Edinburgh and the National Prion Clinic (NPC)
in London (see Contacts – Appendix D) with a copy to the local CCDC (see Appendix E
for form). Failure to notify would prevent early action to trace and withdraw blood
donation and to institute other infection control measures.
Staff from the National Prion Clinic will visit and provide patients/families with an
information leaflet explaining the work of both the National CJD Surveillance Unit and
the National Prion Clinic. Details of the CJD Support Network are given in Appendix F.
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On encountering patients whom they suspect to be suffering from CJD/vCJD, or a
related prion disease, consultant neurologists (or other clinician) will:
 complete the national reporting form and obtain consent from the patient, lead
relative or carer/patient representatives;
 fax or post the completed form, with consent, to the National CJD Surveillance
Unit and the National Prion Clinic;
 advise the patient, carer, or independent representative that staff from the
National CJD Surveillance Unit will visit the patient for national surveillance
purposes (with their consent).
 consider utilising the expertise in diagnosis and management of patients offered
by staff at the National Prion Clinic;
 advise the patient, carer or independent representative that staff from the
National Prion Clinic will visit
 make available samples of blood and cerebrospinal fluid (CSF), and the results of
Magnetic Resonance Imaging (MRI) scans to the two units.
5.5 Quarantining of surgical instruments and endoscopes
This applies to instruments used while awaiting confirmation of the diagnosis of
CJD/vCJD. Destroy single use instruments by incineration.
Re-usable instruments should be washed to removed gross soiling. Take care to avoid
splashing and aerosols holding instruments under the surface of the water in a sink into
which water is running and draining out continuously. Wear gloves and eye protection.
Take care to avoid sharps injury.
Allow to air dry in disposable bag. Then seal in impervious rigid plastic container with
close fitting lid (available via Decontamination Contracts Manager (Ext 6947) or
Decontamination Manager (Ext. 6948)). Label with patients ID (Name, Hospital Number
and DOB), the surgical procedure performed, and name of responsible person (e.g.
theatre manager). Securely store in the secure cupboard in the HSDU Dirty Hub (one
on each site) until the outcome of any investigation is known. If confirmed CJD/vCJD,
incinerate without further examination.
Only if an alternative definitive diagnosis is confirmed, remove from box by responsible
person, decontaminate in usual way and return to normal use.
Keep a record of all action taken.
5.6 Staff records
Occupational Health Records will need to record employees exposed to CJD/vCJD. The
routine clinical care of patients with CJD or a related disorder is unlikely to pose a
significant risk of exposure to CJD of any type and staff working with such patients
would not need to be included on such a list.
The list should be kept where there is a likelihood of exposure and not simply when
there has been a known incident or accident, although it should also include details of
these.
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Examples:

Staff performing invasive clinical procedures on patients suspected to be
suffering CJD, especially where there is risk of exposure to CNS/eye.
 Laboratory staff handling tissue specimens (not blood) from patients with
CJD.
 Staff undertaking postmortem examinations of patients who may have died
of CJD.
The list must include
Name (full name and maiden name), NI number, DOB, dates of employment
Type of work
Any specific exposure/accident/incident which may be reportable under RIDDOR
The list must be kept for 40 years.
Occupational Health.
Staff must report any such exposures to
5.7 After death
A body bag is required (see Chapter 16 Infection Control Manual). The relatives may
view the body. Even if post mortem has taken place, viewing and possible superficial
contact such as touching or kissing need not be discouraged. Body bags may be rolled
down temporarily to allow superficial contact. None of the patient’s tissues may be used
for transplant purposes. Embalming is not permitted. There is no need for special
arrangements for burial or cremation.
Advice for Pathologists on the post mortem examination of patients with
known/suspected prion disease is available on:
http://www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/@dh/@ab/documents/di
gitalasset/dh_116540.pdf
Glossary
BSE: Bovine Spongiform Encephalopathy
CCDC: Consultant in Communicable Disease Control
CJD: Creutzfeldt-Jakob Disease
CNS: Central nervous system
CSF: Cerebrospinal fluid
DOB: Date of birth
EEG: electroencephalogram
GSS: Gerstmann-Straussler-Scheinker syndrome
NCJDSU: National Creutzfeldt Jakob Disease Surveillance Unit
NPC: National Prion Clinic
RIDDOR: Reporting of Injuries, Diseases and Dangerous Occurrences Regulations
TSEs: Transmissible Spongiform Encephalopathies
vCJD: New variant CJD
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References
1. Transmissible Spongiform Encephalopathy agents: safe working and the prevention
of infection. Advisory Committee on Dangerous Pathogens Spongiform Encephalopathy
Advisory Committee, June 2003.
http://www.dh.gov.uk/ab/ACDP/TSEguidance/DH_098253
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Appendix A: Classification and diagnostic criteria
Sporadic CJD
Definite: Neuropathological/immunocytochemical confirmation is required for a
diagnosis of definite sporadic CJD.
Probable: Probable sporadic CJD patients will have rapidly progressive dementia,
and at least two of the following four symptoms:
a.
myoclonus
b.
visual or cerebellar problems
c
pyramidal or extrapyramidal features
d
akinetic mutism
plus typical electroencephalogram (EEG with generalised triphasic periodic complexes
at approximately 1 per second.
or clinical criteria for possible sporadic CJD (see below) and a positive assay for 14-3-3
protein in the cerebrospinal fluid (CSF).
Possible: Possible sporadic CJD patients will have rapidly progressive dementia, two of
the symptoms listed in (a) – (d) above and a duration of less than 2 years.
Iatrogenic CJD
Iatrogenic CJD patients display progressive cerebellar syndrome in a pituitary hormone
recipient or sporadic CJD with a recognised exposure risk (i.e. treatment with human
pituitary growth hormone, human pituitary gonadotrophin or human dura mater graft;
corneal graft in which the corneal donor has been classified as definite or probable
human prion disease; exposure to neurosurgical instruments previously used in a case
of definite or probable human prion disease or transfusion of blood from a donor
subsequently diagnosed with vCJD). A definite diagnosis or iatrogenic CJD still require
a neuropathological examination.
Familial CJD
Patients with familial CJD will have definite or probable CJD (as 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.
variant CJD (vCJD)
Definite: Definite vCJD patients will have a progressive neuropsychiatric disorder
and neuropathological confirmation of the disease, showing spongiform change and
extensive PrPSc deposition with florid plaques throughout the cerebrum and cerebellum.
Probable: Probable vCJD patients can be classified under two sets of criteria:
i.
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 five symptoms:
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a.
b.
c.
d.
e.
early psychiatric symptoms (depression, anxiety, apathy, withdrawal,
delusions)
persistent painful sensory symptoms (including both frank pain and/or
unpleasant dysaesthesia)
ataxia
myoclonus or chorea or dystonia
dementia
An EEG will not show the typical appearances of sporadic CJD, or no EEG has
been done and there is symmetrical high signal in the posterior thalamus on a
MRI brain scan.
These patients would have had no history of potential iatrogenic exposure or
evidence of a familial form of TSE.
ii.
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 iatrogenic exposure or evidence of a familial form of TSE, plus a tonsil
biopsy which is positive for PrPSc
Possible: 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 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.
Patients who do not fulfil the criteria for possible CJD
The NCJDSU have designated three additional categories for patients who are referred
to the Unit but who do not meet the criteria for possible CJD. These can be summarised
as:
i.
Diagnosis unclear – the diagnostic criteria for definite, probable or possible CJD
are not met, nor is there a reasonable alternative diagnosis. CJD, therefore,
remains a possibility.
ii.
CJD thought unlikely – information indicates that a clinical diagnosis of CJD is
very unlikely because of atypical disease features, and/or an atypical course,
and/or atypical clinical investigation results, and/or a reasonable alternative
diagnosis is made but it not confirmed. This category includes cases which
recover clinically without a firm alternative diagnosis.
iii.
Definitely not CJD – information indicates that CJD is not the diagnosis and
there is an alternative definite diagnosis proven on the basis of clinical
examination, clinical investigations or pathology.
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Appendix B: Categorisation of patients by risk
Patients should be categorised as follows, in descending order or risk:
1. Symptomatic patients
1.1 Patients who fulfil the diagnostic criteria for
definite, probable or possible CJD or vCJD (see
Appendix A for diagnostic criteria).
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.
Asymptomatic patients at 2.1 Individuals who have been shown by specific
risk from genetic forms of CJD.
genetic testing to be at significant risk of
developing CJD.
2.2 Individuals who have a blood relative known to
have a genetic mutation indicative of genetic CJD;
2.3 Individuals who have or have had two or more
blood relatives affected by CJD or other prion
disease
3.
Asymptomatic
patients
potentially at increased risk of
CJD/vCJD through iatrogenic
exposures.
Issue Date: 1 October 2012
3.1 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
human-derived gonadotrophin was discontinued in
1973, and use of cadaver-derived human growth
hormone was banned in 1985. However, use of
human-derived products may have continued in
other countries after these dates.
3.2 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).
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
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St Helens & Knowsley Teaching Hospitals NHS Trust- Infection Control Manual - Chapter 27 – CJD Policy – Version 08 (clinical)
components from 80 or more donors since January
1980;
3.6 Individuals who have received blood from
someone who went on to develop vCJD;
3.7 Individuals who have given blood to someone
who went on to develop vCJD;
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
N.B. Recipients of ocular transplants, including corneal transplants, are not considered
to be “at increased risk” of CJD/vCJD.
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St Helens & Knowsley Teaching Hospitals NHS Trust- Infection Control Manual - Chapter 27 – CJD Policy – Version 08 (clinical)
Appendix C: Categorisation of tissue infectivity & handling of instruments
Patients with, or ‘at increased risk’ of, CJD (other than vCJD)
Tissue Infectivity
High*
Brain, Spinal cord
Cranial nerves, specifically
the entire optic nerve and the
intracranial components of the
other cranial nerves
Pituitary gland
Cranial ganglia
Definite or
Probable
Status of patient
Possible
At increased
risk
Single use
Single use
Single use
or
or
or
Destroy
Quarantine for
re-use
exclusively on
the same
patient
pending
diagnosis
Destroy
or
or
Posterior eye, specifically
the posterior hyaloid face,
retina, retinal pigment
epithelium, choroid, subretinal
fluid and optic nerve
Quarantine for
re-use
exclusively on
the same
patient
Quarantine for reuse exclusively
on the same
patient
Medium
Spinal ganglia
Single use
Single use
Single use
or
or
or
Destroy
Quarantine for
re-use
exclusively on
the same
patient
pending
diagnosis
Destroy
No special
precautions
No special
precautions
Olfactory epithelium
or
Quarantine for
re-use
exclusively on
the same
patient
pending
diagnosis
Low
No special
Blood, bone marrow, urine CSF, precautions
placenta, anterior eye, cornea,
peripheral nerve, skeletal
muscle, dental pulp, gingival
tissue, dura mater*, tonsil,
lymph nodes, gut-associated
lymphoid tissue, appendix,
spleen, thymus.
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or
Quarantine for reuse exclusively
on the same
patient
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St Helens & Knowsley Teaching Hospitals NHS Trust- Infection Control Manual - Chapter 27 – CJD Policy – Version 08 (clinical)
Handling of instruments – patients with, or ‘at increased risk’ of, vCJD
Tissue Infectivity
Definite or
probable
High*
Brain
Spinal cord
Pituitary gland
Cranial nerves, specifically
the entire optic nerve and
the intracranial
components of the other
cranial nerves
Cranial ganglia
Posterior eye, specifically
the posterior hyaloids face,
retina, retinal pigment
epithelium, choroid,
subretinal fluid and optic
nerve.
Medium
Spinal ganglia
Olfactory epithelium
Tonsil
Appendix
Spleen
Thymus
Adrenal gland
Lymph nodes and gutassociated lymphoid
tissues
Low
Blood, bone marrow, urine
CSF, placenta, anterior
eye, cornea, peripheral
nerve, skeletal muscle,
dental pulp, gingival
tissue, dura mater.*
Issue Date: 1 October 2012
Status of patient
Possible
At increased risk
Single use
Single use
Single use
or
or
or
Destroy
Quarantine for re- Destroy
use exclusively
on the same
or
patient pending
diagnosis
Quarantine for reuse exclusively on
the same patient
or
Quarantine for
re-use
exclusively on
the same
patient
Single use
Single use
Single use
Or
Or
Or
Destroy
Quarantine for re- Destroy
use exclusively
on the same
Or
patient pending
diagnosis
Quarantine for reuse exclusively on
the same patient
Or
Quarantine for
re-use
exclusively on
the same
patient
No special
precautions
No special
precautions
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No special
precautions
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*Although dura mater is designated low infectivity tissue, procedures conducted on
intradural tissues (i.e. brain , spinal cord and intracranial sections of cranial nerves) or
procedures in which human dura mater has been implanted in a patient prior to 1992,
are high risk and instruments should be handled as such.
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Appendix D: USEFUL CONTACTS

The National CJD Surveillance Unit in Edinburgh can provide advice on clinical and
neuropathological aspects of CJD.It should be notified of any patient suspected on
clinical grounds of having CJD. It can be contacted at::
Director, National CJD Surveillance Unit
Western General Hospital
Crewe Road
Edinburgh EH4 2XUT
Tel: 0131 537 2128 (Clinical office)
0131 537 1980 (Pathology Telephone)
Fax: 0131 343 1404
Email: jan.mackenzie@ed.ac.uk

The National Prion Clinic at St Mary’s Hospital, London specialises in the care of
patients
suffering from CJD. It should be notified of any patient suspected on
clinical grounds of having CJD. It can be contacted at:
National Prion Clinic
Department of Neurology
St Mary’s Hospital
Praed Street
London W2 1NY
Tel: 020 7886 6883
Fax: 020 7061 9889
Email: help.prion@st-marys.nhs.uk

The CJD Support Network is a voluntary organisation set up to provide help and
support for patients of all types of CJD and their families. The Network had
undertaken a case co-ordination initiative aimed at facilitating the co-ordination
enabling cost effect care and ensuring appropriate responses to carer’s needs. It
can be contacted at:
National CJD Co-ordinator
CJD Support Network
PO Box 346
Market Drayton, Shropshire TF9 4AR
Telephone number 01630 673993 (admin)
Telephone number 01630 673973 (helpline).
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Appendix E: NATIONAL CJD REPORTING FORM
Fax to: NCJDSU 0131 343 1404, NPC 0207 061 9889 and also to your local CCDC
when completed.
Patient details
Surname:………………………………………Forename(s):…………………………
Postal
address:………………………………………………………………………………………
…………………………………………………………………………………………………………
…………………………………………………………………………………………
Postcode:………………………………………...
Telephone number:…………………………..
Fax Number:…………………………………….
Email Address:…………………………………...
NHS Number, if
known:…………………………………………………………………………………
Family, carer or independent representative details (if appropriate*)
* This may be appropriate if the approach is made via a lead family member, carer or
independent representative (i.e. when a patient is too ill to be approached directly or has a
preference for this route).
Surname:……………………………………….Forename(s):………………………………
Postal Address:…………………………………………………………………………………………
……………………………………………………………………………………………………………
……………………………………………………………………………………………
Postcode:………………………………………...
Telephone number:…………………………..
Fax Number:…………………………………….
Email Address:…………………………………...
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Neurologist details (or other hospital clinician)
Surname:……………………………………….Forename(s):………………………..
Hospital Postal
Address:……………………………………….……………………………………………..…
……………………………………………………………………………………………………
……………………………………………………………………………
Postcode:………………………………………..
Telephone number:…………………………..
Fax Number:…………………………………….
CCDC details
Surname:……………………………………….Forename(s):…………………………
Postal Address:……………………………………….……………………………………………..
…………………………………………………………………………………………………
Postcode:………………………………………...
Telephone number:…………………………..
GP Details
Surname:……………………………………….Forename(s):……………………………
GP Practice Postal
Address:……………………………………………………………………….……………...
…………………………………………………………………………………………………………
…………………………………………………………………………………………
Postcode:………………………………………...
Telephone number:…………………………..
Fax Number:…………………………………….
Brief clinical details: (please attach recent letter or discharge summary)
…………………………………………………………………………………………………………
…………………………………………………………………………………………………………
…………………………………………………………………………………………………………
…………………………………………………………………………………………………………
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Consent:
*please delete as appropriate
I have been provided with the patient information leaflet which explains the
roles of the National CJD Surveillance Unit and the National Prion Clinic.
YES/NO
I agree to my/the patient’s* details being forwarded to the National CJD
Surveillance Unit and the National Prion Clinic.
YES/NO
I agree that staff from the National CJD Surveillance Unit in Edinburgh and
the National Prion Clinic in London can visit myself/the patient* and
my/their* relatives at a mutually convenient time for clinical assessment
and surveillance purposes and to provide the opportunity, should we wish,
to discuss ongoing research, including clinical trials of potential
treatments.
YES/NO
I understand that this may mean providing further information to help in the
organisation of my/the patient’s* care, and to contribute to a better understanding of
the illness.
YES/NO
Signed:……………………………………………………………………………
Print:………………………………………………………………………………
Date:………………………………………………………………………………
On completion, please fax to NCJDSU 0131 343 1404, NPC 0207 061 9889 and also to
your local CCDC.
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Appendix F: PATIENT/CARER INFORMATION
Many patients and carers find it helpful to talk to others with experience of the same
diseases. The CJD Support Network is a UK charity for people with any type of prion
disease and their families and carers. The confidential helpline offers support and
practical information. There is a website, and regular newsletters, with lots of useful
information.
Because these diseases are very rare, many local medical and care services have no
experience of them. But you are not alone. The support groups, together with the
specialist centres, are committed to making sure you don't miss out on whatever help is
available. You don't have to wait until you have a firm diagnosis - support is there, if you
want it, from the moment you have to consider the possibility of prion disease.
The CJD Support Network is a UK charity for people with any type of CJD.
Helpline: 01630 673973
http://www.cjdsupport.net/index.php
Information leaflets for patients can also be obtained from the HPA website:
http://www.hpa.org.uk/Topics/InfectiousDiseases/InfectionsAZ/CreutzfeldtJakobDisease/
InformationLeafletsForPatientsAndHealthcareProfessionals/
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Appendix G: ASSESSMENT FOR ALL PATIENTS UNDERGOING ELECTIVE OR
EMERGENCY SURGICAL OR ENDOSCOPIC PROCEDURES
All patients about to undergo any elective or emergency surgical or endoscopic
procedure should be asked the question:
“Have you ever been notified that you are at increased risk of CJD or vCJD for
public health purposes?”
Any patients undergoing surgery involving high risk tissues should have a more
detailed risk assessment performed (see Appendix H) unless the patient has already
answered YES to the question above.
The patient’s response should be documented in the patient’s medical records.
The actions to take following the patient’s response to the above question are outlined
below:
Patient’s response
Action
No
Surgery or endoscopy should proceed using
normal infection control
procedures unless the procedure is likely to
lead to contact with high risk
tissue.
Please ask the patient to explain further.
Special infection control precautions should
be taken for all surgery or
endoscopy involving contact with medium or
high infectivity tissues and the Infection
Prevention and Control Team should be
consulted for advice as soon as possible
before the procedure is carried out.
Yes
Unable to respond
Issue Date: 1 October 2012
Surgery or endoscopy should proceed using
normal infection control
procedures unless the procedure is likely to
lead to contact with high risk
tissues referred to in Appendix C - the only
type of surgery under taken on high risk
tissue at St Helens and Knowsley Teaching
Hospitals is posterior eye surgery. In this
case refer to Appendix H.
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Appendix H: RISK ASSESSMENT FOR PATIENTS ABOUT TO UNDERGO
ELECTIVE OR EMERGENCY SURGICAL PROCEDURES LIKELY TO INVOLVE
CONTACT WITH HIGH-RISK TISSUES
All patients undergoing surgery involving high risk tissues must have the following risk
assessment performed and documented in their medical notes.
The only type of surgery carried out on high risk tissues at St Helens and
Knowsley Teaching Hospitals is posterior eye surgery.
Procedures should not be delayed whilst information is being collected, and clinicians
should be careful not to prejudice overall patient care.
Question to patient
Have you a 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:
i) Genetic testing, which has indicated that they are at
significant risk of developing CJD or other prion
disease;
ii) A blood relative known to have a genetic mutation
indicative of genetic CJD or other prion disease;
iii) 2 or more blood relatives affected by CJD or other
prion disease
2
Have you ever received
growth hormone or
gonadotrophin treatment?
If yes, please specify:
i) whether the hormone
was derived from human
pituitary glands
ii) the year of treatment
iii) whether the treatment
was received in the UK or
in another country
Recipients of hormone derived from human pituitary
glands, e.g. growth hormone or gonadotrophin, have
been identified as at increased risk of sporadic CJD.
In the UK, the use of human-derived growth hormone
was discontinued in 1985 but human-derived
products may have continued to be used in other
countries.
In the UK, the use of human-derived gonadotrophin
was discontinued in 1973 but may have continued in
other countries after this time.
3
Have you ever had
surgery on your brain or
spinal cord?
Patients who underwent intradural neurosurgical or
spinal procedures before August 1992 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 humanderived dura mater before 1992 are at increased risk
of transmission of sporadic CJD, but not vCJD.
1
Note that NICE guidance emphasises the need for a
separate pool of reusable surgical instruments for high
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risk procedures on children born since 1 January 1997
and who have not previously undergone high risk
procedures. Any such reusable instruments should
not be used on patients born before 1 January 1997 or
those who underwent high risk procedures using
reusable instruments before the implementation of this
policy.
4
Since 1980, have you
had any transfusions of
blood or
blood components (red
cells, plasma,
cryoprecipitate or
platelets*)?
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
received blood or blood
components.
The actions
as follows:
Patient’s
response
No to all
questions
Yes to
any of
questions
1,2 or 3
Patients who have received blood from more than 80
donors have been identified as at increased risk of
vCJD.
Information on this is available from the HPA:
http://www.hpa.org.uk/vCJDpresurgicalassessment
Patients treated with UK sourced plasma products
should already have been assessed and informed of
their risk by their Haemophilia Centre or other relevant
clinician.
* This does not include:
 Autologous transfusion
 Plasma products such as intravenous
immunoglobulin, albumin, coagulation factors
and anti-D
to be taken according to the patient’s responses to the above questions are
Action
Surgery can proceed using normal infection control procedures.
Further investigation into the nature of the patient’s CJD risk should be
undertaken, and the patient’s CJD risk assessed. This assessment of CJD
risk should be recorded in the patient’s medical notes for future reference.
If the patient is found to be at increased risk of CJD or vCJD following
investigation, or the risk status is unknown at the time of the
procedure, special infection control precautions should be taken as
described in this policy. The Infection Prevention and Control Team
should also be contacted.
If the patient is found to be at increased risk of CJD or vCJD they should be
referred to their GP, who will need to inform them of their increased risk of
CJD or vCJD and provide them with further information and advice. This is
available from the CJD Incidents Panel:
http://www.hpa.org.uk/CJDIncidentsPanel
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Yes to
question 4
Unable to
answer
Patients who are at increased risk of genetic forms of CJD should be
offered the opportunity of referral to the National Prion Clinic, based at the
National Hospital for Neurology and Neurosurgery, Queen Square, London:
http://www.nationalprionclinic.org/
Patients who are at increased risk of sporadic CJD due to receipt of
human-derived growth hormone or gonadotrophin should be offered the
opportunity of referral to the UCL Institute of Child Health, London. Contact:
L.Davidson@ich.ucl.ac.uk, 020 7404 0536
The infection prevention and control team should be contacted for further
assistance.
In the event that a patient about to have emergency surgery is physically or
otherwise unable to answer any questions, a family member, or someone
close to the patient (in the case of a child, a person with parental
responsibility), should be asked the CJD risk questions as set out above
prior to the surgery.
If the family member, or someone close to the patient, is not able to provide
a definitive answer to the CJD risk questions, the surgery should proceed
but all instruments should be quarantined (see section 5.5). The patient’s
GP should be contacted after the surgery, and enquiries made as to
whether the patient is at increased risk of CJD/vCJD according to the
questions above. Based on this, after discussion with the Infection
Prevention and Control Team, a decision can then be made as to what to
do with any quarantined instruments.
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