Interim guidance on incidents involving inherited prion disease

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January 2007 1

CJD Incidents Panel

Interim guidance on incidents involving inherited prion disease

Aims

1. This document is for neurologists caring for patients with, or at risk of

inherited prion disease, and Consultants in Communicable Disease

Control (CCDC) and their equivalents throughout the UK.

2. Inherited prion disease incidents arise when patients with a genetic

mutation for an inherited form of prion disease, or their blood relatives,

have previously undergone invasive medical procedures or donated

blood or tissues.

3. The document sets out how inherited prion disease incidents should be

reported to the CJD Incidents Panel (Panel). It has been prepared by

the Panel, in consultation with the National Prion Clinic and the

National CJD Surveillance Unit.

4. This document complements the guidance on local reporting by

clinicians of CJD cases to public health departments

http://www.cjd.ed.ac.uk/guidance.htm.

Ethical concerns

5. Ethical difficulties may arise if patients choose not to tell their family

that they are at risk of inherited prion disease. In these situations,

family members who are not aware of their at-risk status might

inadvertently put other patients at risk of prion disease.

6. The Panel advises that infection control precautions should only be

taken once individuals are aware of their at-risk status. This means

that infection control precautions would not be taken for family

members who are not told about their at-risk status, should they need

surgery. The Panel is aware that this might put other patients at risk of

prion disease.

7. The Panel advises that the confidentiality of patients with inherited

prion disease, and their wishes with regard to informing others about

their condition, over-ride public health protection issues. This is

because there have not been any reports of iatrogenic transmission of

inherited prion disease. If such cases are reported, or there is new

evidence on infectivity in inherited prion disease, then the Panel will

review this position.

Incidents

8. Inherited prion disease incidents should be reported to the Panel, an

expert committee which was set up by the UK Chief Medical Officers.

The Panel advises local health teams throughout the UK on how to

manage these incidents which could pose a risk of spreading CJD to

other patients.

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9. Abnormal prion protein present in the tissues of people with or ‘at risk’

of inherited prion disease may not be completely removed by routine

decontamination processes. This means that surgical instruments that

have come into contact with the following tissues could pose an

infection risk to other patients:

• high-infectivity tissues (i.e. brain, spinal cord and posterior eye),

• medium-infectivity tissues (olfactory epithelium)

• medium-low infectivity tissues (anterior eye)

10. The risk of transmitting CJD via such surgical instruments decreases

each time they are used and decontaminated, but may not be entirely

eliminated.

Which patients to include

11. This protocol applies to three groups of patients:

a) Patients with inherited prion disease. These patients have a disease

specific mutation in the prion protein gene, and have symptoms of

inherited prion disease.

b) Asymptomatic patients who have been tested and found to have a

disease specific mutation in the prion protein gene.

c) Any blood relatives of patients in groups a) and b) who know about

their relative’s CJD status and about their own risk of developing

inherited prion disease, but have not themselves been tested for a

genetic mutation.

12. Patients in group b) and c) are ‘at risk’ of inherited prion disease and

‘presumed infected for public health purposes’. This is because nearly

all people in group b) and up to 50% of those in group c) will develop

inherited prion disease. Being ‘at risk’ of CJD has infection control

implications. Being ‘presumed infected for public health purposes’ has

implications for incident management.

13. The Panel advises that blood relatives who have not been informed of

their relative’s CJD status, and who are not aware of their own risk of

developing inherited prion disease, should not be managed as ‘at risk’

of CJD. Incidents involving these patients should not be reported.

14. Blood relatives who have been tested and do not have a disease

specific mutation in the prion protein gene, are not ‘at risk’ of CJD.

Incidents involving these patients should not be reported.

Which procedures to report

15. Please report all invasive medical procedures ever carried out on

patients with or ‘at risk’ of inherited prion disease (see 11a. b. and c.).

16. Please report all blood, tissue and organ donations made by these

patients. The Panel will then liaise with the UK Blood Services and

tissue/organ donation services as necessary.

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17. The Panel does not currently advise taking any action following medical

procedures which only involve low infectivity tissues or following blood

donations. These should still be reported, as Panel advice may change

in the future to reflect new evidence on CJD transmission risks.

Reporting incidents

18. The neurologist caring for a patient with or ‘at risk’ of inherited prion

disease should report incidents to the Panel using the reporting form

on the Health Protection Agency website

http://www.hpa.org.uk/infections/topics_az/cjd/incidents_panel.htm.


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The neurologist should include the patient’s name, date of birth, and

address in this correspondence (these are not included on the standard

reporting form).

19. Ideally, the neurologist will collaborate with the patient’s local CCDC to

complete this reporting form. If this is not possible, then the

neurologist should send a copy of the report to the patient’s local

CCDC, either directly or via the CCDC covering the neurology centre.

The hospital infection control team should be able to provide the

CCDC’s contact details.

20. The patient’s local CCDC should review the primary care records to

ensure that all relevant procedures have been reported to the Panel.

The CCDC should make sure the GP is aware of the patient’s ‘at risk’

status so that infection control precautions can be carried out.

21. Some individuals with disease specific mutations in the prion protein

gene may decide that their relatives should not be informed about their

risk of this inherited condition.

22. These patients’ wishes for confidentiality should be respected. The

Panel advises that public health measures can only be implemented for

‘at risk’ patients who are aware of their status. Therefore public health

precautions can not be implemented for relatives who have not been

informed of their risk of developing inherited prion disease.

23. In these cases, the clinician responsible for the index patient’s care

should satisfy him/herself that the patient has made an informed

decision that family members should not be told about their CJD risk.

24. The patient with a disease specific mutation may choose to inform their

family at a later date, or permit NHS staff to inform their relatives. If

this happens, the clinician should contact the GP and the patient’s local

CCDC to ensure that public health precautions are implemented, and

that any incidents are reported to the Panel.

Panel advice

25. The Panel may advise that other patients exposed in certain inherited

prion disease incidents should be contacted and considered as ‘at risk’

of CJD.

26. Instruments that may have been contaminated with medium or

medium-low infectivity tissues and have been through more than ten

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cycles of clinical use and satisfactory decontamination, are generally

unlikely to pose a significant infection risk to other patients.

27. Similarly, instruments that may have been contaminated with highinfectivity

tissues, and have been through more than twenty cycles of

clinical use and satisfactory decontamination, are generally unlikely to

pose a significant infection risk to other patients.

28. This general approach to managing surgical incidents should not be

interpreted as implying that potentially contaminated instruments can

be put prospectively through repeated decontamination cycles in an

attempt to remove any remaining abnormal prion infectivity.

Infection control precautions

29. All individuals with disease specific mutations in the prion protein gene,

blood relatives who know about their risk, and clinicians involved in

their care, should all be aware of the infection control precautions

which relate to CJD.

30. Healthcare professionals who are carrying out invasive medical

procedures will need to know about their patients’ ‘at risk’ status. Staff

directly involved in further invasive procedures on these patients

should be aware of the recommended infection control precautions,

and should ensure that preparations for any future medical procedures

are undertaken in conjunction with the Infection Control Team and the

Sterile Services Department.

31. Advice on infection control for these at risk patients is provided

through the ACDP Working Group on TSE’s guidance ‘Transmissible

Spongiform Encephalopathies: safe working and the prevention of

infection. Department of Health’. This is available on line at

http://www.advisorybodies.doh.gov.uk/acdp/tseguidance/Index.htm.

32. Routine dental procedures are considered low risk. Patients are asked

to inform dentists about their at risk status so that dentists can take

the opportunity to confirm that their decontamination procedures are

satisfactory and in case they refer them for head or neck surgery that

could involve contact with medium- or high-infectivity tissues. This

advice will be reviewed in the light of any new scientific evidence on

infectivity in human dental tissues.

33. Other doctors who care for the patients may need to be aware of the

at risk status to help them assess any clinical signs and symptoms.

We last revised this guidance in January 2007.

To check for any updates, please see the current version of this document,

‘Interim guidance on incidents involving inherited prion disease’.

You can find this at

www.hpa.org.uk/infections/topics_az/cjd/incidents_panel.htm


http://www.hpa.org.uk/infections/topics_az/cjd/inheritedCJD.pdf


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