This guidance is primarily for the use of clinicians caring for cases and suspect cases1 of Creutzfeldt-Jakob Disease (CJD) and Consultants in Communicable Disease Control (CsCDC) and their equivalents in the UK regions. It was prepared originally in 1999 as a collaborative exercise between the National CJD Surveillance Unit, Edinburgh, the Public Health Medicine Environmental Group, the Health Protection Agency Centre for Infections, Health Protection Scotland and the UK Health Departments. This updated version (2006) of the guidance will be kept under review and updated as necessary.
1Will RG, Zeidler M, Stewart GE, et al. Diagnosis of new variant Creutzfeldt-Jakob disease. Annals of Neurology 2000; 47: 575-582.
There is a need for CsCDC to be informed of cases and suspect cases of all types of CJD resident in their area. CsCDC are expected to notify the CJD Incidents Panel of all cases and suspect cases of all types of CJD that have undergone invasive medical and dental procedures. A local response may be required with respect to the potential secondary transmission, infection control, and any issues that may arise over time concerning the protection of the wider community. A local response should be complementary to national initiatives on investigation, control and research.
This document intends to provide guidance on reporting by clinicians of cases and suspect cases of all types of CJD, including variant and sporadic cases, to local CsCDC and on what action should be taken on receipt of such information.
Since July 2004, a national reporting system for CJD, announced by the UK Chief Medial Officers, has been in place. Local clinicians are asked to report all new suspect cases of CJD and other prion diseases to the National Creutzfeldt Jakob Disease Surveillance Unit (NCJDSU) in Edinburgh and the National Prion Clinic (NPC) in London with a copy to the local CCDC.
The NCJDSU currently collects, manages and analyses clinical and epidemiological information on all suspect cases of CJD in the UK. When a suspect case of CJD is referred to the NCJDSU, following consent from the patient/family, a neurologist from the NCJDSU visits and examines the patient and a research nurse completes a detailed risk factor questionnaire with a relative as part of an on-going MREC-approved case control study. Limited data are shared in strict confidence for epidemiological and public health purposes with a number of national bodies, including the UK Health Departments and the National Blood Authorities. As for other infections of substantial public health importance, it is essential to extend sharing of limited data with local public health colleagues as explained in this guidance. The NPC may also wish to visit cases of CJD as part of their research activities.
Action to be taken by LOCAL CLINICIANS and NCJDSU
At the initial visit to the case by the neurologist from the NCJDSU, he/she will ask the local neurologist, or other relevant clinician, to inform the local Consultant in Communicable Disease Control (CCDC) (or their equivalent in Scotland) of cases and suspect cases of CJD using a standardised form if they have not already done so (Appendix). If the local neurologist has already informed the CCDC with the initial referral to the NCJDSU and NPC, then no further action is necessary.
The neurologist should pass sufficient information on the case to the CCDC to enable public health follow up. If this has not already occurred, a letter, including a form for this purpose, will be given to the local clinician by the visiting NCJDSU neurologist (Appendix). Information passed to the CCDC should include the name of the patient, the NCJDSU unique patient number, type of CJD diagnosed or suspected, general practitioner details and what is known of the patient's history of invasive medical, surgical and dental procedures.
The local neurologist may ask the NCJDSU to pass on the information to the CCDC. It will be the responsibility of the CCDC to inform the Regional Epidemiologist of cases/suspect cases.
For cases referred to the NCJDSU after death, the clinician that was responsible for the patient will inform the local CCDC of the case following the post- mortem result (if available) or visit by the NCJDSU neurologist to the relatives of the case.
Referral to the relevant local Infection Control Team should also be carried out. The local Infection Control Team should be able to help you identify the contact details of your local CCDCC, if necessary.
Action to be taken by CsCDC on being informed of case of CJD or suspect case of CJD
CsCDC action will concern
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Control of a communicable disease. |
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Infection control. |
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Care of the patient. |
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Informing responses for the Primary Care Trust, Strategic Health Authority, Health Board, media etc. |
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Establishing if other CsCDC need to be aware of the case because of contact with different hospitals and/or other places where invasive procedures may have taken place. |
CsCDC will confirm with a neurology specialist at the NCJDSU that they are already aware of cases and suspect cases of CJD referred to them (NCJDSU tel. 0131 537 2128). The NCJDSU will carry out the epidemiological investigation of all cases/suspect cases of CJD.
CsCDC will refer all cases of CJD and those suspected of having CJD, who have a history of invasive medical or dental procedures, to the secretariat of CJD Incidents Panel, for advice on managing potential public health risks. The CJD Incidents Panel should be contacted through its secretariat at the Health Protection Agency, Communicable Disease Surveillance Centre (Dr Nicky Connor, Consultant Epidemiologist, tel. 0208 327 6411; nicky.connor@hpa.org.uk). Reporting forms are available here. All cases should be reported, including those who do not fulfil the surveillance criteria for CJD, but where the diagnosis of CJD is being actively considered. NCJDSU and the UK Blood Services (UKBS) identify any blood donations from patients with variant CJD. If the CCDC is informed by UKBS of an incident involving blood, the CCDC should inform the Incidents Panel .
CsCDC will ensure that if the patient has treatment or is resident in another health authority/health board area that the relevant local CCDC in that other area(s) is informed of the case.
CsCDC should be aware that the diagnostic classification might change over time as more information becomes available. For example, cases may be suspected as having CJD, but may not reach initially the diagnostic criteria of possible CJD. For these 'suspect' cases appropriate public health measures, according to this guidance, need to be implemented and updated accordingly.
Infection control - checks should be made with the relevant Trust Infection Control Team to ensure that all relevant guidance is being observed and appropriate action is taken if it is not. (HSC 1999/178 Variant Creutzfeldt-Jakob Disease(vCJD): Minimising the risk of transmission.
Advice on infection control for patients with CJD, suspected of having CJD, or at risk of CJD, is contained within the Joint ACDP/SEAC Working Group on TSE's Guidance "Transmissible Spongiform Encephalopathies: safe working and the prevention of infection" (revised July 2005).
Dental guidance was provided by the Chief Dental Officer in 2005 about the management of patients with CJD.
Care of the patient - ensure that those in Primary Care and the relevant Trusts caring for the patient are aware of current guidance and that a key worker has been identified. The CCDC is not responsible for the clinical care of the patient, but should know who is responsible. The National Care Co-ordinator for CJD is based at the NCJDSU (tel. 0131 537 3073 or 0131 537 2128).
Information management - in most cases there will be no need for any proactive dissemination of information, but the CCDC may be required to prepare informed reports and comments for the Primary Care Trust, Strategic Health Authority, Health Board, UK Health Departments and the media.
Normal rules of medical confidentiality apply to all cases and suspect cases of CJD. It is always desirable that patient's or relative's permission is sought prior to passing on named patient details to others who need to know. It may not always be possible to obtain permission, particularly if the patient has died.
Tables of definite and probable cases of variant CJD by place of last residence will be sent quarterly by the NCJDSU to each Regional Epidemiologist and UK Health Departments. The former will circulate to relevant Districts.
Possible connection between cases of variant CJD noted by the NCJDSU will be reported to the relevant local CsCDC, or their Scottish equivalent, and Regional Epidemiologist(s) following discussion by the National vCJD-Geographically Associated Cases Investigations Working Group. A collective decision will then be made whether to carry out a local investigation in collaboration with the National Working Group.
Any possible connection between cases of vCJD noted by those in a local area should be reported to the NCJDSU before any local epidemiological investigations are carried out.
It is imperative that there is a standard approach co-ordinated by the National vCJD-Geographically Associated Cases Investigations Working Group when considering whether to investigate and when investigating a potential cluster or connection between cases of vCJD. According to the National Protocol for the Investigation of Geographically Associated Cases of vCJD this approach will be lead locally and involves a close collaboration between the NCJDSU, HPA CDSC (Heath Protection Scotland, in Scotland), the Regional Epidemiologist, local public health, environmental health and veterinary specialists and local clinicians (http://www.cjd.ed.ac.uk/geo.html).
This guidance will be reviewed in November 2007, if not earlier. Comments on this guidance are welcome and should be sent to Dr Hester Ward, Consultant Epidemiologist, National CJD Surveillance Unit or to Dr Martin Schweiger, CCDC, Health Protection Agency, Leeds.
November 2006
To: All Neurologists in the UK November 2006
Dear Colleague,
RE: REPORTING CJD CASES, INCLUDING SUSPECT CASES, TO LOCAL PUBLIC HEALTH (HEALTH PROTECTION) TEAMS
The National CJD Surveillance Unit is working with public health doctors to prevent any possible spread of CJD between people. This is vital, as patients with CJD may have had surgery, or donated blood or other tissues.
To carry out this important public health action, when you refer cases and suspect cases to us, PLEASE ALSO INFORM YOUR LOCAL CONSULTANT IN COMMUNICABLE DISEASE CONTROL (CCDC) (or equivalent in Scotland), who are part of your local Health Protection Team (or Health Board in Scotland) if you have not already done so. You may want to use the accompanying form when making referrals to your local CCDC. Your Trust’s Infection Control Team, who should also be informed of the case/suspect case, should be able to help you identify the contact details of your local CCDC.
Public health doctors should be informed about ALL SUSPECT CASES OF CJD (INCLUDING SPORADIC AND VARIANT) using the accompanying form. All cases should be reported, including those who do not fulfil the surveillance criteria for CJD, but where the diagnosis of CJD is being actively considered.
The public health doctors will then work with the CJD Incidents Panel (secretariat provided by the Health Protection Agency Centre for Infections) to investigate and manage any public health risk.
All cases of suspect CJD should continue to be notified to the National CJD Surveillance Unit (NCJDSU) in Edinburgh and the National Prion Clinic (NPC) in London in accordance with the system outlined in the CMO's letter of July 2004.
Many thanks for your help.
Yours sincerely,
Dr Richard Knight, Consultant Neurologist, NCJDSU
Dr Hester Ward, Consultant Epidemiologist, NCJDSU