Creutzfeldt-Jakob disease (CJD) biannual update (August 2019) Health Protection Report Volume 13 Number 28 9 August 2019
Creutzfeldt-Jakob disease (CJD) biannual update (August 2019) Health Protection Report
Volume 13 Number 28
9 August 2019
Creutzfeldt-Jakob disease (CJD) biannual update (August 2019)
Health Protection Report Volume 13 Number 28
Creutzfeldt-Jakob disease (CJD)
biannual update (August 2019)
This six-monthly report provides an update on the enhanced surveillance of potential iatrogenic (healthcare-acquired) exposures to Creutzfeldt-Jakob Disease (CJD). The data is correct as of 30 June 2019. For numbers of CJD case reports, readers should consult
data provided by the National CJD Research and Surveillance Unit
(NCJDRSU, http://www.cjd.ed.ac.uk/surveillance/data-and-reports).
Monitoring of patients 'at increased risk' of CJD
Individuals who have been identified as ‘at increased risk’ of CJD as a consequence of their medical care are informed of their exposure and asked to follow public health precautions to avoid potentially transmitting the infection to others. They are also followed up to help determine the risks of CJD transmission to patients through different routes and to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.
Public Health follow up activities include clinical monitoring, General Practitioner (GP) updates, and post mortem investigations to determine whether asymptomatic individuals in these groups have been infected with the CJD agent. Some individuals also provide blood or tissue specimens for research purposes. A number of different organisations are involved in these activities: Public Health England (PHE), Health Protection Scotland (HPS), UCL Institute of Child Health/Great Ormond Street Hospital (ICH), NHS Blood and Transplant (NHSBT), National CJD Research and Surveillance Unit (NCJDRSU), National Prion Clinic (NPC), and the UK Haemophilia Centre Doctors' Organisation (UKHCDO).
The PHE CJD Section coordinates the collation of data on individuals identified as ‘at increased risk’ of CJD, and who have been informed of this. These individuals are followed up through public health monitoring and research activities by different organisations.
Creutzfeldt-Jakob disease (CJD) biannual update (August 2019)
Health Protection Report Volume 13 Number 28
The PHE CJD Section currently holds data on the following groups of patients who have been identified as ‘at increased risk’ of CJD:
• recipients of blood components from donors who subsequently developed vCJD
• blood donors to individuals who later developed vCJD
• other recipients of blood components from these blood donors
• recipients of certain plasma products between 1990 and 2001 (non-bleeding disorder patients)
• certain surgical contacts of patients diagnosed with CJD
• highly transfused recipients.
Data on the following risk groups are not held by PHE, but are held by other organisations:
• bleeding disorder patients who received plasma products between 1990 and 2001 (UKHCDO)
• recipients of human derived growth hormone before 1985 (ICH)
• patients who could have received a dura mater graft before August 1992 (data not currently collected)
• individuals treated with gonadotrophin sourced from humans before 1973 (data not currently collected)
• family risk of genetic prion disease (NPC).
Patients with bleeding disorders who received UK sourced plasma products (UKHCDO)
The definition of those considered at increased risk of CJD within this group was revised in 2012, following a re-assessment of the risk for vCJD transmission though blood. The change concerned the date from which the UK blood (and plasma) donor population was considered to have been significantly infectious for vCJD. The risk period was narrowed from 1980-2001 to 1990-2001. Individuals who were treated with UK sourced plasma products during the 1980s, but not between 1990 and 2001, have been informed of this change, where possible.
The data from the UKHCDO are likely to be a slight underestimate of the true number of patients with bleeding disorders who received UK-sourced clotting factors (1990 to 2001), as there was incomplete reporting of identified patients by haemophilia centres to the UKHCDO database. Notified patients are given the option of removing their details from the UKHCDO database, and are then removed from the ‘at increased risk' totals. The data on patients who received human-derived growth hormone held by the ICH is also a slight underestimate of the total as a small number of these patients are not included in the ICH follow-up.
Creutzfeldt-Jakob disease (CJD) biannual update (August 2019)
Health Protection Report Volume 13 Number 28
Summary of all ‘at increased risk’ groups on which data are collected (Data correct as of 30 June 2019)
‘At increased risk’ Group Identified as ‘at increased risk’ Number notifiedAll Alive Cases Asymptomatic infectionsa
Recipients of blood from donors who later developed vCJD 67 27 14 3 1
Blood donors to individuals who later developed vCJD 112 108 100 0 0 Other recipients of blood components from these donors 34 32 13 0 0
Plasma product recipients (non-bleeding disorders) who received UK sourced plasma products 1990-2001 2 2 2 0 0
Certain surgical contacts of patients diagnosed with CJD 299 253 205 0 0 Highly transfused recipients 3 3 1 0 0
Total for ‘at increased risk’ groups where PHE holds data 517 425 335 3 1
Patients with bleeding disorders who received UK sourced plasma products 1980-2001 b,e 3,601 3,248c 2,706 0 1
Recipients of human derived growth hormoneb 1,883 1,883 1,452d 80 0 Total for all ‘at increased risk’ groups 6,001 5,556 4,493 83 2
a. An asymptomatic infection is when an individual does not exhibit any of the signs and symptoms of CJD in life but abnormal prion protein indicative of CJD infection has been found in tissue obtained at post mortem.
b. These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and a small number of patients have opted out of the central UKHCDO database. A small number of ‘at increased risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of the ‘at increased risk’ growth hormone recipients have been notified. There is no central record of who has been informed.
c. These are the minimum number of people notified based on those patients who were seen for care after the notification exercise. It is likely that many more of the ‘at increased risk’ patients received their notification letter but as they were not subsequently recorded as being seen for care this cannot be confirmed.
d. Data is correct as of 31 December 2016. Information on non-CJD related deaths is currently not available.
e. Including a small proportion of individuals known to have been treated with UK plasma products 1980-2001, and presumed to have been treated 1990-2001
About Public Health England
Public Health England exists to protect and improve the nation’s health and wellbeing, and reduce health inequalities. We do this through world-class science, research, knowledge and intelligence, advocacy, partnerships and the delivery of specialist public health services. We are an executive agency of the Department of Health and Social Care, and are a distinct delivery organisation with operational autonomy. We provide government, local government, the NHS, Parliament, industry and the public with evidence-based professional, scientific and delivery expertise and support.
About Health Protection Report
Health Protection Report is a national public health bulletin for England and Wales, published by Public Health England. It is PHE’s principal channel for the dissemination of laboratory data relating to pathogens and infections/communicable diseases of public health significance and of reports on outbreaks, incidents and ongoing investigations.
Public Health England, Wellington House, 133-155 Waterloo Road, London SE1 8UG.
Tel: 020 7654 8000 www.gov.uk/phe
Twitter: @PHE_uk Facebook: www.facebook.com/PublicHealthEngland
Queries relating to this document should be directed to:
CJD Section, National Infection Service, PHE Colindale, 61 Colindale Avenue, London NW9 5EQ.
© Crown copyright 2019
You may re-use this information (excluding logos) free of charge in any format or medium, under the terms of the Open Government Licence v3.0. To view this licence, visit OGL. Where we have identified any third party copyright information you will need to obtain permission from the copyright holders concerned.
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