Research and analysis

Creutzfeldt-Jakob disease (CJD) biannual update (August 2014), with updated guidance on decontamination of gastrointestinal endoscopy equipment

Updated 10 February 2017

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 at 30 June 2014. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU).

Following the enhanced surveillance report, an update on the guidance for the decontamination of equipment for gastrointestinal endoscopy is presented.

1. 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 (formerly the Health Protection Agency), 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.

The PHE CJD Section currently holds data on the following groups of ‘at risk’ patients:

  • 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)
  • people who have been treated with gonadotrophin sourced from humans before 1973 (data not currently collected)
  • family risk of inherent prion disease (NPC).

The data from the UKHCDO are likely to be an underestimate of the true number of ‘at risk’ patients with bleeding disorders who received UK-sourced clotting factors, as there was incomplete reporting of identified ‘at risk’ patients by haemophilia centres to the UKHCDO database. Notified ‘at risk’ patients are given the option of removing their details from the UKHCDO database, and are then removed from the ‘at risk’ totals.

The data on ‘at risk’ patients who received human-derived human growth hormone held by the ICH is a slight underestimate of the total as a small number of these patients are not included in the ICH follow-up.

Table 1. Summary of all ‘at risk’ groups on which data are collected (data correct as at 30 June 2014)

At risk Group Identified as ‘at risk’ Number notified as being ‘at risk’   Cases Asymptomatic infections [b]
    All Alive    
Recipients of blood from donors who later developed vCJD 67 27 14 3 1
Blood donors to individuals who later developed vCJD 112 108 104
Other recipients of blood components from these donors 34 32 [c] 19 [c]
Plasma product recipients (non-bleeding disorders) who received UK sourced plasma products 1980-2001 11 10 4
Certain surgical contacts of patients diagnosed with CJD 196 161 [d] 140 [e]
Highly transfused patients 11 10 5
Total for ‘at risk’ groups where PHE holds data 431 348 [f] 286 [f] 3 1
Patients with bleeding disorders who received UK-sourced plasma products 1980-2001 [a] 3,977 National information incomplete National information incomplete 1
Recipients of human-derived growth hormone [a] 1,883 1,883 1,503 75
Total for all ‘at risk’ groups [a] 6,291 At least 2,231 At least 1,789 78 2
           

a. 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 risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of ‘at risk’ growth hormone recipients have been notified. There is no central record of who has been informed.

b. 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 from them. In these cases the abnormal prion protein was identified at post mortem.

c. One patient was notified by proxy.

d. Five of these notified by proxy.

e. Three of these were notified by proxy.

f. Includes patients notified by proxy.

2. Updated guidance on decontamination of equipment for gastrointestinal endoscopy

The British Society of Gastroenterology have updated their guidance on decontamination of equipment for gastrointestinal endoscopy [1]. The update includes revised advice for management and decontamination of endoscopes after they have been used for procedures on individuals at risk of variant CJD. This aligns with Department of Health guidance, CFPP 01-06, published in April 2013 [2], which recommended that: There is no longer a requirement to quarantine endoscopes following an “invasive” procedure on patients at risk of vCJD (with very few exceptions); a single quality assured decontamination cycle following recommended guidelines is considered sufficient, but the endoscope should be decontaminated separately from others with a single-use disinfectant; routine traceability data should be available to demonstrate thorough reprocessing; ‘Single use’ accessories should always be used in preference to reusable accessories.

The ACDP TSE specific guidance on endoscopy, Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex F [3], which builds on the recommendations from the BSG guidance has been updated.

2.1 References

  1. SG Guidance on Decontamination of Equipment for Gastrointestinal Endoscopy (2014).
  2. Management and decontamination of flexible endoscopes (CFPP 01-06) (2013): Policy and Management.
  3. Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex F (2014).

This report was published in Health Protection Report volume 8 issue 31.