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Blood, tissue and organ screening, CJD, Creutzfeldt Jakob disease, government response, UK blood safety, after the storm

Government pledges do more to reduce risk of vCJD transmission

17 October 2014

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The Science and Technology Select Committee has today received the Government’s response to its report on UK blood safety and the risk of variant Creutzfeldt-Jacob Disease (vCJD), the human form of bovine spongiform encephalopathy (BSE).

In its response, the Government acknowledged that “uncertainties remain” about the potential for vCJD to be spread across the population, either through the blood supply or use of contaminated surgical instruments. It denied that its current approach to this risk was “more relaxed” than it had been in the past, but agreed with several of the Committee’s recommendations.

The Government has pledged to review its current guidance on the decontamination of surgical instruments and to discuss with the Care Quality Commission the need for this to be included in regulatory checks. It also promised to consider funding a UK-wide study to collect evidence on the potential rate of ‘silent infection’ with vCJD.

Andrew Miller MP, Chair of the Science and Technology Committee:

"This inquiry highlighted several potential weaknesses in the Government’s handling of blood safety issues in general and the risk of prion transmission in particular. I am therefore happy that the Government has promised to act on so many of our recommendations.

Current Government policy in this area is rightly based on a precautionary approach but the objective must remain to collect more evidence and thereby reduce uncertainty. I am pleased that the Government recognises the value of further research into this area and I look forward to hearing more detailed plans about future studies in due course.

In the meantime, I trust that our report will act as a reminder that the Government cannot afford to be complacent when it comes to blood safety."

Background

A prion is an infectious agent comprised of protein folded into an abnormal form. Prions are responsible for a family of fatal brain diseases known as transmissible spongiform encephalopathies (TSEs). Examples include livestock diseases such as bovine spongiform encephalopathy (BSE) and scrapie and, in humans, Creutzfeldt-Jakob Disease (CJD).

CJD is a debilitating neurodegenerative disease caused by a build-up of abnormal protein in the brain. Symptoms of CJD are similar to those of dementia and include loss of balance, coordination and mobility, loss of memory, slurred speech, personality change and progressive loss of brain function. CJD is invariably fatal and most people die within a year of first experiencing symptoms. 

Prior to the mid-1990s, three types of "classical" CJD had been characterised:

  • an inherited form that runs in families (typically 5–10 cases per year in the UK);
  • an acquired form, transmitted through contact with human tissue contaminated with prions (2–3 per year), and
  • a sporadic form of unknown cause, historically responsible for the majority of cases (50–100 per year).

Following the BSE epidemic of the late 1980s and 1990s, the first cases of a new form of CJD were identified. Variant Creutzfeldt-Jakob Disease (vCJD) shared some symptoms with classical CJD but tended to affect younger people and led to a longer period of illness before death.  Primary transmission was thought to be caused by exposure to BSE-infected material, such as contaminated meat. Since vCJD was first identified in 1995 it has been attributed to 177 UK deaths, the majority occurring between 1996 and 2003.