Category Archives: sCJD

#InfectedBloodInquiry Update and Clarification

The Infected Blood Inquiry emailed Haemophilia Scotland and others today to provide an update following the close of the terms of reference consultation.

The consultation on the terms of reference closed last Thursday and the team has started work on analysing the responses we have received online, by letter, in emails, by phone and at meetings. On Tuesday Sir Brian Langstaff started work as full time chair of the Inquiry and is very grateful for your contributions to the terms of reference, including at the meetings with him during the consultation period.

They have also clarified that the Inquiry will cover all of those infected by contaminated blood or blood products.  This is the same approach to the different routes of infection that was taken by the Penrose Inquiry.  In Scotland, since the Penrose Inquiry, we’ve been working closely with those infected by blood transfusion as part of our relationship with the Scottish Infected Blood Forum.

I’m getting in touch today because I heard that following this Monday’s Victoria Derbyshire programme there had been some concern about how the full range of people affected by the infected blood scandal have been described. As Sir Brian made clear in his meetings with different campaign groups, the Inquiry will be investigating what happened to all those people who were infected, whether they were haemophiliacs or not, and also considering the impact on families.

The Inquiry has also indicated it is interested in holding further meetings to give more people the opportunity to engage.   We are already in touch with the Inquiry to look at ways of making sure people in Scotland have the chance to take up this opportunity.  We will give you more details as soon as we can.

The inquiry team is now starting to consider how we can best engage with the wide range of people affected as we go through the Inquiry process. Andy Burnham, the Mayor of Greater Manchester, has invited the team to meet affected people who have been in contact with him. This will be an opportunity for them to hear first hand how the Inquiry will work and to give their views about how they would like to engage with the Inquiry.

We would like to hold some more meetings outside London to get views on how people would like to engage with the Inquiry, including from people who have not yet had the opportunity to meet the team, so please let me know if you would like to suggest a particular location, and/or a group of people to meet. And of course, please don’t hesitate to get in touch with any other questions related to the Inquiry, and I hope you will share this note with your wider range of contacts.

They have also provided information about their new offices.  This is where the administration of the Inquiry will take place but it does not have the facilities to hold any of the oral hearings.  The oral hearings will be held in other locations.

Finally, you might like to know that we have now moved into the Inquiry office premises which are close to St Paul’s Cathedral,
Fleetbank House
1st Floor
2-6 Salisbury  Square
London
EC4Y 8AE

 

Sporadic CJD cases in the UK bleeding disorders community reported

CJD

A recent paper from the Centers for Disease Control and Prevention (CDC) covers the death of two people with inherited bleeding disorders from Sporadic Creutzfeldt-Jakob disease (sCJD) in 2014. Both of the people concerned were women. One had Type 3 von Willebrands and the other carried the Haemophilia B gene. These are the first deaths from sCJD in the bleeding disorders community anywhere in the world.

Most of the concern in the bleeding disorders community around CJD has been about variant Creutzfeldt-Jakob disease (vCJD). This is thought to be the human equivalent of bovine spongiform encephalopathy (BSE), known as mad cow disease.  Many of our members were informed in 2004 that they are at increased risk from vCJD for public health purposes.  This risk relates to being treated with blood products made from plasma donated by people in the UK who had eaten BSE implicated beef or went on to develop vCJD.  Although in 2008 vCJD implicated prions were found at post-mortem in the spleen of a man with severe haemophilia A who died of other causes, nobody with a bleeding disorder has ever developed vCJD.  To put that in context, a study found that 1 in 2000 people in the UK carry the implicated prion, which is the equivalent to 32,500 people.  At the moment there have been 178 cases of vCJD in the UK.

The news from the CDC related to a different form of CJD, sCJD appears to occur at random in the population at the rate of about 100 a year in the UK (fewer than 10 in Scotland). It mainly affects people of middle age and older and has been known about since the 1920s. It is a rare condition which is why there being two cases in recipients of blood products is unusual enough to trigger an investigation. It is not known whether these cases are related to being treated with plasma derived clotting factor products or whether these cases are coincidental. The CDC paper says, “Assuming an annual incidence rate of sCJD of 1.5–2.0 per million population the occurrence of 2 cases of sCJD in this total population may not imply a causal link between the treatment and the occurrence of the disease.”

There is active surveillance both in the UK and internationally for both sporadic and variant CJD. In the UK the National CJD Research and Surveillance Unit is based in Edinburgh and examines all cases of CJD in detail.  In these new cases they determined that they were sCJD (rather than vCJD ) by looking at how the disease progressed and where the CJD associated prions were found.

Prion diseases such as vCJD and sCJD are not well understood. There are still a lot of unanswered questions.  Haemophilia Scotland have highlighted the CDC paper to the Scottish Government, the Scottish Inherited Bleeding Disorders Network, and are looking into any potential legal implications.  We will be carefully monitoring the situation and will report any further developments on our website and to our members.

Further information about these cases can be found in a briefing paper produced by the European Haemophilia Consortium (ECH). There is more information about CJD on the NHS Choices website.  The best place to go with specific medical questions about your health is your Haemophilia Centre.