A Bleeding Disorders priority setting partnership (PSP) has been formed in conjunction with The James Lind Alliance and they are asking for your help.
The PSP is compiling a list of the 10 most important unanswered questions about bleeding disorders. Once finished, the list will be used to promote research and attract funding. It’s is very important that research goes into areas which have an impact on the lives of individuals and families with inherited bleeding disorders. That is why the PSP want to encourage those who live with the conditions to feed in their ideas and propose research questions.
All you need to do to take part is complete a short online survey.
The JLA is a non-profit making initiative which is a branch of the National Institute for Health Research (NIHR). The experience of the JLA with conducting PSPs is helping us shape and organise this project. You can find details about the JLA and other PSPs here.
Haemophilia Scotland is not a member of the PSP Steering Group but we are supportive of their objectives.
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.
In the run up to our AGM this Saturday we wanted to publish the final report of the Needs Assessment we conducted in partnership with the Scottish Inherited Bleeding Disorders Network. We are grateful to The Lines Between who independently carried out the assessment. Thank you to everyone who took part by completing the survey to have their say.
There report highlighted that there is unmet demand for physiotherapy and psychosocial services. The services already offered in these fields are greatly appreciated but more provision is need. Some of the other key findings from the report were,
- Some people with less severe bleeding disorders suffer anxiety about how to manage their condition.
- Over half of people have additional social, emotional, or practical support needs.
- People are experiencing stigma and discrimination at work.
- While it is understood that activity is important, activity levels are low.
- People wanted more local and specific support services.
The top three priorities for the future were,
- Reducing the number of bleeds
- Finding a cure
- Reducing the frequency of infusions
Read the Report
You can download a copy here or by signing up for a free account on Issuu.
We formally launched the report on the 15th of March and then presented it to MSPs, patients, and healthcare professionals at a reception at the Scottish Parliament.
Photos from the Scottish Parliament Reception
Photos courtesy of Elspeth Parsons of The Lines Between.
We are grateful to Baxalta (a Shire company) for the unrestricted grant which made the Scottish Needs Assessment possible.
Many Haemophilia Scotland members got a chance to meet Alex Dowsett, the record beating elite cyclist who also happens to have Haemophilia, at our recent Try Something Different Day at Crieff Hydro.
He is the face of the Miles for Haemophilia Campaign which is raising awareness of bleeding disorders and the benefits of staying active for those that have one.
As part of the campaigning he will be doing a live Q&A on Twitter from 3.45 – 4.45pm. All you need to do to take part is tweet your questions including @alexdowsett and #HaemophiliaChallengesMe. If you have space to include us at @HaemophiliaScot too that would be great!
If you aren’t on Twitter but would like to take part, it only takes a few seconds to get started. Please make us one the first people you follow too.
Sadly, the Q&A had to be cancelled. However, it is going to be reorganised an we will post new details as soon as we have them.
We are closing the needs assessment survey on Thursday morning (1st December). We’ve had a really good response so far but the more people who take part the more useful our results will be. We are running the needs assessment in partnership with the Scottish Inherited Bleeding Disorders Network so the results will influence the services we offer but also the services of the Scottish Haemophilia Centres.
There are some parts of Scotland that are a little under-represented at the moment. We are keen that the results reflect everyone in Scotland. So if you are reading this in Argyll & Bute, Dumfries & Galloway, East Renfrewshire, Orkney, Stirling, or The Western Isles please make a particular effort to take part.
If you do take part then you might also win one of three FitBits that we are giving away. They would make a great early Christmas present for yourself or could let you cross at least one name of that Christmas shopping list!
Thanks to everyone who came to our day out at Crieff Hydro. It was a wonderful, action packed day! We started by launching The Miles for Haemophilia campaign in Scotland with an inspirational talk by Alex Dowsett. Alex told us of his journey to being a record breaking cyclist, how haemophilia led him to cycling and how he manages his condition. It was great to hear his talk but also to get to chat to him in person too.
During his talk, Alex stressed how important keeping active safely is for all our community and spoke of the value of bringing us together to support and encourage each other.
After lunch, we had an adventure at the brilliant Action Glen. We tried out a range of activities which kept us moving on a cold but beautiful day. Amazing views could be seen by everyone who braved the zipwire.
The climbing wall looked testing but we met challenge!
We also had time for, possibly, the friendliest game of lasertag ever.
Although we did get competitive too…
There was also the chance to learn bushcraft survival skills, including fire lighting.
(which was greatly helped along by toasted marshmallows and biscuits!).
Thank you all for an amazing day, special thanks to Elspeth for taking our photos, but most of all to Pfizer for the funding this event and for bringing Alex to us!
The European Haemophilia Consortium (EHC) has been working with acclaimed director Goran Kapetanovic. He has created a powerful documentary which reveals the variety of experiences people have of living with bleeding disorders across the continent.
His 53 minute documentary, “What is it like to live in Europe with haemophilia?” is available to all for free online on the Haemophilia Stories website.
We hope that everyone who cares about haemophilia and related bleeding disorders will enjoy the film and share it as widely as possible.
For the high quality version of the documentary and to see individual stories please go to Haemophilia Stories website.