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.
The Medicines and Healthcare products Regulatory Agency (MHRA) is an executive agency of the Department of Health (England) and regulates medicines, medical devices and blood components for transfusion in the UK. In the January addition of their Drug Safety Update the agency changed their advice on the use of Direct Acting Antiviral therapies for the treatment of Hepatitis C.
All patients should be screened for hepatitis B before starting treatment for chronic hepatitis C with direct-acting antiviral interferon-free regimens. Patients who are co-infected with hepatitis B and C viruses are at risk of hepatitis B reactivation, and should be monitored and managed according to current clinical guidelines.
This changed advice reflects a concern that the successful treatment of hepatitis C with the new direct acting antiviral products could allow a previously supressed hepatitis B infection to reactivate.
Both hepatitis C and hepatitis B are blood borne viruses and can be transmitted in very similar ways. As a result we are concerned that people with bleeding disorders who were infected with hepatitis C might also have been infected with Hepatitis B at the same time. As with hepatitis C, hepatitis B can also be transmitted sexually so we are conscious this change in advice could be relevant to some partners too. With so many people recently completing the treatment using direct acting antivirals we are taking the new advice from the MHRA very seriously.
We raised these concerns at the recent meeting of the Steering Group of the Scottish Inherited Bleeding Disorders Network. We were assured that Scottish Haemophilia Centres have not seen an increase in hepatitis B cases or in unusual liver function test results.
However, in response to our concerns, all Scottish Haemophilia Centres have been asked to contact their local hepatology teams and discuss this issue. They will ascertain that appropriate hepatitis B reactivation screening in patients receiving these antiviral regimens is being undertaken. Anyone who is anxious about potential hepatitis B reactivation, and has recently completed treatment with direct acting antivirals, can also request a test from their Haemophilia Centre. The Network has asked to receive reports of any cases of Hepatitis B reactivation which are discovered so that the situation can be monitored.
Further information about hepatitis B can be found on the NHS Choices website.
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!
Today, on International Nurses Day, we wanted to thank all the amazing nurses who work in Scottish Haemophilia Centres.
We are extremely fortunate in Scotland to have extremely dedicated nurses, many of whom have many years of experience in Haemophilia. Our members often tell us that their nurse is like a member of the family and that they are deeply grateful for the support and care they receive.
As a charity, we are also very grateful to the support we’ve received in setting up Haemophilia Scotland, running our events, and in our wider work. So many Scottish Haemophilia Nurses go above and beyond on a regular basis to provide the best possible treatment and care. They are extremely active in working with us and other healthcare professionals, through initiatives like the new Scottish Inherited Bleeding Disorders Network, to drive continuous improvement in Scottish care. Specialist nursing is vital to providing the comprehensive care package that is the gold standard in the treatment of bleeding disorders.
The theme for this year is Nurses: A force for change: Improving health systems’ resilience.
International Council of Nursing (ICN) President, Dr Judith Shamian, has said,
As the single largest group of health professionals, with a presence in all settings, nurses can make an enormous impact on the resilience of health systems. By promoting the nursing voice, we can help guide improvements in the quality of health service delivery and inform health systems strengthening.
The first meeting of the Quality Improvement, Audit and Data Group of the Scottish Inherited Bleeding Disorders Network took place on April 25th with Bill Wright in attendance on behalf of Haemophilia Scotland.
It was agreed that one of the key ways of reporting back to patients on its work would be via the Haemophilia Scotland website. The main subjects that the group looked at were:-
- ‘Do not appears’ and patients not filling in their returns for use of products and the action taken in various centres to improve matters.
- The take up on the psychology pilot project being conducted in the Edinburgh Centre.
- The lack of funding for physiotherapy support in some centres and the challenges in securing that support under present administrative structures.
- The revisions to the clinical audit system which is the administrative system for recording the overall use of various products and other trends among patients.
- The ‘auditing’ of Haemophilia Centres and the development of standards that each Centre are tested against.
Anyone wishing further information about the group, please contact Bill via the office.
Regular readers will know that we are very excited about the new Scottish Inherited Bleeding Disorders Network which was launched in November last year.
Things are developing will and three working groups have already been set up.
- Communications and Stakeholder Engagement
- Best Practice, Policies, and Protocols
- Quality Improvement, Audit and Data
There are already patients or parents on all of the working groups but they are all open to new members if you are interested in getting involved. If you are interested you can find out more about the work of the network and sign up to the Working Groups here.
There was a meeting of the Communications and Stakeholder Engagement Group yesterday. Most of the meeting focused on getting a website set up and there was a discussion about what the logo should look like. If you have any ideas for the logo, or would like to help design it, please email Dan Farthing-Sykes who is doing some of the early work.