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.
On 8 April 2017 we held our first every joint meeting with the Scottish Infected Blood Forum (SIBF). The SIBF campaigns on the contaminated blood and blood products disaster, regardless of the route of transmission, in Scotland. We wanted to come together to update members of both organisations on the current situation and give everyone a chance to have their say.
Tommy Leggate from the SIBF provided information about the new Scottish Infected Blood Support Scheme (SIBSS) and some of the work that the SIBF and Haemophilia Scotland have done together in response.
Leone Bissett spoke about the proposal for a memorial to those who had passed on as a consequence of the disaster. She urged everyone to support the Contaminated Blood Memorial Fund. The memorial will be a lasting tribute to those who died as a consequence of the disaster. The location needs to be accessible preferably in Edinburgh and durable enough to be withstand any possible weathering or other damage. It needs to be physically accessible to all ages and physical capabilities and will include words to explain what happened. Individual names need not appear but there may be ways of including individual messages. The style needs to be clear that it denotes a disaster but is also +ve and forward looking. A budget of £45,000 might be needed and so far £8,500 had been raised. It was agreed that the project had full support of those attending and agreed that the small steering group who had driven the project to date should continue to lead it.
There was then a discussion about what still needs to be done and how the new scheme could be improved. This discussion identified a number of potential issues including,
- The availability of income top-up support. Grants will be reviewed in Oct.
- The availability of lower value one-off grants from the support and assistance grant fund.
- Widow(er)s could choose a nominated doctor to help their case for gaining Stage 2.
- The term ‘Sustained Viral Response’, rather than cure, is to be applied where new the viral treatments for HCV have been ‘successful’.
- More case studies were needed to feed into the clinical review. For this, survivors access to their own or loved ones medical records might be necessary.
- Some concerns / anxieties were expressed about the application process. The first port of call should be NSS. However there was concern expressed about the telephone manner of one NSS response to a query.
- The phrase on one of the forms ‘if you really need it’ was regarded as insensitive.
- It was agreed to invite NSS to the next meeting in the autumn.
- It was noted that ‘Stage 1’ widows in particular were missing out.
- Concern was also noted about the composition of the Appeals Panel.
- All agreed that a future guarantee on the level of payments as a minimum is needed. It was agreed to write to all the political parties seeking their agreement to maintain the levels of payments set so far as a minimum.
- It was noted that Stage 0s may have particular problems in securing access to the scheme due to missing medical records.
- The issue of the widows pension payments not being made to those who have remarried.
- The cross border issues caused by the requirement that people were infected in Scotland and lived in Scotland when they first applied for support.
Although not know at the time of the meeting, the BBC were working on a Panorama programme to highlight the impact of the disaster and some of the evidence of wrong doing. The programme, Contaminated Blood: The Search for the Truth, was aired on 10 May 2017 and can be viewed below. It will be available to view for 12 months if you missed it when it went out.
There are still places left for our free cruise and AGM on Saturday 20 May.
Once everyone has arrived and registered we will have some lunch together (12 noon) before holding our AGM. At the AGM you’ll hear reports of our activities and find out how we are managing our finances. It will also be your chance to elect trustees to our board.
Once the AGM is concluded we will all be going on a cruise on the SS Walter Scott out on Loch Katrine (2:30pm).
All members of all ages are very welcome. If you need help with the costs of travel then please get in touch with Dan Farthing-Sykes.
Today, the Scottish Government has issued a press release “Support for those affected by infected blood.”
The press release says,
New scheme launches.
The new Scottish Infected Blood Support Scheme is now operational and will make its first payments to beneficiaries this month.
The scheme, managed by NHS National Services Scotland (NSS), has taken over this role from the existing UK support schemes as a result of the recommendations made by the independent Scottish Financial Review Group in December 2015.
Health Secretary Shona Robison said:
“The needs of patients and their families are very much at the heart of the new Scottish payment system, which will deliver improved support for those affected by infected blood in Scotland.
“The new scheme recognises that their needs are complex and will continue to change over time. It will be more responsive to them, simplifying the approach to support which was previously delivered by several different UK organisations.
“The Scottish Government is committed to doing all we can to help the people affected by this terrible chapter in the history of our health service. We remain the only country in the UK to have held a full public inquiry and I’m proud that we can now offer the most generous package of support in the UK to those infected and their families.”
CEO of Haemophilia Scotland, Dan Farthing-Sykes said:
“The increased payments that have already been received have made a big difference to the lives of many of those affected. Many of our members have chosen to free themselves from some of the debt that had built up as a result of years of inadequate support.
“Haemophilia Scotland is committed to working closely with the Scottish Government to make the new Scottish Infected Blood Support Scheme as simple and user friendly as possible.
“There is still work to do to fully implement the recommendations of the Financial Support Review Group but the launch of the new scheme today is a very significant and welcome step on that journey.”
The Financial Review Support Group Final Report, which has been fully accepted by the Scottish Government, says that the scheme should keep means testing to a minimum. How that can be achieved without endangering payments to those on the lowest incomes is going to be one of the largest challenges for the new scheme.
We are expecting to hear more details about the Clinical Review Group under Professor David Goldberg once the Scottish Infected Blood Support Scheme is launched. The Clinical Review Group recommendation will be vital. They will cover what non-liver health impacts will be taken into account for those applying for the on-going advanced payments (Stage 2). They will also guide the scheme in determining which of the widow(er)s of those who died at Stage 1 will be entitled to the widow(er)s on-going payment. Once this work is complete attention will turn to the terms for those who want to exchange their entitlement for on-going support for a one of payment in final settlement.
Haemophilia Scotland and the Scottish Infected Blood Forum (SIBF) are holding a joint meeting to discuss a range of issues related to the contaminated blood campaign in Scotland. The meeting will start at 10am at the Mercure Glasgow City Hotel on Sat 8th April.
We intend to discuss the new Scottish Infected Blood Support Scheme and the establishment of a new Clinical Review Group. There will also be an update about the progress of the Contaminated Blood Memorial Fund – you can donate today online.
Tea, coffee, and sandwiches will be provided for those who book.
Today, Haemophilia Scotland received a letter from the Cabinet Secretary for Health and Sport, Shona Robison MSP.
We had raised concerns that the discretionary grants scheme of the new Scottish Infected Blood Support Scheme (SIBSS) was not going to be sufficiently changed from the Caxton Foundation and MacFarlane Trust system it is replacing. In particular that means testing has to be kept to a minimum and the need to keep things as simple as possible,
In her letter Shona Robison made it clear that the SIBSS is still in an early, transitional phase. The priority is making sure that it launches on time and is able to make payments and receive applications. However, she assured us that the approaches of the scheme will continue to evolve and adapt, informed by the needs of the people who be using it. She highlighted the complexity of the old schemes and the importance of people still getting the payments they are replying on.
We had also suggested that the new scheme be launched as the Maguire Scheme in recognition of the work of the late Frank Maguire campaigning on behalf of those affected by the contaminated blood disaster. However, this suggestion is not going to be taken forward to reduce the potential for confusion.
Haemophilia Scotland and the Scottish Infected Blood Forum (SIBF) will be meeting with Scottish Government officials next week to discuss how the scheme might evolve in the future. We are also holding a joint meeting for members in Glasgow on the 8th of April to keep everyone updated.