Sickle Cell Anaemia

Pat McFadden Excerpts

Wednesday 10th December 2014 Westminster Hall

[Mr Pat McFadden (Wolverhampton South East) (Lab)]

It is a pleasure to debate this matter under your chairmanship, Mr Owen. Sickle cell anaemia affects an estimated 12,000 to 15,000 people in the UK and around 400 people in the west midlands, where my constituency lies. The associated condition, thalassaemia, affects around 1,000 people, although hundreds of thousands more in our country carry the trait for these conditions. They mainly, but not exclusively, affect the Afro-Caribbean community.

Sickle cell anaemia is the most common genetically inherited condition in the UK. Roughly one baby every day is born with the condition in our country. There is no known cure and for those who have it, the symptoms can vary from relatively mild, infrequent pain to much more serious episodes of crisis, frequent hospital admissions, strokes, often in very young people, organ damage and reduced life span. At the heart of the debate about treatment for the condition lies one simple question: why, with all the progress that has been made—there has been progress—is there still such variability in the treatment for sufferers, leading to enormous and debilitating pain, unnecessary and expensive hospital admissions and damaging consequences for the families of those who suffer from sickle cell anaemia?

Some hospitals and some specialist care units do a fantastic job, but patients with sickle cell anaemia do not want islands of excellence. They want an excellent system that offers high quality integrated care that minimises hospital admissions, manages pain treatment and enhances quality of life wherever they live in the country. The experience of patients, as told to me and to others, is that we are still a long way from having a system in place that delivers that standard of care wherever people live.

I pay tribute to the work of the Wolverhampton sickle cell care and social activity centre based in Bilston in my constituency and to the other patient-led groups around the country, to the Sickle Cell Society, which is the national organisation that speaks up for sufferers, and to the work of the all-party group on sickle cell and thalassaemia, which is chaired by my hon. Friend the Member for Hackney North and Stoke Newington (Ms Abbott).

[Jim Shannon (Strangford) (DUP)]

The right hon. Gentleman and the House know that people are living longer. On average, people live into their 80s or thereabouts, but life expectancy for those with sickle cell anaemia is 40 to 50, which is better than it was. Can the Minister and the NHS do more with private pharmaceutical companies to try to find a method of prolonging their life? It may not be as good as 80, but it could certainly better than 40 to 50.

[Mr McFadden]

That is a very good point. Shortened life span is one effect of the condition, and there is certainly more to do on the research and treatment fronts.

Organisations such as those I mentioned are doing a tremendous job in explaining what the condition means for sufferers, calling for more attention to it, and pressing for better training for NHS staff and a more rounded way of providing treatment. I have had extensive contact over the past seven years or so with the Wolverhampton sickle cell care group. It provides hugely needed and valuable support for sufferers, which may be anything from helping patients to deal with different governmental agencies and helping their families through crises when they have to be admitted to hospital, to a broader advocacy role. The group praises many NHS staff locally, but their biggest and most consistent complaint is about lack of understanding of the condition among GPs, A and E staff and some other NHS staff, leading to pain and delay for sufferers and bad consequences for their families.

The group told me about Sharon, a single parent of two children under the age of 10 who has full sickle cell disease and is in frequent crisis. Every time she is admitted to hospital, there is a desperate scramble among friends and relatives to ensure care for her children. There is no proper system in place to support her children, and the stress on her and her family is enormous. She discharges herself from hospital early, often before proper treatment is complete, because she has to look after her children, so the whole pattern soon begins again. Is there not a better way to manage Sharon’s pain than through frequent hospital admissions and incomplete treatment? If she does have to be admitted, should there not be a better system of support for her children?

The group also told me about a local man in his 40s who has gone for years without treatment for bleeding at the back of his eyes as a result of sickle cell disease. His sight is now at risk, but earlier treatment might have brought about a different outcome.

The Sickle Cell Society told me about a patient who was costing the NHS a fortune through repeated hospital admissions, exacerbated by the fact that her children slept in the one bed in her flat and she did not have a bed. Eventually, the charity was able to help her to buy her a bed and that made a huge difference. The cost of her hospital admissions could have paid for a hundred beds.

We will never reach the stage where no patient with sickle cell anaemia needs to be admitted to hospital, but getting the care right has the potential to reduce hospital admissions, making the condition easier to manage for the patient and saving the NHS a considerable amount of money. The fact that there is no cure does not mean that good professional care cannot make a difference.

That brings me to the national picture and the peer review of specialist care carried out by the west midlands quality review service and the UK Forum on Haemoglobin Disorders, which was published last year. The review visited 29 hospitals and reviewed the care provided. The aim of the care standards that have been developed is quite simple: there should be specialist haemoglobinopathy teams based in hospitals, backed up by a local haemoglobinopathy team. In other words, the system should have both the expertise to offer the best care and properly connect primary and hospital care. That is a good aim, but the reality is much more varied and therein is one of main causes of frustration for sickle cell disease sufferers and the organisations that speak out for them.

The peer review process found instances of excellent joined-up practice. It found committed staff going the extra mile to deliver the best care, but it also found overstretch, patchiness, breakdowns in care pathways and an unacceptable degree of variability across the country. The report says:

“Cooperation between acute and community nursing teams was sometimes very good but this was not universal. The availability of social work and psychology support was variable”.

It continued:

“In many teams there were inadequate numbers of nursing staff for the number of patients, or in some cases no acute nurse specialist at all. Even where these posts were in place they often had a very high workload which included inappropriate tasks”

such as

“filling in benefit forms…with very poor cover arrangements.”

The peer review process also found that, particularly in non-haematology parts of the system, patients

“perceived they were viewed as ‘second class’ haematology patients.”

That point about how sickle cell patients view their own experience is critical. They often feel that they are treated either with a lack of understanding or, even worse, sometimes with suspicion when they try to explain their condition or arrive at A and E in need of urgent pain relief. Lack of understanding can lead not only to poor care, but to patients feeling they have not been treated with dignity and that their need for treatment is not respected.

I do not need to remind the Minister that the very principle of the NHS means there should be no such thing as first-class and second-class patients. I do not want to see anyone treated as, or feeling as though they have been treated as, a second-class citizen. The whole basis of the NHS, paid for collectively with treatment on the basis of need, is that everyone is a first-class citizen. If there are sufferers of a genetically inherited condition who do not feel they are being treated as such, that is not acceptable and something we should take very seriously indeed.

Another issue identified by the peer review process is poor quality of data. That leaves us unsure about the number of sufferers and unsure, beyond emergency admissions, about the resources devoted to treating the condition. How can we ensure that there is the right treatment if we do not know how many sufferers there are or where they are? What is the Minister doing to improve those clearly identifiable data problems?

The peer review made a number of good and important recommendations about access to specialist care, staffing levels, training, psychological support and managing the important transition between paediatric and adult care. Those issues are absolutely central to the experience of sickle cell patients. Can the Minister tell us this morning what is happening to those peer review recommendations? Will he undertake to go through them not in a general way, but point by point, and to give a progress report to the House if not today, then soon and in writing? Such a report would be welcomed by sufferers and would ensure that there was follow-up on these important recommendations.

Then there is, for patients, the basic cost of living with the condition. To live with sickle cell is to live with pain, and that often means frequent prescriptions. For some patients, the cost can be prohibitive. This is not just a matter of money, but of behaviour. If people do not use their pain relief efficiently because they cannot afford more, it can affect their condition. I appreciate the cost pressures on the NHS, but will the Minister agree to commission a departmental analysis on whether free prescriptions for sickle cell sufferers would cost money or lead to net savings because they would reduce avoidable hospital admissions?

What of how sickle cell care is to be delivered in the future? NHS England is currently the body responsible for commissioning care for rare diseases. That is sensible, because sufferers might be concentrated in different parts of the country and sometimes there may be few sufferers. However, a review is taking place into how that will be done in the future, the results of which are due next year. There are three possible models: continuing with national commissioning, co-commissioning with clinical commissioning groups locally, or leaving it all to CCGs. Given that concern already exists about the large variation in the quality of treatment, knowledge of the condition and the priority given to it, sickle cell organisations fear that a move to only local CCG commissioning will exacerbate the problems. Can the Minister ensure that the way treatment is organised in future is in line with the central aim of the peer review exercise—to deal with the variability of treatment issue—rather than its being organised in such a way as to make it more difficult? I repeat that our aim should be a system that makes the best the norm, rather than balkanising care and creating a lottery, depending on where sufferers live.

Sickle cell anaemia is an issue that deserves more attention than it has received and a greater priority in the delivery of high-quality health care. In some ways, it is less about knowing what the best care looks like and more about ensuring that it is delivered to the highest standard throughout the country, regardless of where patients live. The condition also requires some smart, joined-up thinking between different agencies. Repeated hospital admissions because of a lack of a bed to sleep on is not a smart way to deal with a condition such as sickle cell.

Sufferers have to put up with a life of pain, but good treatment, the right information and the right lifestyle can make an enormous difference. That treatment, information and help vary so much is not acceptable. Dignity and respect are essential for all NHS patients: sickle cell patients feeling they do not always get that should be a concern for us all. It is time we ensured that the best care is available to all sufferers, delivered by a system that understands the condition, fully respects the patient and allows sufferers to live as full a life as possible. I hope the Minister can respond to the issues I have raised today in a way that makes that more likely. On the issues for which he does not have immediate answers, I hope he will go back to the Department and press his officials to make sure the changes we know are needed happen.

[The Parliamentary Under-Secretary of State for Health (George Freeman)]

It is a great pleasure to serve under your chairmanship, Mr Owen, and to respond to the right hon. Member for Wolverhampton South East (Mr McFadden), whom I congratulate on securing the debate. I hugely welcome the opportunity to discuss this issue.

Sickle cell anaemia is a really terrible condition and diagnosis for all those who are affected, but especially for our African and Afro-Caribbean communities. I want to start by acknowledging the work that the right hon. Gentleman has done for his constituency and his community, and I join him in paying tribute to the all-party parliamentary group on sickle cell and thalassaemia, chaired by the hon. Member for Hackney North and Stoke Newington (Ms Abbott). I also pay tribute to the Sickle Cell Society, the Wolverhampton sickle cell care and social activity centre and the patient groups. As with so many rare diseases, it is the advocacy of the few that in the end leads to changes in mainstream provision, and I am serious about paying tribute to that. The right hon. Member for Wolverhampton South East made a number of important points that I will try to deal with in detail. If I run out of time, perhaps he would allow me to follow them up in more detail in writing.

I stress that for those who have had a sickle cell diagnosis, it is a life-changing moment. All of us who are involved in policy making should not take our eyes off the personal suffering that patients—those with the diagnosis—and families and loved ones experience. Some 250,000 people in the UK carry the sickle cell trait, with about 15,000 affected by sickle cell anaemia, as the right hon. Gentleman knows. For those affected, it can mean a life of constant pain management, including, often, extensive periods of hospitalisation when the pain is bad, blood transfusion and red cell therapy, and tiredness, dizziness, palpitations, jaundice and gallstones. All those in combination mean that people are denied the quality of life that the rest of us take for granted.

However, as the hon. Member for Strangford (Jim Shannon) pointed out, the worst aspect of all is a substantially reduced life expectancy. Even today, sickle cell sufferers will, on average, survive until their 40s or 50s. Even though that is a massive improvement on the position 40 years ago, when the average life expectancy was only 14 years of age, it is still a shock when the rest of us are expecting to live very much longer than that. We can only pay tribute to the bravery shown by the people who have to deal with all the problems that this condition brings. However, bravery and resilience are not enough; we need to look at the way in which we support and treat people and bring on innovative care pathways and medicines. That is why we are continuing to invest in improving services, especially blood, bone marrow and stem cell services, which are vital for the condition.

Let me say something about what we are doing. In England, NHS Blood and Transplant provides blood for transfusion services. There is targeted donor recruitment, extended donation testing, and supplements, through a national frozen blood bank suitable for the long-term storage of blood for those with rare conditions. NHSBT’s therapeutic apheresis services provide a range of services to patients through NHS trusts from its six units situated across England, in Bristol, Liverpool, Oxford, Sheffield, Manchester and Leeds. Those units undertake procedures that provide direct treatment to patients with a range of medical conditions, as well as collecting stem cells from both patients and donors. Therapeutic apheresis treatments and services provide both life-saving and life-enhancing treatments for patients referred in sickle cell crisis or for ongoing sickle cell management. NHSBT is working with commissioners further to improve access to automated red cell exchange for sickle cell patients as part of those services.

One option for patients is a stem cell transplant, which requires genetically matched stem cell units, either from bone marrow donated by an adult donor or through stem cells harvested from cord blood. In the past four years, the Government have provided an additional £12 million of new money to help with transplant services. The Department of Health, working in partnership with NHSBT and the Anthony Nolan charity, has overseen the delivery of improvements way above what we originally anticipated.

The achievements include the following. More than 60% of black, Asian and minority ethnic patients are able to find a well matched donor now, compared with 40% at the beginning of the Parliament, and 258 more UK patients received a potentially curative stem cell transplant in 2013-14 than in 2010-11. The process for stem cell provision has been significantly streamlined, with single access searching in both England and Wales. I am delighted to say that there are now 60,000 young donors on the so-called fit panel, whose volunteers are eight times more likely to have donated stem cells than other registry volunteers.

Increasing use of UK-sourced cord blood to meet the needs of UK patients is crucial. This year, more than 25% of cord blood transplants will use donations from UK donors, costing about half the price of imported units. That compares with 10% in 2010. The time taken to provide stem cells from adult donors has improved. The right hon. Member for Wolverhampton South East knows that that is a crucial issue. Samples for confirmatory HLA—human leukocyte antigen—typing are provided from more than 80% of donors within 15 days now, compared with 35% in 2010.

The NHS and its key delivery partners are committed to continuing service improvement—I will say more about that in a moment—in collaboration with patients and patient group representatives, which is crucial. In the NHS today, if a child is diagnosed with sickle cell anaemia, they will be referred to a care team in a specialist sickle cell centre. Those are specialist units usually based, as the right hon. Gentleman knows, in large hospitals and staffed by front-line health care professionals with a high level of expertise in treating people with sickle cell anaemia. A detailed treatment and care plan, which outlines future medical care, is now drawn up for each patient, and parents are given information and support to help them to manage their child’s condition.

Due to the complexity of sickle cell anaemia, multidisciplinary teams are now assembled. Typically, they include paediatricians, haematologists, clinical psychologists, social workers and specialist nurses. The purpose of the care plan is to avoid sickle cell crises and to provide adequate pain relief when a crisis does occur, as well as reducing the risk of serious complications developing, such as infections, stroke and other associated symptoms of sickle cell anaemia.

Clearly, we want to see improvements in health care services for all types of patients. The right hon. Gentleman made that point well. The Government have committed to specific strategic plans in key areas. One of those plans is “The UK Strategy for Rare Diseases”, which covers sickle cell anaemia. The strategy sets out a shared UK vision for all those affected by rare diseases. It is owned by each country in the UK and commits them to more than 50 commitments. The strategy focuses on five areas: empowering patients, identifying and preventing rare diseases, diagnosis and earlier intervention, the role of research and, most importantly of all, co-ordination of care. As I have said, people suffering from long-term conditions are resilient, but that resilience can easily be undermined by the constant to-ing and fro-ing that occurs when a patient’s care pathway is not properly managed.

[Mr McFadden]

I thank the Minister for outlining the nature of the specialist care teams that are in place. He is right, but the critical point made by the report from the peer review exercise was that although that approach worked well in some places, it did not work as well in others. The specialists whom he talked about—the specialist consultants and specialist nursing staff—were not always there in sufficient numbers, and there is still something of a lottery, some might say, or at least unacceptable variability in the quality of treatment and the understanding of the condition, depending on where the patient lives. I therefore want to press the Minister on the recommendations from the peer review exercise, which were all about making the best the norm. What will he and the Department do to ensure that those recommendations are followed through on in that way?

[George Freeman]

The right hon. Gentleman makes a good point. I have my eye on the clock, and I will deal with it.

I conclude my opening remarks by saying that it is no longer acceptable to make the patient fit the pathway. We need to fit the pathway around the nature and progression of the disease in patients. These patients in particular have to see a wide range of professionals, and we need to look at that model. The final thing that I want to mention in terms of our strategic response is the genome project. We are funding 100,000 full genome sequences, to be put together with phenotypic data, with cancer and rare diseases as the initial focus. I am confident that that will quickly start to unlock some insights into possible preventions and new treatments.

The right hon. Gentleman made a number of important points, and I want to acknowledge them. If I cannot deal with all of them now, I will come back to him in writing. He made points about the lack of understanding among GPs across the system; the need for better care pathways to try to reduce hospital admissions; the importance of data underpinning our understanding of good outcomes, best and worst practice and variability; and the important insights in the report. I want to come back to his two specific requests. I would be delighted to ask the various organisations involved to give me a progress report on where they have got to in implementing the various measures, and I will obviously share that with him; perhaps we will have an opportunity to debate it. I will also happily ask NHS England and the National Institute for Health and Care Excellence to look at the health economics of free prescriptions in terms of short-term costs unlocking longer-term savings. I cannot prejudge the outcome of that, but I will happily look into the issue.

In the three minutes left to me, I want to touch on a couple of the specific points that the right hon. Gentleman made. How do we promote understanding and get sickle cell disease higher up the agenda? The 100,000 genomes project also includes a substantial investment in training in rare diseases for clinicians across the NHS as we launch our genetic medicines service. NHSBT and the Anthony Nolan charity continue to promote donations of blood and stem cells, but there is also the issue of the training that goes with that. The Department of Health is working with those key delivery partners to see what more can be done to improve not only donation, but the understanding of the condition and the training across the system.

The right hon. Gentleman asked what measures were being put in place to support care for people with long-term conditions. Our aim is to make the NHS among the best in Europe at supporting people with long-term conditions such as sickle cell disease. In the past, we have not done as well in that area as we would have liked. Through the mandate, we have asked NHS England to make measurable, tangible progress and commitments to supporting people with ongoing health problems to live healthily and independently. The NHS outcomes framework contains a range of improvement areas, and I will happily ask it to give me a progress report on that work.

NHS England is tasked with responding to the UK rare diseases strategy. Earlier this year, as the right hon. Gentleman will be aware, it issued a statement of intent that sets out how it intends to play its part in delivering that strategy; and in the recent NHS England “Five Year Forward View”, it has set out various commitments on exploring specialist centres for rare diseases to improve the co-ordination of care for patients in line with the strategy. I understand that NHS England will be looking to those specialist providers to develop networks of services, integrating different organisations and services around patients. As I said, I will happily ask for a progress report and share it with the right hon. Gentleman. Possibly we will have a chance to debate that in a format similar to this.

I again pay tribute to the right hon. Gentleman’s leadership on this issue. I think that the advocacy of Members of Parliament and particularly those with high concentrations of patients who are especially heavily affected by this condition, along with that of patient groups and charities, will be seen in years to come to have played a major part in helping to drive new care pathways and the integration of research, medicine and care, so that patients who are suffering are given the support that they need.