Sickle Cell Anaemia

Jim Shannon 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.