Cystic Fibrosis

Kerry McCarthy Excerpts

Thursday 10th April 2014 Commons Chamber

[Kerry McCarthy (Bristol East) (Lab)]

Thank you, Madam Deputy Speaker, for this opportunity to discuss an issue that is important not just to me personally and to my family, but to those constituents who have families or friends with cystic fibrosis and have written to me recently in support of the Cystic Fibrosis campaign. I thank also the Cystic Fibrosis Trust and the Bristol Adult Cystic Fibrosis Centre for their assistance in preparing for today’s debate, and those with whom I trekked across the desert in Jordan back in February to raise funds for the Cystic Fibrosis Trust. I am so grateful to the many people who sponsored me on that trek and, in particular, to those constituents, many of whom were not known to me personally, who were very generous with their contributions.

I chose to join the trek because I have a nine-year-old niece, Maisie, who has cystic fibrosis. Almost everyone who went on that trek to Petra had a relative, spouse, partner or friend with CF. so as well as being a fantastic if physically challenging experience, it was also useful for me, as we went through the mountains and the desert, to speak to people about their experience of dealing with family and friends with CF.

CF is an inherited disease that affects more than 10,000 people in the UK. One in 25 people carries the CF gene, and if both parents are carriers there is a 25% chance that their child will have CF—so one in 2,500 babies born in the UK has cystic fibrosis. My niece was diagnosed on Christmas eve when she was only a few weeks old. She had been losing weight and her parents were worried so they took her into hospital. But, thankfully, since 2007 newborn babies have been routinely screened, and that is very welcome.

CF is a genetic deficit that causes the production of thick mucus which predominantly affects the lungs and digestive system. The symptoms include a persistent cough, breathing difficulties and repeated chest infections. It also affects the pancreas, meaning that fatty foods in particular and fat-soluble vitamins are not digested or absorbed properly, causing malnutrition, poor growth and diarrhoea. This means that people with CF generally need between 20% to 50% more calories than average, and one third of them go on to develop CF-related diabetes.

Treatment varies because the condition can vary from person to person. Some people are not diagnosed and do not realise they have the symptoms of CF until they are an adult, as was the case with one lad on the trek with me. He was in his mid-20s and had been diagnosed at 18, so he had a milder form of the disease without the pancreatic symptoms. Treatment can vary according to how severe a person’s condition is, but it usually involves daily time-consuming physiotherapy, an oral nebuliser and, occasionally, intravenous antibiotics and having to take enzyme tablets when food is swallowed.

Patients are susceptible to cross-infection, which sadly means it is not a good idea for people with CF to come into contact with each other. That is where online forums have become valuable, because people with cystic fibrosis can now talk to each other about their condition. I want to flag up the Twitter account @CFAware, which I have only just come across. It retweets people who have contacted it with anecdotes about how cystic fibrosis affects their lives and about their treatment. It has been really valuable to me in informing me about the illness.

Cystic fibrosis used to be known as a childhood disease because life expectancy was so short. When the Cystic Fibrosis Trust was founded in 1964, a child born with CF was not expected to live beyond the age of five. Advances now mean that half the people with CF are expected to live beyond 41 years, and the life expectancy continues to rise—indeed, I was told the other day that a child born today with CF could expect to live into his or her 50s. That progress is, of course, very welcome, but it has implications for people with CF and their families, and for treatment and public policy, which I want to focus on this afternoon.

One recent breakthrough was the development of ivacaftor—the brand name is Kalydeco—a tablet treatment that addresses the genetic causes of CF, but that is valuable only for those with the G551D mutation, who comprise just 4% of CF patients. For them, the fact that this drug is now available reduces CF to a manageable lifelong condition—it is something they can live with, rather than a deteriorating condition—so the confirmation that this drug would be funded in England from January last year was an unimaginable relief. The drug demonstrates why investment in research and drug development, and in gene therapy, is so crucial. If a drug were found that could help the remaining 96%, the cost of providing it could be prohibitive, but of course that should not prevent our carrying out the research and continually trying to find better treatment, better genetic experiments and, ultimately, a cure for CF.

In the more immediate term, patients need a focus on the care and support available to them. Bristol is fortunate to have both a specialist paediatric centre at Bristol children’s hospital and, next door, an adult CF centre at the Bristol Royal infirmary, where I met staff and patients when I visited last week. It has a multi-disciplinary team comprising consultants, nurses, dieticians, physiotherapists, pharmacists, psychologists and a social worker. It was clear from my visit that each and every member makes a vital contribution to the treatment and well-being of CF patients there. The dieticians’ input is crucial in trying to help them maintain a healthy weight and deal with the added complication of diabetes. Psychologists are there to help with the psychological impact of having a life-limiting disease or the trauma of waiting for a lung transplant—that is an issue I will return to later. The medics I met also emphasised the importance of the social worker’s role.

The team also helps patients and families cope with the transition from paediatric to adult services at the age of 18. That comes at a time when not only are teenagers struggling with all the usual things that teenagers struggle with and they are perhaps making that transition into managing the condition themselves—by carrying out their own physiotherapy and administering drugs themselves, rather than having their parents do it for them—but their condition is likely to start deteriorating. Young adulthood is often the time when there is a dramatic decline in the health of CF sufferers, so having expert specialists, as well as exercise facilities and in-patient beds, at the specialist unit in Bristol, in a dedicated space for CF, which stops the risk of cross-infection, too—patients have their own rooms—is really important.

It is important to remember that many patients are trying to balance employment with their treatment: 70% of adults with CF who completed an employment questionnaire for the CF Trust were in work or education. Many of these patients are trying to administer treatment, such as IVs, at home and have to struggle with the equipment; they are trying to manage the costs of travelling to the specialist centre; and they may well be trying to apply for the assistance to which they are entitled from the Department for Work and Pensions. Indeed, the financial pressures are considerable, and one consequence of CF having been a “childhood disease” is that it was not included in the prescription charge exemptions, so patients now reaching adulthood face significant bills for their cocktail of medication. As we know, the prescription charge increased last week to £8.05.

[Sir Bob Russell (Colchester) (LD)]

I hope that when the Minister responds she will explain why successive Governments refused to acknowledge the very strong arguments that the hon. Lady has made about how the disease was once a childhood condition whereby people never made it beyond being teenagers but now people make it to adulthood and they are having to pay prescription charges—if they are in employment—to stay alive. People who abuse their body and become drug addicts receive state funding, but people who have been served by mother nature in this way do not get the help. The Government should give that help.

[Kerry McCarthy]

I thank the hon. Gentleman for his intervention. I know that he has campaigned on this issue for many years. To people who think that early-day motions are a magic bullet that will solve things overnight, I point to the hon. Gentleman, who managed to get an early-day motion calling for the removal of prescription charges for cystic fibrosis patients. I think that his early-day motion has been No. 1 on the list for many, many years, but it has not yet managed to progress. He also had a debate on the issue last year. I sometimes use him as an example of how early-day motions do not automatically mean that the Government will sit up and listen, but I hope that the Minister will listen today and hear our plea.

The previous Prime Minister, my right hon. Friend the Member for Kirkcaldy and Cowdenbeath (Mr Brown), recognised this issue, and his Government committed to phasing out prescription charges for people with long-term health conditions, but we did not manage to do it before the 2010 general election.

Some people with cystic fibrosis will be forced to make the difficult decision to give up work, but research conducted last year by the Cystic Fibrosis Trust with Parkinson’s UK, the Multiple Sclerosis Society and the National Rheumatoid Arthritis Society reported that Atos found 45% of people with these progressive conditions fit to work. Obviously, it has now been belatedly acknowledged that the assessments carried out by Atos were far too unreliable. Most worryingly, some people with cystic fibrosis were given “prognosis reports” of when they would recover from the illness—an illness from which it is impossible to recover.

Critically, welfare reforms may increase the barriers to getting to work and hospital appointments—not least the new personal independence payment and the 20 metre mobility limit. A survey of people with CF found that more than a third would no longer be able to work if they were not awarded the higher rate of mobility under the new PIP, while 90% said they would find it difficult to access hospital appointments. In a survey for the Cystic Fibrosis Trust, a third said that they would not be able to attend hospital appointments. The trust also warns that patients fear isolation and loss of independence, which would affect their mental health.

When I visited the Bristol adult cystic fibrosis centre last week, it was explained to me how the trust and the centre fundraise to help the service and the patients. For instance, they buy more nebulisers to lend to patients. The ordinary nebulisers are huge and cumbersome, but the nice neat little ones are far more expensive. The trust and centre want to help patients manage their condition with the least amount of inconvenience. They fundraise to buy the smaller nebulisers. They also buy physio equipment, because it is really important for CF patients to undertake exercise to loosen up the mucus on their lungs. They buy fridges for patients’ medication and desks for their rooms—as I said earlier, patients need their own rooms to avoid cross-contamination.

The Bristol centre is clearly making an invaluable difference to its patients’ lives, but it has a growing patients list, which is due in part to the very welcome increases in life expectancy. I understand that Bristol’s patient numbers are increasing in line with the national average—by around 10% each year—and the patient case load has doubled over the past six years to around 200. I think the Manchester centre has about 600 patients and is the largest in the country.

With the Bristol centre, the transfer of patients from further afield, such as from Taunton and Swindon, has contributed to the growth in numbers. This year, it is also taking on patients from Cheltenham and Bath.

Patients are often admitted for a course of intravenous antibiotics, which they will generally need about three times a year, with the antibiotics being administered about three times a day for a couple of weeks. Some patients can self-administer at home, but that is not always appropriate or safe, depending on their living conditions and how ill they are. Sometimes it may just be that they have not been monitored by the team for a while, so they will be admitted to the centre. I met a young woman who had just started work. She had an understanding employer. She had been in her new job for two weeks and then decided that she needed to go into hospital to have the intravenous antibiotics. She felt much safer there than administering a course of treatment at home, which was her normal way of doing things. The condition clearly requires considerate employers.

It seems that hospital admissions at specialist CF centres are becoming increasingly difficult to plan for and more prone to cancellation because the units cannot guarantee a bed will be available. It seems that, particularly in winter months, the specialist beds also come under pressure from other departments, which have a shortage of beds. Ordinary patients may be put in a CF unit, and the CF patients cannot get the treatment that they need. In Bristol, the annual number of in-patient “bed days” required by patients for their IV antibiotics has increased from just over 500 in 2005 to nearly 2,500. That is due to the number of patients, and not because the length of stay is increasing.

Over the past few years in particular, there has been a steeper rise in the number of bed days at home, which is now at more than 3,500 annually. The unit is trying to facilitate more self-care at home and has developed an outreach service so that patients do not always have to travel to Bristol. It also tries to offer community liaison and health care at home, for example to help with IVs, and is developing innovative ways to help patients, including through an agreement with BUPA to deliver supplies to the homes. There can be a huge amount of equipment and pills and so on, and patients who have to use public transport to get to the hospital, perhaps not feeling very well as they are travelling, do not want to have to carry huge amounts of drugs and equipment around with them. That delivery service is proving very useful. Staffing and resources, however, mean that the centre has limited capacity for outreach and community services.

Importantly, the University Hospitals Bristol NHS Trust is working with the centre and has increased funding for specialist staff but, more generally, the Cystic Fibrosis Trust told me that specialist CF centres around the UK are reporting that resources, personnel and space provision are inadequate and warns that in light of the increasing patient numbers nationally, the lack of capacity for in-patient beds, combined with a lack of funding for out-patient staff, threatens to curtail the clear progress made over the past few decades. I would therefore be grateful if the Minister could update us on the Department of Health’s plans to ensure that CF services are equipped to keep up with increasing patient numbers and the complexity of the condition and assure me that she will work with the Cystic Fibrosis Trust, the specialist centres and their expert staff. Will she also consider the issue of co-ordination between trusts? For example, CF patients who phone an ambulance will often not be taken to the specialist centres even though their consultants recommend it.

Some patients will be assessed and referred for a lung transplant and, as the Minister will be aware, last month the Cystic Fibrosis Trust launched its “Hope for more” campaign. Although a lung transplant is not appropriate for everyone with CF and does not cure it, it can mean the priceless gift of a few more years for the patient and their family. Tragically, though, one in three people with CF on the lung transplant waiting list will die before they can receive one, so the Cystic Fibrosis Trust is campaigning to maximise the use of organs and increase the number of organs available for transplant.

One tragic case was that of Kerry Thorpe, a very brave young woman who became the face of the organ donor campaign. She died only a few weeks ago at the end of March at the age of 23, because new lungs could not be found for her. She spent seven years on the waiting list. Today, I was sent an account from the Daily Record of another 23-year-old, Lynette Armitage, who had a lung transplant in December in an eight-and-a-half-hour operation. She asked surgeons whether she could have a photo of her old lungs when they removed them, but they told her that the lungs had completely disintegrated when they had taken them out. They were only held together by her rib cage and the surgeons said that if she had not had a transplant within the next two weeks, she would have died. The lungs got to her just in time. It might be that she lives for another seven years or so, which is the average, but some people who have had transplants can live into their 50s or 60s. She is talking about having a normal life, having children and looking forward to going to T in the Park this year. That just shows what a difference can be made if lungs can be found: two 23-year-olds with very different outcomes.

There are a number of ways to reduce the length of time people are waiting and, critically, to reduce the number of deaths. That includes increasing organ donation rates and promoting more public discussion of organ donation. Less than a third of donors are registered, so it is not necessarily an obstacle if someone is not on the register. The Cystic Fibrosis Trust reports that the UK has one of the highest rates of family refusal in the western world. I am not suggesting that this is an easy decision for bereaved families to make during such a difficult and emotionally charged period, particularly if the family member has died suddenly, but it is nevertheless an indication of the need to focus on consent as well as registration and to ensure that more people understand what an incredible and generous gift they can give to other people if they sign up to be donors.

The organ donation taskforce led to a 50% increase in the number of deceased donors and a 30.5% increase in transplants in the five years to 2013. That was important progress, but I am sure the Minister will agree there is still more to be done. I would be grateful if she could update us on the “Taking Organ Transplantation to 2020” strategy and the latest assessment of the case for an opt-out system. I know that Wales is moving to a system of presumed consent next year.

As the Cystic Fibrosis Trust has highlighted, it is not only a case of increasing donor rates, as significant numbers of donor lungs are not used, despite the next of kin giving their consent. Lungs from fewer than 25% of donors after brain death are utilised in transplantation. Medical innovation means that more lungs could be used, and the trust has called for more transplant surgeons to be trained in downsizing donor lungs. It has also funded a study at the university of Newcastle on ex-vivo lung perfusion, a technique for the assessment and potential repair of sub-optimal lungs. That was explained to me as a service where damaged lungs are reconditioned so that they are fit for use in transplants. I urge the Minister to consider the results of that study, and whether such techniques could increase the number of suitable donor organs.

In recommending the development of a national lung allocation system, the report focuses on a concern amongst the cystic fibrosis community about the equity of lung allocation, and whether they go to those most in need, irrespective of where the person lives. I am aware that NHSBT has developed a new super-urgent group, separate from the main waiting list, but the Cystic Fibrosis Trust emphasises that that is unlikely to have a significant impact on people with cystic fibrosis waiting for a transplant. It also highlights the evidence from the USA, where the introduction of the lung allocation system led to an increase in the number of transplants and a significant reduction in the number of people with cystic fibrosis dying while on the waiting list.

[Kate Green (Stretford and Urmston) (Lab)]

I am grateful to my hon. Friend for raising the issue of allocation of lungs for transplant. In the north-west of England we have poor levels of transplant. People wait longer and are more likely to die on the waiting list. Does she agree that it is important that the Minister presses for the modelling, which I understand is being carried out, on the impact of a national allocation scheme to be given great attention and accelerated if possible, so that we can have the evidence on the benefits of such a scheme in this country as quickly as possible?

[Kerry McCarthy]

I agree entirely with my hon. Friend. As I have said, it makes such a difference if more people come forward as donors, not just for lung transplants but for many other types of donation as well, from blood through to other organs. I urge the Minister to give this the utmost priority, because, as we have said, it can make such a difference to people’s lives.

The Department of Health has advised that the most recent analysis showed no significant difference in allocation across UK lung transplant centres. But the Cystic Fibrosis Trust clearly believes that an improved allocation system could address the shocking fact that one in three cystic fibrosis patients die before they get to the top of the waiting list. So I ask the Minister to review this with NHSBT, and consider the evidence from the Cystic Fibrosis Trust. It would also be helpful if she could provide more information on the current review by the cardiothoracic organs advisory group, and say whether it is considering a national allocation system, as referred to by my hon. Friend, or consulting stakeholders such as the Cystic Fibrosis Trust and specialist centres, and when it is likely to report.

The “Hope for more” report also highlights that 62% of survey respondents reported that psychosocial support during the whole transplant process is insufficient, and concludes:

“The demand for services that assist the family and individuals in coping with cystic fibrosis is drastically underestimated”.

In response to a parliamentary question that I tabled, the Minister explained that decisions on psychosocial support are a matter for clinicians and commissioners, but I would urge further consideration of the report’s findings and how the Department of Health can help to improve provision and ensure that clinicians have the resources that they need, working with commissioners to address gaps in information, support and psychosocial services for patients waiting for a transplant and post-operative.

It is clear that the specialist centres, working with the Cystic Fibrosis Trust, are making an incalculable difference to their patients’ lives. The trust is putting real money into the provision of services for cystic fibrosis patients. In 2012-13, the trust contributed more than £1.6 million to research projects, and more than £1.1 million to clinical care. It also provides a helpline that takes more than 3,000 calls every year, and it is able to provide limited welfare grants to people with cystic fibrosis and their families who are, understandably, struggling with the impact the condition can have on their lives.

I congratulate the Cystic Fibrosis Trust on its work and all those people that I met at the cystic fibrosis centre in Bristol, who were so dedicated and keen to get across to me the fact that with more resources they would be able to do an even better job in coping with the increased number of patients. I hope that the Minister takes that on board.

[The Parliamentary Under-Secretary of State for Health (Jane Ellison)]

I congratulate the hon. Member for Bristol East (Kerry McCarthy) on securing this debate. She made a wide-ranging speech demonstrating a very deep knowledge of this subject. She alluded to her own family connection to this condition. Obviously, I convey my best wishes to her constituents, particularly to her family and especially to her niece, who is, as she described, suffering from the condition.

I commend the hon. Lady’s recent achievement in raising funds for the Cystic Fibrosis Trust. That is an excellent achievement for an excellent charity; she is right to be generous in paying tribute to it. I am sure she will be interested to know that in recent weeks many Members of Parliament have contacted me in support of the trust’s current campaign on behalf of the 10,000 or so people in the UK who battle with the everyday challenge, which she so eloquently described, of living with cystic fibrosis.

Let me take this opportunity, as I like to do, to pay tribute to those who work in our NHS and their dedication, determination and commitment to provide a first-class care service to all patients, not least CF patients. I pay tribute to them for their efforts, all the time, on behalf of all of us and all our constituents.

Let me first speak more generally about organ transplants and the challenges of organ transplantation. In the UK, the need for an organ is greater than the number of donor organs available. About 8,000 people are on the national transplant list waiting for a transplant that will save their lives or significantly improve their quality of life. Unfortunately, too many people wait too long for a suitable organ to be donated. About 1,000 people a year die waiting—about three adults or children every day. That applies to organs in general. Many others lose their lives before they even get on the transplant list. As of 3 April this year, 75 people with cystic fibrosis were waiting for a lung transplant. About 50 cystic fibrosis patients receive a transplant each year, but unfortunately about 20 patients die each year on the transplant list. We can see the clear challenge to meet that need and assist those people.

This means that there has to be a system to ensure that patients are treated equitably and that donated organs are allocated in a fair and unbiased way based on the patient’s clinical need and the importance of achieving the closest possible match between donor and recipient. A number of factors are involved. The rules for allocating organs are drawn up by the medical profession in consultation with other health professionals, specialist solid organ advisory groups, and health administrations. Factors such as the blood group, tissue type, and age and size of the donor and the recipient are taken into account to direct the allocation of the organ and identify the best-matched patient or, alternatively, the transplant unit to which the organ is to be offered.

The Cystic Fibrosis Trust report “Hope for all”, published on 10 March this year, makes a number of recommendations focusing on three key aspects: increasing the number of organs donated for transplantation; making sure that we make best use of the donated organs; and making sure that patients are fully involved in decisions about their care. We continue to invest in the donation programme to optimise transplantation in the UK. In the five years between April 2008 and April 2013, donation rates rose by 50.3% and transplant rates rose by 30%. That is a record of good and significant progress in recent years. I pay tribute to NHSBT for the work it has done in this regard, alongside other health professionals and the charities. Encouragingly, donor and transplant rates continue to rise, and we see that pattern this year as well. However, we know we can do more to match the successful donation programmes in some other countries —as the hon. Lady said, there are other countries with better records—and to give more people the opportunity of a transplant.

As the hon. Lady and other hon. Members may know, a new seven-year UK-wide organ donation and transplantation strategy, “Taking Organ Transplantation to 2020”, was published in July last year. The strategy expressed the desire to make the UK system comparable with the best in the world. Within that, it aims for a rate of consent—the hon. Lady specifically talked about consent rates—of above 80%; it is currently 55%. Increasingly, consent is the most important strategic aim—interestingly, more so than donation. Spain achieved a consent rate of 84% in 2011—a remarkable achievement. We know that we have particular challenges in relation to consent rates in black, Asian and minority ethnic communities, which I have discussed at length with NHSBT and which hon. Members are aware of. I know of hon. Members not present here today who have done specific work in some of their local black and minority ethnic communities to raise awareness on this point. I would like to see us do more of that and use parliamentarians to do so.

[Kerry McCarthy]

It is perhaps remiss of me not to have mentioned in my speech the fact that in the past year Bristol has had its first Muslim lord mayor who, during his year as lord mayor, chose to focus on encouraging blood and organ donation from the BME communities. As his term of office is almost up, I ought to take this opportunity to congratulate Councillor Faruk Choudhury on that effort.

[Jane Ellison]

I join the hon. Lady in congratulating the lord mayor. That is exactly the sort of local leadership that can help. One of the big pluses of the devolution of public health to local government is that we see such leadership from people who know their community best and understand the diversity in their locality. I am keen to encourage that. Only recently we celebrated examples in other areas, where we saw that specific leadership in some communities where health outcomes were not as good as they could be. We are always looking for such opportunities, and I am delighted that the hon. Lady has taken the opportunity to highlight local leadership in that regard.

Our focus in the strategy is initially on increasing consent rates. We want people to support transplantation. We can all imagine that families are being asked to agree donation at probably one of the worst times in their life, but many families find that they get comfort from knowing they have helped others to live. We will keep a close eye on what happens in Wales following the changes there, to which the hon. Lady alluded. NHSBT also keeps international experience under careful review. I mentioned the good success rates in Spain, for example.

We need to make sure that we make the best use of the donated organs. Currently donor lungs are procured by a retrieval team and allocated to the transplant centre on a zonal basis, based on the location of the donor. The transplant team at the centre will decide whether or not to accept the lungs and will select the most appropriate recipient.

The trust’s report recommends the implementation of a national lung allocation system whereby donor lungs are given to the most urgent patients, regardless of where they live.

This is something that NHSBT’s cardiothoracic organs advisory group, which includes both lung clinicians and lay membership, will be considering very shortly, and in particular whether we should introduce a national lung allocation scheme for people who need a lung transplant urgently, with all remaining donor lungs continuing to be allocated on a zonal basis. The advisory group’s recommendations will then be considered by NHSBT’s transplant policy review committee, and if a change of allocation procedures is agreed, it will be implemented as soon as the governance arrangements can be put in place.