Polycystic Kidneys

Jim Shannon Excerpts

Tuesday 6th November 2012 Commons Chamber

[Mrs Moon]

The hon. Lady is absolutely correct. This is a little-known condition, and the horrific nature and impediments caused by the condition as it progresses are little known and little appreciated. Often, people can look quite normal but be suffering tremendously. I commend her work in trying to raise awareness.

For individuals with ADPKD, it is a truly disabling, painful and limiting condition. Kidneys become enlarged, leaving patients disfigured and appearing prematurely pregnant. The kidneys continue to function normally, but eating, sleeping and even breathing can be difficult because of the size of the kidney and the pain experienced. The liver can be affected, too, and many patients are often diagnosed with PKD and liver disease. Besides the effects on the kidneys and the liver, there is a range of other complications: heart disease, bowel problems, hernias, infections and a high risk of intracranial aneurysms.

The kidneys can weigh up to 17 lb—upwards of 10% of a person’s body weight—and in one case a patient was recorded as having kidneys weighing 48 lb. An affected liver can grow more quickly than the kidneys, effectively squashing them. PKD affects people of working age and is characterised by premature mortality. Tragically, end-stage kidney failure is common at an average age of 55—within working age, as the hon. Member for Chatham and Aylesford (Tracey Crouch) mentioned.

PKD affects an estimated 12.5 million people around the world and is the fourth most common cause of kidney failure. It is more common than sickle-cell anaemia, cystic fibrosis, autonomic dystrophy and Down’s syndrome combined. We simply do not know how many people are affected by PKD in the UK. On the one hand is the answer to my parliamentary question in July. The Department of Health said that a total of 1,100 hospital admissions in 2010-11 were identified as resulting from PKD, although I was informed that that might include repeat visits by the same individuals. On the other hand, PKD Charity’s own figures suggest that the number is far higher. On the basis of the number of people requiring a kidney transplant and dialysis as a result of PKD, it estimates that as many as 60,000 to 70,000 people could have the condition in the UK.

[Jim Shannon (Strangford) (DUP)]

I congratulate the hon. Lady on bringing this matter to the Floor of the House. My nephew, Peter Shannon, was born with posterior urethral valves, which meant a kidney transplant for him. Had he not had one, he would be dead. Does she agree that we need a bigger push to get people to sign on to the organ transplant donor list in the interest of those with kidney diseases, and those with PKD specifically, who are currently being kept alive by dialysis treatment? Transplants are important. If Members have not signed up, they should do so and encourage others in their constituency to do the same.

[Mrs Moon]

I completely endorse the hon. Gentleman’s comments. It is imperative that kidneys be available for these transplant patients. It is the most generous commitment that anyone can make.

I am particularly grateful to Sandra Buckland and her husband for allowing me to quote directly from her remarks at the kidney group meeting. She bravely shared with us her experience of PKD, and I would like to share them with the House. Sandra’s powerful remarks underscore many of the points I want to make about what needs to be put in place to tackle this condition. Sandra was particularly brave in doing so, because she had also recently lost her sister, who died from PKD. She said:

“I suffer from polycystic kidney and liver disease. I am married with two sons, both with polycystic kidney disease and the youngest with polycystic kidney and liver disease. My elder son has an eight-year-old daughter with PKD and my younger son, a four-year-old son with both polycystic kidney and liver disease. My father died at age 39 with heart failure due to side effects of PKD.”

She left the group with the following question:

“Why, when I lost my own father at 17 to this dreadful disease, are lives still being lost and progress appears to be at a standstill? If more successful research could be performed, halting the genes that allow PKD to continue, it would remove the stress, heartache and trauma for many families.”

Sandra Buckland clearly demonstrates the cost of PKD to an individual and her family. It is a long-term, devastating diagnosis. At a national level the costs to the NHS are unknown, although an estimate is possible. The PKD charity believes the annual cost is £330 million. As I said in my opening remarks, PKD is currently incurable and can be managed only with dialysis and transplants, combined with monitoring, all of which are expensive. It was only in 1994 and 1996 that the two ADPKD genes were discovered. The ARPKD gene was characterised only in 2002.

Funding for research is limited. In the US, $360 million has been invested in research over the past 15 years —$45 million in 2009 alone. The House will be shocked to learn that the total investment in research in the UK and the European Union is zero. The PKD charity recently funded a small biobank of PKD kidney cells at the UCL-Royal Free, and together with the US PKD foundation, it has funded one small laboratory study in Sheffield. This funding is all reliant on donations. It is telling that in the past 12 years there has been no improvement in the life expectancy or average age of renal replacement therapy of 55 years for someone diagnosed with ADPKD.

It cannot be acceptable for PKD to remain a neglected condition in terms of research and the development of treatments. Transplants and dialysis are costly. Developing treatments would not only improve the quality of life for patients, but reduce the cost to the NHS. The PKD charity has recently begun work with the UK renal registry to develop an analysis of PKD patients on renal replacement therapy. Would the Minister consider supporting the extension of this work to include pre-dialysis patients by asking the Renal Association to develop quality standards relating to ADPKD? Would she also support changes to governance requirements that would enable the UK renal registry to collect the data, and provide the necessary funding of £500,000 for the analysis and publication of these data?

I appreciate that £500,000 may appear a large sum, but compared with the long-term savings, it is a small investment for a huge return. Funding the registry would overcome a major barrier to understanding ADPKD, support care planning and counselling, and provide cohorts for clinical studies. The information is not available at present, so there is no foundation in place on which to build a meaningful research programme.

New drug treatments are being developed, but getting access to them is not without its challenges. One PKD patient with polycystic liver disease contacted me to describe how she self-funded her involvement in a drugs trial in Italy over three years, travelling back and forth to Italy monthly for three years at her own cost. During the trial, her symptoms improved considerably. The trial has finished and her condition is rapidly deteriorating. Her local PCT refused to fund ongoing treatment. Her long-term survival is being determined by finances.

Last December the Prime Minister announced a package of support for the life science sector, which included an early access scheme for seriously ill patients with limited treatment options to receive promising new drugs in clinical trials. This accurately describes PKD patients. Will the Minister take steps to ensure, as a matter of priority, that PKD patients are included in access to drugs in clinical trials?

Last weekend a drugs trial in the Netherlands reported positive results in altering the progression of ADPKD. The drug will now be presented to the European Medicines Agency for licensing—

--- Later in debate ---

[Anna Soubry]

I absolutely agree with everything that my hon. Friend says. I was horrified to discover in a recent meeting that unfortunately in England we are not making the progress on organ donation that we should. It is a serious problem. We know that so many lives can be saved or seriously improved if people are good enough to indicate that they are willing, on their death, for their organs to be donated.

Let me go further and say this: of course I understand why, when somebody dies, the family struggle in their bereavement to give permission to allow the loved one’s organs to be donated. However, I urge people to do so, even in those very difficult situations. It is a most wonderful way to create a real legacy by enabling somebody literally to live on through someone else. If more people could, in those dark moments, see that, it would make a profound difference to improving, and indeed prolonging, lives.

[Jim Shannon]

I thank the Minister for her pertinent words about transplants. I understand that when people renew their driving licence there is a box they can tick if they want to be on a donor register for the rest of their life. It is a painless exercise that commits them for ever and provides the authority for all their bodily items to be transplanted. Given that it is so easy to tick that box, perhaps more of those who are renewing their licence should do so to say, “Yes, I want to be a donor.”

[Anna Soubry]

The ability to do that is a golden opportunity for people, and I wish they would take it. One of the problems, though, is that someone applying for a licence will think, “Well, I’m applying for a driving licence and I can’t deal with all that now; I’ll come back to it another day”, and unfortunately they do not return to it having got their driving licence.

I would very much welcome a serious look at how we can solve this problem by campaigning harder to ask people to tick the box, make their views known, and speak to their families. Each and every one of us should talk among our families about the things that we want on our death. I know that these are difficult subjects, but this is, as we all know, a wonderful legacy that people can leave which makes a huge difference to the quality and length of the lives that people could lead.

As provision could be improved with the introduction of evidence-based best practice guidance both at diagnosis and for the management of people with polycystic kidney disease, I will ask my officials to raise this with the appropriate agencies to see what further actions can be taken.

I pay tribute to the hon. Lady and all those who have contributed to this debate, and thank them for drawing attention to this specific and important disease area. I also congratulate those at PKD Charity on all their hard work. I look forward to meeting them and the hon. Lady to make sure that we raise the profile of polycystic kidney disease.

Question put and agreed to.