Huntington’s Disease

Liz Saville Roberts Excerpts

Wednesday 9th November 2022 Westminster Hall

[Hilary Benn (Leeds Central) (Lab)]

I beg to move,

That this House has considered Huntington’s disease.

It is a great pleasure to serve under your chairmanship, Mr Robertson, and to see the Minister in her place. I thank all the Huntington’s disease organisations in England, Scotland, Wales and Northern Ireland for the vital work they do and for the help and expertise they have given me in preparing for this debate. Back in May I tabled early-day motion 72 on Huntington’s, and I am really grateful for the support it received from Members on both sides of the House. My colleague Jackie Baillie tabled a similar motion in the Scottish Parliament, which I am told secured record support. I should also declare that my interest in this subject is the direct result of knowing someone who has the disease.

Huntington’s is a rare genetic neurodegenerative disorder that, over time, basically stops the brain working properly. It affects some 8,000 people in the UK, but around 32,000 people are living at risk of developing it for the simple but deadly reason that Huntington’s can be passed from generation to generation. This means that a diagnosis of Huntington’s does not just affect the person who has it; it also affects their children, who have a one in two chance of carrying the gene. There is a predictive genetic test to find out whether someone has the faulty gene that causes the disease, but I am told that on learning of, say, their parent’s diagnosis, the vast majority of people do not want to know and prefer to travel in hope. For all those reasons, it is a devastating diagnosis, because there is no cure, and there is only limited palliative care.

Imagine for a moment the questions that go through somebody’s mind when a loved one gets the diagnosis. How long has my spouse or my parent got? What is going to happen when they can no longer look after themselves? Which of our children has it? Should we tell the rest of the family? Will my loved one’s personality change, so that they end up shouting at me or even assaulting me? In many ways that is probably the most difficult thing to cope with, because we are losing the person we love not just physically but emotionally. This horrible disease makes them no longer the person they once were, and there is nothing—absolutely nothing—we can do about it.

It is very hard fully to convey what this means, so let me quote a letter I have received from someone who writes of

“being forced to witness the agonisingly slow degeneration of someone you have known and loved for nearly forty years, and how hard it is to keep remembering the person that they once were. That man—a loving, supportive spouse and utterly devoted father—was erudite, kind, attentive, with a wickedly dry sense of humour. He bears little resemblance to the unsteady figure with unclear speech, alternating between bouts of aggression, anxiety and apathy, with whom I now share my home.”

That is what sufferers and their families have to live with.

The symptoms of Huntington’s are many, and the list I am about to give is by no means exhaustive. They include early onset dementia; difficulty concentrating; lapses of memory; cognitive decline; depression; anxiety; obsessive disorders; psychosis; stumbling and clumsiness; involuntary jerking or fidgety movements of the limbs and body; difficulty moving; mood swings; personality changes; irritability; apathy; disinhibition; problems swallowing, speaking and breathing; fatigue; loss of weight; incontinence; and sexual dysfunction. In the later stages, full-time nursing care is needed.

[Liz Saville Roberts (Dwyfor Meirionnydd) (PC)]

I am immensely grateful to the right hon. Gentleman for giving way, and I congratulate him on securing the debate. He is speaking very movingly.

One thing that has been emphasised to me is how important it is to remember the carers—particularly the family carers—affected by this condition. I am sure the right hon. Gentleman will join me in impressing upon the Minister the importance to families who are going through this experience of granting the facility of respite care for family carers at a time of real carer shortages.

[Hilary Benn]

I am profoundly grateful to my right hon. Friend for attending this debate, and I agree completely with what she has said. I will come to the question of the needs both of people who have the disease and of those who care for them.

In short, those affected will lose the ability to walk, talk, eat, drink, make rational decisions and care for themselves. Partners and children are turned into carers, and children know that they themselves have a 50% chance of going through what they see unfolding in front of their eyes—a prospect that often results in self-harm, low esteem, low confidence, low mood, anxiety or depression.

Professionals, and indeed families, can sometimes mistake Huntington’s for other conditions such as Parkinson’s or Alzheimer’s. That is especially true when the family has no idea that Huntington’s exists in their family, and those living with it face a great deal of stigma and discrimination. It is not uncommon for a loved one to be wrongly accused of being drunk or on drugs due to their symptoms. The symptoms can start at an early age, and about 5% to 10% of sufferers experience them before the age of 20, although they usually appear between the ages of 30 and 50, and some 10% of sufferers develop them after the age of 60. The average survival time from first onset of symptoms is about 15 to 20 years.

Needless to say, people living with Huntington’s and their families face extraordinary challenges in their lives because the condition affects everyone who comes into contact with it: those experiencing symptoms and their families, those who have tested positive but do not yet have symptoms, and people at risk of developing it. Even those who test negative can suffer from survivor guilt, and in some cases might be ostracised by their families.

--- Later in debate ---

[Jim Shannon (Strangford) (DUP)]

It is always a pleasure to speak in this Chamber. As the DUP health spokesperson, I wanted to add my contribution today. I congratulate the right hon. Member for Leeds Central (Hilary Benn) on setting out the case so well and on doing so from a passionate, intimate and obviously knowledgeable point of view. It was hard to listen to some of the things he said, not because he does not put them over right, but just because, when we hear the emotion in his voice, we understand that he has a very personal interest in this subject. So, again, I thank him personally, as I think we all do in the Chamber today. I just want to add my contribution and, as I always do, to give a Northern Ireland perspective.

This is a difficult subject to deal with. As the right hon. Gentleman said, this disease does not just affect the person who has it; it can potentially affect the children as well. I think that makes things harder, because if someone has any doubt whatever as to whether they carry the gene, that will impact what they do when it comes to marriage, having children and having relationships, but also what will potentially happen to them in the latter part of their life. Therefore it is important that we take the right action to make our services better, and that is what we want to try to do.

May I say what a pleasure it is to see the shadow Ministers, the hon. Members for Leicester West (Liz Kendall) and for Paisley and Renfrewshire North (Gavin Newlands), and to see the Minister in her place two days running? We are doing well here, so we are. The Minister tries extremely hard to answer the questions that we put forward, so I thank her for that. I am very pleased to see the Labour shadow Minister—a fellow Leicester City supporter—in her place. We won 3-0 last night, which was a good result. That is by the way and nothing to do with this debate; it is just for the hon. Lady and me to glory in that victory, as we do, personally and collectively.

We need to have better mental health services for patients suffering with this disease and to encourage more funding into research. The right hon. Member for Leeds Central was right about the importance of research; I will comment on research later, but he was right to mention the need for it. In a way, this debate follows on from some of the questions about research in the debate on pancreatic cancer that I secured in Westminster Hall yesterday. We have to focus on research in a deeper and stronger way, and I look forward to hearing the comments of others about what we can do for our constituents.

Health is a devolved matter for Northern Ireland and is not the Minister’s responsibility, but I want to sew a Northern Ireland perspective into the debate. I will replicate the perspective heard in the right hon. Gentleman’s comments, and talk about what we in the United Kingdom need to do in Northern Ireland, Scotland and Wales. I look forward to hearing from my friend and colleague in the SNP, the hon. Member for Coatbridge, Chryston and Bellshill (Steven Bonnar), who always makes a significant contribution on health issues.

Huntington’s services across the UK lack efficiency and funding, especially in Northern Ireland. That is not the Minister’s fault, but it shows what this is all about. One of my constituents made me aware of the fact that there are only two Huntington’s-qualified nurses across the whole of Northern Ireland—for a population of 2 million. Wow! It shocks me to the core when I read that and have to convey the situation in Northern Ireland. As a result of the right hon. Gentleman raising my awareness of this matter, I will take a deeper interest in it from a Northern Ireland perspective. I will follow this up with Robin Swann, the Northern Ireland Health Minister.

In that population of 2 million, the rate of Huntington’s has increased from 6.4 people per 100,000 in 1991 to 12 per 100,000—almost doubling over that period of time. Approximately 223 people have been diagnosed with the disease back home, leaving many with the possibility of getting it genetically. That is one of the worst things: someone could be carrying the disease without knowing—this rare condition is also known as the disease of families.

The hon. Member for Blaydon (Liz Twist), who has left her place, is the chair of the all-party parliamentary group on rare, genetic and undiagnosed conditions. Huntington’s is a rare condition, and sometimes the symptoms are not as prevalent, making it even more essential for people to be aware of them. I would subscribe to the hon. Lady’s line of thought that this should be categorised as a rare disease: statistically, the numbers suffering from this disease are not large, and it is rare in the effect that it has. Government have a policy to deal with rare diseases, so maybe it is time to consider this as one of those, Minister.

Huntington’s Disease Association Northern Ireland has been instrumental in providing support for families—it is not all doom and gloom in Northern Ireland for the families and those who support them. The association has a lovely motto: “Families at the heart of all that we do”. That conveys the importance of what it does, bringing families together so that they can help and reach out to each other. The right hon. Member for Leeds Central referred to that point, and I would reiterate it.

The organisation offers care to loved ones and encouragement throughout the process. Not only that, but it provides hope for those dealing with Huntington’s. Currently, 15 clinical trials of different treatments are under way. We should take some encouragement from that and have hope of a cure. With 15 clinical trials taking place, there is hope that one day soon—not too far away—we will have a cure. If we have that cure, we can deal with these issues better personally.

Sorcha McGuinness of HDANI has stated that, by the late stages of the condition, people will require 24-hour care, as the right hon. Gentleman referred to. They will be unable to move, speak or sometimes even swallow. Other diseases we have spoken about, such as motor neurone disease, are similar.

[Liz Saville Roberts]

The hon. Gentleman is speaking movingly and powerfully. Members will be concerned when we hear from constituents who are being refused personal independence payments. The procedure that applicants—people with Huntington’s disease and their families—have to go through to get PIP, to which they are entitled, is almost a test of their perseverance. As MPs, many of us have to deal with these things, but there must be a better way of dealing with families affected by conditions such as Huntington’s. Given what they are likely to need and that their care needs are so great, we must find a better way of dealing with this issue.

[Jim Shannon]

I thank the right hon. Lady for her intervention, which clearly outlines another issue. It is not always health alone that is an issue; it is also about not being able to work again, as the right hon. Member for Leeds Central said. There is the financial impact on families. There is going on to benefits, which are probably alien to those applying. The system needs a wee bit more compassion for those who are under financial pressure. When they state that they have Huntington’s disease, the reality of what that means should figure in how they are helped through PIPs and other benefits. More often than not, we—elected representatives—come to an acknowledgment and knowledge of those matters through constituents who apply for PIPs. We understand a bit better what they are doing. There is one lady in my office who does nothing but benefits—five days a week. That gives Members an idea of the magnitude of this issue. The right hon. Lady is right, and I thank her for her intervention.

As the disease becomes increasingly debilitating, there is a need for more trained specialists in it. I ask again the question asked by the right hon. Member for Leeds Central, and we look to the Minister for help: what can be done, and what is being done, to increase the numbers of trained nurses? The disease affects the whole of this great United Kingdom of Great Britain and Northern Ireland, so what discussions has the Minister had, or will she have, with the devolved Administrations to ensure that there is a universal strategy for moving forward that encompasses us all?

A nurse who is qualified in Huntington’s plays a key part in the patient’s life, as the link to mental health. Physically, the patient’s body is dying—that is what is happening. Mentally, the disease affects them with anxiety, depression and all those issues. They feel it as it overtakes them and as their bodies decay. The nurse is also the link to neurology, GPs, social services and occupational therapy. I come back to the comment about PIPs made by the right hon. Member for Dwyfor Meirionnydd (Liz Saville Roberts). When our staff fill in PIPs forms, we look at how occupational therapy can add aids that help patients around the house. There is only so much that can be done for Huntington’s, and perhaps other diseases, but there is a key role for the occupational therapist in helping families to deal with it, whether that means a bed downstairs, an extension to the house or a walk-in shower. At certain stages of the game, of course, those things will not help, but perhaps early on they can.

In the area covered by the South Eastern Health and Social Care Trust, which includes my constituency, patients are referred to the Belfast Health and Social Care Trust specialist nurse, and the cost is covered by the Health and Social Care Board. Patients living in the western, northern and southern trust areas in Northern Ireland have no access to Huntington’s disease specialist nurses. Some sufferers have described the condition as a vacuum of silence—that is what it is. They feel almost isolated—on their own—and they are very much dealing with all the issues without help. When people are living in complete isolation, with no assistance, it is important that there is someone they can turn to.

Although I appreciate that health is devolved in Northern Ireland, the situation unfortunately remains the same in the rest of the UK, as the right hon. Member for Leeds Central said and as others will as well. There is no equality of care, and Huntington’s disease patients still feel left behind. Through this debate and through awareness raising, we must try to address that. If the number of people diagnosed with Huntington’s disease continues to rise, the Government must review its status as a rare disease. I said that earlier on, and I say it again. Perhaps we need to move it into a priority category as soon as we can.

I look to the Minister, as I will continue to do whenever she is responsible for the answers, and to her counterparts in the devolved Administrations to initiate funding for greater care for those in the early and latter stages of the disease. We referred to those 15 clinical trials earlier. The right hon. Member for Leeds Central referred to the clinical trials and how important it is to find a cure, help that research and bring new symptoms to light.

Familial carers desperately need their loved one to have professional care, so that they can seek some respite, without being sick with worry about them. They need respite care—we say that often, but Huntington’s disease is such an all-encompassing disease that it totally takes over the life of families. That respite care has to be there to give them a half-day or even a few hours off from the 24/7 focus they have. Some indication in the Minister’s response that there will be some help with respite care would be helpful.