Huntington's Disease
The final item of business today is a members' business debate on motion S3M-3000, in the name of Hugh Henry, on Huntington's disease. The debate will be concluded without any question being put. I call Hugh Henry to open the debate.
Motion debated,
That the Parliament recognises that Huntington's disease is a devastating, high-risk, inherited, neurological condition that causes a profound form of dementia and mental ill health; notes that the Scottish Huntington's Association (SHA), based in Paisley, was established in 1989 by families living with the disease because they could not get help with health and social care services; congratulates SHA in approaching its 20th anniversary in 2009 and on providing Huntington's disease services staffed by skilled nurses that make a significant difference to the lives of those affected by the disease; expresses its concern that only nine out of the 15 NHS boards provide this service, and considers that all people living with the condition would benefit from access to the type of support provided by SHA.
I am pleased to have the opportunity to debate the motion. I thank all the members who signed it and all those who intend to participate in the debate. It is appropriate that we debate the motion during the Scottish Huntington's Association's 20th anniversary year. A number of visitors in the gallery this evening have an interest in the issue because they have supported people with Huntington's disease. In particular, I welcome the families who are suffering because of the effects of the disease. I hope that members can speak to them after the debate.
Families who were living with the disease established the Scottish Huntington's Association in 1989 because they could not get help or support from health or social care services. The association continues to provide vital help and its focus is the goal of improving the quality of life of families who live with this devastating illness. The organisation provides Huntington's disease services in nine of the 15 health board areas across Scotland. Staffed by skilled nurses, those services make a significant difference to the lives of those who are affected by HD.
The nurses ensure that families always have someone to talk to who understands the problems that they face. They advocate on behalf of families to ensure that they have access to the correct health and social care services and they manage and co-ordinate that care. They educate other professionals about the disease so that they are in a better position to help ensure that symptoms of the disease are well managed. They also run a national youth service that supports young people who live in Huntington's disease families, 70 per cent of whom are young carers. Many of them experience social isolation and have to live with the psychological burden of having a 50:50 risk of inheriting Huntington's disease. The service provides one-to-one support, group work, information and advice.
Huntington's disease is devastating. It is a high-risk, inherited neurological condition. Its impact is so terrible because it destroys a crucial part of the brain, which leads to loss of control of the affected individual's muscles. That manifests itself as speech difficulties, involuntary movements and loss of the ability to swallow. The disease also causes a profound form of dementia. Mental ill health is common, as is depression, anxiety and personality change. Those three groups of symptoms gradually worsen over 15 to 25 years until complications from the disease—often pneumonia as a consequence of swallowing problems—lead to death.
The number of people living with Huntington's disease is three times the number with the more widely known motor neurone disease, which led to the premature death of the great Jimmy Johnstone, and because it is a genetic condition a large number of people in Scotland are living at high risk of inheriting the disease—the current estimate is between 4,000 and 6,000.
Huntington's disease is a family disease; it often affects two or three generations at the same time. In one family that the Scottish Huntington's Association has supported recently, a child of eight years died of the juvenile form of the disease. Her father has it and he needs a great deal of support to remain at home. Two of her father's siblings also have Huntington's disease and her grandfather died of it only three years ago.
Those who live in a family with the disease but who are not at risk of inheriting the gene are also profoundly affected. Loss is a constant visitor in Huntington's families. One member of the family I described did not inherit the gene that causes HD and will not therefore develop the disease, so it cannot be passed on to his children—a glimmer of light in the bleak picture that is Huntington's disease—but that young man says:
"People tell me I'm lucky that I won't get HD, and I am, but I will still never escape from it. I still have to watch most of my family suffer and eventually die. I might end up caring for them. When I look ahead I see a future on my own."
In addition to all those problems, Huntington's disease brings further pressures to bear on families. People with HD have to give up work very early and spouses have to give up work to become carers. Up to 80 per cent of affected families live in poverty. Relationship breakdown is sadly very common and it is estimated that 70 per cent of marriages end during the course of the disease.
The disease also carries a terrible stigma and has in the past often been a family secret. The mental health problems that are commonly caused by the illness, and the physical disabilities that frequently lead to falling and loss of confidence, often lead individuals to become withdrawn and isolated. Unfortunately, the involuntary movements, poor co-ordination and slurred speech have led to many people with the disease being accused of public drunkenness and, as a result, being wrongly arrested.
One of the core goals of the association is to ensure that, in every health board area where a need exists, a specialist nurse is provided. There is currently an inequity of service provision in Scotland. That leaves families who live in areas where the Scottish Huntington's Association has, as yet, been unable to deliver a service without the specialist help that they so desperately need and deserve. There are three health board areas where the association knows of an unmet need. In its 20th year, the association wants that to change. It wants, as a minimum level of service, every family living with Huntington's disease to have access to a specialist nurse, regardless of where they live in Scotland.
For services to be sustainable, they need support from all the statutory agencies. The best funding model exists where local authorities join forces with the regional national health service boards. That has already happened in Highland, Tayside, Fife and Glasgow.
I ask for the minister's support in trying to influence those health boards and local authorities that currently offer no assistance. I also ask the minister to recognise that the landscape of care for people living with Huntington's disease needs to change throughout Scotland, so that those affected by this terrible condition are cared for properly. [Applause.]
Visitors are obviously very welcome to what is, after all, their Parliament, but I ask them not to applaud either during or after speeches.
I thank Hugh Henry for securing tonight's debate. I think that this might be the first time that Huntington's disease has been debated in the Scottish Parliament. Members will correct me if I am wrong, but I was not able to find a previous debate.
I, too, would like to welcome the people who are in the public gallery. It is from such people that we learn what is happening in our own areas.
Although Huntington's disease is more common than motor neurone disease, the facts relating to Huntington's are often less well known. In Scotland, 850 people live with Huntington's disease. As Hugh Henry said, it is estimated that between 4,000 and 6,000 people could have a high risk of inheriting this genetic disease.
I apologise for the fact that I may use some figures that Hugh Henry has already used; I have tried to score out the parts of my speech that duplicate what he said.
It is clear why Huntington's is considered by many to be one of the most devastating of diseases. As Hugh Henry said, in those afflicted by the disease, a crucial part of the brain is destroyed, leading to a loss of control of muscles, speech difficulties, involuntary movements and, perhaps worst of all, an inability to swallow. What makes Huntington's all the more disturbing is the genetic link and the pressure that is put on future generations. Effects such as those explain why the Scottish Huntington's Association was established two decades ago.
The association does tremendous work, providing skilled nurses for sufferers. The nurses also help families, who are often under considerable pressure caring for loved ones. The decision that families living with the disease made two decades ago to set up the association highlighted the shortage of skilled staff to care for patients and their families. The association has worked hard ever since to raise the profile of Huntington's.
It was distressing to read in some of the briefings for this debate that sufferers have to give up work early, that many spouses become carers and are unable to work, and that more than 80 per cent of families with a Huntington's sufferer live in poverty. However, we are here tonight to acknowledge and commend the work of the Scottish Huntington's Association and its national youth service, which supports youngsters in families with Huntington's sufferers. Some 70 per cent of those young people are also carers. They can experience problems with friendships and schoolwork, and they may live with the knowledge that they have a 50:50 chance of suffering from the same disease as those they are caring for.
I believe that the Scottish Huntington's Association is right to seek to ensure that every family that lives with this disease has access to a specialist nurse and that there is no postcode lottery around that provision. No matter where someone lives, that vital support should be available to them.
I am pleased to say that, in the Highlands, a dedicated group works out of Raigmore hospital, with funding from Highland Council and NHS Highland. Thanks to this debate, I will get in touch with that group so that I can find out what is being done locally in the Highlands.
We need to raise awareness of this disease so that people in society do not mistake sufferers for another drunk who is unable to walk properly or is exhibiting slurred speech.
I was moved by the story of Sarah Winkless, an Olympic bronze medallist at the 2004 games and an HD gene carrier. Her story should prove to young people with a family history of this disease that their ambitions need not be limited by the disease or defeated by their worry about getting the disease. Her closing remarks about caring for her mother and living with the Huntington's gene sum up better than any politician could what the Scottish Huntington's Association does. She writes:
"I can't pretend that I don't sometimes look at Mum and wonder if how she is now is what the future holds for me. What I do know is that as, when, if I am affected by the disease, those around me will be better equipped physically and emotionally to deal with it than I was with Mum, thanks to the work of organisations such as the Scottish Huntington's Association".
I join other members in congratulating Hugh Henry on obtaining this debate on the 20th anniversary of the Scottish Huntington's Association.
I will not cover the ground that my colleagues have already covered—like Mary Scanlon, I have been crossing things off my list, so I will not talk about numbers, symptoms, the huge impact on families or the mistaken identification of intoxication.
When I was serving on the chief scientist's committee in the 1990s, we funded a study to promote genetic counselling services in the Lothian area as a pilot scheme. Can the minister say whether that counselling service is now universal? It is vital that the families in which there is a 50:50 chance of inheritance should receive appropriate counselling. It is also important, now that we have the availability of predictive testing, that it is available to all who wish it. Not all will seek it, which is quite important to remember, but those who wish to have it should, after counselling, be able to obtain it. Can the minister confirm that predictive testing is available to all who seek it?
We have moved on since I started in practice and first dealt with a family with Huntington's chorea—testing is now available. In those days, the risk was unknown and people simply had to live with that. There is also now the possibility of family planning through in vitro fertilisation, and approval for two cycles of that has been given by the Scottish Medicines Consortium. Is universal counselling available in that regard, too? I gather that that process has only a 25 per cent success rate and that, at the moment, the service for the group of patients that we are discussing is available only at Guy's hospital. Is the minister prepared to consider having discussions with the regional health authorities in the north of England about the possibility of establishing a new centre for such treatment somewhere further north than London, so that people might have easier access to that way of creating a new family?
We must consider how we can support these families in an integrated way. There needs to be integrated planning, integrated care pathways, effective standards of care, effective information in written form and on the internet and adequate advocacy services for families that are affected by the disease and individuals who are suffering from the disease beyond the stage at which their marriage has broken down, when they no longer have a spouse to support them.
There need to be local audits of the mental health effects on individuals. I know that the SHA is keen for the Scottish intercollegiate guidelines network to reconsider its decision on guidelines. I cannot say whether there is adequate research to allow it to establish further guidelines, but I ask the minister whether that has been considered recently and, if not, whether she is prepared to ask SIGN at least to reconsider its decision.
Huntington's disease is the sixth most funded subject of research in the United States, and the European Union network of research is already making an effective contribution. I ask again what the Scottish Government is doing to contribute in the form of collaborative research approaches.
I was disturbed to see that among the health boards that are named as not providing a service for the condition is the one in which I practised as a doctor—NHS Forth Valley. However, I was delighted to see that Fife, which is an area that I represent, was commended for its multidisciplinary teams. I say to the SHA that, after tonight's debate, I will write to NHS Forth Valley to ask what steps it will take to ensure that an effective service is provided. I am also aware that, once again, we are faced with short-term funding in some areas, such as my colleague Cathy Jamieson's area of Ayrshire and Arran, and I know that she will take up the issue to ensure that there is funding beyond 2011.
Patients with Huntington's disease deserve our support and attention. I hope that the minister will be able to answer some of the questions that I have put to her.
I join other members in congratulating the Scottish Huntington's Association on approaching its 20th anniversary. It is based in my colleague Hugh Henry's constituency of Paisley South, and I congratulate him on bringing this vital debate to the chamber.
The Scottish Huntington's Association started in the late 1980s as a consequence of the fact that families living with the disease were experiencing a deficit of help and support from health and social services. Mary Scanlon is probably correct to say that this is the first debate that the Scottish Parliament has had on Huntington's disease. Like her, I have been here since 1999 and I do not recall a debate on the matter. However, if there had been such a debate, would I have put my name down to take part in it? I did not know much about Huntington's disease. I knew a family that had Huntington's within it, but I did not know them well enough to get to know what the condition was about. Now, however, I have become aware of the disease because it is touching my family and we are having to learn how to deal with it.
It concerns me that today, 20 years after the SHA identified a lack of support, families still do not have the support that specialist nursing staff can bring. Having support from organisations and nursing staff can make a big difference, so I support the association's call for a specialist nurse in every health board area where a needs assessment demonstrates that such a service is required. I hope that the Scottish Government will support the measure in order to make the services sustainable.
Every family that is affected by Huntington's disease deserves a nursing service that provides advice and help to support individuals to manage symptoms—but it should also act as an advocate for the family, ensuring that they have access to the correct care and that there is awareness on the part of the other health professionals who are involved. Given that Huntington's disease is a genetic condition, the work of the SHA's nursing teams in supporting young people and families who are affected by the disease must also be supported. Whether they have inherited Huntington's or they care for family members with the disease, it is crucial that young people receive the support that they need to live full lives. I would like the Scottish Government to work with the SHA to provide consistent support for young people, who can be particularly isolated due to the disease.
Other care givers of all ages also deserve the best support that can be made available. I hope that the minister will acknowledge the lack of long-term care placements for those who have Huntington's disease. For sufferers and carers alike, the lack of respite care is particularly acute and the Government must act to fill that gap in provision.
As for my constituency, I am concerned that Big Lottery Fund money that was secured for specialist nursing services in Lanarkshire for those who have to live with Huntington's disease will run out in 2011. I realise that that is some time away but if we are planning for the future we have to look at services now.
I again congratulate Hugh Henry on securing a debate on such an important and underrepresented issue and ask the Scottish Government to give careful consideration to the points that I and other members have raised.
I welcome to the public gallery those who have come to listen to the debate, and I congratulate Hugh Henry on bringing the issue to the chamber.
I am not going to describe the disease, because that has already been done. My experience is from providing support to people who were suffering from the later effects of what in those days was called Huntington's chorea. Psychiatric symptoms would begin to appear, the most common of which was depression. In some cases, people were admitted to hospital, and the disease was discovered only when they were in the system and had become worse, exhibiting aggression, delusions and paranoia. Sometimes many other diagnoses were proposed before it became clear that the person was suffering from Huntington's disease.
As Hugh Henry said, some families keep the disease a secret. Families have been known to split up as a result; in one case, I had to deal with a young man who had been adopted and did not know that he was going to develop the illness. In those days, people who were adopted did not necessarily know who their father was.
There was also the emotionally charged issue of having to talk to parents, one of whom might be carrying the gene, to highlight the possibility that, if the woman became pregnant, the child had a 50:50 chance of inheriting the gene, and to inform them that the onset of the disease might not happen until their child was in their 30s, 40s or 50s. There is also for parents the very difficult question whether a child should be tested or told.
Richard Simpson is correct to say that helpful systems are now in place to carry out the test during pregnancy and that there is greater understanding of inheritance, prognosis and management. Much more information is certainly available than when I was practising and working with families, and awareness has been raised through books and, whether we like it or not, through television series such as "ER" and films such as "Alice's Restaurant". Indeed, we must not forget the work of the foundation that was set up by Marjorie Guthrie, Woody Guthrie's wife, after his death from HD complications. The United States has a national HD awareness day on 6 June and, of course, the UK has a national HD awareness week.
This morning, MSPs debated hospital waiting times and, as Richard Simpson has pointed out, ministers committed to increasing specialist nurses and support systems for those who suffer mental illness. I hope that any such approach will include not only waiting time guarantees but training for those who deal with people suffering from Huntington's disease and support for their families.
I understand that research from the University of Leeds has found that one of the body's naturally recurring proteins might—and I stress that word—cause some of the disruption in the brains of those who suffer from Huntington's disease. The effects might be modified with drugs that are used to help cancer patients, but such treatment is in its very early stages, and it might be years before it is fully developed.
My mother died from tuberculosis. She had it all the time that I was growing up, and she, my father, my two brothers and I spent every day hoping that someone would find a cure. I know that the incidence of TB has increased in some areas, but a cure for the disease was nevertheless found. I hope that the same happens for HD.
On behalf of the Scottish Government, I welcome this debate on Huntington's disease and thank Hugh Henry for bringing it to the Parliament. As has been said, this is the first time that we have discussed the condition in the Parliament. I, too, welcome the people in the public gallery who have come to listen to our debate. The Scottish Government is well aware of the excellent work that the Scottish Huntington's Association does in supporting people who have the devastating condition and their families. I congratulate the association on its 20th birthday. The association's contribution to the review of genetic services in Scotland, the findings of which were published in 2006, is a tribute to its effectiveness at a strategic level.
As members have said, living with the condition is hard enough, but its inherited nature compounds the problems and pressures. Several generations can often be affected at the same time and children have to watch a parent deteriorate, knowing that the same might happen to them. It is no wonder that the Scottish needs assessment programme pointed out back in 2000 that the number of people whose lives are affected is far greater than the number of people who live with the condition. It is therefore essential that NHS boards and their local planning partners are aware of the scale of the issue and provide services accordingly.
The standards that NHS Quality Improvement Scotland is developing for neurological conditions will require boards to collect the best possible data on those conditions. Boards will also need to define and publish details of the services that they provide for people with neurological conditions, which will of course cover Huntington's disease. Those reports will include information about the agreements that boards have entered into with the Scottish Huntington's Association. We strongly advocate that form of joint working in "Better Health, Better Care".
Probably the most important thing that we all need to do is to raise awareness of Huntington's, which can be done in several ways. One possibility is to revisit the Scottish needs assessment programme report of 2000 and the follow-up report of 2004. That is now the responsibility of the Scottish public health network, and we have asked the network to consider adding that to its work programme. Another possibility is to develop a SIGN guideline, which Richard Simpson mentioned. The association believes that the evidence base has developed greatly in the three years since it last approached SIGN. I understand that it has submitted another application in time for this year's deadline, and I hope that SIGN will look favourably on that.
The motion makes particular reference to the contribution of specialist nurses, which is an issue that several members have mentioned. We are aware of how highly those nurses are valued by people with the condition and their families and carers. I heard what Richard Simpson said about Forth Valley NHS Board, but I hope that he recognises that the board is developing the idea of a neurology specialist nurse. In following that example and adopting such a model, boards would need to be clear that the needs of those with Huntington's were met properly. However, we are interested in the idea. Specialist nurses fit in well with the idea of managed clinical or care networks, which need to define clearly the roles of all members of the team. Huntington's lends itself to an MCN approach because of the complex nature of the condition and the need for fully integrated services. "Better Health, Better Care" gave priority to the development of MCNs for neurological conditions. The Scottish Government health directorates would be happy to advise the association on that.
Managed care networks promote integration of health and social care, which is essential in the case of Huntington's. That integration must include access to mental health services, given that the disease can cause a profound form of dementia, as well as depression and anxiety. There are a number of other levers for achieving that integration. For example, NHS QIS standard 4 emphasises the role of community health partnerships in integrating primary care and specialist services with social care.
Richard Simpson asked about genetic counselling services. The single-gene complex disorder project, which is part of the implementation of the genetics review, has a particular focus on the integration of health and social care and draws on the expertise that the association has acquired in the past 20 years. The implementation of the review also involves appointing more genetic care co-ordinators, who will enhance the interface between health and social care. Genetic counselling is a key aim of the implementation. I can tell Richard Simpson that in Lothian there is a project co-ordinator and an administrative post for the project. The other four centres will have a co-ordinator post. All those posts are now in place, except the Tayside post, which is being taken forward. Progress has been made, which I hope that people will welcome.
The focus of the community care outcomes framework on improving partnership performance through collecting data that shape the design of specific care packages is important. The single shared assessment is about identifying each person's needs so that the necessary services and interventions can be put in place.
Greater uptake of self-directed support has a key role to play in progressive long-term conditions such as Huntington's. We want people to have more independence and control over the services that they receive. That also has an application in end-of-life and palliative care services. I am sure that the needs of people with Huntington's will be kept in mind as we implement our national strategy on palliative and end-of-life care. I shall certainly ensure that that happens.
It would be wrong not to pay tribute to the central role of unpaid carers, especially in relation to a condition that affects whole families in such a profound way. I heard what Cathie Craigie said about the need for greater access to respite care, which is of course one of our main concerns. That is why we have been working with the Convention of Scottish Local Authorities to provide an extra 10,000 weeks of respite care. We are also funding the implementation of carer information strategies in each NHS board area.
The clearest message that I want to send from the debate is that Huntington's needs to be rescued, to some extent, from the lack of awareness that exists in society and among some of our health professionals and service providers. I hope that today's debate will play a crucial role in raising awareness. The Scottish needs assessment programme reports of 2000 and 2004 were very clear about what needs to be done to improve services. They must now be placed on the soundest possible long-term footing throughout Scotland. The best models are those in which the NHS and local authorities join forces to provide the funding. That is something for which a number of members have called in the debate. I assure them that we will certainly encourage the NHS and local authorities to go down that path, which is the best way of ensuring that services improve throughout Scotland in the way that we all want.
Meeting closed at 17:42.