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Huntington's Chorea

Volume 888: debated on Wednesday 19 March 1975

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10.52 p.m.

I am grateful to the Minister of State for coming to reply to the debate, which is on a topic of immense importance to a small minority of people.

The subject that I wish to raise is the problem of those suffering from Hunting-ton's disease and those who may suffer from that ghastly disease in the future. It is a disease that is inherited. It attacks the nervous system and results in all adverse effect upon bodily movement. It sometimes eventually affects the mental faculties. Frequently the disease does not develop until the person affected is in his forties, or even later.

The first problem is to identify the disease. I hope that the Minister will have considered the problem of notification. I think that the Department does not have accurate information about the numbers affected. In 1972 we learnt from a reply to a Question that the Department estimated that the number of people suffering from the disease was 2,000. Less than two years later the estimate from the Department was that it was probably 3,000. I do not think that it would have calculated that there had been a 50 per cent. increase in that time.

I have spoken to a number of those actively interested in the topic from a medical point of view. They tend to think that probably the figure is nearer 6,000, with many people not having been identified as suffering from the disease. Therefore, probably one in every 10,000 people in this country suffers from the disease. In a typical constituency, if we have the average incidence of the illness, we probably have 10 of our constituents suffering from it, and perhaps 40 of their children and grandchildren who live in dread that they have inherited the disease and will suffer from it. Half of them will almost certainly develop the disease before they die, and will perhaps die of it.

It is usually a fairly slow-developing disease, although sometimes the development can be quite rapid. It usually results in the sufferer having to spend the last years of his life in hospital. Many sufferers have to spend those years in either psychiatric or geriatric hospitals, and the medical profession holds the strong opinion that many sufferers in psychiatric hospitals should not be there. The main reason for their being in that type of hospital accommodation is that a long period of hospital attention is required because of the nature of the disease. I ask the Minister of State to consider the possibility of providing for sufferers of the disease hospital accommodation which is not necessarily psychiatric or geriatric.

There is need for a positive approach by the Department. This is a serious disease, ghastly for those who suffer from it and terrifying for those who know that they may have inherited it and that it will develop later in life. I wish to put forward eight constructive suggestions. I recognise that in replying to an Adjournment debate the Minister may be unable to commit himself, or to give a direct answer tonight, but I ask him to give careful consideration to my suggestions and to get his Department to come to conclusions on them. When he has done so I ask him to tell the medical profession and the sufferers from the disease what constructive approach the Department will develop.

I ask the Minister to consider making the disease notifiable. If any disease should be officially notifiable it is surely this one. It is one which children and grandchildren are likely to inherit, and there is an even chance that they will inherit it.

Notification of the disease could be linked with my second suggestion, which is that there should be an automatic routine of contacting the children and grandchildren of the person concerned, so that advice may be given to them by a genetic counsellor. It is vital that people should know immediately that they are likely to be carriers of the disease. Children often lose contact with their parents, and grandchildren often lose contact with their grandparents. It is therefore important for all who could have inherited the disease to be told and to be counselled on the pontential danger of their children inheriting it.

Next, I ask the Minister to review the research programme. Some important work is being done. Those who are interested in this disease recognise the diligent work of Dr. Caro in East Anglia, which is producing interesting facts which would otherwise never have been discovered. The incidence of the disease in certain localities of East Anglia, for example, in Lowestoft, is far greater than might be thought. I hope that the Department will review its research programme and give help to the official organisation "Combat ", formed of interested people and sufferers from the disease.

I am glad that the right hon. Gentleman has brought up this subject. Is he aware of the research institution in Glasgow in which is being carried out genetic engineering, not for the purpose of curing the disease—because it cannot be cured—but of eradicating it by determining before a child is born or at a very early age whether it carries this gene? I hope that the right hon. Gentleman will push the Minister of State to institute further research of that kind.

I am grateful to the hon. Member for East Kilbride (Dr. Miller). I knew of that work. It is a valuable piece of research, which, over a period could assist in eradicating the disease. Unless it is done and done well, and conclusions are drawn from it and action is taken on it, this disease is bound to increase in the years ahead.

The next suggestion I should like to make to the Minister is that more should be done to brief the medical profession and social workers about this disease. Because of its comparative rarity, those who have some knowledge of the disease feel that many doctors in general practice do not correctly diagnose it, and that many social workers who are brought into contact with families who have a member suffering from the disease do not recognise the considerable emotional tensions and anxiety that arise in the family. I hope that the medical profession and social workers who are likely to come into contact with the disease will be able to obtain a more positive and complete briefing, and that there will be more of a rethink than has happened in the past.

The next suggestion I wish to make is that there should be a study of the feasibility of providing suitable hospital accommodation. Cases have been brought to my attention of people placed in psychiatric and geriatric hospitals having been made doubly unhappy by the surroundings in which they find themselves. Their medical advisers consider that they would be far better off if they were put in general hospitals rather than psychiatric or geriatric hospitals. In the localities where the known density of sufferers is higher than normal—and there are some where it is—it would be in the interests of sufferers to see that appropriate long-term hospital accommodation is made available to them.

The next of my eight points concerns the question of attendance allowance. This varies in different parts of the country. Certainly all those in the medical profession whom I have contacted who are aware of the effects of this disease are unanimous in the belief that anybody suffering from it needs proper attendance. I hope that it is the objective of the Department to see that those making judgments on attendance allowances make them in favour of providing such an allowance in a family where there is a sufferer.

Likewise, the Department should give careful consideration to the overall social welfare and community care programme for those with this disease.

Occupational therapy and treatment of that type can be very important to the patients, particularly in the early stages of the disease.

It is interesting that in the United States, as in this country, this topic was largely neglected until a famous folk singer, Woody Guthrie, died of the disease. His widow devoted her time to trying to organise public opinion to urge the United States Government and the governments of the states to recognise the suffering and dangers resulting from the disease. That has had an important impact in the United States, where a committee has been formed to combat the disease.

It is also interesting that before the American Senate there are proposals for legislation to provide, in each of the next three years, $500,000 in grants for screening, treatment and counsel ; $½ million in grants for research and $150,000 for education and information.

I believe that anybody who has ever come across a family suffering from this disease will recognise the real nature of the suffering. Not only do patients suffer from this creeping disease which gradually destroys the movement of limbs, retards speech and has an effect on mental processes; they also know that their children and grandchildren may have inherited the disease from them. The tensions are immense, and the anxieties on the part of those who may have inherited the disease are also very great. This is a small minority but a minority that suffers badly. I hope that the Minister will give careful consideration to what I have said and that his Department will take all possible action to alleviate the situation.

11.7 p.m.

The right hon. Member for Worcester (Mr. Walker) is to be congratulated on raising this small—" small "in the sense that it affects a limited number of people—but major issue. It also raises a number of interesting concepts of how we deal with these problems.

The right hon. Gentleman raised eight specific points, and I shall do my best to answer each and every one. Perhaps it will help the House if I begin by saying a few words about the nature of Hunting-ton's chorea. It is, as the right hon. Gentleman said, an inherited degenerative disease of the central nervous system. The problem is that the first symptoms of the disease generally manifest themselves at about 35 or 40 years of age, although on occasion they can appear either in childhood or in elderly people. The disease is a dominantly inherited genetic disorder, which means that each child of an affected parent will have a fifty-fifty chance of inheriting the disease. Early signs of the disease, such as unsteadiness when walking, or forgetfulness, are difficult to detect, and a firm diagnosis cannot always be made until the disease has progressed for a considerable time. This is more likely to occur if the sufferer is not known to come from a family affected by the disease. The symptoms become progressively worse until patients invariably have to enter either a hospital or residential unit, because nursing them in their own homes poses too many problems.

Let me deal with the first of the right hon. Gentleman's points—namely, the incidence of the disease. He was right to say that estimates of its incidence have varied. The current estimate is about 3,000 sufferers, but I should be the first to admit that the estimate may not be correct. The right hon. Gentleman mentioned that it was possible the figure might be nearer 6,000. I hesitate to endorse such a figure, but I would not like to stand pat on the figure of 3,000.

What can we do about the situation? The first thing I was asked was whether the disease could be made notifiable. That would involve problems. It is a difficult matter to trace and to cope with the problems and complexities of seeking to establish a register of known sufferers and members of families at risk. A systematic approach has much to recommend it, and I shall look carefully at the question of whether we could make the disease notifiable. Registers have been tried experimentally in some ranges of genetic disease. The Department is considering whether it would offer worthwhile opportunity to reduce the occurrence of the disease. A further difficulty in making the disease notifiable is that it is difficult to diagnose and, as the right hon. Gentleman said, it would involve briefing members of the medical profession and social workers on early diagnostic signs. I shall look into that matter.

Secondly, the right hon. Gentleman raised the question of follow-up and counselling. These are vital matters. The child of an affected parent will have a fifty-fifty chance and many parents will conceive before diagnosis. If we are to stop the prevalance of the disease, it is important to trace sufferers at an early stage. Genetic counselling for prospectice parents is important. It has already been provided under the National Health Service through general practitioners, and special genetic advisory centres have been set up in each region. Late onset of the disease is one of the biggest problems in achieving effective counselling.

That leads me to the right hon. Gentleman's point about briefing the medical profession and social workers. I admit that more needs to be done about this. One way of doing that is to make it a notifiable disease, which tends to concentrate more attention on it. It is a formidable task to guarantee that all professional staff are in possession of all the relevant information to pick up the early signs. However, I shall look at any way in which we can improve information about the disease. When considering notification we are bound to take into account the need to ensure that the early symptoms are known.

The third point raised by the hon. Gentleman concerned the research programme. I shall be interested to hear whether the United States Senate approves this ambitious research project. Research is international. Research that is being done in many countries will help the United Kingdom.

The hon. Gentleman mentioned East Anglia. The Department has recently approved expenditure of more than £16,000 over a period of three years from its central research funds to extend a project which has been financed to date by the East Anglian Regional Health Authority under its locally organised research scheme. The project aims at identifying sufferers and potential sufferers from the disease in East Anglia with the intention of assessing social and welfare needs, enabling improved planning of required facilities for care, and determining the advantages of tracing family groups to aid the provision of genetic counselling.

The Medical Research Council is supporting a wide range of basic neurological work which could advance the knowledge of Huntington's chorea. The major part of the research programme at the MRC neurochemical pharmacology unit is concerned with studies of the biochemical changes underlying the disease. Other biochemical investigations are in progress at the MRC brain metabolism unit and in the Division of Molecular Pharmacology at the National Institute for Medical Research. The Department of Social Medicine at Edinburgh University is engaged on a project on the incidence and family distribution of the disease, and the Department of Psychiatry at Sheffield University has recently finished a project on cerebrospinal fluid amines in Huntington's chorea and other neurological conditions. The Department of Neurological Surgery at Addenbrooke's Hospital, Cambridge has also recently completed a research project on rigidity in Huntington's chorea from funds provided by the Mental Health Trust, which was concerned with biochemical aspects of the disease. The trust is now considering a proposal from the Department of Clinical Genetics at the Welsh National School of Medicine dealing with some of the genetic aspects of the disease. A great deal is being done, but difficult problems are posed.

The fourth point raised by the hon. Gentleman concerned the Association to Combat Huntington's chorea. He is right; the association has played a very important part since its foundation in 1971. It exists mainly to foster research into the disease but also makes a significant contribution to the welfare of sufferers and their families. My hon. Friend the Under-Secretary of State ressponsible for the disabled, and officials in the Department, met representatives of the association. At both of these meetings there were very full and useful discussions of the many problems involved. Following these discussions I am pleased to say that the Department has been able to increase its funding of research into this disease.

I am considering other ways in which sufferers might be helped. I want to keep in close contact with the association. It is performing a valuable task in making more people aware of the problems involved in this disease.

I have dealt with the briefing of the medical profession and social workers.

The sixth point raised by the hon. Gentleman concerned available and suitable hospital accommodation. I have great sympathy with this. However, there are problems. When these patients become severe sufferers they are difficult to treat. They require heavy nursing facilities. It would be nice to promise that they would be treated close to their homes. I think that is an important factor. Many are admitted to psychiatric hospitals because of the mental aspect of the illness. However, I should not want to think that those were the only places to which they could go. We need additional smaller units for the chronic sick and the severely disabled. Much has already been done about that problem.

I do not think we can look on this as a special disease category in terms of its treatment, because many of its manifestations are common to some other ailments. However, the fact that the disease is progressive and is genetic causes real concern. I will consider that, but I do not think that I can offer any more than the overall provision, which I know is inadequate, for chronic disability.

The right hon. Gentleman's seventh point related to the attendance allowance. Again, I do not think that we can say that all those who suffer from Huntington's chorea must have an attendance allowance. In the easly stages of the disease, they would not necessarily require it. But, as with other progressive conditions, when they become severely disabled there is no doubt that these patients should be eligible for the attendance allowance. I know that the Attendance Allowance Board shares the view that it is best and fairest for all concerned that entitlement to the allowance should be decided on an individual basis, depending on he facts in each case. It is important that the relatives of sufferers should know that they can claim the attendance allowance.

I cannot comment on individual cases on decisions given by the board as the board is an independent authority set up under the Act of Parliament which introduced the allowance. If, however, a claimant is dissatisfied with a decision by or on behalf of the board, he or she has only to write to the attendance allowance unit, Norcross, Blackpool and ask for the decision to be reviewed. What is more, it may need reviewing fairly frequently, because the deterioration can go on. A person may be rejected on one occasion for attendance allowance and year later be a candidate for it.

The right hon. Gentleman's final point dealt with the need for more national concern. How do we generate a feeling of concern about this and many other genetic diseases, and how do we arrange screening programmes? This relates also to community support and how much we are giving overall. It is not easy. It is a question of competing limited resources. But some of these degenerative diseases, where we know there is this dominant inheritance, pose very real problems. The early identification of families at risk is crucial. Genetic counselling prior to the decision to have children is of vital importance. There is no doubt that money spent sensibly and wisely there will not only prevent a great deal of hardship and misery to those families but will save a considerable amount of money. Much has been done in research.

The right hon. Gentleman's point that the incidence of the disease may be far greater than we currently think needs looking at seriously. I shall put this to my professional advisers. I shall look again at the question of notification. It has considerable attractions, on the face of it, but the problems of making it a notifiable disease are formidable. Experience seems to indicate that when a disease it made notifiable without being certain that cases can be picked up, an illusion of notification is given, almost generating the very atmosphere hoped to be avoided. But I am prepared to look at this. I am sure, from the right hon. Gentleman's knowledge and the research that he has done on the subject, that a number of professional people have put it to him that this is what they would like to see done. In the light of that, I shall take that suggestion away and perhaps look at the experience of other countries who have notification to see whether this would help us.

I cannot offer to those who suffer from Huntington's chorea any immediate prospect that a magic cure will be found for the disease, but I think that the research going on into the biochemical nature offers some prospect at some time. We are trying to discover more about its basic aetiology.

As I said just now, the right hon. Gentleman spoke about public concern. He has raised the matter in this debate. He had brought it to my attention. It is an illness which, unfortunately, I had to deal with in a number of cases when I was involved in neurology myself. I will take up his points and see whether anything further can be done.

Question put and agreed to.

Adjourned accordingly at nineteen minutes past Eleven o'clock.