Sue Watkin - Chairperson of the Huntington’s Disease Association England, UK.

Today’s world in relation to Huntington’s Disease is a vastly different one to that of twenty, ten or even five years ago. I am sure that we would all agree that most of the changes that have occurred are changes for the better - but old problems remain and new problems have been created.
    Around the world genetics has become a hot topic. Hardly a day goes by without another story appearing on the television news or in a newspaper or magazine. These stories range from Dolly, the cloned sheep, to journalists’ flights of fancy - seemingly absurd notions - like the cloning of Michael Jackson (probably a popular idea with cosmetic surgeons). There are of course sensible discussions of the latest advances in science and technology, and the benefits they may bring to mankind. Media interest has resulted in families affected by HD suddenly finding themselves in the spotlight - not necessarily as individuals but as a ‘collective’. We are part of the’affected by genetic disease community’ upon whom journalists, broadcasters and the general public sometimes feel free to sit in judgment. It was ever thus - but it is just more common now that genetics is always in the news. Surely this is a benefit - think of the awareness raising ! Isn’t it always said that there is no such thing as bad publicity?.
    Let’s look at the awareness raising from the point of view of the individual. Has it taken away an option? Many people do not speak of the illness affecting the family. In the past especially, people felt that nothing could be done for them - no one was interested - there was no hope - so in the interests of protecting themselves and their children from the negative effects of the disease they kept quiet. An elderly lady recently said to me that she was terrified that friends and neighbors might find out that her grandchildren, in their early twenties, were at risk.
    She said: They won’t have any girlfriends - no one will want to marry them - no one will want their children - they won’t get promotion at work. Why do they have to make programmes about HD?
She was distraught - she felt that publicity about HD would ruin her grandchildrens lives.
    Perhaps one of the hardest things a parent ever has to do is to tell their children about the illness in the family. All parents want to do is to protect their children, protect them from learning painful information. You and I might think that not telling them about HD is harmful - that it will be a source of future anger, resentment and pain - that everyone has the right to knowledge which might profoundly affect their life. But not everyonr thinks that way - some people feel that the longer you live without the knowledge, the longer your life will remain carefree, unencumbered with fears for the future. But in today’s world keeping HD a secret is very difficult. Let’s look at the classroom - in many schools HD is part of the teaching of genetics. It is an illness used to demonstrate the autsomal dominant form of inheritance. Certainly in the UK all fourteen to fifteen year olds will be given information about HD without revealing its hereditary nature. Perhaps they are waiting for the right time to reveal more facts. When they are confronted with their child’s questions they feel at a loss and unprepared.
    Let’s look at this from the child’s point of view. How many children in the class room are confronted with knowledge that they feel they cannot share. How many children are trying to protect their parents from the hurt of realising that they already know. There are children suffering from being unable to talk about their fears - creating in their minds the worst scenario. I would just like to illustrate how children can be affected by being given some information but then having answers to their questions denied. - one of our Regional Care Advisers holds a drop in clinic for people to come in and talk about their problems. It is held in a community facility in the heart of a major city. Recently two young brothers, aged 11 and 9, unaccompanied, came to her for help. They had taken a day off school and travelled alone to the city centre. They told her that their mum had HD - they said she was at home but she was qite ill - they said that they had asked the social worker if their mum would get better or would she die - they had asked their doctor the same question - they had then asked their teacher. They had been told - don’t worry - everything will be all right. They didn’t believe any of them. They were young children - desperate for truth - aware that they were being told lies.
    How should we view the fact that information about HD is being brought to the attention of the general public? It is certainly a change - something new. The option of secrecy is being lost.
    Should we see this as a benefit, preventing the anger, distress and pain of the young adults who have made lifestyle decisions while having the facts withheld from them. Will greater openness help the children come to terms with HD while they are more accepting of painful information? Should we see it as an erosion of parental choice?
    This year, in the UK, public awareness of HD has been raised enormously as a result of being featured on the ‘soap’ Emmerdale, a twice weekly TV serial. Apart from the problems relating to how well or badly HD has been pertrayed the serial has caused a great deal of controversy.
    The cause of the greatest concern can be summed up in the well known saying - ‘a little learning is a dangerous thing’. The storyline has spawned opinions from people with no prior knowledge of HD - dogmatic opinions which have not been thought through.
    Probably the cause of greatest anguish was the element in the story which implied that having children or even contemplating starting a family was not an option for those at risk. There are people within the HD community with these beliefs but many people take a different view. The guilt heaped upon people who already have children or people who are planning a family has been overwhelming. One couple, expecting their first child, said they had spent night after sleepless night that their carefully considered decision was immoral and would only end in grief and heartache. Sadly, it isn’t only opinionated programme makers that promote these views. I know of a neurologist who told a young couple who had just found out that the wife was at risk that it would be best to make an appointment for sterilisation immediately - this wasn’t in the dim and distant past - it was earlier this year !.
    The media so often present matters in black and white and their audience is all too readily accepting their stance. I am sure that we all welcome debate on the issues which face families affected by genetic disease. We don’t want to be lectured by programme makers and journalist who are only interested in promoting their own opinions. There needs to be balanced discussion. We, as representatives of lay associations need to be ready to refute their opinionated stance and protect our members from the hurt that they cause.
    On the positive side Emmerdale has brought about a great deal of public awareness. It might only be that people have a vague idea of what HD is about - but it is a beginning. Perhaps it has been of greater benefit in making people affected by HD aware that they are not alone. It has certainly helped them have open discussion..
    One young woman said when she realised that HD was being featured on the programme she cooked her boyfriend’s favourite meal, opened a bottle of wine, sat down and watched the programme together, told him she was at risk. She had not been able to tell him before - there had never been the right moment. He said it didn’t matter - now she has someone to share her future without the hidden secret.
    As I have already said, many people have contacted us in a distressed state. But although the immediate cause of their distress has been the content of the programme has it helped them to confront their feelings - has it assisted them in seeking help? Has it given people information that they were not aware of? Of one thing we are sure - more than eight hundred people have contacted our Association for information as a result of the programme. The genetics clinics have seen an increase in the number of people coming to them for advice.
    Let’s consider now the interest that is created inthe lives of real people. I am sure we are alll familiar with the newspaper reporter or the television company requesting names of families to interview. In our newsletter we often ask willing individuals to come forward. But people need to be aware of the potential problems. So many times people give interviews without thinking of the impact on the wider family. Families can be torn apart by injudicious airing of their problems. Children may feel that their parent has jeopardised their future. Brothers and sisters may feel that the family illness is not for popular consumption. It is so important that we all realise the possible consequences of going public.
    The media are notorious for poor reporting - unsuspecting people can have their words taken out of context - they can be made to feel silly by incorrect interpretation of their statements, they can be attributed with statements never made. It is impossible to prevent the worst of the media abuses but it is our duty to do our best to avoid them. In our quest for a greater understanding of HD we often feel that there is no such thing as bad publicity - it isn’t true !.
    Let’s turn to the public perception of predictive testing. This is a subject in which the media has taken a strong interest, especially in the light of the discovery of new genes predisposing people to the common diseases. One journalist with a regular Saturday column about all the soaps took the view that the young man in Emmerdale was pathetic for not taking the predictive test immediately he found out that he was at risk. Week after week she trivialised the test making it seem no more important than choosing some new wallpaper ! This one journalist probably caused more hurt and anger than the programme itself. Eventually after being bombarded by letters from our members, a grudging apology appeared in the column.
    Recently a story was run to expose the unfairness of a person of fifteen being denied the opportunity of taking a predictive test. There is a lot of debate around this issue but the article failed to discuss the problems facing everyone regardless of age in relation to testing. It was based solely on the right of the individual, regardless of maturity, regardless of the pitfalls to excercise this option.
    There was no mention of practical matters of insurance, or the psychological implications, or the degree of understanding - testing was a right - end of story.
    One of our deepest concerns is that public knowledge of predictive testing should result in unfairness in unemployment and insurance.
    How many people are afraid to take a predictive test because they may become uninsurable?.
    If they go ahead and have a positive result how many people are afraid to tell their employer in case they should be classified as already ill?.
    Many people around the world are looking at the problems posed by predictive testing in the area of employment and insurance. Some countries have legislated over the use or misuse of genetic information, others have adopted a "let’s wait and see" stance. of one thing we can be sure - it is very difficult to keep information you may obtain on your owwn future, for your own reasons, to yourself.
    What about the problems around diagnosis? How many people jeopardise their motor insurance because they are afraid to inform their insurer that a diagnosis of HD has been made.? How many fear prohibitive premiums? How many fear that their licence will be unjustifiably taken away. After all HD is often diagnosed in the very early stages these days when impairment is minimal. But do insurers know that?.
    How many people have problems with obtaining travel insurance, sometimes refused outright - at other times quoted exorbitant premiums.
    All we ask, in the interests of fairness, that the people making decisions that have such impact on our lives are in full possession of the facts and have the ability to interpret them.
    But what of ourselves, our own associations, our societies. The impact on families of information becoming public knowledge is not just a matter for the national media. Knowledge can be shared through our own national newsletters, by local branches and support groups. Sometimes confidentiality is the victim. Very often within a branch or a chapter or a support group there is a special person acting in a counselling capacity - a listening ear. This is the sort of person who is told the result of a prdeictive test for example. How often does this information end up for public consumption in the local newsletter. If someone tells you their news it does not mean they want the world to know. Confidentiality is a major issue for us all.
    Very often breaches of confidentiality are made with the best of intentions - with an enthusiasm for information sharing which it is thought will improve the condition of people affected by HD. Often local social services, will say - " We need to know how many people with HD live in this locality. We need to ascertain the scale of the problem before we can help. You need to justify your requirements ". Very often they use this ploy to put off the day when they have to find the personnel and the resources to provide for the families needs. Local branches often feel that they must go all out to ascertain prevalence figures when the local genetics clinics could provide much of the information. In their efforts to obtain services, questionaires are formulated. It is all too simple for the research to become intrusive and unwelcome. The people approached often feel they are letting the side down if they don’t participate. They often, unwittingly, give information on other family members without their permission.
    As an example of this quest for information, and the ensuing disregard for confidentiality, one of our local branches displayed a local area map - very detailed with every village and hamlet - at an Annual Meeting to which local dignitaries and the press had been invited. On this map were a series of coloured pins showing by their different colours every person with HD, those at 50 and 25 per cent risk, those with a positive test result. In a tight knit community this was a gross violation of confidentiality. How many people are, with great justification prevented from seeking the help they need because of this kind of insensitvity. Even if this kind of action meets with the approval of one family member what about the rest? There are too many people afraid of joining lay associations for fear of being on record. The Gung Ho attitude of some well meaning people can only destroy confidence in an organisation.
    There are many ways that information becomes public knowledge and confidentiality is breached. There is the well meaning person who tries to obtain help for a friend, from social services perhaps, without seeking permission.
    But what about the professional - the person for whom confidentiality should be second nature. How often are our advisers given the telephone number of a person by a professional, asking them to make contact. How many times has the affected person not been asked for permission. We have had instances where our advisers have been asked to offer assistance only to discover that the family is unaware of the diagnosis.
    There are numerous examples in this vein - the nurse in charge of a specialist unit who attempted to trace family members of an individual in her care through the Salvation Army. She thought it was her duty to inform others of the family illness. She hadn’t thought to discuss this idea with her patient. She was probably motivated by the best of intentions but that is no excuse for lack of professionalism.
    Unthinking sharing of confidential information can be so damaging - recently a member of our staff made a joint visit with a social workerto the offices of a social services unit specialising in providing care in the community. On display was a large notice board detailing their success stories - people they had enabled to stay at home. Together with photographs there were names and diagnoses. The social worker spotted a picture of her aunt whom she thought had Parkinson’s disease. As she was currently dealing with another HD client she was aware of all the implications for herself. As you can imagine, she was devastated.
    Then there is the other side of the coin. How often is information given to family members while excluding the person who is ill. How often do doctors tell other family members of the diagnosis without telling the affected person. Again this is often done with the best of motives. Can you imagine how the affected person must feel. All around him are aware of the diagnosis while he lives in bewildering, often frightening, ignorance.
    How many of us come across the caring professional who feels that he or she should be the custodian of the knowledge. How many of us have been subjected to the denial of information on the grounds that it might upset us. We might be affected in some way by HD but it does not mean that we need to be patronised - that some other person knows best. Of course information may be upsetting but being denied it will not make our problems go away. Professionals need to be aware whose problems they are trying to solve - their own or ours. Denial is said to be a feature of HD - whose denial is it?.
    There is no doubt that a huge change is occurring in public knowledge and the perception of hereditary disease. Attitudes are changing - we must hope that stigmatisation and shame will soon belong to the past. We must strive to ensure that new information is correctly interpreted by the people and organisations that have an effect on our lives. In our attempts to raise awareness to obtain better sevices, to alleviate suffering, to achieve a better understanding - in our attempts to achieve fairness and equity we must be aware of our responsibility to help protect one another. No one should suffer from unwelcome interest. No one should be subject to the unfair use of confidential information or from discrimination. No one should be expected to conform to other people’s expectations or suffer from stereotyping. We are all affected in some way by HD but we have the right to be treated as individuals with different aspirations, different ideals and beliefs.

Sue Watkins    August 1997