Bigger picture presentation

Editor's Notes

• #3: Good morning… my name is Errol Walsh and as you can see I’m responsible for Member Services and Development in HDANI and today I want to look briefly at the impact of HD on the wholefamily… and particularly on young people. and how they interact (or not) with other family members.
• #4: Most people here, if you’re working with HD clients or families, will already know some of the basic facts about Huntington’s so this is the briefest of brief outlines.However, if this is your first contact with HD then I’d like to refer you straight away to our Nurse Specialist MajellaMcConville who will be your most knowledgeable reference point for any queries you may have today or in the future. You can also contact me if you have any questions about my presentation today. So, for the purposes of this presentation about the impact on the wider family, and particularly young people, the last two points in this slide are the important ones…..“Because the damage caused by HD is only inside the brain, the person with HD may look relatively able bodied until the later stages of the disease..”:More and more the scientific research points to the fact that brain cells may begin to die anything up to 20 years before physical symptoms become apparent… this is extremely significant if you think that the average recognisable onset occurs between 35-50. We’re now talking about potential brain cell deterioration from quite an early age.Most of those unseen early symptoms can be very subtle changes in behaviour, cognition and judgement… changes that may easily go un-noticed among the many ‘normal’ developmental stages of a young person…. So, if he/she inherits the gene, a young person could be dealing with HD long before an actual diagnosis or physical onset “Children who have one parent with Huntington’s disease have a 50% chance of inheriting the faulty gene”This creates a huge psychological burden for young people who may be faced with the milestone of genetic testing at eighteen years of age.Some young people grow up with this knowledge and adapt gradually to the information. Some grow up unaware of their genetic risk… Some, because there is no family history of HD, learn about it suddenly following a parent’s diagnosis. As a symptomatic parent grows more unwell and needs more support, changes in their behaviour and family life becomes more focused on the needs of that adult, and then, all too frequently, young people may have to move into a caregiving role.
• #5: So… every individual in the family, whether or not they inherit the gene, will be impacted to different degrees by it’s incidence in the family. They may become patients themselves…. or carers… they may suffer guilt at having been spared the genetic inheritance….. or they may suffer from the stigma that has traditionally been associated with HD…Until recent times it has been a shameful secret in many families… seldom, if ever, mentioned or discussed.
• #6: This slide very briefly addresses two of our strongest emotions: ----- Guilt and Fear…Parents often struggle with feelings of guilt about whether they may have passed the gene on to their children. They may wish to talk to them about Huntington’s disease, but be fearful of causing harm by saying the wrong thing. Some parents struggle with knowing at what age to tell children/ young people…… or they worry about the impact that telling an older sibling may have upon younger ones. Symptomatic parents, especially if family breakdown has occurred, may be very fearful of losing contact with their children…. while unaffected parents may harbour concerns about the impact the disease is having on children in the family. Even in families where the ‘at risk’ parent has received a negative test result they may have worries about wider family members (aunts, uncles, cousins etc)Nowadays, information…. and often inaccurate or frightening information…. is readily available to young people via the internet and social media… so it’s vitally important that they learn things from an informed and empathetic source…. And… at their own pace. So its vitally important that everyone working with an HD family becomes aware of these potential hazards and with the emotional impact that this may have on young children and young adults… as well as the wider family… It’s essential to be able to see the ‘Bigger Picture’. 
• #7: Huntington’s disease creates unique challenges for families and for the young people who live in them…………. there is now a considerable body of evidence worldwide to support this and I’d like to focus, particularly, this morning on the considerable challenges faced by young people in HD families.At an Open Weekend hosted by HDANI in Fermanagh earlier this year, (part of a project funded by the Public Health Agency) we consulted extensively with young people, and their parents, about their experiences of living with Huntington’s disease in order to explore and understand it’s wider impact on family life. From the evidence gathered it is clear that young people living with Huntington’s disease experience a number of common challenges…. Such as: Family breakdown:Family life can be complex at the best of times… but where there has been family breakdown e.g lone parent families, or families where there are step parents and siblings…… the impact of Huntington’s disease may be greatly magnified… or made much more complicated.Young caregivers. Many young people in HD families are…. or will become, through necessity, young caregivers… with all the challenges that that brings … a few of which we’ll look at briefly now. My own grandson Leon became, in a very real sense, the main carer for his mother at age 8! His mother, Rachael (my step-daughter) was a single mother after her relationship broke down… but, as long as she could possibly manage she was fiercely independent….. and remained so until she eventually recognised that her illness was having a very detrimental impact on Leon’s development.Isolation ,Secrecy & Stigma are, of course, closely linked:Young caregivers will inevitably have less free time to themselves…. and often become much more isolated from their peers.  The stigma and fear that may surround Huntington’s disease…. …. being a caregiver…. the family breakdown … problems with attachment formation with symptomatic parents….. secrecy and shame…… difficulties for families in communicating about the illness…. All of these are significant contributing factors to a very real sense of isolation for all family members….. but particularly for young people who may be struggling with peer pressureFor example, some young people can become isolated within their peer group and the wider community, because the behavior… or physical changes caused by the disease… and reflected in affected family members… often attract negative reactions and stereotyping. If a young person lives in a lone parent family where parenting capacity has become an issue.. (as happened with my grandson).. obvious changes, like going to school in dishevelled clothes, or frequent lateness can quickly mark a child out as ‘different’.Information Needs: There is often a lack of accurate , age appropriate information.. especially if HD is a taboo subject at home. So young people may have very different information needs. Those growing up in families with a long generational history of the illness are likely to know more, but it is still the case that misinformation can be transmitted within the family. Those who grow up in a family where there is no family history have far greater information needs.  Disadvantage and lack of opportunity: This can significantly impact the quality of life of young people living with Huntington’s disease…. The long-term nature of Huntington’s disease…. and the fact that partners and spouses frequently have to give up work to take on a caring role, means that a high percentage of families living with the illness…. (including young carers)… may experience significant levels of poverty.It is now widely recognised that many young carers have poorer experiences of school…. achieve a lower level of education…. and are more likely to be unemployed…. especially if they live in families that experience greater financial restraints.As young people make the transition to young adulthood… they face further disadvantage in that their career choices may be impacted by their genetic status…. Some organisations may not be willing to employ a young person with a 50% risk of inheriting Huntington’s disease…. This is generally true of the Police Force and the Armed Services. Obtaining life insurance can be a complex and difficult process too.Increased stress, anxiety and emotional pressure:Young people frequently have to cope with greater levels of stress, anxiety and emotional pressure at home, at school…. and in their peer group…. and often have to juggle multiple caring responsibilities which are not shared by their peers. The Future: A unique and cruel feature of Huntington’s disease is that young people…. particularly those in a caring role… can vividly see what the future might visit upon their own health if they have inherited the gene that causes Huntington’s disease.Serial loss and Bereavement:Young people in a caregiving role are caring for an adult (or sibling) who is deteriorating over time, whose level of function is constantly changing… and who will die from the condition…. making anticipated loss.. and actual loss a constant feature of family life. Finally…. Genetic testing: You will be aware that there is an accurate predictive test for Huntington’s disease (a simple blood test) which is made available to young adults at age 18.Deciding whether or not take the genetic test is an intensely personal and complex decision shaped by personal beliefs…. aspirations… emotional maturity… sibling choices about testing.. and family history of Huntington’s disease. There are many significant and long lasting implications for test results whether those results are positive or negative. This is a very complex and challenging subject which, unfortunately, we won’t have time to discuss at length here today. 
• #8: Growing up in an HD family is not unlike walking a tightrope for your whole life!Genetic Risk: Of course, in terms of risk, there is the huge and constant looming shadow of a young person’s own genetic risk….. and , as I mentioned earlier… young people constantly, and acutely, feel the psychological burden of living with that genetic risk…. and they also worry about parents, siblings and other extended family members. How young people learn about their risk… who tells them… and when… has a significant impact on how they go on to cope with that information. It’s generally accepted that young people want to be told about Huntington’s disease… but where families have difficulty communicating… or where knowledge of it is a secret… young people may feel very isolated… struggle with misinformation… or create ‘fantasies’ about what is really going on in their family. It is a constant shadow that hangs over every aspect of their lives.There are, of course, other mental, emotional and physical risks:The individual impact of the isolation and disadvantage faced by young people can often.. and easily be seen in terms of poor mental health… low levels of confidence.. and low self-esteem. Young people may experience depression anxiety… and may engage in self-harming behaviour… or even have suicidal ideation They may also be at risk of physical and emotional harm at home…. because the cognitive impact of Huntington’s disease can significantly reduce parenting capacity. A parent’s organisational skills are frequently an early casualty of the illness making them much less able to plan for the needs of their children. These problems may be worsened by a characteristic loss of empathy (a direct result of brain changes caused by the disease) which may impair a parent’s ability to recognise or understand the needs of their child. Common behavioural changes caused by the illness may lead to loss of temper control… or very rigid , or apparently irrational, behavior… which can also be distressing for children and can lead to physical harm. The experience of some of our young people who have attended specialist HD summer camps in Scotland and Europe over the past 5 years… identified many of these issues in workshops during their time there….This evidence clearly highlights the need for more attention to be paid to the physical, mental and emotional development of young people as they grow up in HD families.  It’s worth mentioning that, young people are often much more capable than we give them credit for… and we can.. and should, support and encourage them in creating their own support networks… on their own terms… with their own aims, objectives and solutions. The “generation gap” is real! I can’t claim to understand young people any more than I understood my own parents’ generation… but I remember how frustrating that was… and where or when I was unsupported and patronised… I rebelled!... and then some! So, we need to respect young people and support them wherever possible.  
• #9: I’d like to recommend that everyone here today, especially if you’re dealing with families where children and young adults are involved, explore in depth the website illustrated on this slide.This is the work of a committed group of young adults, living with HD, from around the world… who reached out to their own generation because they recognised a significant and urgent need for accurate and age appropriate information and problem sharing among young people…. and access to accurate and valuable information for parents and professionals too. It is a great forum for young people of all ages to share concerns and information on HD (see tabs)…. HDANI has been very fortunate to have had the opportunity of working closely with some of the founders of HDYO to explore the value of a youth led, HD network here in N. Ireland …which will not only help our young people to support each other… but help to bridge some of the barriers that exist between them and their parents. The board of HDYO take their responsibility very seriously and so the accuracy of the information they carry is overseen and checked by experts before being uploaded onto the site.
• #10: Finally:The Scottish Huntington’s Association’s experience and perspective after nine years of working with people living with Huntington’s disease (including many of our own young people from N. Ireland), has highlighted this crucial message. “Huntington’s disease is a family disease, and only an approach which recognises and embraces the needs of whole families, can succeed in adequately enabling them to meet the extraordinary challenges that confront them”So please remember… especially when dealing with young people… always be aware of the Bigger Picture!If anyone wants a copy of this presentation, or Ed Wild’s research video please know and I’ll email them to you.