15 minute read
HUNTINGTON'S
Shining a light
on Huntington’s Disease
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Huntington’s Disease (HD) is a genetic condition affecting thousands of people across the UK, with every child born to a parent with Huntington’s having a 50 per cent chance of inheriting it. But while research is underway across the world, there remains no cure and the profile and understanding of HD in wider society remains low.
Here, consultant clinical psychologist Dr Inga Stewart from specialist HD service provider St Andrew’s Healthcare, and forensic and clinical psychologist Dr Vincent Harding, share their expertise to explain more about the disease, its symptoms and how they manifest, how patients can be supported by clinicians and families alike, and why personcentred care is so important to enabling someone to be able to live well with HD
What symptoms could we routinely expect to see in someone living with HD?
Dr Inga Stewart (IS): If you think about
HD with the triad of cognition, movement, and also changes in mood, then you can imagine how HD can affect every aspect of somebody's life. It can have an impact on their levels of independence, but also their social relationships. And as the disease progresses, then this can become more profound.
Dr Vincent Harding (VH): The span from
the onset of symptoms tends to be around 15 to 20 years, however can be more or less. The onset of symptoms is most typically during middle age, between the ages of 30 and 50. There is a juvenile form of HD, which is when a person is affected before the age of 20, and that tends to progress more rapidly. The trajectory of HD tends to be quite long, which means we are able to work together to maximise functional independence and quality of life.
IS: I think it's important to note that although you have this collection of symptoms with HD, when you've met one person with HD, you've met ONE person with HD. There are commonalities, but everybody is individual. And so therefore, everybody needs to be treated as an individual. But there's always an acknowledgment that it's a progressive neurological condition, so it will get worse over time and those living with HD and supporting people with HD will need to adapt to change.
Is early intervention important?
IS: I would always advocate that people seek support as early as possible, because there's a combination of getting the right support in early but there's also the opportunity to think about your future and to think about advanced wishes that you might want to make, knowing that it's a progressive condition. It also enables you to consider the support that’s out there. We have organisations like the Huntington's Disease Association (HDA), which is a fantastic resource, because there's lots of information there and access to support.
VH: I think it's about having the right support at the right time, what is right for that person. There is no one approach or ‘one size fits all’. We do this holistically, in a person-centred way, by working together as a team with the person themselves at the heart of it. We're thinking about those things from day one, working collaboratively with the person with HD, and thinking, What's important to you? What are your goals? And what are your wants in life? How can we support you by working with you?
Is it possible to still have a fulfilling life
after an HD diagnosis?
IS: It’s very important to know that that you can live well with HD. If we make the right adaptations and provide the right support, then somebody can have a really fulfilling life.
VH: A big part of living well with HD is about planning for the future and being able to adapt to change with support. But that's no easy process in a lot of cases. It takes time and it's about adjustment to change. It can be quite a process for the person to be able to adapt to changes in their life and make reasonable adjustments so that they can still live well and have a good quality of life.
How important is a person-centred approach to HD care and support?
IS: Co-production is how we describe it, working with people as equal partners, and that is the approach we take. You've got the care team, the person living with HD, and the people in their lives - carers, family members - who are partners in care. We're always trying to work together. As one example, it could be about an advanced decision about PEG feeding (a PEG allows food and fluids to be put directly into the stomach through a flexible feeding tube). People with HD are at risk of developing dysphagia, which is difficulty swallowing. So on a day to day basis we’ll work with the speech and language therapist to support with communication, eating, drinking and swallowing, the dietician for food and nutrition choices, the occupational therapist might be looking at what equipment can help the person eat as independently as possible, their routine and learning ways to adapt to changes, the physiotherapist might be working on posture and positioning, and they’ll also be supported by the wider team. Psychologists will also be involved in looking at someone’s capacity to make an advance decision, and then we can support that person in co-creating their care plan for the future. We can support them in starting those conversations and looking at what their future may look like. We’ve worked with more than one person who has made the decision that they don't want to be PEG fed in the future. So working around that, we can create risk feeding care plans, which means that we support somebody to be able to eat the foods that they like in the safest way that they can. That is a decision for the future, but we're working with somebody in the moment to be able to do that.
VH: A big part of our role as psychologists is not just about direct work with the person with HD, but it’s the indirect and systemic work with the whole clinical team, providing regular spaces for reflective practice and formulation sessions. This gives us an opportunity as a team to ‘sense make’ of what we're seeing and how we're responding to it, and what the right approach looks like. But we also bear in mind that the right approach can and does change over time as we flex to meet people's changing needs.
What are the mental health challenges for someone with HD?
VH: People who come to us, often will have comorbid mental and physical health conditions alongside HD. So in terms of mental health conditions, that can often be anxiety, depression, sometimes symptoms of OCD, and sometimes psychosis. Again, it's making sense of that person's individual experience. We do that through a formulation-led approach, co-produced with the patient and their families and friends. It's almost putting the pieces of a puzzle together which then informs the evolving care and treatment plan.
IS: Somebody might have had traumatic experiences or difficult times that have not been directly related to symptoms of HD, so we need to give attention to that separately, but also consider how it fits in with the person’s whole experience. But the other factor is that HD is an inherited condition, many people living with HD have witnessed relatives already live and potentially die with HD. And so you can think about the impact that that might have on somebody's lived experience. Finding out that you have not inherited the condition is also not as simple as people might initially think. I think you learn to never make assumptions about how life might be for someone affected by HD. So here, you’ve got the psychological reaction to having a diagnosis or to being at risk of developing HD, which may cause further anxiety and depression, but you then also have the changes to the brain that are happening as a result of HD, which can also cause mental health challenges. So you have the mix of organic changes to the brain, and also the psychological experience, all mixed together.
How important is the Life Story approach that St Andrew’s use?
VH: I think it is very important. We use the Life Story Book as a form of narrative therapy. As soon as a person comes to us, we're having those discussions about the person's background, their life, their career, achievements and any special moments in their life. We use that to help contextualise that person, their experience and what's important to them, and get that down on paper. Because, as a team, the better understanding we all have of that person, the better we can support them. But we also need to be mindful that a person's needs and wants can and do change over time. So just because that is their history, we have to remain flexible in working with them. These are live stories, so works in progress. Wherever appropriate, we will involve that person's family and friends in sharing the story, so that we get the best possible understanding.
IS: We want to see the person first, not the HD. They are a person who happens to have HD. So if an individual's communication should be affected, or their memory, it means that doesn't need to negatively impact building relationships with other people in the future. So you might have somebody in the later stages of HD whose communication is very poor, and sometimes gets to a point where they can't communicate at all. Something like the Life Story Book then becomes an advocate for who they are as a person. If somebody is new, like a new staff member for example, they can look in the book and understand more about the person and their life. I think it’s our responsibility to hold and protect that person's identity, which includes how many sugars they like in their tea and what music they love. Really important things which you need to understand, and which the person is relying on other people to anticipate to be able to support them.
Working with people who may have behaviours that challenge is a key area of expertise at St Andrew’s, within the context of HD care how can that be best approached?
IS: Communication is a big part of it and is very important in ensuring that the person has a good quality of life and positive wellbeing. What we might see, for example, is that if somebody's communication is really affected, they might communicate their needs through behaviours that other people see as challenging. So, for example, if somebody gives you a big woolly jumper because it’s cold outside, they will no doubt think that’s the best thing to do. But if actually you get quite hot, but you can’t communicate that, you might physically resist having the jumper put on, and that could be interpreted as behaviour that challenges. Actually, all you're doing is communicating your need, which is ‘I get hot, and that great big jumper is going to be too hot for me’, so that's where you really need to know the person. It might be that we know that person tends to feel hot, and therefore doesn't need to be wearing a big woolly jumper. When communication is a challenge, and a person’s information processing is slowed, it’s important to pause after you've asked a question, and give them time to think about what you've asked and to provide an answer. You do have to wait, and it can feel like quite a socially awkward amount of time. For example, if you said to somebody; ‘Are you thirsty?’, and you don't get a response straightaway, you then might say, ‘Would you like a drink?…you can have a hot drink or a cold drink… you normally have a cup of tea, don't you? Would you like a cup of tea? Would you like two sugars? If the person says ‘Yes’, you then have no idea which question they’re answering. And that can be incredibly frustrating for them. You have to ask your question and then you have to wait, it could be for 60 seconds or more, but you have to wait. A big part of this is understanding who the person is, holding their identity and supporting them to feel like themselves. Supporting their personhood is crucial. The examples of the jumper and the tea are the kinds of things that might keep somebody in hospital if they are presenting with behaviours that challenge, but if you get the communication right, it can really help with their discharge.
VH: There is a phrase put forward by Jimmy Pollard, who is well known in the HD community, which is about being able to hurry up, but also to wait. So in terms of the hurry up, it's that we need to be able to anticipate somebody's wants and needs and meet them in a timely way to support them with their perhaps impaired ability to plan, problem solve and reason, to tolerate frustration and to engage in consequential thinking. But with that comes the need to wait. So when we ask a question or make a statement, we need to give that person the time they need to process and respond.
IS: Structure is also really important for someone with HD, but you need to have flex within that structured day. So you might have somebody, for example, with a brain injury who has a structured day, and it's really
important for their rehabilitation that they do the same thing each time, and that structure helps them learn what they need to re-learn. Whereas with HD, you want to have that structure, but if you're too rigid with it, you will cause distress and amplify cognitive impairment, rather than trying to minimise the impact of cognitive changes. Another factor is something we call the frontal lobe paradox. Here, it can be that through planning, the person can come up with a really great strategy of how to manage a situation in the future - but in the moment, their ability to action that plan was reduced, or disappeared entirely. They can ‘talk the talk’ but not ‘walk the walk’. Like the idea of ‘hurry up and wait’, it can feel contradictory to those supporting the person with HD. That’s where the understanding of that person and of how the condition manifests is so important, so we can support them as best we can. We use Positive Behaviour Support Plans with all of our patients, which in addition to the Life Story Books, helps to give the information the team need to support people in their lives. These plans are ever changing, depending on a person’s changing need, and we’ll do everything we can to co-produce these plans.
Is there enough awareness of HD?
IS: If you think that there are around 850,000 people with dementia within the UK, but less than 10,000 people with HD. So it's a really small proportion of people. But I think it has helped showing a character in Casualty living with HD, and we’ve seen more in the news recently, some headlines around the research. There are some big international research projects underway to look at halting the symptoms and progression of HD, and ultimately finding a cure. But when it comes to research, we see a lot looking at genetics and medication to treat and hopefully eventually cure HD. But we don't have a cure now, and we don’t have a lot of research into how you manage HD while you find that cure. So I think the psychosocial interventions as opposed to the pharmacological interventions are an important area where progress would be very valuable.
VH: The research field is growing, but there's still a need there. We need more in terms of the psychosocial experience of having HD so we can promote a person living well with HD. And again, what it means to them, and what support they require, not just in terms of pharmacological treatment which might manage some of the symptoms, but the experience of living with HD and how to live well with HD.
A loved one reaching the point where they need inpatient care must be a significant challenge for families?
VH: Wherever appropriate, and wherever agreed, we actively support families and friends to remain involved with the person at every step of the way. When we think about contact, we think about how often do they want to maintain contact? And what does that contact look like? So it's about giving people opportunities, choice and control, both for the person with HD, and for their family and friends.
IS: I think a contextual factor is the experience of being admitted to hospital. Some people can feel they want to be in hospital and feel safe and empowered by the fact they're getting specialist support, and other people don't want to be in hospital, they want to be back in the community with their families. This helps to show the need to support both them and their families through all of that. I imagine it would be really difficult for family members who might not want their relative to go to hospital, but know that they need to, and that must be very difficult to manage the opposing feelings. Somebody might feel a real sense of distress, but also a sense of relief all at the same time.
Does St Andrew’s play a role in bringing families together?
IS: At St Andrew’s, we’re very interested in creating support for family and friends and looking at how we can develop that. We’re a national resource, so do have people coming to us from across the country. For that reason, we do tend to signpost them to support within their local area, and of course the pandemic has posed problems as we haven’t had any family events during that period. But I do think the opportunities for virtual connections presented by the pandemic, to bring people together online, are exciting. That wouldn’t require everybody coming down to Northampton and could really help with peer support across the country. So that’s something we’re looking at.
To find out more about HD services at St Andrew’s visit: stah.org/HD
