The hidden challenges of Huntington’s disease:

UK mental health charity and hospital, St Andrew’s Healthcare, has been supporting the Huntington Disease Association's (HDA) campaign to raise awareness of the condition. Speaking to NR Times, the team discussed how the charity is using an enhanced approach to Huntington’s disease care that incorporates multidisciplinary collaboration to support mental and physical health.

Huntington’s is a rare, neurodegenerative disease which affects movement, mental health and cognition. A recent HDA survey found that 85 per cent of people within the Huntington’s community had to educate a doctor, nurse, or other healthcare professional about the condition, highlighting an urgent need for increased awareness.

With Huntington’s affecting every individual differently, those living with the condition often face their own unique challenges, requiring personalised, mindful and multifaceted approaches towards their care.

A hallmark of Huntington’s is chorea - involuntary movements that can affect all the muscles of the body. Huntington’s can also affect the ability to make voluntary movements. Movement disorders can often lead individuals to experience falls, requiring the need for additional support or intervention.

While this support is vital to help improve the safety of an individual living with the condition, it can also impact a person’s independence.

With this in mind, St Andrew’s Healthcare has been trialling an enhanced, formulation-led multidisciplinary approach to support an individual with Huntington’s who had been experiencing increased falls.

Integrating Acceptance and Commitment Therapy (ACT) and utilising multidisciplinary collaboration and co-production with the person, the intervention aimed to balance their desire for independence with staff’s need to maintain the person’s safety.

“The traditional approach to care within mental health provision is paternalistic, and as a result things have primarily been ‘done to’ patients using services rather than ‘done with’ them” explains Dr. Inga Stewart, Consultant Clinical Psychologist and Head of Progressive Neurological Conditions Research Programme at St Andrew’s.

“We have known for a long time – and it's even mandated within the Care Act – that we need to co-produce care with the person and consider them as an expert on themselves with valuable knowledge to contribute, however, how we do that is a bigger question and can be really challenging in practice, particularly within the healthcare system as it is currently set up.

“The traditional way that you would approach a risky situation would be to make a falls prevention plan as a multidisciplinary team and then to implement it, and as part of that to hopefully have a conversation with the person about what the intervention is and why you are doing it.

"Someone repeatedly falling would be seen as needing a physical health intervention, and it would be something that primarily physiotherapy and occupational therapy would look at.

“This situation wasn't that simple, because we were working with somebody who was aware that they were falling and had an understanding that the falls were linked to their Huntington's - they understood that we needed to use these interventions – but when it came to the ‘here and now’ in the moment when they were potentially about to fall, they were often unable to accept the intervention.

“One way we conceptualised this was to think of the "frontal lobe paradox". This describes a phenomenon where what a person demonstrates in a controlled setting – such as a care planning session – and their actual abilities in real-world situation – such as when they are about to fall – are different.

"This disconnect means that clinicians might overestimate a person’s ability and fail to provide the support they need. Consequently, we weren't able to implement the plan in the way that we wanted, so we needed to take a new approach.”

The psychology team’s approach was multifaceted. With the Falls Prevention Lead, they worked with both the individual and the wider multidisciplinary team to understand the formulation. Together, they realised that there was often a mismatch between the aims of the staff who wanted to keep the person safe from falls, and the person who just wanted to be independent.

To help assess the falls problem, they used a dashboard to track and understand the context of the falls, like when and where it happened, what was happening before and how they fell.

ACT helps people develop ‘psychological flexibility’ – the ability to accept difficult emotions while committing to meaningful actions – and they used this with the person and the team to understand why the falls plan had not always worked. They then worked together to adapt the care plans to respect the individual’s autonomy while maintaining safety as far as possible.

One of the interventions implemented after looking at the data was to encourage the individual to rest after going out on leave, and therefore reduce the risk of falls due to fatigue. But prompting them to sit and rest was often experienced as interfering and frustrating, so guided by the formulation the team instead encouraged the person to engage in sociable seated activities they enjoyed - such as having a cup of tea and chatting with staff, or having their nails painted.

Carrying out the intervention in this way helped the individual rest while maintaining a sense of autonomy and independence. The new approach was a start, but to be more successful in reducing the number of falls they had to take the intervention further.

“We co-produced a care plan with the person around how they would like staff to approach them to offer support in order to effectively decrease their falls,” said Sahana Mehta, a Trainee Clinical Associate Psychologist studying through the University of Exeter at St Andrew’s, who implemented the intervention.

“It also helped the individual to understand where the staff were coming from – that they just wanted to help and keep them safe.

“The top line was that they would ask for help if they needed it, but that they also wanted to be independent and wanted to keep walking - it was important that it was a care plan written in their own words so that they felt heard.

“The Falls Prevention Lead joined some reflective practice sessions with the ward staff and the Physiotherapist also got involved in talking about the benefits of supporting people with Huntington’s to keep moving and walking as long as possible to help maintain their mobility – this itself reduces the risk of falling.”

The approach was successful – helping to reduce the number of falls for the individual.

“It had become clear that psychological inflexibility was a factor for both the individual and the team – the individual wanted to be independent and the team wanted to prevent falling – and that the intervention therefore supported everyone to meet in the middle.”

Mehta highlights that there are currently no NICE Guidelines for Huntington’s in relation to the use of ACT, but that the therapy is well established for treating anxiety and depression, substance misuse, and in physical health care and chronic conditions.

It is starting to be considered in individual therapy for people affected by Huntington’s but not as a systemic approach with inpatients and ward teams.

“Huntington’s is a progressive neurological condition – sadly it's not going to get better.

"As it advances then people naturally become more dependent on help from others. So it's about two stages - acceptance and commitment. It's thinking about how to reframe and find the value in living a life as best as you can,” says Mehta.

“Then there's the commitment side, where you have to be committed to making those kinds of changes.”

Stewart adds: “We are working with the whole system, which includes the staff and the individual – instead of focusing just on the individual. It can be difficult for staff when they are caring for someone who is increasingly likely to fall.

"You can see how they want to do anything they can to prevent it. Then when you think about Huntington's and the cognitive impairment that comes with that, there can be a mental inflexibility and change is often difficult.

“Information processing can also be slowed, and these cognitive changes can make even the best planned interventions more tricky - if you put those alongside the physical changes that are happening due to Huntington’s there is this greater risk of falls. So the intervention has to be adapted to meet the needs of that person and to consider the context of the staff supporting them.”

The intervention itself allows for flexibility - and the team say that as the individual’s needs change, the intervention can be adapted to suit these changes.

“This is the first time that we've used ACT explicitly in this way with the whole multidisciplinary team,” explains Stewart.

“What this is about is a physical health approach being formulation led - what Sahana has done is use this formulation to find a shared middle ground – the shared narrative that brings these two really valid, important motivations together – between keeping the individual safe and respecting their wish to remain independent, all while also considering the cognitive changes that happen within Huntington's that may make it harder for the individual to be able to manage the help offered in the moment.

“With options such as using walkers or wheelchairs, we know that could reduce the likelihood of somebody falling if they can use the equipment safely, but we also know from our specialists that if someone isn't staying physically active and using their muscles involved in balance and co-ordination, they'll get muscle wastage and lose strength and stability - so they're then more likely to fall.

Mehta adds: “We may not be able to write a care plan in the direct words of all of our patients as they might not be cognitively able to do that - so, we might do a similar intervention using the same framework, but working with their family or friends as ‘partners-in-care’ and by learning from what we observe when working with the person.

“This way, we can still co-produce a care plan, but it might be done more indirectly.

“This formulation-led, multidisciplinary approach is something that we are both passionate about within the service and is something we will continue to do more of.”

If you would like to find out more about St Andrew’s neurobehavioural services you can contact Rob Walsh, Service Development Manager:

T: 01604 616592 / 07714 600699
E: ​robert.walsh@nhs.net

Or visit the website: Neuropsychiatry services - Huntington's Disease (HD) » St Andrew's Healthcare