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Huntington’s Disease Awareness Month: Insights from the expert



Huntington’s Disease Awareness Month: Insights from the expert

Huntington’s disease (HD) is a progressive neurological condition with no known cure.

The disease gradually changes an individual’s ability to walk, talk, think and communicate.

NR Times called up Dr Vincent Harding, forensic and clinical psychologist at St Andrew’s Healthcare, to learn more about HD and the innovative new approaches helping patients to live well, stay engaged with their care and meet the values that matter to them.

Hi Dr Harding. How do people develop HD?

HD is inherited from one or both parents, though the latter is quite rare. It affects both males and females and approximately 8,000 people in the UK have a diagnosis of HD.

If you have one parent with the faulty gene, you have a 50 per cent chance of developing HD at some point in your lifetime. If both parents have the faulty gene, that increases to a 75 per cent likelihood.

And in terms of onset that is normally between the ages of 30 and 50, that’s the standard variant. 

Less than 10 per cent of those affected with HD have juvenile HD, where symptoms present before the age of 20.

There are some differences in symptoms between adult and juvenile HD.

People with juvenile HD are more likely to experience involuntary movements and be affected by muscle contractions and rigidity, for example.

How and when is HD diagnosed?

Diagnosis is made at the point of onset of physical symptoms, and often they’re the most obvious, such as a slight, uncontrollable movement, stumbling, physical clumsiness and so on.

It could also be that the individual starts to have falls, for instance.

The cognitive and behavioural symptoms can often be much more subtle and they can come some years before years before physical symptoms and a diagnosis of HD.

We think of a triad of symptoms: cognitive, mood and behavioural and physical or motor symptoms. 

Cognitive symptoms could include difficulty with attention and concentration, planning and problem solving, organising and multi-tasking.

The person might have difficulty with memory recall but fare better with recognition memory. Processing speed can become slower over time and the person might perseverate or get stuck on a thought, behaviour or action. 

In terms of mood and behaviour, the person might have episodes of low mood or irritability. This can become more pronounced as HD progresses.

There may be difficulties in their relationships with friends or family or at work.

They might appear to act without thinking, presenting as more impulsive or disinhibited, seemingly without anticipation of consequence to themselves and/or others.

This is driven by changes to the brain, not an active choice of the person themselves. 

People with HD are also more prone to psychiatric symptoms including anxiety and depression, and some go on to develop OCD or psychosis.

I imagine that there’s no set path when it comes to the progression of the disease. But what is the average life expectancy for HD?

Generally speaking, from symptom onset to death is usually between 10 and 20 years. But that can and does vary from person to person.

Some people can live much longer than that while others will live for less than 10 years.

Many who live longer will go on to develop multiple cognitive impairments and may receive a diagnosis of dementia alongside their HD.

The longer a person lives with HD, the greater the likelihood there is of this happening.

HD is a whole-person condition. It affects the way a person thinks, feels, behaves, speaks, moves, swallows and eats.

How does HD impact the individual’s relationships and life choices?

HD affects individuals differently, depending on their set of circumstances.

People can experience practical and emotional difficulties. The nature of their relationships and roles within them can change as their abilities and needs change.

HD changes the way a person thinks, it can alter their personality and their behaviours. 

That can be difficult, particularly for family members because social relationships and support needs change as the disease progresses.

The person may also be contending with decisions about whether to whether to take a genetic test, whether to start a family for instance. 

And as the disease progresses, there are questions about advanced directives.

Does the person want to be resuscitated? Do they want to PEG feed if they can no longer eat safely and effectively?

It’s helpful to start those discussions early when the person has the capacity and ability to engage and make those decisions.

What can organisations like St Andrew’s do to help people with HD live better?

One of the big pieces of work we do with people and their families is psychoeducation. 

The person may already have a good understanding of HD because they’ve seen it first-hand in their family.

But even then, they might not relate to those symptoms or have an understanding of how they could live with them.

We start by identifying what’s important to the person: What they like, what they dislike and what their goals are.

Our focus is on person-centred care.

To do that, we need to co-produce their care plans with them, should they want to, and actively look for ways to support people to overcome any barriers to participation.

It’s also important to establish whether the individual is agreeable to including their families and friends as equal partners in their care. 

We work together with the person in our care, their partners-in-care and the clinical team, using that triangle of care to achieve the best outcomes and experiences for all. 

This allows us to learn from each other to have the best possible understanding of that person that we can, so we can help them live well.

There is so much that we can do to support improved wellbeing and quality of life.

How do you tailor care?

The interventions are tailored to what the person wants and needs and what’s likely to benefit them most.

For some, it may be acceptance and adjustment work to help them live better with HD and the mental health impact it can have.

We also take into account the individual’s values and look at how they can continue to meet those values, but perhaps in a different way as the disease progresses.

Again, we work with the person in our care, their partners-in-care and the clinical team to make sense of their experiences and needs, helping us to shape the intervention.

Meaningful engagement and occupation is really important. People are often fiercely independent and they want to continue doing things for as long as they possibly can.

When they can no longer do these things, we have to be creative, flexible and innovative to help them find another way to meet the same values.

For example, someone who has a passion for cycling may not be able to continue doing so as the disease progresses and their coordination becomes more impaired.

Here, we would work with the person to establish what it is that cycling brings them and look at finding new ways to meet that need.

It could be that the outdoors gives them a sense of freedom and independence. 

Working with the person and our occupational therapists and physiotherapists, we might look at using mobility aids to enable the person to meet that need but in a safer way.

What do you see as the most promising treatments? How are you applying these at St Andrew’s today?

There is a long way to go in terms of finding a cure. 

But in the meantime, there’s a lot of work that’s being done and we’re making great leaps with the help of technology.

Voice banking is already making a huge difference at St Andrew’s. This technology allows us to capture the patient’s voice, preserving that aspect of their individual identity.

This means that when the disease progresses, they can continue to talk using a smartphone or iPad, with their own voice, for the rest of their lives.

That allows them to continue sharing their voice and what’s important to them so we can continue to work with and learn from them.

And equally, John’s Campaign, which is again about working closely with the person in our care, their families and our clinical team to provide opportunities for that joined up working.

There’s no obligation but the opportunity should be there.

It’s about being more explicit in our offering and creating opportunities for people to work more closely with each other, learn from one another and improve experiences and outcomes for everyone.

HD changes the way a person thinks, it changes their personality and it changes their behaviours. 

That can be difficult, particularly for family members because social relationships and support needs change as the disease progresses.

It’s important that we establish and maintain that joined-up approach so we can provide the consistency, familiarity and predictability to enable them to live well – whatever that means for the individual.

It all comes back to identifying what’s important to the person and thinking about how we, alongside family and friends, can provide the wraparound support they need to help them reach their goals.

Find out more about St Andrew’s’ Huntington’s provision here.