Maximising function and quality of life for people with Huntington's disease (HD)

Delivering holistic management of psychiatric, cognitive and motor deficits

At St Andrew's we have a deep understanding of the physical, emotional and psychological impact of living with Huntington's disease (HD)

What is Huntington's disease? Decoding the symptoms

Huntington's disease (HD) is a genetic neurological condition which affects the central nervous system and usually develops when individuals are in their thirties to fifties.  In the UK in 2016 there are an estimated 8,000 people living with HD. The effects of HD are wide ranging and change how people think, feel, speak, move, swallow and eat.

These effects can be broadly grouped into three key areas; Physical, Cognitive and Emotional. An understanding of the impact of the unique symptoms of HD are key to developing person-centred care.

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Physical

Physical symptoms include:

Greek for ‘dancing’, a movement disorder causing ‘jerky’ uncontrollable actions. The person may overcompensate for this with big bursts of movement.

Difficulty in swallowing and sometimes bringing food and drink back up, increasing the risk of choking. Speech and Language Therapists can recommend graded foods.

More often at latter stages making fine motor control, such as holding objects, difficult.

Changes in appearance caused by weakness in facial and body muscle tone may appear as boredom or anger, and poor posture may look like ‘attitude’.

Muscle deterioration in the throat means the person may switch between whispering and shouting for no obvious reason, making it difficult to gauge mood and needs.

Muscle deterioration can also change the tone and coherence of speech, and is sometimes mistaken for drunkenness.

An on-going challenge as changes in metabolism, combined with their constant movement, mean that people with HD burn more energy, so require a high-calorie diet.

Understandably a cause of distress, and requires support from urology teams.

Underactive immune protection makes the person more prone to illness, and requires careful management.

Cognitive

Cognitive disorders include:

A deteriorating ability to plan tasks and stick to schedules.

The person may become fixed on a task or activity, to the detriment of other things such as hygiene.

A preference for scheduled times for eating, bathing, dressing etc..., where changes can make the person obstinate. Inform and plan changes with the person.

Difficulty getting started with tasks and activities, such as maintaining hygiene.

Can affect short and long-term memory, requiring prompts.

Repeating the same questions and getting ‘stuck’ on a subject, make it difficult to move topics on.

It may appear that the person is oblivious to the mood of others, but they have difficulty reading other people’s faces.

It may take longer, sometimes minutes, for the person to respond to a question or request.

An inability to gauge the impact of their behaviour on others as the disease progresses.

Emotional

Emotional and psychological symptoms include:

Family may notice the person becoming moody and irritable more frequently and for longer periods of time.

Changes in the brain may make the person more impulsive in their speech and actions.

This is not because the person is selfish or impatient but a direct impact of the disease on the brain.

“I know exactly what I am saying… why can’t you understand me?” Difficulty relaying feelings, information and requests can lead to misunderstandings with carers.

Cognitive changes can cause frustration which can occasionally lead to temper outbursts.

People who have always been active may suddenly find it difficult to motivate themselves, and may just prefer to watch tv.

Common throughout all phases of the disease, making it important to liaise with specialist services, such as GP and psychiatry.

The person faces the constant re-realisation that their present, future, independence, family-life etc... will change.

Some people may develop obsessions (e.g around objects or people), and displace some of the social norms that govern our lives, such as undressing in public.

Whether self-imposed or as a result of the impact on friends and family.

"I used to have a great job and an active lifestyle. Just because I have HD doesn't mean I have forgotten to have fun."

Ian, living with HD

We deliver person-centred care for people with Huntington's disease

Our specialist teams focus on maximising functional ability and quality of life, across a dedicated HD pathway from secure to locked environments.

  • 1st to be awarded the RAID® Centre of Excellence

  • 10 months average length of stay for discharged patients (2011-2015)

  • 24 hours timetabled activity each week

  • 64% of discharged HD patients return to a lower level of security

 

"You need to give me time to answer, don't repeat what you said or put it another way as this makes it difficult for me to answer."

Person with HD

 

 

Click here for our latest That Makes Sense Now campaign in association with the Huntington's Disease Association.

To download a PDF copy of the booklet click here or on the front cover image.

Our services at a glance

We provide tailored pathways focusing on three core treatment components; neuropsychiatry, cognitive deficits and physical / motor deficits, including:

  • in-patient service for adults aged 18+ at any stage of HD
  • adapted environments and specialist equipment
  • admission for full and comprehensive time-limited assessments
  • highly specialised HD professionals form ward-based MDTs
  • specialist input including Dieticians, GP and podiatry
  • ability to manage complex and challenging behaviour
  • HD active care wards designed to maximise functional ability
  • gender specific neuro-palliation wards
  • therapies and activites to enhance patient quality of life
  • secure services for forensic admissions
  • dedicated social workers to support placement transitions
  • Harper HD unit voted 'Ward of the Year' 2014/15*

*St Andrew's Healthcare awards