FOI 24/25-0367 | DOCUMENT 12

Huntington’s Disease Disability Snapshot

SGP KP Publishing

Exported on 2024-10-18 03:12:32

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

1 Peak body consulted …………………………………………………………………………………………….. 4
2 What is Huntington’s Disease? ……………………………………………………………………………… 5
3 How is Huntington’s disease (HD) diagnosed? ………………………………………………………. 6
4 Communicating and supporting people with HD …………………………………………………….. 7
5 Language and terminology ……………………………………………………………………………………. 8
6 Enabling social and economic participation …………………………………………………………… 9
7 How can I tailor a meeting to suit a participant with Huntington’s disease? ………….. 10
8 What people with Huntington’s disease want you to keep in mind ………………………… 11
9 Helpful links ………………………………………………………………………………………………………… 12

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

This Disability Snapshot provides general information about Huntington’s disease to help you to communicate effectively and support the participant in a planning meeting. Each person living with Huntington’s disease is an individual with their own needs, preferences and experiences. This information has been prepared for NDIA staff and partners and is not intended for external distribution.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

1 Peak body consulted

In developing this resource, we consulted the Consortium of Australian Huntington’s Associations.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

2 What is Huntington’s Disease?

Huntington’s disease (HD) is a rare, hereditary, chronic, neuro-degenerative neuromuscular disease with no cure. However, there are treatments and strategies to minimise the impact of the disease. Signs and symptoms often begin to appear when people are in their 30s and 40s.

SIGNS are changes as noted by others.
SYMPTOMS are changes noticed by the HD affected person.

Common early signs and symptoms include:

loss of fine motor coordination
muscle stiffness
involuntary body movements (often pronounced) known as chorea
cognitive or behavioural changes (for example, memory loss, mood swings, trouble with concentration, difficulty in making decisions, and behaving impulsively).

Not all people with HD will have the same changes, even those from the same family.

Huntington’s disease will progressively affect how the person functions each day, and will change their everyday needs. Eventually, HD will significantly impact a person’s ability to make rational decisions, walk safely, speak clearly and swallow normal food safely.

Most people with HD have a life expectancy of 10 – 20 years after measurable changes begin, but they have usually had signs and symptoms for much longer.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

3 How is Huntington’s disease (HD) diagnosed?

Huntington’s disease is often diagnosed when a person with a family history experiences mild changes in their functioning. Their GP will usually organise a general physical and neurological exam, and should refer them to a Huntington’s disease specialist clinic if one is accessible. They will likely recommend an MRI scan of the brain.

A genetic test will confirm the diagnosis, usually following genetic counselling for the family in a genetics clinic.

It is important to understand that while most people with HD are aware of a family history of the disease, this is not true for everyone. The family history issue needs a sensitive, tactful approach.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

4 Communicating and supporting people with HD

People with HD may struggle with communication because they become less aware of other people. They may also experience psychological symptoms such as anxiety, depression and personality changes.

To successfully communicate with people with HD, it’s crucial to understand that they may not be aware of their own physical and cognitive changes.

The progress of the disease varies between individuals with HD, and over time for the same person, so a flexible, individualised care plan will work best. The changes tend to be gradual. However, if a sudden change in ability is noticed, professional help may be needed to treat any reversible conditions such as infection, or to understand and manage what may be triggering behavioural responses.

The participant’s plan may need to be reassessed often, and there may be frequent changes in how they manage to do things.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

5 Language and terminology

You should use language which reflects the person first, for example “person living with Huntington’s disease”. Some of the language used when talking about Huntington’s disease is explained below.

Chorea: involuntary, dance-like movements – the person may be unaware they have chorea even when it’s obvious to others
Hereditary: passed on from one family generation to the next
Dominant: one mutated copy of a gene is enough to cause disease
Neurodegenerative: a condition of the nervous system that will continue to get worse over time.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

You’re best able to support a participant with HD when you know what challenges and barriers to social and economic participation they’re likely to face.

For example, a participant with HD could experience challenges in their personal relationships because of the impacts of the disease. They will gradually lose their physical independence and often experience psychological and/or behavioural symptoms which can place a strain on their relationships.

Counselling or other types of psychological support may help them and their carers better cope with their changing life circumstances. Arranging appropriate social activities, especially outings, can make a big difference to their quality of life.

Although the cognitive impacts of HD may be subtle, they can affect the participant’s ability to communicate their needs clearly during a planning meeting. Keep this in mind when talking to the participant and/or their carer.

It’s important to include support coordination for the participant to implement their plan. For example, because of the impact of HD the participant may find it difficult to communicate with their service providers. Having a support coordinator to help them will make sure they keep getting the supports they need. If the participant experiences a behavioural crisis, the support co-ordinator may be able to adjust the supports the participants needs instead of needing a change to the participants plan.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

7 How can I tailor a meeting to suit a participant with Huntington’s disease?

It’s important to remember that Huntington’s disease is a degenerative disease and the participant’s needs are likely to change over time. Make sure to check with the participant and their carer for any adjustments they might need for the meeting beforehand. It’s important to check with both a family member or carer as well as the participant to gain a full understanding of their symptoms, diagnosis or problems.

While sensitivity is needed when discussing future support needs, it’s important to be realistic about the participant’s growing and potentially unpredictable need for support as their Huntington’s disease progresses.

You should consider supports that will:

enable the participant to make the most of their independence, and keep them safe for as long as possible
help maintain the participant’s social and economic participation
help maintain their relationships.

You should also ensure;

there is funding in the plan for specialist training in providing support to participants with HD as many service providers often have no background or experience with HD.

Funding in the plan may include things like assistive technology or psychological support. It’s important to listen to the participant as well as their carers to make sure you get all the information you need to make your decisions.

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SGP KP Publishing – Huntington’s Disease Disability Snapshot

8 What people with Huntington’s disease want you to keep in mind

In building a successful relationship with a person with HD, it’s very important to convey a strong sense of respect, and be direct and simple in your choice of words. Always request feedback to check they have got the message you wish to convey, without hurrying the person as they may take much longer than expected to formulate their response.

Huntington’s disease affects everyone differently, even within the same family. Keep this in mind when supporting multiple participants from the same family.

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9 Helpful links

[Huntington’s Australia](Huntington’s Australia)
NSW Huntington Outreach Service

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