At ORS, we’re passionate about delivering high-quality Positive Behaviour Support to both NDIS and private participants from all walks of life. Our team of skilled and dedicated Positive Behaviour Support practitioners are committed to continuous professional growth, seeking opportunities to upskill and stay informed on the latest research.
This ongoing learning allows us to design and implement Behaviour Support Plans (BSPs) that are not only more effective but also person-centred, ensuring that every individual we work with receives the best possible care tailored to their unique needs.
Recently, two of our fantastic Behaviour Support Practitioners, Katie Southwell and Shelby Lee, attended a one-day conference and workshop at Westmead Hospital in Sydney, where they deepened their knowledge about Huntington’s Disease (HD). Huntington’s Disease is a genetic condition that impacts a person’s movement, mood, thinking, and behaviour.
In Australia, more than 2,000 people live with this incurable disease. This opportunity for Katie and Shelby was not just about expanding their expertise in managing behaviours of concern but also about understanding how this complex condition affects participants on a physical and emotional level.
Understanding Huntington's Disease (HD)
Huntington’s Disease is a hereditary neurodegenerative disease passed down through families. The disease is caused by a mutation in the “huntingtin” gene.
Everyone has the huntingtin gene, however, those who inherit the mutation in the gene will develop HD. The Huntington gene then leads to the production of a protein known as huntingtin protein. This protein gradually damages specific brain cells and areas, which control movement, mood, and cognition.
Genetic testing can identify whether someone carries the mutated gene, and those who have parents with HD have a 50% chance of developing HD. If parents do not carry the mutated gene, then their child cannot inherit the mutated gene. People can get genetic counselling if they wish to know their gene status. It equally impacts all genders.
Typically, HD manifests between the ages of 30 and 50, although a small amount of juvenile cases have been observed. Currently there is no cure or treatment to slow or stop the progression of HD, but symptom management can greatly improve a person’s quality of life.
One of the first signs of HD is often cognitive decline, which might appear as difficulty with planning, organising, processing information, or using judgement. These changes in thinking are frequently followed by shifts in mood and behaviour. Many people with HD experience irritability, depression, and other emotional symptoms.
Additionally, as the disease progresses, motor symptoms such as involuntary movements (known as chorea) begin to emerge. These movements can interfere with everyday tasks and cause weight loss. Some medications, such as atypical antipsychotics may reduce the severity of chorea.
Supporting Individuals with Huntington's Disease
Supporting someone with HD requires a holistic and person-centred approach. Due to the progressive nature of the disease, individuals often need help managing their symptoms and completing daily activities. Communication becomes a significant challenge, as people with HD may have trouble verbalising their needs, processing verbal information, or engaging in conversation.
In Positive Behaviour Support, our goal is to identify what behaviour is communicating and find ways to support functional communication so that need is met. This is especially crucial in cases where verbal skills are compromised. By collaborating with Speech Pathologists, we can teach individuals alternative communication strategies, such as using nonverbal cues or assistive technologies, to ensure they are heard and understood.
In addition to communication, individuals with HD may need help with daily routines. Our Positive Behaviour Support team often works alongside Occupational Therapists to break down complex tasks into smaller, more manageable steps. For someone with HD, maintaining independence is vital to their sense of identity, and having structured, achievable goals can help foster a sense of accomplishment. Implementing routine schedules that avoid overstimulation or fatigue is also critical, as stress can exacerbate symptoms like chorea or mood disturbances.
The environment in which a person with HD lives can significantly impact their behaviour. Creating a calm, predictable setting can reduce anxiety and prevent overstimulation. By recommending environmental modifications—such as reducing noise levels, minimising clutter, and ensuring spaces are safe and accessible—we aim to enhance the individual’s quality of life.
Supporting Caregivers and Families
Caring for someone with Huntington’s Disease is an emotionally and physically demanding role, and caregivers, often family members, need support just as much as the person with HD.
At ORS, we believe that empowering families through education and coaching is a key part of effective Positive Behaviour Support. Our team works with caregivers to help them understand the behavioural symptoms of HD, recognise triggers, and develop strategies for managing difficult situations. This may involve learning how to de-escalate crises, redirect attention during moments of distress, or create a more structured daily routine.
Caregiving can be an isolating experience, particularly when dealing with the unpredictable nature of HD symptoms. Many caregivers face the emotional challenges of knowing that HD is genetic, which means they may be caring for multiple family members at different stages of the disease, or they may be grappling with the possibility that they or their children could develop the condition in the future. Providing caregivers with psychological support, resources, and opportunities for respite is critical to preventing burnout and ensuring they can continue to provide the best care possible.
How the ORS Positive Behaviour Support Team Can Help
At ORS, we’re dedicated to improving the lives of individuals and families affected by Huntington’s Disease. Our Positive Behaviour Support team at ORS utilises evidence-based practices to deliver person-centred support that is responsive to the evolving needs of each individual. We work within a person’s support network, collaborating closely with families, caregivers, and other professionals to ensure a coordinated approach. Our comprehensive functional behaviour assessments help us understand the factors influencing behaviour, allowing us to develop tailored strategies that promote positive outcomes.
In addition to addressing behavioural concerns, we assist in collecting meaningful data about an individual’s daily functioning. This information can be shared with medical teams to ensure that healthcare providers have a clear understanding of the person’s progress and needs. We also work to evaluate the effectiveness of environmental and communication strategies, adjusting plans as needed to ensure ongoing support.
As our understanding of Huntington’s Disease continues to grow, so does our commitment to learning and improving our practice. By providing compassionate, evidence-based support, we help our participants, and their families navigate the complexities of HD with greater confidence and hope.
For more information about our services, please feel free to call us at 1800 000 677 or click here to refer!
Together, we can provide the support and care needed to navigate the challenges of Huntington’s Disease with confidence and hope.
References
Alzheimer’s Association. (n.d.). Huntington’s disease. Alzheimer’s Association. https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/huntington-s-disease
Barisic, S. & Kwong, P. (2024, September). Teasing Out Triggers. [Conference presentation]. Huntington Disease (HD) Professional Conference, Westmead, Australia.
Huntington’s Disease Society of America. (n.d.). Overview of Huntington’s disease. Huntington’s Disease Society of America. https://hdsa.org/what-is-hd/overview-of-huntingtons-disease/
Loy, C. (2024, September). Introduction to Huntington’s Disease and Clinical Trials. [Conference presentation]. Huntington Disease (HD) Professional Conference, Westmead, Australia.
Murray, N. (2024, September). Neuropsychology of Huntington’s Disease. [Conference presentation]. Huntington Disease (HD) Professional Conference, Westmead, Australia.
Resources
- Huntington’s Australia (NSW, ACT, QLD, SA, NT, TAS, WA)
- Huntington’s NSW and ACT — 02 9874 9777
- Huntington’s Queensland — 07 3064 3222
- Huntington’s SA and NT — 0424 062 121
- Huntington’s Tasmania — 03 6431 3403
- Huntington’s Victoria — 1800 063 501
- Huntington’s WA — 08 6457 7599
- Huntington’s Disease Network of Australia
- Huntington’s NSW ACT
- Huntington’s Disease and Genetic Testing Booklet
- Huntington’s Disease Youth Organisation (international)
- Carer Gateway
- Crisis Support – Lifeline 13 11 14
