Legislative Council, Thursday 19 October 2023

Ms FORREST (Murchison) - Mr President, I wish to use this opportunity on adjournment to speak about Huntington's disease and request the Government take certain action.

I have spoken before in this place about Huntington's disease, but I wanted to remind members that Huntington's disease is a genetically inherited neurodegenerative disease. It progressively diminishes physical, cognitive and psychological functions to severe levels. It has devastating impacts on individuals and families. Each child of a person with Huntington's disease has a one-in-two chance of inheriting the faulty gene and if they do, they will develop Huntington's disease in the future. Because of this genetic inheritance, Huntington's disease often appears in numbers within a family and across generations, creating complex and very challenging circumstances.

Huntington's disease not only impacts a person; it impacts families, relatives, generations and communities. The cognitive symptoms of Huntington's disease include impaired judgment and difficulty thinking clearly, and lead to a loss of decision-making capacity. The most common motor symptom is involuntary movements, but this eventually extends to a lack of control of most muscle functions such as in talking and swallowing.

Psychological symptoms include fluctuations in mood and emotional responses, depression and anxiety, and many other more serious and often psychiatric problems. It is the behavioural changes that are most often distressing and create the greatest challenge for the person with the disease, their family and carers.

The most common age of onset is between 35 and 50 years, although it can occur at earlier or later stages. Juvenile Huntington's disease is rare in Australia but does occur. The course of Huntington's disease is usually between 10 and 20 years, with complications of the disease eventually causing a premature death. There are no textbook cases with Huntington's disease. It affects each person differently. Generally, the multiplicity of impacts causes progressively difficult circumstances to deal with, not only for the person with the disease but for their family members. Gradually, the person with Huntington's disease loses the ability to eat, walk and talk, and becomes dependent on others for their care. People with Huntington's disease are generally economically disadvantaged, and physically and socially isolated. A sad history, ignorance and stigma about Huntington's disease, and a lack of understanding and support for people with Huntington's disease only increases the disadvantage.

Families are also impacted, often becoming increasingly isolated - especially those rife with Huntington's disease across generations. Many families are also psychologically burdened with the risk of developing Huntington's disease and the risk for their offspring. Individuals and families within Huntington's disease communities struggle to understand what Huntington's disease is doing to their minds, their bodies and their lives.

Limited resources are currently available to help people with Huntington's disease and their families navigate this incredibly stressful journey. Due to the lack of resources, most people with Huntington's disease and their families are trying just to cope to survive, let alone move forward with any understanding or confidence.

Equally, a range of government and community support providers struggle to understand how to deal with the Huntington's disease symptoms and behaviours, as access to Huntington's disease education is also scarce. This lack of service support further isolates people with Huntington disease and their families, making participation and contribution to the community more difficult for them.

The circumstances of Huntington's disease families are fraught and complex. A myriad of social, health or economic issues are often not dealt with and tend to worsen over time; and without adequate supports or interventions, lead to crises. Without the necessary infrastructure, proactive support and intervention, this often leads to long and expensive stays in hospital, helping to clog up the health system.

As the disease and associated disabilities progress, the person requires increasingly high levels of care. Such care is usually too onerous for the person caring for them at home, especially considering that the need will continue to increase over the years. Eventually, for most people, residential care of some kind becomes the only option. Tragically, the age at which someone with Huntington's disease enters residential care is between the ages of 40 and 60.

Mr President, the key point I wish to raise is that there is no dedicated Huntington's disease accommodation for people suffering from Huntington's disease in Tasmania. There are challenges in securing appropriate specialist disability accommodation through the NDIS to meet the complex and critical clinical care required by those impacted by Huntington's disease.

For those who are in care, most are young but they are placed in aged care facilities and subject to aged care activities and mentality. It is not an appropriate location for young people with Huntington's disease. In countries of Western European ancestry, 7 to 12 people in 100 000 have Huntington's disease. These numbers are increasing by 10 to 20 per cent every decade primarily due to increased testing and prevalence. In Australia it is around 10 in 100 000 and Tasmania is double that prevalence. I have spoken about this before the prevalence in Tasmania, Mr President.

The latest 2022 prevalence study in Tasmania puts prevalence conservatively at 19.4 to 22.6 in 100 000, double the Australian figures, and that is dependent on input variables. This is the second highest prevalence in the world next to Venezuela.

The former association, Huntington's Disease Tasmania, has recently merged with other state associations to form the new national peak body, Huntington's Australia. This not for profit organisation provides much-needed specialist support and services for people with Huntington's disease and their families, providing outreach, information, education, counselling, program support groups and advocacy, connecting families to the right services for the stage of their Huntington's disease journey, and generally supporting them through their complexities and challenges.

Huntington's Australia also provide education to the government service groups who interact with Huntington's clients, including care organisations and accommodation facilities. Their services also extend to supporting the youth who are at risk, sharing the burden of Huntington's disease or contemplating starting families. The only way to prevent it, or stop it, is to stop having children through carriers, and that is a decision that is enormous for some people to take and how they can manage it.

Huntington's Australia is extending the provision of its NDIS services such as support coordination for people with NDIS plans from a few states to nationally. They have recently employed an NDIS specialist resource to commence those activities in Tasmania, which is good news. This is an area of great need and is a challenge at the best of times for Huntington's disease families with complex needs. Huntington's Australia has also recently employed a full time Huntington's disease specialist to better proactively, through outreach, support Huntington's disease families.

Through this process alone, in just three months, Huntington's Australia had discovered seven new and previously unknown Huntington's disease families to support. A part-time administration person to support operations has also been employed. Given the shoestring resources that have previously been in place for Huntington's Disease Tasmania and the enormous Huntington's disease challenges facing Tasmania, Huntington's Australia is keen to further increase its services and provide better support for both families and the wider health and community services in Tasmania. For example, they work closely with the neurological personnel at the Older Persons Mental Health Service in Hobart to complement services.

The Tasmanian Government has a small service provision funding agreement with Huntington's Australia, but given the challenges and needs in Tasmania, Huntington's Australia is keen to put a business case to the Tasmanian Government to increase these arrangements.

Mr President, my request to the Government is in part to seek the Government's favourable consideration of this business case. The business case will focus initially on extra Huntington's disease specialists to support the growing number of Huntington's disease families and/or support the facilitation of Huntington's disease clinics in Hobart to start with, in conjunction possibly with the Older Persons Mental Health Service, which will require government funding and support. This is critically important to Tasmanians and their families living with Huntington's disease, particularly as there is currently no specialist Huntington's disease clinic for Tasmanians impacted by that disease. This is despite other states such as Queensland, Western Australia, New South Wales and South Australia all having specialist clinics with less than half the prevalence of Huntington's disease cases than in Tasmania. I request the Government, or urge them, to consider this request favourably to support people and families with Huntington's disease.