MOTION - Huntington's Disease

Published: 23 March 2023

Ms FORREST (Murchison) - Madam Deputy President, I move -

(1)That this House notes:

(a) Huntington's disease is an inherited degenerative neurological condition affecting the brain and central nervous system that results in the progressive loss of mental and physical capacities impacting affected individuals' ability to walk, talk, eat, think and reason with death ensuing from complications;

(b) Huntington's disease is a neurological condition that has both physical and mental symptoms;

(c) that the services to provide care and support to individuals with symptomatic Huntington's disease are currently delivered through mental health services, specifically Older Person's Mental Health Services;

(d) Huntington's disease does not skip a generation. An individual who does not inherit the expanded HTT gene will not develop the disease and therefore will not pass it on to the next generation;

(e) Huntington's disease symptoms typically manifest at the prime of the person's working life, thus creating financial challenges;

(f) five to 10 per cent of all Huntington's disease cases are classified as Juvenile HD, which has an age of onset between infancy and 20 years and in most cases, the rate of progression of Juvenile HD tends to be faster than in the adult form;

(g) early features of Juvenile HD include strong behavioural changes, learning problems, decline at school and speech problems;

(h) according to Tasmanian statistics:

(i) Huntington’s disease affects one in 5000 individuals, not
including those that are considered at risk or pre-symptomatic;

(ii) that this number is three times the Australian average; and

(iii) per capita, Tasmania has the second highest prevalence of Huntington's disease in the world;

(i) Huntington's Tasmania continues to work with and support Huntington's disease families and calls for additional resources and dedicated facilities to support affected Tasmanians; and

(j) Huntington's Tasmania has recently resolved to join a national body, Huntington's Australia as the peak national body representing families impacted by Huntington's disease.

(2) This House calls on the Government to:

(a) deliver services to those with symptomatic Huntington's disease through robust model of care, developed in consultation with the Huntington's disease community with greater emphasis on access to neurological services, allied health and the establishment of regular clinics in the three most affected regions of Tasmania, within the health sector to ensure all support, expertise and services necessary are available that actively reduces the stigma associated with Huntington'sdDisease;

(b) consider the inclusion of Specialist Neurologist Nurse Practitioners across the state to support the care of people with Huntington's disease;

(c) consider the alignment of Huntington's disease case managers aligned to Huntington's Disease Tasmania rather than Older Person's Mental Health Services;

(d) ensure appropriate purpose-built residential care facilities are available to assist members of the community impacted by Huntington's disease that require full-time care;

(e) ensure the justice system is adequately resourced to respond appropriately to those interacting with the justice system who have symptomatic Huntington's disease; and

(f) support Tasmanian health services to improve facilities and services to enable Tasmanians with Huntington's disease to be eligible for and qualify for clinical trial status in Tasmania, particularly in regard to neurological and neurosurgical services.

In making my contribution, I will read through the motion. I know it is a long motion, but I think it is really important we really focus on what we are talking about here. The rest of my contribution will be reflecting on those points because most of it is contained within the motion itself.

The motion calls on this House to note:

(a) Huntington's disease is an inherited degenerative neurological condition affecting the brain and central nervous system that results in the progressive loss of mental and physical capacities, impacting affected individuals to be able to walk, talk, eat, think and reason with death ensuing from complications.

I think you will all agree that is not the sort of life or death that any of us would want.

(b) Huntington's disease is a neurological condition that has both physical and mental symptoms.

I think it is really important that we focus on this because so many people think it is just a mental health condition, and it is not. It is a neurological condition that has both mental and physical symptoms.

(c) that the services to provide care and support to individuals with symptomatic Huntington's disease are currently delivered through Mental Health Services, specifically, an Older Person's Mental Health Services.

When you think about the comment I just made that seems entirely inappropriate. Firstly, these people often are not old, they are often in their 40s. Secondly, it is not a mental health condition; it is a neurological condition.

(d) Huntington's disease does not skip a generation. An individual who does not inherit the expanded HTT gene will not develop the disease and therefore not pass it on to the next generation.

(e) Huntington's disease symptoms typically manifest at the prime of a person's working life [as I mentioned, these are not old people] thus creating financial challenges for them and their families.

(f) five to ten per cent of all Huntington's disease cases were classified as Juvenile HD; which has an age of onset between infancy and 20 years. In most cases, the rate of progression of Juvenile HD tends to be faster than the adult form;

So again, it is highly inappropriate that these young people are cared for through older persons mental health services.

(g) Early features of Juvenile HD include strong behavioural changes, learning problems, decline at school and speech problems;

(h) according to Tasmania statistics:

(i) Huntington’s disease affects one in 5000 individuals, not including those that are considered at risk or pre-symptomatic;

These are the ones who are affected.

(ii) that this number of one in 5000 individuals is three times the Australian average.

In Tasmania we have three times the Australian average number of cases. A lot of those people live on the north-west coast.

(iii) Per capita, Tasmania has the second highest prevalence of Huntington's disease in the world;

It should shock us because that is the reality that the Huntington's families are living with. However, when you understand it is an inherited genetic disorder that is passed down through a gene in a small population of Tasmania where a lot of people, like it or not, are related, then the risk of passing that gene on is much higher and concentrated. That is why Tasmania has such a high rate. It came from one family. In the other jurisdictions where it is high, they are small communities as well.

(i) Huntington's Tasmania continues to work with and support Huntington's disease families and calls for additional resources and dedicated facilities to support affected Tasmanians.

I will come back to this point, but Huntington's Tasmania is very grateful for the support they have got and the Government increased their funding not so long ago after we had lobbied quite significantly for that. That is great, we - I say 'we' because I am the patron of Huntington's Tasmania. I have very close connections to the main voluntary workers and only recently I am in the position of CEO for the organisation. I have worked closely with them and with some of the families. So, I do say 'we' are grateful for that, but I also know that additional support and assistance is required, particularly, in appropriate facilities and - as we will get to - the reallocation of the support base from health, not mental health, and particularly not Older Persons Mental Health Services.

Fairly recently, Huntington's Tasmania resolved to join a national body, Huntington's Australia as the peak national body representing families impacted by Huntington's disease. This was a decision that was agreed by all states, except for Victoria. Victoria is still on its own. All other states have come into the one organisation, Huntington's Australia, which does help our Tasmanian organisation because those organisations are much better resourced. They have many more staff. Even though we have the higher number per capita, they are much better resourced.

When the organisation recently had some challenges with staff resignations, for example, the other states stepped up to offer their help and support which was very gratefully received. The actual establishment of the national body has not quite occurred yet, there has been a bit of a hold up with some of the processes involved as these things tend to do. There has been broad agreement and even without that formalised process, other jurisdictions are reaching out to help Huntington's Tasmania when needed.

Part two of the motion calls on the Government to do a number of things. Firstly:

That is not to say that there is not a need for mental health support. There is. You can only imagine the family themselves might need mental health support considering the journey their loved one is on when they have a positive and symptomatic person in their family.

We also call on the Government to:

(b) consider the inclusion of Specialist Neurologist Nurse Practitioners across the state to support the care of people with Huntington's disease;

Nurse practitioners in this area come with both the psychological, the mental health aspect, and also the physical aspect of the symptoms because they are focused on the neurological condition that Huntington's disease is.

We also ask the Government to:

(c) consider the alignment of Huntington's disease case managers aligned to Huntington's Disease Tasmania rather than Older Person's Mental Health Services;

I have already touched on that. Also:

(d) ensure appropriate purpose-built residential care facilities are available to assist members of the community impacted by Huntington's disease that require full-time care;

There was a period - I cannot think what year it was because it was a little bit before COVID-19 started - when there was a new facility being built in Devonport. Originally, there was going to be some dedicated Huntington's disease accommodation in that. It changed. It became emergency accommodation I think for youth, at the time - which is fine because they need it too - but it means that there is still no dedicated facility to provide care. Unfortunately, the majority of people who are not necessarily old and usually are not old end up in aged care facilities.

One of them, in particular, who I am aware of, had a very unfortunate experience in an aged care facility. It was not set up to care for her particular needs. It was dreadful and shocking. We do need purpose-built residential facilities to support these families.

We also need to ensure the justice system is adequately resourced to respond appropriately to those interacting with the justice system who have symptomatic Huntington's disease. If you think about some of the behaviours, that are not of their own making, but are symptoms of their disease that Huntington sufferers can experience and display, you can understand how they may come into contact with the justice system.

The justice system is not set up to manage people with symptomatic Huntington's disease. Again, I have supported Huntington's Tasmania to try to support some of these people and they have ended up in prison. I am not sure that is a very good place for a person with Huntington's disease, but that is where they have ended up in our prison.

I also call on the Government to:

I will come to that a little further in a moment.

To reiterate, Huntington's disease is a progressive, neurodegenerative disease, for which there is no current effective cure and there is no current effective treatment, except for symptomatic treatments to manage the psychiatric and movement symptoms.

This is particularly important because Tasmania has one of the highest rates of prevalence with Huntington's disease in the world, affecting about one in five thousand individuals. We need to remember, these are the people who we know have it, this does not include people who are considered at risk.

They may have a person with Huntington's disease in their family, or they may know they have it, but they are pre-symptomatic before they get the symptoms but they will all get symptoms. Everyone with Huntington's disease with the ACTG will go down the path.

For every gene positive person, in Tasmania and everywhere else, it is estimated that there are between five to seven people at risk of inheriting the disease. There are currently 114 presently symptomatic cases in Tasmania, with an unknown number of people who may have the gene, and thus become symptomatic in the future.

With approximately 85 per cent of those who may be at risk who know that somewhere in their family there is a person who has Huntington's disease, they choose not to be tested. It is impossible to know the full extent of cases in Tasmania. It is not the case that some with this gene will not progress to an untimely death and with all the unpleasant symptoms, because all cases do.

This does make it extremely difficult to prevent in many cases and this has particularly been a problem in the past. Many cases may well be at the age where they have already had children and potentially passed on the gene, prior to becoming aware of the presence of the gene in their family or certainly in themselves.

I do not condemn anyone for making this difficult choice regarding testing, as I personally would find it hard to know what I would do. Is it better not to know? Because you cannot change it or do you just need to find out? That is a very individual call, no-one can make that call for any one person, they have to make that decision themselves. They make that knowing that it is the most debilitating and dreadful disease, but it is still not fully understood. The symptoms are severe and distressing for the person and for the family and friends as the disease progresses.

Back in the day, people with Huntington's disease were shut away, locked away, because of the stigma and the shame. It made it even harder to even try to talk about the passing on of the gene, particularly as these people are perfectly healthy, perfectly productive and very healthy normal members of our community, often having children before the symptoms appear.

This disease also significantly degrades a person's mental health and that of their family. Research on the rates of suicide with people with Huntington's disease have found rates between 5.7 to 12.7 per cent. Sadly, for people with Huntington's disease, the risk of death by suicide has been estimated to be between two to seven times greater than that observed in the general population according to research published by the Cambridge University in 2021.

This research focused on people in advanced stages of Huntington's and does not adequately account for all those at risk, including those people who are grappling with the possibility of the disease or watching their family suffer with no support or very limited support within our current systems.

We can all agree we do need to do better for those impacted by this disease and ensure that a person with Huntington's disease and their family have access to timely and appropriate care and support. This means purpose built facilities and education for the families, as well as the broader community.

This includes mental health and mental health care, as well as neurological care and the care for the physical symptoms. It also requires wellbeing support, as the mental health burden for the entire family, not just the individuals, and also individuals with Huntington's disease but those at risk of inheriting the disease. The trauma and grief resulting from caring for a loved one with Huntington's disease is quite significant.

The trauma experienced by a person and their family is compounded further given the potential for impulsive, aggressive, violent behaviour or verbal angry outbursts that are outside the control of the person but it is pretty hard looking after someone who you love whose character changes completely as the disease progresses.

The behavioural and cognitive effect on the individual are such they may be unable to empathise or comprehend the danger they may cause to their own family, physical or mental. The symptoms are visually confronting and their needs are very high. People with Huntington's disease may be treated poorly and ostracised in society, as the symptoms of the disease are not well understood or recognised.

People impacted can be and often are misidentified as being under the influence of drugs, alcohol or other medications because their behaviour may reflect some of those behaviours you might see that someone who is really high on ice or something, for example. They are often shunned and ostracised in our community, making it even harder for the family to get the support they need.

We need to be providing support services, particularly mental health services to young people as they navigate their understanding of the disease, their at-risk status and the decline of their loved ones. We need to ensure that the Department of Justice is also adequately resourced and informed of the impact of Huntington's disease for those who may find themselves engaging with the justice system. I have already mentioned one particular case where the justice system - whilst the person did commit offences as a result of their condition or predominantly related to that, the justice system was not built up to properly support or deal with that person. The law is pretty black and white in some things and that is part of the problem, but we do need to ensure we really need to consider what is the best outcome for this person. I do not think prison is a good place to care for a person with Huntington's disease in the advancing stages, which is the case I am referring to.

It is vital there is inter-governmental cooperation across all areas of Government services to ensure that families with Huntington's are adequately supported, understood and managed in a way that meets the needs of the individual, while ensuring the safety and wellbeing of families.

One of the worst parts of this disease is the cognitive and behavioural decline that often leaves families struggling to manage a person who no longer acts like themselves. They basically become someone who is completely different to the person they knew. Individuals with Huntington's disease may become difficult to manage, violent and sometimes a risk to their families. They can be quite physically violent and quite strong, which makes it very difficult for sometimes their partners or other loved ones to actually provide that care.

Understanding their loved one's inability to make decisions, avoiding misinterpretation of these behaviours between these actions and gender based violence and ensuring adequate guardianship is pivotal to ensuring the risk is appropriately managed, particularly where there is diversion from the criminal justice system. When you have a partner whose behaviour changes enormously and it presents as a family violence situation, you have to be sure that you not condoning the family violence aspect of it, you would need to deal with that - but you need to look at what sits behind that and work to help that family and not facilitate that family violence any further.

Huntington's disease can place considerable financial burdens on those diagnosed with the disease, given that the changes people generally start to experience typically happen at the prime of their working life. They probably have a mortgage, if they are lucky enough to have a mortgage these days. They often have a lot of financial commitments, they often have children at school and things like that and a lot of financial pressures, and suddenly, they have no source of income if their partner is not also working. Then the partner often becomes the carer, and these people often cannot be left on their own, for obvious reasons.

The onset of symptoms tends to appear in one's mid-30s, which is a critical time in terms of career, earning potential and superannuation contribution. It is also a time when many have already had children, potentially passing on the gene.

This leaves families having to rely on financial assistance that is often inadequate, especially as others within the family may have to prioritise care for their family member over their own career. These factors can lead to intergenerational poverty, and that is a matter we need to be aware of in providing support and care for impacted families.

Discriminatory practices of insurance providers, and pre-existing condition exclusions, often leaves families without insurance cover, especially with regard to income protection or total permanent disability - which is what this is.

This discrimination also has a negative impact on testing where a diagnosis would render a person ineligible for insurance. If you think you might get a positive test and you are wanting to get your health insurance, or your income protection insurance, if you do not have a test, you do not have an answer. Then we do not know. We cannot necessarily put those mechanisms in place to try to prevent the passing on of that gene. People with a family history may elect not to be tested to avoid confirmation of their own status for this and a range of other reasons.

Furthermore, access to specialist services, clinics, clinical trials, specialist care facilities and housing in Tasmania can be challenging, given that Huntington's disease families are spread out across the state, with many outside the major metropolitan areas of Hobart and Launceston. Many live across the North-West coast and a lot of them live in rural areas.

Travel - both intra-state and interstate - can be cost-prohibitive and not feasible due to the symptoms of their condition. They need care closer to home. The local specialist care facilities and accommodation, in particular, causes significant hardship for those experiencing more severe symptoms. Sadly, and inappropriately, residential age care is the only available option for many, even those with juvenile onset Huntington's disease.

There are other very real challenges for young people at risk of Huntington's disease who are unable to test until they are 18. There is a legal requirement to be 18 before you can be tested, so those with juvenile Huntington's disease are only tested if they become symptomatic. Once they are symptomatic, they can be tested, but they do need to be 18 otherwise.

International standards guiding the genetic testing processes require that an individual have three appointments with a genetic counsellor, spaced a minimum one month apart, before they are able to test. You think about the challenges with accessing a genetic counsellor for those three appointments. It is important to do it, I am not saying we should not do it; but where is the nearest genetic counsellor? Most will have to go to Melbourne.

While protecting someone's mental health is critically important, the process of testing often takes more than six months from the first referral. You can only imagine what a stressful time this is, firstly waiting to get your appointment, going through the process, and then waiting for the result.

There needs to be greater support and education for young people to inform their decision to test or not, and efforts made to remove barriers to testing and receiving results, with mental health support ongoing throughout the process, including after the test, regardless of the result. Even if the test is negative, people still need mental health support because of the stress of going through that whole process.

The testing experience itself is inherently traumatic, however the messaging and long delay also has the potential to discourage people from testing, altogether. Given that IVF has the potential to eradicate Huntington's from families, a different approach to managing risk, supporting and encouraging testing should be considered, and more consideration on resource needs and options needs to be a priority. This is another area where the government can help, making access to IVF and the technologies that we can use around that, to work with these families to still enable them to have children, but healthy children that do not carry the gene.

We have a real chance to make a difference while we wait for researchers to find treatments and a cure, which we hope they do but they may never fully succeed in this.

Prevention of future cases is possible in the longer term but requires much greater understanding, support, mental health care support and planning in the interim.

Right now, we can empower our young people and families to take action; to build awareness and understanding; and to consider and plan what life will look like when they are unwell, if they know they have Huntington's disease and to focus on Huntington's-free children where possible, to reduce the burden on families and to give freedom to people who feel trapped by a horrible disease with little or no option. That is how it stands currently.

The golden objectives that Huntington's Tasmania outlined in this strategic plan are: to build our community; to ensure quality care for Huntington's disease families; and for effective governance, management and funding of Huntington's disease Tasmania.

Since 1978, the Huntington's Disease Association of Tasmania has taken a leading role in advocating for and educating individuals, organisations and professionals to meet the needs of people effected by Huntington's disease. I commend their work. It is a small and, until recently, a fully volunteer organisation. That is not the case in other states; they have funded positions for much longer. I particularly want to mention the incredible work of my good friend, Pam Cummings. Some of you may have met her and will know how incredibly dedicated she is. As a member of this community, she does an enormous amount of work and picks up the slack when anyone else cannot. I have worked with her for years, advocating for financial support for the organisation that provides invaluable support to families. The advocacy work Pam and her small team, including another one of my constituents, Wendy Weeks, and their volunteer board members, has been phenomenal.

Thanks to the hard work of the leadership team of the organisation and the Huntington's Disease Association of Tasmania, we received an additional funding grant from the state Government which enabled the organisation to employ an executive officer for the first time to help them realise their strategic goals; and that was only last year.

After much lobbying and much begging, and in my typical form of never giving up or shutting up, and the work of Pam, in particular, and Wendy and others, the Government came through and supported that, for which we have been very grateful.

The organisation has continued to transition to a more professionally operated organisation and further restructuring is taking place, including the processes around becoming a part of the national body.

As I said, more recently Huntington's disease organisations around the country, with the exception of Victoria, have agreed to form a national body to work collaboratively. This will assist Tasmanians as we have the highest percentage of people living with Huntington's disease. Really, Huntington's Tasmania is the most under-resourced organisation financially; not without human will power, I can tell you, but this will assist the organisation greatly.

Huntington's Tasmania is currently advertising for a Huntington's disease specialist practitioner role and further positions will follow later, including providing support for coordination for people navigating the NDIS. A lot of these people are young enough to fit into the NDIS Scheme. We all know how difficult that can be to navigate, particularly for someone with Huntington's disease. This work is ongoing and part of the progress that will occur as part of the national body.

Another matter I wish the Government to consider, especially as Huntington's disease does not only manifest in mental illness, Huntington's disease sufferers have many physically debilitating symptoms. As I said at the beginning, it is a neurological condition and therefore we ask the Government to fully consider why the funding and support for Huntington's disease sufferers still sits in Mental Health Services, particularly in Older Persons Mental Health unit. I think for all the reasons I have outlined, that is highly inappropriate. It is also inappropriate because it adds stigma and shame to many Huntington's disease families and their experience and has done for years. This can and should change. If anyone knows of a family who had a person with Huntington's disease many years, they were often referred to as the mad old person. A lot of them were not old and they certainly were not mad. They had mental health challenges and neurological challenges and physical challenges because they had Huntington's disease.

Just a brief focus on part two of the motion, which I have already spoken to a bit.

We urge the Government to deliver services to those with symptomatic Huntington's disease through robust models of care that are developed in consultation with the Huntington's disease community, with a focus on and greater emphasis on and access to neurological services, allied health and the establishment of regular clinics in the three most affected regions of Tasmania. I note, the recruitment of the Huntington's disease specialist practitioners and further positions and urge the Government to continue to assist with that work. As mentioned, this care should be provided from within the health sector to ensure all support, expertise and services necessary are available to actively reduce the stigma and to provide the appropriate care to those suffering with Huntington's disease, as the majority of these are not elderly, they do not even get to live to be elderly.

I urge the Government to consider the inclusion of specialist neurological nurse practitioners across the state to support the care of people with Huntington's Disease as the motion calls for, particularly as travel can be very difficult for families or people suffering from Huntington's disease. We also need the Government to ensure an appropriate purpose built residential care facility is available to assist members of the community impacted by Huntington's disease who require full time care, noting that it often becomes impossible for the families to provide this care full time. Aged care is inappropriate but sadly, that is where a lot of people with Huntington's disease end up being placed.

As I said, having been patron of Huntington's Tasmania for a number of years, I know that sadly some of Huntington's Tasmania clients, due to the nature of their symptoms they experience and have very little control over, find themselves interacting with the justice system. As I have mentioned previously, this is another area I want the Government to look at. It does take a broader look than just health, mental health. It requires housing, it requires justice, all to be involved in this very insidious and tragic disease. We also need better education and awareness of the disease itself.

On that final point about access to clinical trials, research is really the only way we are going to have any hope of finding effective treatments and the holy grail of a cure. If we can have our own people participate in research as well. I urge the Government to seek to do what they can to support that.

As I have noted, in closing there is no current cure and very little treatment, hardly any that is fully effective. Everyone who carries the expanded HTT gene will develop Huntington's disease and will suffer the symptoms and face an early death. We do need to do all we can to support medical research and enable Tasmanians to participate. It is a progressive neurodegenerative disease and has both psychiatric and physical movement symptoms. It is often misdiagnosed or misrecognised in the community. It is a devastating neurological disorder, a disease that destroys a person's life in their prime.

If I have not said enough, I will just say it again, that Tasmania has the highest per capita number of cases in the country and the second highest in the world. People with Huntington's disease have specific and special needs and need to be provided with a range of health care and support across a range of areas, including but not limited to mental health care. Sadly, these people do die young, often with families, young families, who all live with the trauma of the disease, even if they do not carry the gene themselves. We owe it to ourselves to be well informed and aware of this insidious disease.

I seek members' support for the motion which actively calls on the Government to respond directly to the needs of a group of Tasmanians who may face more challenges than any of us can even imagine.

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