While Huntington’s Disease is not unique to Tasmania, it is unusually prevalent across the state. In fact, per capita, Tasmania has the second highest prevalence of Huntington’s Disease in the world, yet there is still a lack of awareness of the disease.
Huntington’s Disease is an inherited degenerative neurological condition affecting the brain and central nervous system, and is progressive in nature, causing a range of motor, behavioural and cognitive disturbances for those living with it.
According to the Huntington’s Disease Association Tasmania website, we all carry the Huntington’s gene, however only those who inherit the expansion of the gene will develop Huntington’s Disease. A child of a parent with Huntington’s Disease has a 50/50 risk of inheriting the expanded gene.
Becauese Huntington’s can cause a range of different symptoms for different people, it can be quite varied in how it’s experienced by Huntington’s families.
Janne Dolbey, a local who lives with Huntington’s spoke to the variety of symptoms it can cause.
“Well, even in the same family, it manifests itself differently.” She said.
Four out of five of Janne’s siblings inherited Huntington’s Disease, and have each experienced different presentations, with Janne describing her brother as unable to talk as the disease progressed and spent the last eight years of his life in a nursing home.
Thankfully testing is available to those at risk of inheriting Huntington’s Disease, however it is not easy to access, with Janne explaining that patients are required to undergo six months of genetic counselling before being able to know whether or not you carry the Huntington’s gene.
“I had to go and have gene counselling for six months to make sure that I was psychologically OK because it has one of the highest suicide rates because they watched it in their families, and so when they found out that they had the gene [they may be at risk of suicide].”
“So, I didn’t get tested until my late forties, but I was worried that I would have passed the disease on to my sons, so I wasn’t worried so much about myself.” She added.
Huntington’s Tasmania has begun a merger with Huntington’s Australia, hoping to achieve a stronger, national approach to supporting those with Huntington’s, and allowing those impacted to receive access to a wider range of support, with the change allowing for new support roles to be implemented across the state.
Last month, both the CEO and Chair of the Board of Huntington’s Australia held a get together in Launceston for those affected by Huntington’s in the north of Tasmania.
For Chair of Huntington’s Australia, Chris Glasson, the movement is close to home.
“I come from a Huntington’s family.”
“15 years ago, I wouldn’t have known of Huntington’s disease, until my mother started falling over, and my mother was in her late sixties, and inexplicably started falling over, she went to doctor after doctor to find out what was going on”.
“She was in hospital one day, and we still didn’t know what was going on, and the doctor brought in a neurologist who we hadn’t met before. The neurologist walked into the room, sat down and said your mother’s got Huntington’s disease, and we said, ‘what the hell’s that?’”
“I’ve got two siblings, both of them are positive, I’m negative. It’s a big issue in my family.”
“My father and my mother used to go to the local community centre for activities and my father dragging me along to an annual general meeting of the association one year, and it had only just really started up and I saw that I had some sort of management and business skills that I thought might be able to be offered, and so I talked to the general manager there and she said ‘why don’t you get on the board?’ and so I’ve been on there five or six years.”
With different Huntington’s associations spread out around the country, it was clear that more could be achieved from collaborating and co-operating with each other, which lead to the national association.
“Some were stronger than others. Some have struggled for sustainability, financial sustainability, staffing. So, we started looking at that over the last two or three years” he added.
Chris also added there is a clear need for extra resources in Tasmania.
“Travelling around, talking to all the people, it’s very obvious, Lauren over here is the practitioner has only been here three months and on top of all the numbers that we do know here, she’s just found seven families who have not identified at all. Not just seven individuals, but seven families in three months, so you extrapolate that out and that’s the sort of thing that we’re looking at dealing with.”
When asked about some of the main challenges faced by those with Huntington’s are, a clear front-runner was a lack of services.
“Getting access to professional services is a struggle all around the country.”
“In some states it’s a two year wait for neurology, it’s sometimes even 12 months just for an OT.”
CEO of Huntington’s Australia, Lenni Duffield, said that it’s not just about Huntington’s disease, but about disease in general.
“Tasmania does not have post-secondary opportunity for young people, or people wanting to skill themselves at a university level to study allied health.”
“So the market conditions at the moment, and Tasmania is no different to the mainland, very hard to get an OT, a [speech therapist], dietician, a physio, all those people in the allied health field, yet the university in Tasmania don’t offer it as a study stream.”
Ms Duffield added that Tasmania currently has two juvenile cases of Huntington’s Disease.
“It’s very aggressive, they’ve started in their teenage years, and they won’t live until they’re thirty” she added.
For more information visit huntingtonstasmania.org.au