ACA: What is motor neurone disease, for someone who isn’t familiar with it?

JM: Motor neuron disease (MND) is where the motor neurons that send the message from your brain down to your muscles stop working. And it's all muscles that you control yourself, so it's not involuntary muscles – and it starts to present in three places. Firstly, in your feet: the muscle wasting there generally tends to people having what's known as drop foot, which leads to people tripping over. The second place is in the arms, and particularly in the hands, and unfortunately you get weakness in the hands, which is often thought of just as a bit of arthritis. But slowly you'll lose use of your hands, and then your arms. And then the third place it presents is in the throat, so a person will start to sort of slur their words, or they'll start to have a bit of a cough that they can't clear phlegm out of their throat because the muscles in their throat are not working.

Eventually you lose all muscle control, and what eventually kills someone with MND is their diaphragm not functioning anymore. And once that's involved, then it's usually a fairly rapid progression from there, with people losing their lung capacity and lung function. It is a terminal disease, and there is no cure. There's no viable treatment readily available on the market. The average life expectancy is 27 months. According to Australian statistics, there's two people diagnosed and two people that pass away every single day.

It is an aging disease. And there is a statistic going around that if everybody lived to 100 you have a one in 300 chance of developing MND. But there’s no known cause – around 10 per cent of cases are genetic. But just because you have one of the identified genes doesn't mean that that will switch on and you will develop MND. So there's also some environmental and lifestyle factors involved, but for the other 90 per cent of people that don't carry the genes, those factors are so far unknown – they don't know what they are.

There are lots of research projects happening around the world to try and work out what the cause is, so that we can move towards prevention and better treatment. There's no viable biomarker, so that is frustrating also for patients, because it can take a long time to be diagnosed correctly. People can go through a number of different stages as part of their diagnosis. We've had various people who have surgeries on their backs or things to fix something that it was misdiagnosed as.

There's also no set progression for the disease, so you can't be diagnosed and be told what you’ll be experiencing in 12 months or two years. Every person is completely different in their way they progress. There are some signals, like I said, around respiratory decline, but for the vast majority of people, it's just an unknown. This means for an early stage diagnosis, they get told to sort of get your affairs in order and go and live your life, because you just don't know what is coming at you. But then we've had people that are eight or 10 years down the track, they've given up their job, they've gone and spent all of their super and they're still here eight years later.

It doesn’t affect someone’s mind or senses. However, there is a very large crossover cohort of people with MND who also develop frontotemporal dementia – around 30 per cent of patients who develop both. So they may have frontotemporal dementia and then start showing MND symptoms, or they may have MND and then start showing FTD symptoms, so they're the only cases where it does have sort of significant impact on mental capacity. But the vast majority have no issues with mental capacity or their main senses, so their sight, hearing, taste.

ACA: What’s the psychological impact for someone who’s been recently diagnosed – what is it like being told you might have just 27 months to live?

JM: I don't know whether it's a scientific term, but we call it active grief.

So that is knowing you have a life-limiting disease, which is very close and tangible. If you know you're going to die in 10 to 15 years, it's a lot further down the track. But when you're given 27 months, as well as losing your mobility. You could be losing your speech, you could be losing your independence. You've got all of that on top of you, so that real active grieving process that the person goes through, as well as their family members around them – add on top of that, the frustration of not knowing how the progression will go is really quite debilitating in their psychological space for a lot of people.

ACA: And for people that are caring for them, family members, loved ones as well, how would you describe the psychological impact on those people?

JM: It’s the same type of active grief – it's watching somebody slowly lose their function and knowing that in the not too distant future, they're going to be passing away. It’s very different to a sudden heart attack or other conditions that have a cure or treatment for people to live well in the meantime. With MMD, every day you wake up, you don't know what new symptom or new issue is going to present itself, and that impacts the carer’s life immensely.

Once a person is losing their ability to walk or isn’t able to feed themselves, or the bigger issue of losing their ability to communicate – when they’re unable to speak and say, “Hey, I need a drink of water,” or “Could you scratch my nose?” – those sorts of things are hugely frustrating. There is modern technology now with eye gaze devices that people can use, but it is still timely and difficult.

ACA: What role does grief counselling play when we’re talking about active grief, and what’s helpful for clients given the mountain of things they’re already dealing with?

JM: It depends on the patient. Everybody has good and bad days. There are some patients who have a really positive outlook on their life, and live their life the days that they have to the fullest. So they're going on excursions, going travelling, going overseas – we’ve got people that are wheelchair-bound, so they can go on cruises. Those families that have children and grandchildren are getting the joy out of spending time with them. Some people get bogged down in the negative and the doom and gloom of it – and that's all encompassing for them.

Grief counselling for those two groups is very different. A lot of the grief counseling for the positive group is reinforcing great things in life. Trying to do that with the other group, you've actually got to get them up to a level where they are appreciating what they've got to then sort of reinforce that. And we've had some really sad cases, including some suicides. We've had a number of patients admitted to mental hospitals.

The voluntary assisted dying (VAD) scheme has also changed some of that landscape as well, and there's counselling that goes along with that. It gives some people the power to be in control of MND – the disease is not going to be the winner, they are able to make the decision of when and how they pass away. And we've seen quite a shift in people's psychology once they get that approval – it really changes their outlook on how they live their life and how they interact with other people as well. For some people, once they have control of that, it makes a marked difference in their presence and the psychology around it. We’re looking for someone to do a research project in this field, as a PhD or otherwise.

ACA: What kinds of best practice guidelines would you recommend for mental health practitioners working with clients with MND?

JM: When you're dealing with anybody with a disability, always be patient focused and make sure that it's all encompassing of the person – not to just pick out certain parts of the disability. And when you're dealing with someone with MND, like I said, their disability is drastically changing over a period of time, it’s not static. With MND, they might be able to stand on their own this week, but next week, they need assistance to be able to stand, and the following week, they can't stand.

So it's being aware of every time they come to visit, the physical restraints that the person is going through may have drastically changed, which is then changing their lifestyle. It's changed their interactions with family members or carers in some of those big milestones that people go through: not being able to wipe their bottom, not being able to feed themselves, not being able to stand on their own, those are sort of major milestones that people go through.

For example, at the beginning people can say, “Well, when I thought I lost my licence, I thought that was a real kicker for me. But now that I've lost my independence and being able to just go to the toilet on my own, I find that that's way more of an impact on my life than not being able to drive.” So best practice would be making sure each visit includes an update on what stage they are at right now, versus assuming it’s the same as a month ago – it could be drastically changed.

ACA: What’s your personal connection with MND and the work of the foundation?

JD: My kids went to school with the kids of Scott Sullivan, the founder of the MNDandMe Foundation. So I saw from his journey from the very beginning, and I was involved when they were first starting the foundation – I was an event participant and a volunteer, and attended the galas and helped with fundraising. Scott passed away in 2014 and one of his best mates took over as CEO, and he was the only staff member. After four years, he was getting to the end of his term, and being the only person, he couldn't just up and resign. He knew of my background in events and fundraising, so he asked if I was interested in coming on board to do a longer handover. That was in the middle of 2018, and I took over as CEO at the beginning of 2020, just as we launched into COVID.

I saw Scott through his time with the disease, but since working here, I've built up really close friendships with a number of people with MND as well as their family members. I’m very close with some past carers and spouses who have lost their loved ones. I find it such a rewarding job – I often say I hate work but I love my job. Coming from a business and fundraising background into a charity realm where I can do all of that but see our impact with families on a daily basis, it’s one of the most rewarding things we do. Things like an MND diagnosis really turns your whole life upside down, and it’s personally changed my own outlook on life.

MNDandME is seeking practitioners specialising in grief counselling and/or terminal diseases for client referrals in Queensland. Please click here for further details.