"I'm towing a jumbo jet" – Life with MND

A note that reads 'You are not a product of your circumstances, but a product of your decisions' is pinned above Justin Yerbury's desk. His daughter also smiles back at him from a photo.

In the corner sits a pencil sketch of a dated Cambridge streetscape; two others hang in his home. The bookshelf is filled with leather-bound theses, years of work from former students, and his computer monitor is propped up by thick textbooks about the brain.

Papers are pushed aside on his desk and research journals published weekly pile up, unread, a sign that now there is only time to focus on certain things.

^{Justin Yerbury gets to work every other day, meeting with students and colleagues, entrusting the future to those around him.}

Two decades ago, three words - Motor Neurone Disease (MND) - would radically change Justin Yerbury's life - for the better and for the worse. Instead of going into business, a commerce degree under his belt, or taking his place with the Illawarra Hawks as a professional basketballer in the national league, Justin stepped into the lab. He stepped into the world of cell biology, protein soups and molecular origami.

"I never would have thought that when I started out that I would be here," Justin says with a smile. "Running a research team, with a PhD, having worked in Cambridge - it wasn't in my realm of possibilities but here we are." As a Principal Fellow at the Illawarra Health and Medical Research Institute, Justin leads a group of scientists who are studying neuro-degeneration and motor neurone disease.

Twenty years ago, those three words crept into his family. First an uncle, then a cousin. "Those words meant nothing to me," Justin recalls. "I had no idea; none of us did. He was a distant uncle. It was something at arm's length. We didn't even know what the prognosis meant, but it didn't take long to figure out this could strike anyone in the family." A few years later, Justin lost his grandmother, mother and aunt in a matter of weeks.

"We knew that motor neurones were dying but what was triggering that was unknown."
- Justin Yerbury

At the time, very little was known about MND (referred as amyotrophic lateral sclerosis, ALS, or Lou Gehrig's Disease elsewhere in the world), only that it caused a rapid and progressive degeneration of motor neurones in the brain and spinal cord. From diagnosis, a person's life expectancy can be counted with the fingers on one hand.

Motor neurones carry messages to our muscles so that we can move, swallow, talk and breathe. As these cells die and muscles waste away, MND insidiously robs people of their strength, mobility and independence. In some cases, there is a genetic link that can be traced through a family, but for most people diagnosed with MND, the disease takes hold without warning.

"Luckily, or not, whichever way you look at it, the first mutations in the SOD1 gene were discovered in 1993, a few years before my uncle was diagnosed. Lucky in the way that we had more information, not that we could do anything, but at least we could pin it to something," Justin says. Before those discoveries, scarce research could be done because there was no way to study what was happening to motor neurones before diagnosis; scientists had no tools to model the disease in the lab.

"We knew that motor neurones were dying but what was triggering that was unknown," Justin explains. He went back to university, first one biology subject - to understand the foreign language of academic papers that he was trying to read - then a whole degree. "I soaked it up. I just wanted more and more," he says.

"But for a long time, I didn't work on MND at all. I tried really hard at the start of my career to be a scientist, not someone who had MND in their family. I wanted to get the skills and training, build up collaborations and respect, so that I was taken seriously."

^{Motor neurones carry messages to our muscles so that we can move, swallow, talk and breathe. As these cells die and muscles waste away, MND insidiously robs people of their strength, mobility and independence.}

"Research is a slow grind"

When his younger sister died aged 27, Justin had just finished his PhD. He was studying molecular chaperones which lend a helping hand to the millions of proteins bouncing around inside each cell in our bodies. Proteins need to be assembled precisely so that they are fit to function, but in MND, like other neurodegenerative diseases, misfolded proteins aggregate and become toxic to cells.

"If protein aggregation is a problem, then chaperones are an interesting thing to study so that's what we did," Justin explains. "No one [at the university] was doing MND research but I could see where we might be able to go.

"Those were shitty days," Justin says of the time when his sister was sick and he wished the field was further ahead. "But I understood fairly quickly that research is a slow grind. All you can do is contribute and hope that it pushes the field forward." Our understanding of MND has come a long way since, the field growing in size and momentum, but there still many questions begging for answers, particularly about the early stages of the disease.

"Apparently, when you're diagnosed, you've already lost half of your motor neurones," Justin says. "And we only have 200,000 motor neurones to begin with - that's a drop in the ocean with the billions of cells in your brain," he continues, quietly in awe of the tangled web inside our head and what we have discovered.

This, from a man who has recently been diagnosed with MND himself.

"The story that I’m trying to tell is that anyone can do this. For people and students out there, if you set your mind to it and work hard then the sky is the limit."
- Justin Yerbury

"I'm towing a jumbo jet"

Justin's family history forewarned what a genetic test would confirm, a few years back, that he too was carrying the faulty gene. Now, it has surfaced. First in his right thumb, then it sidled into his shoulder, and for a while he could walk holding his stray arm with the other. Eighteen months later, he sits in a wheelchair. His steely attitude hasn't changed.

"It felt like a change in gear. I was towing a bus before; now I'm towing a jumbo jet. [With the diagnosis] I knew it was going to be slower and harder but my mindset didn't change. To keep going, seemingly at the same pace, was therapeutic to me and a bit of normality for everyone."

For Justin, knowing so much about MND - the gritty details of the path that lies ahead, but more importantly, who to contact, which clinical trials are available and that there are many research groups pushing the envelope to understand more - has helped in a way.

"It makes me feel a little bit more in control. I've been there at the end [with my family], it's devastating. Had I known no science, I think [the diagnosis] would have been scarier. Knowing the best clinicians has been a big benefit to me." Justin has been the first person in the world to trial an experimental drug called copper-ATSM at a therapeutic dose. There is still no cure or effective treatment for MND.

"My own personal condition is inextricably linked to the research that we are doing," Justin says. "Of course, there has always been that motivation of MND in my family but from very early on I've tried to keep the two things very separate - but it's impossible now."

Even still, Justin doesn't dwell on the past. He stops to reflect on the steps he has taken, what he has learnt, and of course, to remember his family in quiet moments. But his gaze is set firmly ahead.

^{"Sometimes I wonder if it is fair for me to drag everyone along on the ride - I think about that all the time. Staff and students are going along this journey with me but it's not something that they signed up for - but then I think, why should I sit at home and do nothing because I'm in a wheelchair?"}

"Let's get to work today"

Justin gets to work every other day. He is busy meeting with students and colleagues, entrusting the future to those around him and in this way, he will still have his part in that research, too. He is engaged and focused, forever asking questions and adding ideas. After one of our conversations, his next appointment is with a visiting collaborator who has been in town for a conference the week before. They talk incessantly for two hours.

Decades ago, scientists had to focus on one protein of interest, one culprit in disease, for simplicity, but now, Justin says, "We're asking questions about how the cell manipulates millions of protein molecules. You take one out, or add one in, and the whole system changes. It's mind-boggling."

He loves the hard science, but it's the people that Justin works with that keep him coming back to the office. "Without them, it would be so hard for me to get here every other day. But it's such a pleasure to be here that I think, 'Let's get to work today'." He talks about his colleagues, in Wollongong and abroad, as they would describe him: a pleasure to work with, someone who always makes time for others, and thankful that they have taken him under their wing.

After his PhD, Justin studied at the University of Cambridge under the illustrious Christopher Dobson FRS. The college town which stands tall over the River Cam quickly became Justin's second home. He has lived with his family in Cambridge on-and-off for ten years, returning to foster collaborations and friendships.

"It's a special place for us," Justin says. "We could have been there for five, or ten, or twenty years. It's a place that - had I not been diagnosed with MND - eventually, Rachel and I could have returned to. We could have packed up, left the kids in the house here, and lived there for a number of years."

Justin met his wife Rachel at a New Year's Eve party when they were seventeen. Mismatched in height but not in ambition, Rachel is part-way through her own PhD looking at how connecting with nature can improve human well-being, but her studies have been sidelined to care for her husband. Justin and Rachel share a love of the outdoors and have two daughters, Talia and Maddison, who each carry with them a thread of their parents: Talia is studying floristry and Maddy has chosen biology.

"I'm trying to take inspiration from Stephen Hawking."
- Justin Yerbury

On their last trip to Cambridge, in April, Justin and his family had the chance to meet the late Stephen Hawking, the theoretical physicist whose name is synonymous with black holes and the expanding universe. Hawking's brilliance was formidable: "We all hid behind Chris [Dobson] when we walked into the room," Justin says. Despite being diagnosed with MND in his 20s, Hawking continued to publish grand theories about the universe and his work is still making waves decades later.

"I said to him, 'You know you've caused me a lot of trouble? MND is this terrible disease, but I'm always asked why you have survived longer than anyone else.' You know what I think it is?" Justin continues, "I think there's this point for people living with MND when they say enough is enough. Every breath is a struggle. But Stephen was able to move beyond that; his mind was in the cosmos.

"Sometimes I wonder if it is fair for me to drag everyone along on the ride - I think about that all the time. Staff and students are going along this journey with me but it's not something that they signed up for - but then I think, why should I sit at home and do nothing because I'm in a wheelchair?

"I'm trying to take inspiration from Stephen Hawking," Justin says. "To meet him and see that even in his condition you can still be a part of society, part of an academic community, contributing, writing books and memoirs. It's amazing. He was cheeky - even after all this time. You couldn't read much into his face, but when he smiled you knew about it."

^{He loves the hard science, but it's the people that Justin works with that keep him coming back to the office.}

"I don't know I could have done it any other way"

Justin has his smile too. It's bright behind his eyes. And with his eyes, Justin can command his computer, share ideas and advise his students. Instead of using a mouse with his laptop, a camera mounted above the keyboard tracks his gaze and voice recognition software sends out emails. More recently, a crown-like headpiece installed on his electric wheelchair means that Justin can take back control - "I'm self-propelled!" - and soon he is zipping up and down the corridor to surprise his students. That was a good day.

Sadly, each new addition has also been a marker of his declining strength but the suite of adaptive technologies enables Justin to continue his work. In the same year, he has co-authored fifteen scientific papers, guided two PhD students to graduation, supervised a handful of others, and received the MND Australia Betty Laidlaw Prize for excellence in research.

His days are getting shorter, a few hours at work at most, but no less busy. Visitors stream through Justin's office and laughter overflows into the hallway. Conversation may be slow, every sentence broken by short breaths, a ventilator clipped onto his nose, but people listen closely.

To live the way that Justin does despite his harrowing circumstances - to hold onto ambition, to channel your energy into the people around you, and to be supported and empowered by those you love - is a life to aspire to.

"The person that you are is not determined by those circumstances but the way you deal with it," Justin remarks, nodding his head toward the note on his pinboard. "That resonated with me. I think if that mindset had changed, I wouldn't still be sitting here. I don't know I could have done it any other way.

"I've been reluctant up until now to share my story but I feel like now is the time. If not now, it's never. But the story that I'm trying to tell is that anyone can do this. For people and students out there, if you set your mind to it and work hard then the sky is the limit."

Justin Yerbury featured in ABC's Australian Story on Monday 26 March, 2018.

• To donate to Justin's motor neurone disease research click here and select Motor Neurone Disease Research in the "Designation" drop-down menu.