Australian doctors are trialling a new drug they hope will help slow the progress of motor neurone disease, which is on the rise.

Before motor neurone disease struck him in his mid-40s, Peteris Ginters was a successful barrister, keen runner and cyclist. Credit:James Brickwood Normal text size Larger text size Very large text size The grand, high-ceilinged banquet hall of the Marigold restaurant in Sydney's Chinatown is a whirlwind of waiters, food trolleys and never-ending dishes as Sunday yum cha gets into full swing. Sitting at a large round table with his wife, Caroline, and their two children, aged 13 and 11, Peteris Ginters picks up his chopsticks and reaches over to pluck a prawn dumpling from a serving dish. The 44-year-old barrister finds himself chopstick-challenged as he struggles to apply the small pincer-like pressure between thumb and index finger required to pick up the dumpling. Taken aback – he's never had difficulty with chopsticks before – he winds up skewering the dumpling and dropping it in his mouth. A few days later Peteris (pronounced "Petris") is emptying his dishwasher when a dinner plate slides out of his grip and smashes on the tiled floor. He dismisses it as a moment of uncharacteristic clumsiness. Over the next couple of weeks, he finds he can't turn the key in the heavy wooden door of his law chambers, nor fasten his top buttons for court without assistance. He's mystified about this worsening weakness between the thumb and index finger on his right hand. And what's with his handwriting? He's been using a fountain pen since he was a teen – while his cursive was never elegant, his sentences are now dissolving into an indecipherable squiggle. It's late August 2016. Only recently, Peteris had cycled the 40-kilometre Akuna Bay West Head loop with his mates, which he did most Sundays. Always supremely fit – exercise was his means of combating the brutal stresses of the courtroom, and he also jogged regularly with his colleagues – he'd suffered his share of bruises and strained muscles over the years. Thus, when he feels an odd heaviness in his thighs, he chalks it up to a pinched nerve from one of those early-morning bike rides. With the symptoms in his hand and legs getting worse, Peteris finally visits his GP, who sends him off for an MRI brain scan. It comes back all-clear, but the GP is troubled by Peteris's mysterious muscle weakness. "You need to see a neurologist," she advises, writing him a referral. And so, on the sunny spring morning of November 7, 2016, Peteris finds himself on the M1 motorway driving towards Macquarie University Hospital, Caroline beside him in the passenger seat. Still convinced it's all related to a pinched nerve in his back, he's only attending this appointment at her urging (she'd looked up the neurologist's areas of expertise – Parkinson's disease and motor neurone disease [MND] – with some alarm). Peteris can only think of all the preparation he has to do for an appeal tomorrow. But he has a premonitory aww f... moment in the waiting room, when he spies a motor neurone disease poster on the wall. Professor Dominic Rowe, a youthful-looking neurologist with a shaved head, barrel chest and bow tie, greets the couple and ushers them into his corner office, a casual, welcoming space with a Sherrin football and teddy bear perched on the window sill and sunlight streaming in. Rowe, 53, gets swiftly down to business; after Peteris runs through the set of strange symptoms, he asks him to remove his shoes for some strength and nerve tests. Rowe quietly watches Peteris as he fiddles awkwardly with his shoelaces and socks. On the examination table, he asks Peteris to push his hands as hard as he can against Rowe's, and to do the same with his feet. Things seem pretty normal to Peteris as the doctor bounces a weight on his knees, so he's surprised when Rowe says his responses are hypersensitive. Finally, Peteris's strength of grip is measured with a dynamometer.


While Peteris puts his shoes back on, Rowe pins the MRI scans on to his light box. "The radiologist's report says it shows nothing," says Peteris casually. "That's not unusual," interjects Rowe without turning. "Sometimes changes can be very subtle." And there he sees it: dots that should be lined up are slightly out. He pauses. "I know this is a lot to take in, but I have absolutely no doubt you've got the most common form of motor neurone disease, sporadic amyotrophic lateral sclerosis [ALS]," he says. For a moment Peteris and Caroline sit in stunned silence, processing those three words – motor neurone disease – knowing that in an instant, their universe has changed forever. ALS, known in the US as Lou Gehrig's disease after the former New York Yankees baseball star who died of it in 1941, is a progressive neurodegenerative disease that erodes motor neurons – cells in the brain and spinal cord that control muscular function – until it becomes difficult or impossible for a person to walk, talk, speak, swallow and breathe. ALS often begins with subtle muscle weakness in the hands or legs, creeping up like a stealth assassin as it wipes out hundreds of millions of nerves, leaving muscles to wither from being powerless to move limbs. "I'm happy to refer you for a second opinion if you'd like one," Rowe adds gently. But Peteris instinctively trusts this doctor who so frankly, but compassionately, calls it as he sees it. "Life expectancy?" Peteris asks quietly. No two sufferers of ALS are exactly alike, answers Rowe, and there's no way to accurately predict how fast a sufferer's disease will progress. The grim truth is that ALS can move swiftly, one of the most traumatic aspects of the disease. Some can be on a cane within a month of diagnosis, which can turn into a walker and a wheelchair shortly after that. In others it can progress much more slowly, beginning with cramps, twitches and slurred speech. But he's sorry to say that on the list of diseases with the worst of prognoses, MND is near the top, with 75 per cent of the newly diagnosed dying within two-and-a-half to five years. At this point, Peteris, who is not a crier, breaks down, his shoulders heaving. Rowe steps forward, and gives him a big hug. "It's a f…in' bastard of a disease," he says kindly.


But, he adds, Peteris is still in the early stages and while there's no cure, it can be managed with medication, physical therapy and speech therapy. The standard treatment for ALS is the drug riluzole, which can buy a little bit of time for some. But there may be a better way of slowing the disease down, Rowe adds – a very promising one being trialled here at Macquarie University. If he's willing and able, Peteris may be a candidate. On the drive back to the city, with the inevitable "What now?" questions thundering through their minds, Peteris and Caroline begin drafting a plan. They agree on the painful decision to close his legal practice immediately. He will wrap up the appeal tomorrow and pass his caseload over to other barristers. More traumatic will be how to prepare his son and daughter, two sisters and parents for the news. But first, he has some urgent business to attend to. He asks Caroline to drop him off at Martin Place, where he walks to the Channel Seven building and takes the lift to State Chambers on the 36th floor. Fiona Hayden, clerk of the chambers, takes one glance at the usually chirpy Peteris as he walks through the glass entrance doors and knows something awful has happened. "Are you okay?" she asks. Peteris is due to sign on the dotted line for the purchase of new chambers – a single room on this floor – and wants to let the owner, who is in ill-health himself, know he can't proceed. "I've just been diagnosed with motor neurone disease," he tells Hayden with a trembling voice. Moved to tears, Hayden gives Peteris a heartfelt embrace. The barristers on the floor had all been looking forward to Peteris joining them here. "Is there anything I – or any of us – can do for you?" she asks.


"That's kind of you, but no," replies Peteris, batting away fresh tears. And with that he leaves the offices, his 18-year career as a barrister now over. MND specialist Professor Dominic Rowe, who’s treating Peteris. Credit:James Brickwood It's April 2018 and Rowe is standing in his office doorway, preparing for a busy Wednesday in what is the largest MND research facility and multi-disciplinary clinic in the country. Today, among his list of patients at various stages of MND, he'll be seeing a former sexual assault counsellor, a former Crown prosecutor and a former model. "If you were to design the cruellest of diseases imaginable, this would be it," says Rowe. "It robs you of your ability to walk, to use your hands, wipe your bum, scratch your nose, swallow, hug your loved ones. Yet it leaves intact all your intellect and your special senses – sight, hearing, smell – so you're able to watch your body waste away." What is it about the neurons controlling eye movement that makes them so resistant to ALS, a fact that enabled the late physicist Stephen Hawking to communicate? "The cells in the eyes, and the bladder and bowels, have different buffering capacities, more resistant to the process that destroys susceptible cells," he explains. Understanding how these cells stave off death may help scientists understand long-term survival. Rowe's longest surviving patient with ALS has had the disease for more than 30 years, but he had one patient, a mother of two in her 40s, who died within two months of developing her first symptom – a weak ankle – while on an overseas trip. "It's this unpredictability that makes ALS one of the most feared diagnoses in medicine," he says.


Many will be surprised to learn that the number of MND diagnoses is as high as multiple sclerosis; the reason MND isn't as prevalent is that sufferers don't live nearly as long. At any one time, 2000 Australians have MND – 60 per cent male and 40 per cent female – and about 800 die each year. To put that number into context, the national road toll each year is around 1200. "The incidence [in cause of death] has almost tripled, from one in 500 in 1985 to one in 180 in 2014," notes Rowe. "Some argue that it's because of better diagnosis, but the diagnostic tools were the same in 2014 as they were in 1985." "Part of that initial shock is 'This isn't supposed to happen to someone like me,' but of course it can happen to anyone. Credit:James Brickwood The mystery at the heart of MND is this: what is the biological spark that sets off the deadly dance of nerve destruction? The human brain has about 100 billion nerve cells, which send signals to the muscles at speeds of up to 300 kilometres an hour. ALS is about the most pernicious form of MND, killing both the upper and lower motor neurons. About 10 per cent of sufferers have a family history of the disease, but for the vast majority the unknown cause is some complex interplay of genes and environment. As he moves from room to room seeing patients, Rowe reminds me of an old-fashioned family doctor. With a warm, solicitous style, he frequently befriends the people in his care. "It's important to tell people the truth, not to withhold anything that they'll be dealing with," he says. "By the same token, you don't have to be some brutalist deliverer of the shit storm ahead." Rowe is known for his salty language, and on the morning I visit his clinic a patient hands him a swear jar. "Five bucks for every time I swear," he smiles. "But I defy anyone who works with MND not to swear." The jar already contains $10. He has no patience with specialists who sit in their ivory towers and dole out impersonal medicine, nor does he have any truck with sugar-coating the truth. "Tomorrow is the first anniversary of my dad's death from renal cell carcinoma," he reveals. "He got sick four months before he died, and not one f...ing doctor involved in his care told him he was going to die. My dad asked me, 'Is this survivable?' and I told him no." Rowe is the principal investigator in the trial of a new drug for MND, Copper ATSM (Cu-ATSM), which began in late 2016. While it's no cure, and cannot reverse pre-existing nerve death, the drug could be the most significant breakthrough yet in slowing the merciless march of the disease. "We've finished one trial and we're in the middle of another trial of therapies that will dramatically slow and eventually stop MND, so we have rubber on the road for the first time," Rowe enthuses. The research, an Australian-first, involved the collaboration of a trio of professors from the University of Melbourne (Associate Professors Anthony White, Kevin Barnham and Paul Donnelly).

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