Motor neurone disease: ‘If you were to design the cruellest of diseases, this would be it’ – The Northern Daily Leader

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

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.

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