Motor neurone disease: Sheffield's Dame Pamela Shaw on a life treating a 'devastating' condition

At primary school in Kuwait, Professor Dame Pamela Shaw joined her classmates on a trip to a hospital in the capital and saw something that changed the course of her life – a boy of about 10 having a technetium brain scan.

Her fascination was the spark which led to a life’s work in neurology, with a specific dedication to helping people with motor neurone disease (MND).

It’s a progressive condition affecting the brain and nerves - leading muscles to weaken, stiffen and waste – whose profile has grown in recent years with media attention on sports stars including former Leeds Rhinos player Rob Burrow, who was diagnosed in December 2019.

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But few can have had the impact on improving the prospects of those with MND as Dame Pamela, Professor of Neurology and Director of the Sheffield Institute for Translational Neuroscience (SITraN), based at the University of Sheffield.

Professor Dame Pamela Shaw with the late Queen, who opened the Sheffield Institute for Translational Neuroscience (SITraN) in November 2010.Professor Dame Pamela Shaw with the late Queen, who opened the Sheffield Institute for Translational Neuroscience (SITraN) in November 2010.
Professor Dame Pamela Shaw with the late Queen, who opened the Sheffield Institute for Translational Neuroscience (SITraN) in November 2010.

“I found neurology fascinating,” she says. “And really it was when I started training in neurology as a junior doctor, I just thought MND was the worst disease in medicine. It always seemed to happen to the nicest people, and it really made me feel like a bit of a useless doctor because we didn't do anything for anybody in those days, really, or their families, and we knew nothing about what caused it. We had no treatments. I thought it was a neglected condition so I thought in this day of major medicine, we should really be trying to do something about this. It's been a long, slow process, but you know, I think we're making some progress.”

While in Kuwait (where her father was a marine pilot and harbourmaster) the family had always kept a house in Northumberland, and when she returned for secondary school, her interest in science continued. She was moved up a year “for no better reason than they couldn't fit enough desks into the classroom,” she says. “So when I did my A levels, I had a year in hand. I did them a year young. And so I then went off to another school in Northumberland and did a crash course in physics and chemistry.”

Later she went to the University of Newcastle, where she earned a first in medicine in 1979. She then undertook her specialist training in Neurology, and in 1988 was awarded an MD with commendation for her work on the neurological complications of coronary bypass surgery.

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It was there that she first developed her interest in MND and in 1997, she was appointed Professor of Neurological Medicine at the University of Newcastle.

Dame Pamela Shaw shows the Queen around in 2010.Dame Pamela Shaw shows the Queen around in 2010.
Dame Pamela Shaw shows the Queen around in 2010.

Supported by Senior Fellowship and programme funding from the Wellcome Trust, she set out to establish the team and research infrastructure needed to investigate the neurochemical, molecular and genetic factors underlying MND, which included the first national Care and Research Centre for Motor Neuron Disorders in 1992 in Newcastle.

She was a leading researcher in evaluating the drug riluzole - the only drug licensed for treating MND in the UK - in the pivotal clinical trial. care commissioners. It is the only drug licensed for treating MND in the UK.

Dame Pamela also instigated research to investigate the effects of non-invasive ventilation on life expectancy and quality of life in MND patients.

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She says: “I think we have made some difference to life expectancy, especially with non-invasive ventilation when the breathing muscles start to significantly fail. If you don't do something about it, you know, people will die from failure of the breathing muscles, so it certainly made a difference. But I don't want to take too much pride in it because we haven't done enough to make it less than a devastating disease.”

In 2000 she was invited to become Professor of Neurology at the University of Sheffield and establish academic neurology, continuing to build up the MND care and research programmes.

A patient from London once asked her what she would do if she had £20m to spend on treating motor neurone disease.

Dame Pamela says: "I thought this was just a dream-like jokey conversation...I thought what would I do if that miracle ever happened? I actually described SITraN. So I said: ‘I would build a dedicated research institute that would have multi-skilled teams of scientists and clinical doctors, neurologists in the same building talking to each other. And it would be a nice building with lots of light coming into it, so it was nice place to work so that attracts great people from all over the world to come and help us solve the disease’.”

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That patient vowed to help Dame Pamela actually do it, putting her in touch with people who could raise funds for such a place. Contributions came from many people including a particularly “huge” sum from a man who asked to remain anonymous but who Dame Pamela calls the “Yorkshire White Knight”.

Queen Elizabeth II and the Duke of Edinburgh opened SITraN in November 2010. They had been invited by the Duke of Devonshire, of Chatsworth, who had taken a keen interest in the building, and Dame Pamela had originally asked him to open the facility.

“I plucked up my courage to ask him, when the building was completed, would he do us the honour of opening it for us and he said no. I thought oh gosh, I’ve breached protocol, I shouldn’t have said anything. I thought he was cross at being asked. But then he got a twinkle in his eyes, he said: ‘No I won’t do it but I’m going to find you someone much more important than me’.”

Reflecting on the Queen’s visit, she says: “I got some really good background information from a professor at Oxford University. I had invited him to come to the opening and he said: ‘Pam, the Queen opened a building for us just a few months ago and what I learned from that is that we kept her standing for too long and she was uncomfortable.’ When she came to us I thought we mustn’t keep her standing for too long. And she liked to go down the stairs rather than in the lift and so I was accompanying her down the stairs, I plucked up my courage and said: ‘We’ve got four little stations to show you the research that we’re doing, but you’ll only be a couple of minutes at each one, but I wondered if you might like to sit down rather than standing all that time’. She was really pleased at that, I think it made it feel much more comfortable for her. So that was a very good tip from my friend in Oxford.”

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It was not her only brush with royalty, as she received her Damehood in 2014 and attended a ceremony conducted by Princess Anne.

Dame Pamela has also identified the tendency of MND to occur in athletes, believing strenuous physical activity can be a cause when combined with a certain genetic background.

It is an issue that has received attention in recent years with the MND diagnosis of rugby players Rob Burrow and the late Doddie Weir, as well as former Bradford City footballer Stephen Darby.

She says: “Particularly when your speech is affected and it’s difficult to communicate, it takes real courage to show the world what you’re suffering from.”

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Dame Pamela praised Burrow and fellow campaigners for putting pressure on the Government to release £50m over five years for research funding.

Meanwhile, there was a breakthrough last year with a clinical trial of the drug Tofersen, which showed it can slow and reduce progression of the disease in patients with MND caused by the faulty SOD1 gene.

She says: “It was really exciting. I've done 22 trials for MND and this is the first one where patients were reporting to us, spontaneously, ‘I haven't changed a jot since I went on to this treatment, I've been perfectly stable’, or even some patients reporting improvement in their muscle strength.”

Dame Pamela, who is married to husband Paul and mother to daughter Sophie, is optimistic that one day a cure will be discovered, but thinks sub-categorising MND could be key.

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“I think we’ve got to learn from oncology, from cancer medicine. If you think about cancers like breast cancer, at one time that would have just been treated as one thing and we would have missed effective drugs. Herceptin, for example, works in a particular sub-group of patients with breast cancer, but if you tested in a trial that includes all breast cancer patients, you wouldn’t see the benefit.”

She adds: “I really do think, if you look at the disaster of the Covid pandemic, if you look at what a disaster HIV/AIDs once was...with focused funding and attention, you can work miracles.”