Professor Dame Pamela Shaw

0:00Hello, I'm Lauren Laverne and this is the Desert Island Discs podcast from BBC Radio 4. Every week I ask my guests to choose the eight tracks, book and luxury, that they'd want to take with them if they were cast away to a desert island. For rights reasons, the music's shorter than on the original broadcast, but you can find a version with longer music tracks on BBC Sounds. Listeners will also get access to episodes 28 days earlier than everyone else. I hope you enjoy listening.

0:45My castaway this week is the neurologist Professor Dame Pamela Shaw. Her pioneering medical career has been spent tackling what she calls the worst disease in medicine, motor neuron disease or MND. Her interest in neurology was sparked by a hospital visit as a girl growing up in Kuwait in the 1960s. She witnessed a young boy undergoing a brain scan. She first encountered MND as a young doctor in the late 80s. Frustrated that so little could be done to help those who'd been diagnosed, she pledged to dedicate her working life to making a difference. She's the founder and director of Citran, the world-leading Sheffield Institute for Translational Neuroscience, which is slowly but surely unlocking the secrets of this and other devastated neurological conditions.

1:34They recently developed a groundbreaking treatment, a drug that can actually affect the progression of MND in certain patients. She says, I've never been bored in my career. Combining research and clinical work gives you enormous variety and the opportunity to make a real difference. There's nothing more satisfying than making a discovery and seeing it translate into something that genuinely improves human lives. Professor Dame Pamela Shaw, welcome to Desert Island Discs. Thank you Lauren. So Pam, take me back to your early medical career then. What made you want to focus on MND? I always knew, even when I was still at school, that I was interested in the brain and the nervous system.

2:18And when I was a trainee neurologist, helping with clinics and so on, just something about the plight of M &D patients really got to me. It made me feel like a bit of a useless doctor, really. What were things like back then? What sort of prognosis did people have? There were no drugs of any sort to help protect the motor neurons. We didn't even intervene very well with troublesome symptoms like swallowing difficulties or breathing difficulties. so I used to almost dread getting a set of notes for an MND patient because I felt I could do so little to help. It feels like these days motor neurone disease is much more visible than it used to be.

3:05I mean, you know, obviously Professor Stephen Hawking lived for many years with the condition and then we had rugby players Doddy Weir and Rob Burrow who shared what they were going through. Former Castaway Kevin Sinfield, you know, a good friend of Rob who's done so much fundraising and awareness raising. How do you feel about that change, about it being in the spotlight? Those high-profile sportsmen, it's so courageous of them to show their symptoms to the world when they have been such powerful athletes themselves. But they've done an awful lot to raise awareness and their families have as well.

3:41I mentioned that, you know, how much you said you enjoy being both a clinician and a researcher. Why is that? What kind of balance does that bring, seeing patients but also spending time in the lab? If you're a researcher as well as a clinician, what you're trying to do is harness your research to do some good in the clinic. And that's why our research institute at the University of Sheffield is called the Translational Neuroscience Institute. So we're trying to harness all the exciting scientific discoveries in neuroscience and translate those into benefits for patients in the clinic. And how big is your team there now?

4:23Oh, there are about 300 researchers now at Citran and we're full. So we're in the middle of building a sister building joined on to Citran. So we'll double our capacity. That new building will be ready in October time. Are they going to name it after you? No, no, no, no, no. Just Citroën. Why not? Pam, come on. No, no. Well, you have had several honours, including being made a dame in 2014. Is that a title that gets used in the workplace? To be honest, it's a very teasable thing. So people use it cheekily, do they? Yes, they sing into my voicemail, you know, there ain't nothing like a dame, that kind of thing.

5:09So, no, usually at work, I'm just Pam. And what about your title? How did you find out that you'd been selected? I was at a conference at the Royal College of Physicians and my husband sent me an urgent, urgent text. And I thought, oh, my goodness, what's happened at home? The house is on fire. My dog's died. So I went out of the lecture, took the message and Paul, my husband, said, Pam, there's a great big envelope here. had just come in the post from the Prime Minister's office. Can I open it? He said. So he did open it and it was absolutely, you know, I was completely flabbergasted. I had no idea that that was going to happen.

5:54Well, well deserved, as we'll hear today. And of course, we're going to hear your music too, Pam. How's it been putting your discs together? So I like all sorts of music, but the ones that I did choose have some special meaning. so it wasn't too hard really to pick them out. Well, tell us about your first then. What's the story behind disc number one? It's the Hallelujah Chorus by Handel and I go up to my study and play it whenever there is some momentous, joyful thing that's happened in life. Whether it was, you know, getting first class honours in medicine from my unconventional educational background, my daughter doing so well in her psychology degree.

6:38Sometimes when my husband wins a golf match, we play it. But also in terms of work, one of the fantastic things is the emergence of Tofferson as a treatment for at least a subgroup of patients with MND. So that's a world first, isn't it? It's an absolute game changer. This is the first time actually a good proportion of patients, they either stabilise and don't change over a period of years, but some of them actually recover muscle strength. So it's a really big moment in terms of MND research.

7:33Alleluia, alleluia, alleluia, alleluia. For the Lord God, give us a chance to reign. Alleluia, alleluia, alleluia, alleluia. For the Lord God, give us a chance to reign. Alleluia, Alleluia, Alleluia, Alleluia.

8:20Handel's Messiah performed by the London Philharmonic Orchestra and chorus conducted by Douglas Gamley. For medical breakthroughs and for your husband, Paul's Hulls in One. So, Pam Shaw, let's go back to the beginning, shall we? You were born in Rothbury, Northumberland, in April 1955, the second of four children to Robert and Valerie Shaw. Tell me about your mum and dad. How did they meet? So they met in Morpeth, which is a market town in Northumberland. My mum was a nurse. She ran Morpeth Cottage Hospital, so she was the nurse in charge of that. Did she enjoy doing that? She did, yes, she did, although they had four children, so she didn't carry on working really once my older brother was born.

9:08My father, when they got married, he was a captain in the Merchant Navy. Just a lovely character. Everyone liked him, very easygoing, very much a family man. And because of his long sea journeys, he didn't see his baby daughter till I was six months old. Oh, my goodness. And that's, I think he decided that wasn't the life for a family man. So he went to Kuwait and used his maritime skills. So he was a marine pilot piloting oil tankers and then ran the harbour as harbour master. But that meant he could be at home. OK, so this is the big move that your family made. You were about three, I think, when in 1958 you moved to Kuwait, which from Morpeth in Northumberland is.

9:56I mean, you couldn't get more different. It's a big move, yes. It was a really nice life to grow up in Kuwait, very much an outdoor life, lots of swimming and beaches and sailing. And the town we lived in called Ahmadi, the sector of it that we lived in, it was all British, European, American people who were working in Kuwait. So there was a very good social life. We used to celebrate New Year, Kuwait time, UK time, US time. To really stretch it out. My only regret is I didn't train myself to speak Arabic fluently. And actually, the local Kuwaitis were always very keen to practice their English.

10:43So if I tried to say something in Arabic, they would just take over in English. I think you also joined the Kuwait branch of the Brownies, didn't you? I did, yes. I was a sprightly sprite with a green badge in the brownies. But I loved the brownies. They taught you things that girls would never really learn. You know, how to tie different types of knots and how to set a table properly and all sorts of useful things I learned in the brownies. Your dad was very easygoing, so he would have adapted to this new adventure quite well. What about your mum? What kind of character was she and how did she adapt to Lysa?

11:21So my mum, very kind person, very well supported by my dad. I mean, my dad devoted his life really to, you know, making my mum's life as happy as he could. And did she tell you much about her former life as a nurse? She did. She used to tell stories. In a way, it was my mum that got me interested in medicine. and so when I was at school I wanted to do subjects that would enable me to study medicine. So she planted that seed? Yes, she did. All right Pam, let's have some more music. It's time for your second choice today. What's it going to be? My second choice is a piece by Frank Sinatra. It just reminds me of childhood in Kuwait but also in Northumberland so my parents would come home to Northumberland to Morpeth every year.

12:13They kept a place there, didn't they? They kept a place there and they would come home for six weeks every year. And my mum in particular just loved Frank Sinatra. I know I stand in line until you think you have the time to spend an evening with me. And if we go someplace to dance, I know that there's a chance you won't be leaving with me. Then afterwards we drop into a quiet little place And have a drink or two And then I go and spoil it all By saying something stupid like I love you Frank and Nancy Sinatra with Something Stupid. Pam Shaw, you just shared the loveliest memory with me. Tell us about it of your mum when listening to that track.

13:06Yes. So when my parents were in their early 80s, their health became quite frail. And we as a family, including my parents, decided it would be best to move them down to near where we were in Sheffield to sheltered accommodation where they could have a bit more ongoing support. so my daughter Sophie and I went up to Northumberland to pack up my parents house getting my mum to choose which things she wanted to bring with her and she was wandering around with a smile on her face gaily unpacking what Sophie and I had packed but humming Frank Sinatra and I think she was humming I'll do it my way Oh, Blassa.

13:54All right. So, Pam, your own interest in medicine was sparked by your mum, but also by an experience that you had in Kuwait. You went to the Anglo-American school in Akhmadi, as you mentioned. Tell us a bit about what it was like. It was a great school. We did lots of sports. You know, we were taught to swim. It was a very good school. And it was a trip to a hospital that really sparked something in you. They took us on a school trip to the big hospital in Kuwait City. And I saw a young Kuwaiti boy who must have only been 10 or 11 years old. And he was having a technetium brain scan where they do an injection of a radio isotope.

14:39It gets taken up by the tumour. So this young boy had a tumour inside his head. And they showed us the scans of this. and I just thought, gosh, that's amazing. So it did hit a chord, sparked an interest in me for sure. So you loved your primary school, but you couldn't stay beyond the primary. There was no real secondary education for overseas kids, so we all had to come back to boarding school at the age of 11. So I went to a school in Annick in Northumberland, just next to Annick Castle. Beautiful part of the world. they filmed Harry Potter. And I went there because some of my friends from Morpeth attended that school as day girls, but it had a boarding facility.

15:27So I was a boarder. How did you adapt? I was okay with it. I had lots of friends. I was keen on my schoolwork. So it wasn't too much of a problem for me. We would go out to Kuwait for Christmas and summer, and then my parents would usually be home for Easter. So quite long chunks where you were together. Yeah, just the school term. And what about the basics? I mean, how was it being back in chilly Northumberland after swimming and playing tennis in air-conditioned Kuwait? When I was about eight years old, I did jump into the North Sea expecting it to be like the Persian Gulf. I've never liked cold water plunges ever since then.

16:11So, yeah, none of that for you. No cold water swimming for you. No. And you knew quite early on that you wanted to study medicine, but you had some challenges with A-level biology. What happened? The school I went to was wonderful for the arts, for languages. I did A-level French just for the love of it. It was my favourite subject. Music, drama, Gilbert and Sullivan operettas, wonderful for all of that. But the headmistress at the time did not think that young ladies really needed to know anything about physics and chemistry. And even biology, I really did A-level biology on my own. I took the syllabus and just studied from textbooks.

16:57The lovely teacher who took on biology was a trained cookery teacher. Yeah, no, I just took the syllabus and... And just learned it at all. Learned it, yeah. How did you get on then? How did you do in your exams? Yeah, no, I got A's in both subjects, but I knew I had a year in hand because they bumped me up a year. Oh, yeah. So they bumped you up a year just for the class size? Yes, they couldn't fit enough desks in the classroom, so they decided to bump me up a year. You sound quite a pragmatic bunch. Yes. In a way, that experience taught me to be self-reliant. If I really want to go to medical school, it's up to me to work hard and make that happen.

17:43So you knew you had this sort of extra year. Did you decide to do some extra study during that time? So I did those two A-levels and then I went to another school in Northumberland in a little place called Hayden Bridge. And I did a crash course in physics and chemistry. And the headmaster there was a chemistry teacher by background. And he really took me under his wing and helped me kind of learn those two subjects from scratch, really. So I was lucky. Pam, let's have some more music. It's your third choice. What are we going to hear? It's Joni Mitchell. I think one of her teachers, schoolteachers, described her as a painter with words.

18:26And if you listen to her songs, she really is. She talks about sunlight coming through windows like butterscotch and things like that. And I always used to play Joni Mitchell when I was about to go into the medical school for an exam and somehow it just sort of calmed me down. So I chose Both Sides Now from Joni Mitchell, just reminding me of those happy days as a medical student. I've looked at love from both sides now From give and take And still somehow it's love's illusions I recall I really don't know love At all

19:22Joni Mitchell and both sides now. So Pamela Shaw, you mentioned a love of Gilbert and Sullivan And I think it was that that clinched the deal for your place at Newcastle University, wasn't it? Yes, I think it was. I applied to Newcastle as my first choice and I was a bit unconventional. You know, I had loads of O levels and these two A levels, but not physics and chemistry. And I was interviewed by the professor of medicine and the interview was going OK. And then he asked me, and what do you like to do when you're not doing your schoolwork? And I said, well, every year at school, we have a Gilbert and Sullivan operetta.

20:06So I've just been pish tush in the micado and his eyes lit up. He loved Gilbert and Sullivan. So he smiled and the interview went very well after that. So I'm convinced that it was Gilbert and Sullivan that got me into medical school. Thank heavens for your pish tush, Pam. Yes, absolutely. So I know how grateful you've said you feel to Newcastle's medical school for taking a chance on you, as you put it. Did you make them proud with your results? Well, yes, I graduated with first class honours. And one of my professors told me that I was the best medical graduate they had had for 10 years. How did that feel?

20:48You must have felt a bit on the back foot when you joined. I didn't quite believe it in some ways. But, yeah. As well as enjoying all the rotations of clinical training, you did orthopaedics, obstetrics and everything like that, you also met your now husband, Paul. Yes. So Paul was, in the year above me, we used to have discos in the doctor's mess and I was helping behind the bar and Paul came to order a drink and he said, ooh, I'd do anything for you. and then a little while later he you know asked me out and it went from there. You also shared a mutual love of flowers that I think still endures.

21:35Yes when I first went back to his flat he had this red painted windowsill and window frames and it was covered with fuchsias from his dad's greenhouse so I thought gosh he likes plants I I like plants. And, you know, we still do. We have a big garden, one and a half acres. Oh, wow. I love roses. So we have a lot of roses. Paul does, while I'm still busy at work, he does most of the heavy lifting in the garden, but I do look after the roses. So you graduated, Pam, in 1979. Tell me about life as a junior doctor. It was very busy. I was based in Newcastle. I did a year, for example, at Dryburn Hospital in Durrance.

22:20So I went as a medical registrar to Durham and then came back to Newcastle. So when I was training in neurology, we were doing one night in two, one weekend and two on call. And we had the only CT scanner in the whole region. So the whole of the northeast. Well, the whole of the northeast and across to Carlisle, Cumbria, up to the Scottish border. Oh, wow. So the on-calls were really busy because you'd get physicians ringing from Carlisle saying, I've got this patient, I think he needs an urgent head scan. So it was really busy for four years doing that training, but really good training. You were going great guns yourself.

23:02Obviously, you were well supported. But in 1988, you wrote a paper on the neurological complications of cardiac bypass surgery. And that actually resulted in a change in clinical practice, I think. Yeah. We were repeatedly being called to the cardiothoracic unit in Newcastle, which was at a different hospital, Freeman Hospital, to see patients who'd had coronary artery bypass surgery and had some neurological problem postoperatively. So I did my higher degree, my MD, studying 300 plus patients before and after their heart surgery. What did you find out? One of the things was little emboli that caused blockages in the small vessels in the brain.

23:50And when the patient's heart stopped and they're having the surgery, you get oxygen into the blood of the patient in different ways. And there were membrane oxygenators and bubble oxygenators. And bubble oxygenators were much more likely to cause these little circulatory problems within the brain. So actually, you know, describing the spectrum of disorders and their recovery caused people to move away from the bubble oxygenator. It raised awareness. And the cardiologists, cardiothoracic surgeons at Freeman Hospital, you would imagine they might be a bit put out by this whippersnapper of a young neurologist coming around looking at their patients.

24:38But actually, they weren't. They were really kind and very interested in the findings. Fantastic. All right, let's have some more music, I think, Pam. It's your fourth choice today. What's next and why are you taking this to the island? My next piece is the clarinet concerto in A major from Mozart. I first heard that, I think, in the 1990s, and I went to a motor neurone disease conference in Berlin and the person hosting the conference took us to this little theatre which was oldie-worldie, all wooden panels and so on and to a live performance of this piece of music and I was blown away by it.

25:21It made my spine tingle. The clarinet and the way it goes from high notes to low notes, it's just about my favourite piece of music altogether. I really like it. neurologist, when you're listening to music and having those feelings, feeling your spine tingle, are you thinking about what's actually happening to your brain? Not really, no, no. But it's interesting, isn't it? What on earth? There must be synapses firing to cause that sensation. But it really did make my spine tingle.

26:20¶¶

27:01¶¶ The Adagio from Mozart's Clarinet Concerto in A Major performed by Thea King and the English Chamber Orchestra conducted by Alan Francis. So Pam Shaw, we talked earlier about your achievements in the specialist field of motor neurone disease. MND is a disease that leads to loss of control of a patient's muscles over time. It can lead to problems with breathing, swallowing and can often progress quite rapidly. When you started, what treatments were available to patients? Really, there was nothing very much at all. One treatment came through in the mid-1990s, Rilisol, which is still used today.

27:49And it does have some effect. It just slows down the course of the condition on average by a few months. How fast is the progression of the condition usually? It's very variable and I always say that to patients that the speed of progression varies a lot between different people. The example of Stephen Hawking who had it for more than half a century, in its worst form it can be really rapidly changing, causing major problems within 12 months of the first symptom. and that speed of change makes it very frightening for patients and their families. But also for you Pam, I mean such a thing to take on as a young doctor, how did friends and colleagues react when you said this is the specialism, this is what I want to do?

28:38Some of them said why on earth would you want to do that Pam? Is it not very depressing? And it is in a way, I write condolence letters to families almost every week But you have to learn to control the sadness that you feel for families, I guess. You don't stop feeling the sadness or compassion, but you learn to control how it displays itself, I suppose. To channel it. And what do you know now? What do we know about why people develop MND? Back in the 1990s, the first gene was found that can cause familial MND. So around about 10 % of people with MND will report someone else in the family had the same condition.

29:26So the first gene was found in the 1990s called SOD1, S-O-D-1. And since then, with all the sophisticated genetic sequencing that you can do now, there are more than 30 genes that have been found. The first one, SOD1 MND, there is the Tofferson treatment. The treatment that your centre has introduced, it's not available on the NHS yet? Not yet. It's been approved by MHRA, but it still has to be considered by NICE, the National Institute for Clinical Excellence, and they're considering it. But in the meantime, the pharmaceutical company that manufacture it, they're making it available to patients free of charge if you know you have that gene change.

30:13How is it delivered? It's given via a lumbar puncture, so a little needle in your back into the spinal fluid that surrounds your spine. And it's given intrathecally into the spinal fluid and it has to be given that way every month. Some patients have been having that monthly lumbar puncture for eight plus years. And I know that you've got some patients who you'll never forget. There was one gentleman that I read about who was a solicitor in Glasgow and he helped with one breakthrough. Tell me about him. The end of the illness in MND often happens because the breathing muscles become weak. The motor neurons supplying your diaphragm and breathing muscles become weak.