Professor Dame Pamela Shaw

Presenter0:00

Hello, 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.

Presenter0:45

My 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. They 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?

Presenter2:11

Yeah.

Pamela Shaw2:11

I always knew, even when I was still at school, that I was interested in the brain and the nervous system, and when I was a trainee neurologist helping with clinics and so on, just something about the plight of M and D patients really got to me. It made me feel like a bit of a useless doctor, really, because

Presenter2:29

Mm-hmm.

Presenter2:33

What what were things like back then? What sort of prognosis did people have?

Pamela Shaw2:36

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.

Speaker 22:46

Slide.

Pamela Shaw3:00

Yeah.

Presenter3:00

It feels like these days motor neuron disease is much more visible than it used to be. I 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?

Pamela Shaw3:18

And so

Pamela Shaw3:25

Those high-profile sportsmen, it's so courageous of them to

Presenter3:30

Two

Speaker 23:30

Uh

Pamela Shaw3:31

Show their symptoms to the world when they have been such powerful athletes themselves.

Presenter3:36

Oops.

Pamela Shaw3:37

But they've done an awful lot to raise awareness, and their families have as well.

Presenter3:41

I mentioned that you 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?

Pamela Shaw3:52

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 a 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?

Pamela Shaw4:24

Ooh, there are about three hundred researchers now at at Citran, and we're full, so we're in the middle of building a sister building joined on to Citran, so we'll d double our capacity. That new building will be ready in October time.

Presenter4:41

Are they going to name it after you?

Pamela Shaw4:42

Uh

Pamela Shaw4:42

No, no, no, no, no. Just sit. Why not? Just sit, Rank.

Presenter4:46

Pass.

Pamela Shaw4:47

Uh

Presenter4:47

Come on.

Pamela Shaw4:47

Yeah.

Pamela Shaw4:49

Yeah.

Presenter4:50

Well, you have had several honours, including being made a dame in 2014. Is that a title that gets used in the workplace?

Pamela Shaw4:57

To be honest, it's a very teasable thing. So people use it cheekily, do they? Yes, they they sing into my voicemail, you know, there ain't nothing like a dame and that kind of thing.

Presenter5:01

So people

Pamela Shaw5:09

So, no, usually at work I'm just Pam.

Presenter5:12

And what about your title? How did you find out that you'd been so

Pamela Shaw5:15

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 dogs died. So I went out of the lecture, took the message and Paul, my husband, said, Pam, there's a great big envelope here, just come in the post, from the Prime Minister's office. Can I open it?

Speaker 25:28

This is on five.

Speaker 25:29

They don't know.

Pamela Shaw5:46

So we did open it and it was ap you know, I was completely flabbergasted. I had no idea that that was going to happen.

Presenter5:54

Oh well, 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?

Pamela Shaw6:01

So I like all sorts of music, but the ones that I did choose have some special meaning, so it wasn't too hard for me to pick them out.

Presenter6:09

I need to put them out. Well, tell us about your first, then what's the story behind disc number one?

Pamela Shaw6:14

It's the Alleluia 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, sometimes 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 tofoson 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.

Presenter6:55

To that is

Presenter6:59

Mm-hmm.

Speaker 27:00

Uh

Pamela Shaw7:01

actually a good proportion of patients, they either stabilize 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.

Speaker 27:11

Musk

Speaker 27:13

Coming.

Presenter8:21

Handel's Messiah performed by the London Philharmonic Orchestra and chorus conducted by Douglas Gamling. For medical breakthroughs and for your husband Paul's Holes in One.

Presenter8:32

So, Pam Shaw, let's go back to the beginning, shall we? You were born in Rothbury, Northumberland, in April nineteen fifty five, the second of four children to Robert and Valerie Shaw. Yes. Tell me about your mum and dad. How did they meet?

Presenter8:44

Yeah.

Pamela Shaw8:44

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.

Speaker 28:55

Uh

Speaker 29:04

One side.

Pamela Shaw9:08

My 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. And that's I think he decided that wasn't the life for a family man. So he went to Kuwait and

Speaker 39:26

Oh my goodness.

Speaker 29:27

Uh

Speaker 29:32

Yeah.

Pamela Shaw9:35

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.

Presenter9:46

Okay, so so this is the big move that your family made. You were about three, I think, when in nineteen fifty eight you moved to Kuwait, which from Morpeth in Northumberland is I mean, you couldn't get more different. It's a big move.

Pamela Shaw9:52

That's

Pamela Shaw9:54

Yeah.

Pamela Shaw9:59

Yes, it was a really um nice life to grow up in Kuwait, very much uh an outdoor life, lots of swimming and beaches and sailing and the town we lived in called Ahmedi, 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, Americ US time.

Pamela Shaw10:30

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. So 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

Presenter10:30

To really stretch it.

Presenter10:49

Yeah.

Pamela Shaw11:01

Girls would never really learn, you know, how to tie different types of knots and.

Pamela Shaw11:08

how to set a table properly and all sorts of useful things I learnt in the Browning.

Presenter11:13

His 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 life there?

Pamela Shaw11:21

So my mum, very kind person, very well supported by my dad. I mean, my dad devoted his life really to

Presenter11:28

I mean my

Pamela Shaw11:31

you know, making my mum's life as happy as he could.

Presenter11:34

And did she tell you much about her former life as a nurse?

Presenter11:39

Yeah.

Pamela Shaw11:39

She did. She used to tell stories. Um 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?

Presenter11:53

Yes, she did. All right, Pam, let's have some more music. It's time for your second choice today. What's it gonna be?

Pamela Shaw11:59

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. They kept a place there, didn't they? They kept a place there and they would come home for six weeks every year.

Presenter12:13

They kept a place that in the

Pamela Shaw12:18

And my mum in particular just loved Frank Sinatra.

Speaker 212:23

I know I stand in line until you think you have the time to spend an evening with me.

Speaker 212:32

And if we go someplace to dance, I know that there's a chance You won't be leaving with me

Pamela Shaw12:32

Do you

Speaker 212:41

And afterwards we drop into a quiet little place and have a drink or two.

Speaker 212:51

And then I go and spoil it all by saying something stupid like I love.

Presenter12:57

Frank and Nancy Sinatra with Something Stupid. Pam sure you just shared the loveliest memory with me. Tell us about it of your mum when listening to that track.

Pamela Shaw13:06

Yes, so when my parents were in their early eighties, their health became quite frail and.

Speaker 213:13

Mm-hmm.

Pamela Shaw13:15

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 Sophy 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 round with a smile on her face.

Pamela Shaw13:42

Gaily unpacking what Sophie and I had packed, but humming Frank's sinatra, and I think she was humming, I'll do it my way.

Presenter13:53

Oh, Flasher.

Pamela Shaw13:53

Uh

Presenter13:54

All 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 Ahmadi, as you mentioned. Tell us a bit about what it was like.

Pamela Shaw14:07

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. It 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

Presenter14:16

really sparked something in you.

Pamela Shaw14:29

Ten or eleven years old, and he was having a technetium brain scan, where they did they do an injection of a radioisotope. It gets taken up by the tumor. 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.

Speaker 214:42

Tumor incited.

Presenter14:49

Yeah.

Presenter14:54

So you loved your primary school, but you couldn't stay

Pamela Shaw14:57

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 eleven. So I went to a school in Annick in Northumberland, just next to Annick Castle, where they filmed Harry Potter. And I went there because some of my friends from Morpeth attended that school as day girls.

Speaker 215:15

What part of the world

Speaker 215:18

Then

Pamela Shaw15:24

But it had a boarding facility, so 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 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.

Presenter15:42

Mm-hmm.

Presenter15:46

So quite long chunks where where you were together.

Pamela Shaw15:48

Yeah.

Presenter15:49

Yeah. And what about the basics? I mean, how was it being back in chilly Northumberland after after swimming and playing tennis in in air conditioned?

Pamela Shaw15:58

When I was about eight years old I did jump into the North Sea expecting it to be like the Persian Gulf. And I've never liked cold water plunges ever since then. So yeah, none of that for you. No cold water swung.

Presenter16:07

Ouch.

Presenter16:10

She has ever seen

Presenter16:11

So yeah, none of that for you. No cold water swimming for you. And you knew quite early on that you wanted to study medicine, but you had some challenges with A-level biology.

Pamela Shaw16:21

Up

Presenter16:21

Yeah.

Pamela Shaw16:22

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 the headmistress at the time

Pamela Shaw16:43

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. The lovely teacher who who took on biology w was a trained cookery teacher.

Pamela Shaw17:05

Yeah, no, I just took the syllabus and

Presenter17:08

And just learned it at home.

Pamela Shaw17:08

How did you Get on then. How did you do in your exams? Yeah, no, I got A's in in both subjects, but I knew I had a year in hand'cause they bumped me up a year.

Presenter17:21

Oh yeah, I

Pamela Shaw17:22

So so they bumped you up a year just b for the class size? Yes, they couldn't fit enough desks in the classroom so they decided to bump me up a year. So I did 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.

Presenter17:24

Guys

Presenter17:27

From Survey

Presenter17:30

December

Presenter17:37

Yeah.

Presenter17:44

Extra

Pamela Shaw17:45

Yeah.

Presenter17:45

Did you decide to do some extra study during that time?

Pamela Shaw17:45

Yeah.

Pamela Shaw17:48

So I did those two A levels, and then I went to another school in Northumberland in a little uh place called Heyden Bridge, and I did a crash course in physics and chemistry and headmaster there.

Speaker 218:01

Uh

Speaker 318:01

Thank you.

Pamela Shaw18:03

Was

Pamela Shaw18:04

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 have we got to hear? It's Joni Mitchell. I think one of her teachers, school teachers, described her as a painter with words. And 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.

Speaker 318:55

I've looked at love from both sides now, Find give and take Instead somehow it's love's illusions I recall I really don't know

Presenter19:22

Joni Mitchell and Both Sides Now.

Presenter19:26

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?

Pamela Shaw19:34

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 okay. And then he asked me, and what do you like to do when you're not doing your school work? And I said, well, every year at school we have a Gilbert and Sullivan operetta. So I've just been pish tush in the Miado. 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.

Presenter20:23

Thank heavens

Presenter20:24

Power

Pamela Shaw20:26

Absolutely.

Presenter20:26

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 the.

Pamela Shaw20:34

I'm proud with your results? Well, yes, I graduated with first class honors, and one of my professors told me that I was the best medical graduate they had had for ten years. So how did that feel? For

Presenter20:48

Uh You know, having you must have felt a bit on the back foot when you joined and to hear that.

Pamela Shaw20:51

Did you hear that?

Presenter20:52

Uh

Pamela Shaw20:52

Quite believe it.

Presenter20:53

in some ways. Um but but yeah. As well as uh enjoying all the rotations of clinical training, you did orthropaedics, obstetrics and everything like that, you also met your now husband, Paul.

Pamela Shaw21:07

So 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 a drink, and he said, Ooh, I'd do anything for you.

Pamela Shaw21:24

And then a little while later he, you know, asked me out and it it went from there. You also shared a mutual love of flowers that I think still endures. Yes. 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 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.

Presenter22:08

So you graduated, Pam, in 1979. Tell me about life as a junior doctor.

Pamela Shaw22:13

It was very busy. I was based in Newcastle. I did a year, for example, at Dryburn Hospital in Durham, so I went as a medical registrar to Durham and then came back.

Speaker 222:20

Yeah.

Presenter22:20

I think so.

Pamela Shaw22:24

um 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 C T scanner.

Pamela Shaw22:37

in the whole region. So the well, the whole of the north east and across to Carlisle, Cumbria to the Scottish border. Oh, wow. So the on calls were really busy because you'd get physicians ringing from Carlisle saying

Speaker 222:43

Oh wow.

Speaker 222:45

Oh wow.

Speaker 222:47

Because

Pamela Shaw22:51

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.

Presenter22:58

Yeah.

Pamela Shaw22:59

Really good training.

Presenter23:01

You were going great guns yourself, obviously you were well supported, but in nineteen eighty eight you wrote a paper on the neurological complications of cardiac bypass surgery, and that actually resulted in a change in clinical practice, I think.

Pamela Shaw23:14

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 post-operatively. 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

Speaker 223:23

Uh

Speaker 323:32

Pick.

Speaker 223:33

Yeah.

Pamela Shaw23:45

Emboli that cause blockages in the small vessels in the brain. And 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 whipper snapper of a young neurologist coming around looking at their patients, but actually they weren't. They were really kind and very interested in the findings. Fantastic.

Presenter24:40

They were

Presenter24:43

Fantastic.

Pamela Shaw24:45

Uh

Presenter24:45

Uh Alright, 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?

Pamela Shaw24:51

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 neuron disease conference in Berlin, and the person hosting the conference took us to this little theatre, which was old-y-worldy, all wooden panels and so on, and to a live performance of this piece of music. I was blown away by it. It 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 love it.

Presenter25:32

Paul, well as a 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?

Pamela Shaw25:41

Not really, no, no. But it's interesting, isn't it? What what what on earth? There must be synapses firing um to cause that sensation. But it really did make my spine tingle.

Presenter25:48

The most

Speaker 325:48

And

Presenter27:04

The adagio from Mozart's Clarinet Concerto in A major performed by Thea King and the English Chamber Orchestra conducted by Alan Francis.

Presenter27:14

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?

Pamela Shaw27:39

Really, there was nothing very much at all. One treatment came through in the mid-1990s, Rilizol, which is still used today, and it does have some effect. It just slows down the course of the condition on average by a few months.

Presenter27:58

Okay.

Pamela Shaw27:59

How fast is the

Presenter28:00

Uh

Pamela Shaw28:00

The process of

Presenter28:00

Regression of the condition usually.

Pamela Shaw28:02

Yeah.

Pamela Shaw28:02

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,

Speaker 228:10

Yeah.

Pamela Shaw28:15

In its worst form, it can be really rapidly changing, you know, causing major problems within twelve months of the first symptom. And that speed of change makes it very frightening for patients and their families.

Presenter28:29

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?

Pamela Shaw28:39

Some of them said, Why on earth would you want to do that, Pam? Is 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.

Presenter29:07

To channel it. And what do you know now? What do we know about why people develop him?

Pamela Shaw29:11

Okay.

Presenter29:11

Uh

Pamela Shaw29:11

Uh

Presenter29:11

Dang.

Pamela Shaw29:11

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. So, the first gene was found in the 1990s called SOD1, SOD1, 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 Topheson treatment.

Presenter29:47

The treatment that your centre has introduced. It's not available on the NHS yet.

Pamela Shaw29:52

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

Speaker 229:57

Uh

Presenter29:57

This is the

Speaker 229:58

Uh

Pamela Shaw30:06

They're making it available to patients free of charge if you know you have that gene change. How 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

Speaker 230:16

So

Pamela Shaw30:22

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.

Presenter30:32

Yeah

Pamela Shaw30:32

Yeah.

Presenter30:32

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. Yeah.

Pamela Shaw30:43

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. But there was a solicitor from Glasgow who came down to my clinic in Newcastle. He was still walking about, but in real problems with his breathing, and had had to give up work and so on because of this breathlessness. And he went back to Glasgow, and this enlightened respiratory physician said there's this new type of support for breathing muscles that's come out. Let's try you with that. So it's a mask that you put on a bit like you'd use for sleep apnea. You put it on when you go to bed at night. It assists your breathing muscles during the night. And so he came back to see me in Newcastle after this had been introduced and he was a different man. You know, he was sleeping well, he'd put on weight, he was able to get back to work. And so that inspired me that we ought to try this more systematically to see if it will help patients. So I joined forces with a respiratory physician in Newcastle, John Gibson, and together we did a formal trial.

Pamela Shaw32:03

It showed dramatic benefits. Yeah.

Presenter32:06

To have a breakthrough where you actually see people improve, it must be mind-blowing.

Pamela Shaw32:11

It must be mind-blowing. What does that feel like? Yeah, no, it's it's um

Pamela Shaw32:15

It is amazing. There there are a few things like that where I really feel, yes, we've done some good there, but we had to do that trial to show the evidence, and now that's available to everyone with M and D in the UK and overseas as well. So it was it was an important step.

Presenter32:36

Alright, time for some more music, Pam. It's your fifth choice today.

Pamela Shaw32:39

What are we going to hear? This one, and my husband Paul, kind of helped me think of this one. I I wanted to think of something that would make me remember happy holidays. So we had many happy family holidays driving through France or Ireland, and so we settled on Sweet and Dandy by Toots and Maitol. So my husband sort of got me interested in reggae music to a certain extent. And this one, my daughter Sophie, used to love to listen to this as we were driving along, and we'd all sing along to it.

Speaker 333:19

We popula, hold our wine.

Speaker 333:21

Are you friends just up in a white pickaxe on that wedding cake? It is no wonder.

Speaker 333:29

In the perfect wonder why they were dunting in right for room or flag.

Speaker 333:38

Uh

Speaker 333:39

There was three dun dunny, three dun

Presenter33:45

Sweet and Dandy by Toots and the Mittals. So Pam Shaw, in the year two thousand you moved to Sheffield and you met another patient there who changed the course of your career. How did you come across her?

Presenter33:59

Uh

Pamela Shaw33:59

The MND Association charity was holding a fundraising event, and so I composed a speech on the train going down about the impact of MND, what a cruel disease it was. And I sat at the dinner next to this professor of obstetrics, and he was chatting, and he said, Oh, my wife over there at the next table was just told last week that she had motor neurone disease. So you can't talk about how devastating it is and all of that. So I had to rejig the speech in my head. So I met with that lady.

Presenter34:29

So you can't talk about how devastating it is and all of that.

Presenter34:36

So

Pamela Shaw34:39

and she asked me could she come from London to my clinic in Sheffield. Fortunately she had a slow form of it and survived it for, I think, six or seven years.

Presenter34:51

So you got to know this lady and, you know, she she asked you about your pitch for fun.

Pamela Shaw34:52

Great.

Pamela Shaw34:58

What happened? She was having some tests in Sheffield and we were just chatting and she said to me, if you had twenty million pounds to do something about MND, what would you do?

Presenter34:58

Yes, so

Pamela Shaw35:12

And I thought we were just having a light-hearted conversation, but I sat back and thought about it. I described CITRAM. I said I would make a dedicated research institute. I'd have clinical doctors and scientists under the same roof, having coffee and lunch together, and keeping each other right. And she said, well.

Pamela Shaw35:35

I'm used to fundraising for my husband's research, so I I'm going to try and help you do that. Go away and write me a b uh a brochure about the vision for this institute.

Pamela Shaw35:48

I love her.

Presenter35:48

Yeah.

Pamela Shaw35:48

Being bossy, having just got the diagnosis. That was one of her character traits. So there were a group of patrons in London.

Presenter35:49

We just got the diagnosis.

Presenter35:53

Love that was

Pamela Shaw35:58

And a group of patrons in Yorkshire, and they helped us raise £12 million. And then one of the high-powered businessmen that was really the head of the Yorkshire patrons persuaded the Vice-Chancellor that, look, we're about to make this big donation. I think you need to put in at least half of that. And of course, the Vice-Chancellor readily agreed. So it was very good.

Presenter36:24

For servers.

Presenter36:26

And what did it mean to you to see the fundraising feats and all that effort that was going into bringing in this money?

Pamela Shaw36:33

I will probably write a book when I retire about all the generous people that helped. One wonderful gentleman gave us the first million pounds from his charitable foundation, and it was like a snowball. Once somebody had committed, then other people would. There was a chap from Tasmania who knew a person in Yorkshire whose son had had M and D, that did the same.

Speaker 236:50

Uh-huh.

Speaker 236:58

Mm-hmm.

Pamela Shaw37:00

Journey that William Bligh did after the mutiny on the Bounty with exactly the same size boat, same conditions, and again raised uh a lot of money, so there were amazing things. And I think it is because MND is such an emotive condition, people who've seen it want to try and help and do something about it.

Presenter37:17

People

Presenter37:22

So the Sheffield Institute for Translational Neuroscience, or CITRAN, was ready in twenty ten, although I'm told that you thought you'd messed up the opening invitation.

Presenter37:32

Oh yes.

Pamela Shaw37:33

Yes. The Duke of Devonshire took an interest in this building, and when I got to know him a bit, I said to him, When the building's finished, would you do us the honour of presiding over the opening of it? and he said no.

Pamela Shaw37:50

And I thought, oh gosh, I've breached protocol by asking directly rather than going through the usual channels. And then he got a twinkle in his eye and he said, No, I won't do it. I'm going to find you someone much more important than me.

Speaker 237:53

Bar yeah, bar bar.

Speaker 338:05

Yeah.

Pamela Shaw38:06

So his job in those days, one of his jobs was to run the Ascot races for the Queen. And he waited, I think, until one of her horses did very well. And then he said, Would you come to Sheffield and open this neuroscience building? And she said, Yes, I'd be delighted, as long as Philip can come with me. So, yeah, so the Queen and Prince Philip opened it for us in November 2010.

Presenter38:33

Alright.

Pamela Shaw38:34

It's time to go to the music. What's next for us? The next piece is from just about my favourite film, so Shawshank Redemption. I've always had a great passion for fairness and justice, and that film really touched a chord with me. But one of the most beautiful moments in that film, and I think in cinema generally, is when Tim Robbins locks himself into the prison library and puts a loudspeaker onto the prison yard with this piece from Mozart's Marriage of Figaro, Duatino Sularia. And it's just amazing. The look on his face, it's a combination of serenity listening to that music, but also mischief, knowing what he's doing, and then the amazement of all the prisoners in the prison yard, and Morgan Freeman saying, I've no idea what those two Italian ladies were singing about. Their voices were soaring, but it gave such hope in the grey, dismal prison environment. So the Duetino Sularia from The Marriage of Figaro by Mozart.

Speaker 340:07

Sotly, software's current softly

Speaker 340:26

There was kick on.

Presenter40:56

The duet between Susanna and the Countess from Mozart's The Marriage of Figaro, as heard in the film The Shaushank Redemption. The soloists were Edith Martis and Gondela Janowitz, with the orchestra of the Deutsche Opera Berlin conducted by Karl Bohr

Presenter41:12

Pam, you've mentioned the breakthrough MND treatment, Toficin, which came through in twenty twenty two. Tell me about some of the benefits that you've witnessed in patients to whom the

Pamela Shaw41:26

That drug is helpful. During the trial, patients would bring videos of themselves walking up their garden steps, which they haven't been able to do for two years. One gentleman in a wheelchair when he first started actually at the end of the trial

Speaker 241:36

Wow.

Pamela Shaw41:43

Walking around his house without any walking aids. So it's a real breakthrough. You know, looking forward, what.

Presenter41:50

What do you hope for the future from this breakthrough for other patients for whom?

Pamela Shaw41:56

There are ongoing trials for patients who have different gene changes. There's one called Fuzz that is looking very promising, but also there are these genetic therapies that tackle some of the damaging mechanisms more broadly. So I think the era of genetic therapies is going to be really important.

Presenter41:56

Such officers are not appropriate.

Presenter42:21

You know, I was going to

Pamela Shaw42:21

Bus

Presenter42:21

What keeps you hopeful today, but it must be seeing that research.

Pamela Shaw42:23

Yeah.

Pamela Shaw42:26

Yes. That's why I can't retire yet. There are several so there are two exciting treatment possibilities that I just want to see come through into clinical trials.

Presenter42:28

There are

Presenter42:38

Mm-hmm.

Presenter42:39

Well, good luck. We've got everything crossed for those. Pam, your own days are clearly incredibly busy. I mean, how important have family and friends been in keeping you going over the years? As you say, you

Pamela Shaw42:39

Uh

Pamela Shaw42:41

Yeah.

Pamela Shaw42:50

Yeah.

Presenter42:51

Um

Pamela Shaw42:51

Reta

Presenter42:51

I am

Pamela Shaw42:51

You still got too much on? Yeah, no, no, very much so. You know, one of the lucky things in my life is really being blessed with a beautiful family. My husband has always been and you know, I work hard and long hours very often, but he's nothing but supportive of me and clearly friends and colleagues as well. Yeah, the human contact and just shared experiences, humour definitely keeps you going.

Presenter43:22

All right, Pam. Music keeps you going too, of course, so I think we should have your seventh choice today.

Pamela Shaw43:27

So the seventh choice is a haunting piece by Jay Ungar called The Ashokan Farewell, and I would have it on my desert island to remind me of people that we've lost. So my parents, my father-in-law, my sister-in-law, my nephew, my best friend that I started school with in Kuwait, Christina, whose mum taught me how to read. I lost her not that long ago. But also I would dedicate it to all those families that have had to cope with M and D and face that condition in one of their loved ones or family members.

Presenter44:38

Ashokan Farewell American folk violinist Jay Unger with the band of His Majesty's Royal Marines.

Presenter44:46

Pam, you said that piece of music was for friends, relatives and patients that you've lost over the years and included your mum and dad in that. Did they get to see some of your achievements?

Pamela Shaw44:56

They did, and they were always very proud. You know that I became a professor of neurology. They weren't alive when the DBAE was awarded. But interestingly, the ceremony at Buckingham Palace was on the twelfth of October, and they both had their birthdays on that day the same day. So several years after they died, the ceremony was on their the date of their birthday. So it's amazing.

Speaker 245:15

Yeah.

Presenter45:20

Uh

Presenter45:23

Oh, how amazing. And of course, as well as the Damehood, you've received a number of awards and accolades, but I wonder what part of your career has brought you the most pleasure over the years?

Pamela Shaw45:33

Uh

Presenter45:33

Uh

Pamela Shaw45:34

The results of the Toferson trial had us dancing, all the research nurses and the researchers. Teaching. Well, yes, jumping up and down with excitement. But also training and teaching people and bringing the next generation along. So one of my he was a trainee a few years ago, but he's just become the Rob Burrow Professor of Neurology in Citran. So just seeing people's careers blossoming that and you've helped them a little bit along the way is very nice.

Presenter45:38

Yeah. The researcher

Speaker 245:59

Go.

Presenter45:59

Uh

Speaker 245:59

Yeah.

Presenter46:07

Yeah, very special. You've just talked about the people, the collaboration and that's a that's such a a big thread in your career. I mean, it's literally what Citron is all about, that dialogue between clinicians and researchers.

Pamela Shaw46:07

Yeah.

Pamela Shaw46:19

And we're setting up.

Presenter46:20

But it does make me wonder how you'll get on on the desert island. You're going to be completely on your own. How will that be for you?

Pamela Shaw46:27

I've always been able to find things that interest me. I would love to have brought one of my animals with me, but I know that's not allowed. So I would I would try to.

Presenter46:37

So I would I would try to start that now, not after 84 years pal.

Pamela Shaw46:42

I would try and find living things on the island because you know animals are great company. If if my husband's away, the two cats and my dog, they they you don't feel lonely in the house. So I would I would try and find some wildlife that I could link with. Well, we'll let you take one.

Presenter47:03

More disc to your island with you, Pam. Your final choice today. What is it?

Pamela Shaw47:06

What is it? So the final one is again ending on a positive note. So I think some female singers have voices like honey, and Alison Krause is one of those. I particularly like her song, The Lucky One. It always makes me feel you've got to you know, if you're having a difficult day for one reason or another, it's a count your blessings song, so I would definitely have that on the island.

Speaker 347:37

You're the lucky one, so I've been told Spree's a wind blowing down the road Loved by many, hated by none I'd say you were lucky, doesn't know what you felt Out of care in the world, not a worry inside Everything's gonna be alright, cause you're the lucky one

Speaker 347:59

You're the lucky one, always having fun. A jack of all trades, a master of none. You look at the world.

Presenter48:07

Alison Grace and Union Station with The Lucky One. So, Pam Shaw, I'm about to cast you away to the Desert Island. I'm going to give you the books, the Bible, the complete works of Shakespeare, and another book of your choice. What's that?

Pamela Shaw48:21

My daughter, Sophie, has just introduced me to the novels of a lady called Kristen Hanna. If I'm allowed to take more than one book, the complete works of Kristen Hanna. She describes, through the eyes of women, really, all sorts of life events, whether it be, you know, smuggling children out of occupied France during the war, nurses going over to Vietnam to look after wounded soldiers and civilians, the Great Depression in the US and the Dust Bowls. Each book has me in tears at some point. And Sophie said, I think you'd like this, mum, so she gave me one called

Presenter49:05

Yeah.

Pamela Shaw49:06

The Nightingale

Presenter49:06

Well, there's a six-book set that we can give you, so that will keep you happy for many hours on the island. You can also, Pam, have a luxury item. What's that going to be?

Pamela Shaw49:16

If I'm allowed three roses, and I would try and find a place where they might thrive, so I would have to have a relative.

Presenter49:23

Or a relatively shady nook. Yes.

Pamela Shaw49:26

Yes. Which which varieties would you take? One of my favourites is called L. D. Braithwaite, so it's a deep red rose. I very much like the new rose that's just come out, Sir David Beckham. So that that's a kind of white one with a yellowish centre. And which other one?

Pamela Shaw49:45

There's one called Lady of Shalotte that's a sort of orangey-yellow.

Presenter49:50

The Lady of Shalot and David Beckham together at last on your island, Pam. I'm all for it.

Pamela Shaw49:51

I have

Pamela Shaw49:52

Gather at last on your island, Pam. I'm all for it. It's all you're nothing like the scent of roses.

Presenter49:55

Yeah.

Pamela Shaw49:58

It it it again it makes my spine tingle when I smell roses.

Presenter50:03

Uh Uh

Pamela Shaw50:03

The

Presenter50:03

Perfect choice. And finally, which one track of the eight that you've shared with us today would you save from the waves first?

Pamela Shaw50:10

Hard choice, but I think it would have to be Mozart's clarinet concerto.

Presenter50:17

Yeah.

Pamela Shaw50:17

Professor

Presenter50:17

Dame Pamela Shaw, thank you very much for letting us hear your desert island discs. Thank you for having me.

Pamela Shaw50:23

me on the programme. I very much enjoyed it.

Presenter50:43

Hello, I hope you enjoyed my conversation with Professor Dame Pamela Shaw. I'm sure she'll be able to get her roses to survive the island climate. We've cast away so many medical scientists over the years, including Professor Stephen Westerby, Professor Dame Leslie Reagan, and David Knott. The studio manager for today's programme was Jackie Marjoram. The production coordinator was Susie Roylands. The editor was Mugabe Turia, and the producers were Christine Pavlovsky and Tom Alban. Next time, my guest will be the singer and songwriter Chaka Khan. I do hope you'll join us.

Speaker 351:41

Hello, I'm Greg Jenner. I'm the host of You're Dead to Me, the comedy podcast that takes history seriously. And we are back for a 12th series. Everyone in that film is wearing their own pants. We'll be joined by comedians like Kyle Smith Bino and Desiree Birch. Can't see how this would go wrong. We'll meet many medieval saints. Motivational speaker saint with a sort of Madonna head mic on. We're doing a history of underwear. Plus, there's loads more. You're Dead to Me. Listen on BBC Sounds.

Speaker 251:56

Yeah.

Pamela Shaw52:07

Uh