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IN TOUCH 鈥� Gene Therapy Aims to Halt AMD听

TX:听 19.02.2019听 2040-2100

PRESENTER:听 听听听听听听听听 PETER WHITE

PRODUCERS:听 听 听 听 听 TOM WALKER, LEE KUMUTAT

White

Good evening.听 Tonight, controlling the biggest cause of blindness in the Western world.听 It鈥檚 a large aim.听 We discuss the significance of a ground-breaking operation for current and future patients.

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And how the loss of vision for one member of a long-standing relationship can affect the other one.

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Clip

She couldn鈥檛 see me, she couldn鈥檛 see me ageing, I wanted her to be able to see my wrinkles, you know, my greying hair.听 I got a new haircut, she couldn鈥檛 see it.

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White

Charmian Cvek, who鈥檒l be talking later about how her mother鈥檚 loss of sight affected her.

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But first, a medical team in Oxford has just taken the latest step in the attempt to halt a form of eye disease using gene therapy.听 AMD or age-related macular degeneration affects around 600,000 people in the UK, when you include those who have not registered as visually impaired.听

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Janet Osborne, who鈥檚 part of a trial to test the safety of the operation, had a synthetic gene injected into the back of her eye to prevent more cells from dying.听 She already has blurred central vision.听 Part of the process was filmed by 91热爆 News, as the leader of the team of surgeons 鈥� Professor Robert MacLaren 鈥� tested Janet鈥檚 current vision.

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Clip 91热爆 News

MacLaren

Can you see the letters on this line, the next one down?

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Osborne

No, it鈥檚 become a blur.听 But I can see your glasses and your ears.

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MacLaren

Can you see the colour of my eyes?

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Osborne

No.

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MacLaren

Approximately 350,000 people in the UK are severely sight impaired by age-related macular degeneration.听 A genetic treatment administered early on to preserve the vision in patients who鈥檇 otherwise lose their sight would be a tremendous breakthrough in ophthalmology and certainly something I hope to see in the near future.

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White

Professor MacLaren admits there can be no certainty yet that the loss of Janet鈥檚 vision will be halted.听 But the ultimate aim of this procedure will be to inject the gene into the eyes of people at risk before their sight has deteriorated.听 But inevitably such publicity raises a mass of questions in the minds of people who already have the condition.

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Cathy Yelf is CEO of the Macular Society, which supports people with the condition.听 I asked her what she thought was the significance of this latest procedure.

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Yelf

Well I think the excitement that this procedure causes, this particular trial arouses, is that it is the first gene therapy trial in dry AMD.听 And the reason that that is important is that there is no current therapy at all for dry AMD.听 And so, it is the great hope of probably the majority of people with age-related macular degeneration, the hope is to find a first therapy for dry AMD.

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White

So, just remind us of the current forms of treatment that are actually available to people who have AMD, perhaps particularly wet AMD, which can be treated.

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Yelf

Yes, wet AMD is the only form of age-related macular degeneration for which there is a treatment and it鈥檚 given as an injection into the eyeball to get the drug to the back of the eye and the drug slows down the development of abnormal blood vessels that grow and leak in wet AMD.听 And so, in most people who have the treatment, not everybody but most people it will significantly slow down the progress of the disease.听 But it doesn鈥檛 work for everybody and it doesn鈥檛 work forever and so longer-term therapies are needed and ones which are less burdensome really because these injections have to be given very frequently.听 And there is no treatment at all for dry AMD.

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White

Now clearly, however level-headed people try to be about this the question it鈥檚 bound to raise for them, when they hear this kind of publicity, is will this restore any of my sight or at the very least will it prevent my AMD from getting worse.听 What do you say to people who come to you with those kinds of questions?

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Yelf

It鈥檚 very hard because people are desperate to regain or prevent the loss of their sight.听 So, it is very difficult with early stage trials, and as you say this is a safety trial at this stage to make sure that the treatment doesn鈥檛 do any harm to patients.听 So, at very early stage in medical research it鈥檚 quite difficult for people to keep their feet on the ground, hope is a very important part of people鈥檚 lives and we do hope very much that this will prove to be an effective therapy but it is at a very early stage.听 The other thing is, that it is designed, I think, not to restore sight but to prevent sight from deteriorating.听 And the other important thing is that this particular gene, that is being targeted with the gene therapy, so the gene that is defective in this particular case, affects only about 5% of people with age-related macular degeneration.听 We know that AMD is a very complex disease, it involves many genes are associated with AMD, as well as lifestyle factors.听 So, it is not a single gene that causes this disease, and in fact it may not even be a single disease.听 So, this particular gene, the CF-1 gene, that鈥檚 being targeted in this trial, affects about, we think, about 5% of people with Macular Degeneration.听 But it鈥檚 a very important proof of concept here and if we can do it with this gene then we might be able to do it with all the others as well.

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White

So, of course, when this kind of thing hits and when you get this kind of publicity, I just wonder what happens in your offices when this happens.

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Yelf

Well people phone us, of course, for more information.听 So, I just spoke to the manager of our advice and information service today and he said 75% of the calls that our helpline has taken this morning have been about this therapy.听 So, that鈥檚 the sort of impact it has, people clearly want to know more about it and whether it鈥檚 something that might be able to help them.

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White

And I guess our role has always been to try and put these things into perspective and I guess even when the treatment does become more available there will still, won鈥檛 there, be the issues of cost, of beds, of staffing with 600,000 potential patients and all the ones that they want to help before they start to lose their sight, this is a heck of a challenge isn鈥檛 it?

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Yelf

It is, there are many steps along the way to get a brilliant idea and even a proven concept through to actually a drug that we can deliver in the clinic.听 It has to be commercially viable, it has to be affordable, it has to be deliverable 鈥� there has to be a way of actually getting into the patient鈥檚 eye or the bit of the patient that it needs to get into.

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White

Cathy Yelf, CEO of the Macular Society.听 And we鈥檇 like your reactions please.

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We鈥檝e already had a number of your reactions to our item about the rising number of attacks on guide dogs and one woman鈥檚 disappointment at the level of support she received afterwards.听 Here鈥檚 an example of what we鈥檝e heard.

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Drewell

I鈥檓 Sandra Drewell.听 I, too, am a guide dog owner and my previous guide dog, he was attacked in December 2015.听 Obviously, I contacted Guide Dogs and told them what had happened.听 Someone did phone up and do an accident report form but that was the only contact I had from them.听 They never came out to make sure the dog was working well, was not scared of other dogs or anything.

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White

And it鈥檚 good to see that some of you are already using our new voicemail system which allows people to leave messages for us with your views and it鈥檚 good to hear the real voices of the people who listen to us.听 Michael, for instance, who said he鈥檇 been a guide dog owner for almost 60 years, was now on his seventh dog and had always had excellent service from the association.听 And Angie Fairclough with the interesting idea of some kind of pendant system, such as those used by older people, so that guide dog owners could call for advice or assistance if they needed it.听 The number to call us 0161 836 1338.听 And we鈥檒l be giving you that number again at the end of the programme.

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We鈥檝e had another statement from the Guide Dogs Association.听 They tell us: 鈥淥n average we supported 12 guide dog owners every month through what can be a very difficult time.听 We鈥檝e also designed a special workshop for guide dog owners which focuses on helping them manage situations around other dogs. 听This is often attended by people who have experienced a dog attack as a means to support them during their recovery.鈥�

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Now, Charmian Cvek鈥檚 mother, was just one of those people we鈥檝e just been talking about, people who developed AMD or age-related macular disease, in her case in her 80s.听 But what effect does someone鈥檚 gradual loss of sight have on the person they live with?

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Her daughter, Charmian, emailed us to suggest that this was something she hadn鈥檛 heard tackled on In Touch and I certainly couldn鈥檛 remember many examples where we had made this the focus of a report.听 So, we invited her into the studio to explain what she meant.听 It was a thought-provoking conversation and Charmian began by telling me a little about her mother.

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Cvek

We鈥檇 always been together.听 She was a teacher.听 She was very artistic.听 She loved painting and she loved gardening.听 She was my best friend.听 She鈥檇 had cataracts in her 60s and that was the first sort of time that I encountered visual problems with her.听 I remember even friends and relatives who knew there was a problem.听 They鈥檇 still, you know you鈥檇 visit them and they鈥檇 give you photo albums to go through and there鈥檇 be me thinking, very protective, thinking she can鈥檛 see them for heavens sake.听

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White

Now clearly a relative鈥檚 loss of sight is distressing because it鈥檚 distressing for them, which is why it鈥檚 partly distressing for you, but you really seem to be describing something more than that 鈥� the loss of visual reference points, I think.

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Cvek

Yes, my mother had a cataract operation, had a new lease of life but then in her 80s Macular Degeneration kicked in and it was a gradual process.听 So, you start off where you find your life is being put in aspic a bit because you can鈥檛 come in, kick off your shoes, leave them there to be fallen over.听 You can鈥檛 just leave your handbag anywhere.听 You can鈥檛 change the furniture around.听 You can鈥檛 鈥� you can鈥檛 change anything really.听 If something breaks down 鈥� my mother was still cooking at this point 鈥� we needed a new cooker, we had to get one as much like the previous one as possible.

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White

And presumably there鈥檚 also a bit of a tendency to over-protect maybe?

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Cvek

I鈥檓 sure there was on my part, absolutely.听 Very, very difficult to come to terms with when it鈥檚 somebody that you love so very much and you鈥檙e finding the visual world is starting to retreat for them.

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White

And that was in a way what you emailed us about, which seemed to be the biggest thing, things you couldn鈥檛 share.

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Cvek

Yes.听 My own loss was profound.听 The things I could not share with her.听 We loved going around art galleries but now I couldn鈥檛 even show her pictures of paintings in books.听 Obviously, we couldn鈥檛 watch television together.听 At one point we鈥檇 used audio description but even that, it just got鈥� She couldn鈥檛 see me, she couldn鈥檛 see me ageing.听 I wanted her to be able to see my wrinkles, my greying hair.听 I got a new haircut, she couldn鈥檛 see it.听 I鈥檇 get her to run her hand over it but she didn鈥檛 know what I looked like in my clothes, if I bought anything.听 If I bought anything for her, she couldn鈥檛 see it.听 I鈥檇 be describing 鈥� oh your new nightie, it鈥檚 got those little rose buds on it.听 And I would never buy her anything that she wouldn鈥檛 have liked, even though she couldn鈥檛 see them.听 It was quite devastating, you don鈥檛 realise how much somebody else鈥檚 perception of you and the things you share brings to your own life and that you need that validation.

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White

And you went on holidays, I think, after she lost her sight and that that created problems too?

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Cvek

It was, it was difficult, yes.

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White

Because?

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Cvek

Well, she couldn鈥檛 do anything without holding my arm.听 I had to tell her absolutely every step, there鈥檚 another step here, put her hand on a banister, she couldn鈥檛 see the sights that we鈥檇 shared.听 She could still smell the sea and the 鈥� feel the sun on her but鈥�

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White

I was going to ask you about that because I mean one of the things this programme鈥檚 鈥� always tries to be about are some of the positives and we try to make the point of how much there is left in life.听 I just wonder if you found any 鈥� any compensations or things that you did that you might not have thought of doing?

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Cvek

One of the best things I ever did was getting a little Dictaphone, so that when I read a book to my mother, I would actually record it at the same time and then later on she could replay it, she didn鈥檛 mind hearing things again.听 And it was my voice, the comfort of my voice.

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White

There was an added problem because you feel that though it wasn鈥檛 diagnosed, your mum had begun to suffer from some elements of dementia before.

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Cvek

People who didn鈥檛 know her, who would come to see her, would say 鈥� oh, isn鈥檛 it wonderful, you know, she鈥檚 still got all her marbles and I鈥檇 be thinking, oh well you didn鈥檛 have a conversation with her last night, she would forget that people had died and keep going over the same things.

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White

People know perhaps now some of the things that can be done to help, as far as that is concerned, triggers and things like that but I think the loss of vision, that got in the way of some of the triggers that people advised you to use.

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Cvek

That鈥檚 right.听 I couldn鈥檛 write her notes to remind her of things.听 I couldn鈥檛 show her old photographs 鈥� family photographs, whatever, places that we鈥檇 been 鈥� those memory triggers, they weren鈥檛 available.

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White

And again, the kind of things that people advise you to use 鈥� technology and the things that are available now 鈥� any good in this instance?

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Cvek

Definitely the radio, that was a great blessing to her.听 I do think there鈥檚 a need perhaps for some more cheerful radio.听 Radio 4 can tend to be rather depressing.听

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White

We鈥檒l see if they allow that 鈥� us to put that out, we鈥檒l see if it survives the edit.

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Cvek

Yes, yes indeed.

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White

I鈥檓 going to ask you a potentially difficult question, so forgive me, but I wonder how well did your mother and your relationship survive this experience?

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Cvek

Very well, yes.听 We loved each other deeply and it was a strain but that鈥檚 one of the things 鈥搚ou become a carer and I don鈥檛 really like that label, to a certain extent, I was her daughter.听 One thing that few people seem to be aware of as well, is the possibility of old people seeing hallucinations because they鈥檙e losing their sight.听 It isn鈥檛 necessarily down to dementia or anything, I believe it鈥檚 called Charles Bonnet syndrome?

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White

It is and we鈥檝e talked about it on the programme.听 Did you mother suffer from it?

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Cvek

She did, yes, earlier on in the process of her losing her sight.听 She was plagued by a boy with ginger hair at one point, we don鈥檛 know where he popped up from.听 But if I hadn鈥檛 been able to reassure her, if I hadn鈥檛 known about it, she would have been afraid, she would have thought she was seeing a ghost.

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White

Just finally, I think you believe also that there were health implications of the loss of sight, not necessarily related to the loss of vision but other associated things.

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Cvek

Well it was the loss of vision that stopped my mother going out, then stopped her moving around even in her own house.听 And if you don鈥檛 keep moving you lose the ability to move and she did end up bed ridden, although there was nothing physically wrong with her.

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White

I just wonder what you would say to people listening to this because you鈥檝e highlighted the things that were a problem?

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Cvek

Well I suppose it is keep talking, keep responding, don鈥檛 withdraw.听 It is a very visual world, we live in, I think it鈥檚 becoming even more so with phones 鈥� everybody鈥檚 looking at them all the time.

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White

So, perhaps keeping talking is the key.

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Cvek

I think so, I wish I could offer some more insights but I think that perhaps my mother鈥檚 age and everything else, there wasn鈥檛 a lot I could offer except for my love and patience and continuing communication.

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White

Charmian Cvek.听 We鈥檇 like to hear your reactions to that and perhaps particularly positive suggestions of how the loss of visual stimuli could be overcome or maybe replaced with something else.

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And that鈥檚 it for today.听 But, as I promised, we can tell you about a change in the way that you can contact the programme to make your comments or give your views.听 You can now call 0161 836 1338 and when you do call this number calls are charged at the standard geographic rate although calls from mobiles may cost more.听 You can leave us a message there, we鈥檒l get back to you.听 Or, as usual, you can do lots of the other things we suggest, you can email intouch@bbc.co.uk or visit our website , from where you can also download tonight鈥檚 programme and previous editions.

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That鈥檚 it, from me Peter White, producer Tom Walker and the team, goodbye.