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IN TOUCH – Orbit Reader 20, Choroideremia breakthrough

TX:Ìý 09.10.2018Ìý 2040-2100

PRESENTER:Ìý ÌýÌýÌýÌýÌýÌýÌý PETER WHITE

PRODUCER:Ìý ÌýÌýÌýÌýÌýÌýÌýÌýÌý KEVIN CORE

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White

Good evening.Ìý Tonight:Ìý The long promised, long delayed electronic braille machine, costing hundreds rather than thousands of pounds is finally with us.Ìý So, has it been worth waiting for?Ìý And there’s also positive news of another holy grail – the ability to treat some eye conditions by injecting missing genes into the eyes is coming closer.

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Clip

Diseases that I was taught during my training were incurable and never would be are now potentially treatable with the new technology that they’ve got.

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White

We’ll be hearing more from the man who’s leading that research and someone who’s been on one of its trials.

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But first, machines which produce electronic braille have been with us for some time now.Ìý They act both as notetakers and reading machines, they enable us to read a far wider range of books and documents than ever before and write material for ourselves which can be transferred into print for friends and work colleagues.Ìý The problem has been that until now they’ve been very expensive, costing somewhere between two and four thousand pounds, putting them out of reach of many braille readers.Ìý But back in early 2016 on this programme we were assured that a far cheaper model would be with us within months.Ìý The result of cooperation between visual impairment agencies throughout the world, with the RNIB taking a leading role.Ìý Then it all suddenly went very quiet and although the Orbit 20 was being made available in other parts of the world no sign of it here.Ìý

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Well finally it has arrived and not simply on the market but here in front of me on the table – I’ve actually got one here and I’ve just switched from my hard copy paper braille script to the Orbit 20, which is making an interesting grasshopper-like noise. I have to say it’s neat, small, it’s about six inches by four, roughly, with comfortable braille – fits nicely under the finger – maybe the lines are rather too short for reading a radio script, which is why I’m a little bit hesitant but that might come.Ìý And also, fine, I’d say, for reading notes or short documents.Ìý I’ll go back to the paper script now, if you’ll forgive me.Ìý It does come in, for visually impaired users, at around £400.

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Well in a moment we’ll be hearing from a user better qualified than me who’s been assessing its performance but first, I asked RNIB services director, David Clarke, why have we had to wait so long.

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Clarke

There were some production delays, it was absolutely our original intention to launch this much earlier but I think what we were really conscious of is to have a product that was actually fit for market.

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White

But it has been available in other parts of the world – USA, New Zealand – and it does seem odd since we were led to believe at the beginning of all this the RNIB was a big player in bringing it to market, so how come it’s got to market here later than everywhere else?

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Clarke

As I say we wanted to make sure it was a product that was fit for market.Ìý I think you’re absolutely right, for example in the USA the product did launch much earlier but shortly after launch had a product recall.Ìý And we were absolutely definite in our minds that we didn’t want anything like that happening, we wanted to continue the consumer testing and make sure the product was ready and we’re now satisfied, after that testing, that it is ready and delighted now to be offering it.

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White

Can you just give us an idea of what the technical problem was?

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Clarke

It was a part that wasn’t working properly which were very keen to make sure it did, we’ve now reached that stage and that’s why we’re launching it today.

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White

Well, that’s some of the history but what’s it like and how does it perform?Ìý Jackie Brown is a very experienced user of notetakers and braille readers and she’s been running her fingers over the Orbit Reader 20 for us and I asked her what she made of it.

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Brown

I think it’s fab, to be honest, I really like it.Ìý I think the braille is very clear.Ìý For some people they may say that it’s quite firm but I actually quite like that because I think it’s really nice braille for reading, it’s very clear and sharp but not uncomfortably so.Ìý And I like its compact sturdy feel and I like the idea that it pairs up with Bluetooth to your phone, if you want to use it with an iPhone or an iPad or an android device and you can also use it with your screen reader as well.Ìý So, it works well with JAWS and NVDA, it’s very nifty.Ìý It does keep up with you, it definitely refreshes well enough for I think anybody that reads very quickly, I think it refreshes well.Ìý The most important thing for me is that it puts braille in the hands of more people than could ordinarily afford to buy a braille display of four figures.

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White

And that, of course, is because of price but people would say you can’t produce something at so much less without losing something, what can’t you do with it?

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Brown

The main thing for purist and people who like to use cursor routing buttons, that’s a button above every braille cell so that you can manipulate the cursor, you can’t use that.

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White

Just to make it clear to people, that’s what you would normally use to edit because you could bring things up in relief and have them exactly where they ought to be so that you can edit.Ìý Does that mean you can’t edit with it?

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Brown

You can edit with it, yes, it is feasible to do it but you just have to – the method of doing it is different, you’ve got to do something else.Ìý For people it might be a deal breaker.Ìý For me, personally, it isn’t because it does what it says on the tin, it’s a reader, it has an SD card slot so you can put loads of formatted braille text on there and also RNIB have put a selection of books – classics and other things – on an SD card to get you started.

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White

That’s the hardware, do you have any other concerns?

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Brown

My only concern is how well it will be supported by RNIB given the fact that if it needs to go back, if there’s a problem with a dot or there’s something that it’s not doing or a customer needs help with setting it up perhaps or pairing with their iPhone, because I think this will be quite a popular device and as I said before, put braille in the hands of a lot more people.Ìý I really just hope that the support will be available.

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White

Jackie Brown, talking to us from her home just outside Belfast.

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David Clarke, we will come back to her question of support, but first of all, who is the Orbit Reader 20 really aimed at?

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Clarke

Well I think the original intention of the Transforming Braille Group was to create a low-cost device that opened this up to a much wider market of people who simply couldn’t afford the much more expensive products.Ìý And I think that’s what we’ve achieved.Ìý There’s currently 20,000 people reading braille, as we understand it at the moment, for whom we hope as many people as possible will be opened up to be able to access braille on this device.Ìý And I think it was really lovely, and I have to say exciting, to hear Jackie talking about the various ways in which that’s possible.

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White

Some of the people who could benefit from this aren’t as tech savvy, so what about Jackie’s point about support, both at the point of buying it and of course when little things start to go wrong?

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Clarke

Yeah, I think that’s a really, really important point she raises and frankly it’s my job to make sure that this happens.Ìý We see ourselves as a bespoke retailer in this kind of area of the market and it’s really important that we provide customers and users with a really good level of service.Ìý We have our tech for life team who are more than happy to talk about this stuff over the phone but we also have an army of volunteers who are prepared to work with people to support them with this device.

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White

Because that really is important, isn’t it, for blind people quite often trying to do stuff over the phone is just not good enough, you need somebody with you, you need hands on, you need to be able to… are you really going to be able to do that?

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Clarke

Jackie referred to the fact that she thinks this is going to be a very popular device and we’ve committed to buying 2,000 of these devices, so we believe it’s going to be very popular too.Ìý But I think she raises a really valid point in that the actual support that people need is essential and I will be, as I say, responsible for that and making sure that it happens.

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White

Well we might have to hold your feet to the fire, if there’s a problem about that…

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Clarke

You’re very welcome to Peter.

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White

…because people will care about that.Ìý There are a number of commercial readers and notetakers available of course but what do you think will be the effect on them of a much cheaper product, will they be driven out of business?

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Clarke

Well that’s certainly not our intention.Ìý I think that this has been a terribly useful experiment in disruptive behaviour and I think you’re already seeing a reduction in prices right across the piece….

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White

You mean disruptive in the sense that it is designed to force the price down?

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Clarke

Well I was asked specific intention to make a cheaper version of it and I think if our actions, on a global basis, that’s achieved this encourages others to look at their devices and find ways in which to bring their own prices down, without compromising on major features of the device, I think that’s a good thing.

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White

I imagine the RNIB have had to put quite a lot money into this project, are you going to get that money back?

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Clarke

I sincerely hope so.Ìý This is not a profit-making exercise in any way by the RNIB, we put $250,000 in from the start in a budget of 1.25 million US dollars to get this project underway and the price has been set to ensure cost recovery but no more.Ìý And we’re very, very hopeful that our stock will very quickly move off the shelves and then we can consider what extra orders we make.

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White

That’s David Clarke, services director at the RNIB.

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Well, today we’ve also got news of what looks like real progress on the medical front.Ìý A number of eye conditions are caused by faulty or missing genes and the hope has been growing that those genes can be replaced by effective ones.Ìý In one specific set of trials a rare form of blindness, called choroideremia, has been treated by an injection into the back of the eye with the gene that’s missing.Ìý Initial results have been encouraging.

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Joe Pepper is one of the small number who’ve been on the trial and he told me more about his condition.

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Pepper

I suffer from a condition called choroideremia, it’s not that easy to say but basically what it gives you is a gradual deterioration of your sight and that will ultimately lead to blindness eventually.

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White

Have you been losing sight as you’ve grown older?

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Pepper

Yeah, so I’m 26 now, from about the age of 10 there’s been a gradual decline in sight loss, particularly from the age of 16 onwards, that then adds that element of real worry and anxiety, you’re losing your sight but you have no – there’s no real hope of that ever stopping.Ìý I quite often describe it as you’re kind of constantly going through grief – you’re losing something which you really don’t have any control over losing.

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White

And explain what’s happened and what’s been the effect of the treatment that you’ve had?

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Pepper

I had an injection in the back of the eye, which essentially gave the cells back the gene that it had never had.Ìý It’s basically guaranteed that I will never lose my sight and also, it’s given me a bit of vision back in my right eye.Ìý It’s the equivalent to four lines on an eye chart.Ìý Many people won’t grasp that when you’re sitting there and you’re contemplating, in your teenage years and in your early twenties, and you wake up and panic attacks and having just dreamt of just pure blackness, to just have that weight totally removed, it has changed my life unimaginably.Ìý

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People want more, our society is all about wanting this and wanting that, I’m just exceptionally happy with the sight that I’ve got and I now know that that’s the sight that I’m going to have for the rest of my life.Ìý Me and my parents were – when I was nine years old and I was told that I had this condition which no one can really say and I was told that maybe in 20 or 30 years there might be something, 15 years down the line I’m sitting with sight that isn’t going to change.Ìý That is phenomenal.Ìý I can move about when the dusk is coming in, I can function.Ìý You know the kids at my school – I’m a schoolteacher – I can mark their work with ease, I can read their work.Ìý I can plan lessons, I can see my friends – all the things that actually we just take for granted I can do and I never have to worry about not doing them ever again.

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White

Joe Pepper.

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Well now the team has produced a more detailed report on the progress they’ve made.Ìý The research has been led, at Oxford Eye Hospital, by Professor Robert Maclaren and he told me a bit more about Joe’s condition.

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Maclaren

Well choroideremia is part of a spectrum of diseases under the banner of inherited retinal degenerations.Ìý These are genetic conditions that cause blindness.Ìý The development of gene therapy is a new approach that enables us to insert the correct gene back into the cells of the retina and therefore slow or prevent the degeneration from progressing.Ìý A gene therapy treatment, Luxturna, has been approved in principle by the European Medicines Agency for the first time for retinal degeneration and that was last month.Ìý So, we are, I think, in an era now where we’re moving from science and research into genuine treatments for patients.Ìý If we get the dose right at the beginning and if we intervene early enough whilst we can still rescue the cells we would expect it to have a lifelong effect.

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White

How much improvement in sight are we talking about?

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Maclaren

We’re looking at improvements in the central vision, a sharpening of the vision, what we call visual acuity.Ìý But ideally, we would like to intervene in the disease much earlier on, before they lose their vision, so that we can preserve the visual field.

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White

So, if you could put that in terms perhaps that people could visualise, can you give me an example of what perhaps somebody would have been able to see and what they can see now?

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Maclaren

Yes, so, if you imagine if you’re looking through a telescope and the telescope is not focused correctly in the distance, what we’ve done is we’ve focused the telescope by improving the clarity of the vision in the centre.Ìý But what we haven’t done is we haven’t brought back the cells in the peripheral vision, you’re still looking through a telescope.Ìý It certainly has been life changing for some of the patients in the trail because they’ve told me so.Ìý We are currently in a phase three clinical trial, which is international, involving several different countries and patients around the world, in order to get approval for the drug or the virus as a treatment.Ìý Once it’s approved as a treatment obviously we’ll be in a position to be able to treat people and give them some reassurances.

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So, it’s a very exciting time at the moment in gene therapy.Ìý Diseases that I was taught, during my training, were incurable and never would be are now potentially treatable with the new technology that we’ve got.

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White

I know you try to resist exaggerating claims for what you can do but how much of a breakthrough is this do you think?

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Maclaren

Well I think the breakthrough is at two levels.Ìý There is the scientific breakthrough of showing a long-lasting effect following the gene therapy and I think everyone would agree that this is something which we predicted based on the laboratory studies but we still needed to prove was the case in humans.Ìý

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And the second thing, I guess, is the ability to take a rare single gene disorder and develop a research programme that then becomes commercialised, if you like, as part of a spinout in order to develop a treatment in a trial that includes many countries around the world.Ìý One of the problems with rare diseases is that there are very few patients, it’s very difficult to get companies interested in the treatment, but we’ve been able to do that as well with the choroideremia programme based on the very successful results we’ve had in the early phased studies.Ìý There are now over 200 patients in the trial across many countries in the EU and North America.

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White

And how do people – you obviously get to know these patients – how do patients react to what’s happened?

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Maclaren

Generally, the initial results are that the vision goes down because the patients are recovering from an operation, that requires retinal detachment and some other procedures at the back of the eye.Ìý But usually the vision comes back to normal around about one to two weeks.Ìý And then between the sort of two- and four-week period they may notice improvements in their night vision or some patients report improvements in their colour vision.Ìý Some patients don’t really notice much difference at all but you’ve got to bear in mind the reason for giving the gene therapy is to prevent further retinal degeneration.Ìý So, it’s through following these patients for many, many years we’ve discovered that the initial improvements that they described have been sustained.

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White

But may be not the kind of eureka moment that perhaps some people would like to imagine.

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Maclaren

Well I’m not sure about that because although the operation itself is quite challenging and obviously quite stressful for the patients I think the eureka moment is when they come into the clinic, having been told for the lifelong that they’ve got a disease that is incurable and nothing can be done, to then suddenly be recruited into a trial for a potential treatment, that I think – that process – is the eureka moment for many of these people.

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White

So, perhaps there’s also a sense that they’re doing something for the whole group of people who’ve got the condition.

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Maclaren

Exactly, I mean I couldn’t agree with that more.Ìý A lot of my patients know that they’ve lost most of the vision but by contributing to a research study and helping in a trial for others, particularly people who may be in their family, for instance grandsons of some of the patients who are affected, have also got the early diagnosis, they know that they can help them as well by being in the trial.Ìý The patients obviously help us but ultimately, we can’t do the trail without patients who are volunteers and we remain extremely grateful to them for coming forward and helping us with these studies.

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White

That’s Professor Robert Maclaren.

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And that’s it for today.Ìý We always welcome your queries and your comments, you can call our actionline for 24 hours after tonight’s programme on 0800 044 044.Ìý You can email intouch@bbc.co.uk or you can click on contact us on our website.Ìý And you can also download tonight’s and other editions of In Touch from there.Ìý That’s it, from me, Peter White, producer Kevin Core and the team, goodbye.