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Adapting to Usher syndrome – Living with both Deafness and Sight Loss.

This is the transcript of the Radio 4's "You and Yours" show which was broadcast on 26th July 2004 in which Nick Sturley who has Usher Tyoe 1 was interviewed about his Usher. He was supported by a BSL interpreter and a hands–on Deaf relay during the interview and the presenter was Peter White, who is blind himself.

WHITE: Now how on earth do you prepare for the loss of your sight when you're already dealing with the effects of having been born partially or profoundly deaf? It's the cruel reality which faces those people in Britain with Usher's Syndrome. Usher's is a relatively rare condition and indeed little is known about it, which is why the charity SENSE has just begun a three year study of it. With the help of the Institute of Child Health and Ophthalmology 200 people are being asked to describe the effects of Usher's Syndrome on their families. The aim is to learn more about how it progresses to bring about earlier diagnosis, which in turn will mean we'll be able to offer more help to those people with the condition to prepare for its effects and make important life decisions. At present people are often not diagnosed until they begin to lose their sight, usually in their teenage years.

Nick Sturley is now in his 30s, he was born profoundly deaf, he began to lose his sight when he was 10. I asked him how he first learnt of the diagnosis of Usher's. His words are spoken for us by an interpreter.

STURLEY: I was diagnosed with Usher's at 12, although my parents knew that when I was about 10. And the way my parents realised that I had Usher's is that once my mother caught me, as I was leaving the house, via the front door and going down some steps, I ran down the steps and my brother's bike was laid on the path outside and I just ran straight into it and fell over. And my mother noticed that something was wrong because I didn't see the bike and that was at the age of 10 – no I was 9 at that time – and that's when they realised that I had a visual problem.

WHITE: How much has your sight deteriorated and can you give us an idea of the pace of the deterioration?

STURLEY: It goes in sort of peaks and troughs – suddenly I'll lose a lot of vision and then it'll stay the same for a while, it's not a gradual decline, although it's not the same for every person – each person that has Usher's will lose eyesight at varying levels. And now it's got to a point that I do need a full–time guide if I'm outside of my home environment.

WHITE: Most people would think that having one disability and then being told that you're going to have the loss of another sense is just about as unfair as life gets – how did you react to it?

STURLEY: It's quite a debatable issue because in deaf culture deaf people don't see themselves as disabled and I feel the same. I don't know whether I would actually accept the label of disability as a deaf person. Certainly as I began to lose my eyesight I started to accept – I've decided to accept that label more. But I certainly wouldn't class myself as being a disabled person for the deafness, I am culturally and linguistically deaf. So I would say an additional disability is a mute point. Some deaf people do see themselves as disabled but I think that is quite a personal view.

WHITE: So does that mean that to you really the loss of sight is far worse than being profoundly deaf?

STURLEY: Yes, but I think that's because I was born profoundly deaf so I have that cultural identity, as a deaf person I've always sign language, I've used British sign language, throughout my life. So in a case I've not missed not being able to hear, as I say, being brought up as a profoundly deaf person that's been the norm for me. But in losing my eyesight obviously eyesight is something that's very valuable to me because it's the way I take in all my information.

WHIE: What kind of information really would help and I mean had this study been done 20 years ago what would you like it to have concentrated on? What would have helped you most?

STURLEY: Generally information on Usher's is now much better than it was 20 years ago but it does need to be a lot more because many professionals – doctors etc. – are still unaware about Usher.

WHITE: Are there really practical things which would have made or would still make a difference to your life?

STURLEY: I think there is a general shortage of people working with Usher's, sort of what are classed as human support, people that become sort of support workers – guides – for people with Usher. I think that's one thing. So certainly people working with deafblind people.

WHITE: How do you face the prospect of losing the rest of your sight?

STURLEY: I have an inner fear of that. When your vision changes it feels like your life's being eroded and it feels like, you know, a layer – a layer of you has gone and it's something that you don't want to lose. But I think the important thing is to actually hold on to what's left, rather than think about what's gone. I compare it to somebody waiting on death row for execution – I know that sounds horrible but that's how I feel, you just feel like you're waiting for something to happen. But I think the important thing is to concentrate on life as it is now and just take things one step at a time and make the most of what you've got for now.

WHITE: Is much of what you do to use the senses you have – is that really a question of adapting practically to situations, not kind of being phased by them?

STURLEY: Basically I receive money to employ various support workers and, as I say, that money is received from Croydon Council, which I think is very, very good. Three years ago there was a provision made in law that this had to be provided for all deafblind people but still many councils do not provide that and there are more than 23,000 deafblind people in the UK, so to say that only 40% of those people are receiving the appropriate help I think is a terrible situation and I think we do need to look for improvements in the law.

WHITE: Nick Sturley, thank you very much indeed.

Thanks to the producers of Radio 4’s “You and Yours” programme for their permission for allowing this transcript into this UsherLife website. © ‘You and Yours’ 2004.

© Nick Sturley / UsherLiffe 2005-10. All Rights Reserved.