Partially deaf, partially sighted, totally Usher

Picture of a retina, that of somebody living with usher syndrome.

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My name is Fabrizio, I have Ushers and I am 35 years old. Or to put it another way, I am 18 with 17 years worth of experience!

Like many Ushers I was born deaf and again like many that is all I thought it was. I did speech therapy, I played lots of sports, particularly rugby, and over time I got confident in myself and really did not see the deafness as a barrier to achieving any realistic goal that I set myself. I did notice that I suffered from night blindness in my teenage years, but thought nothing more of it. I also found playing rugby a bit more difficult because I started to struggle to catch balls thrown to me from the side (but not from the front or up high) and I put it down to being outside a centre with a really weird passing technique. With hindsight, it is one of the interesting things that you realise as an Usher, the brain will very subtly adapt to accommodate any shortcomings without you even noticing.

And so life went on. I went to university, I learnt how to drive a car and then I got a job in the civil service. Around 2004, at 25 years old, I thought that I really should look into the night blindness. I was working in London at the time and walking among the daily commuters in the dark was proving to be quite a bruising challenge. So my GP referred me to the Chelsea and Westminster for a specialist eye examination. The doctor assigned my case was a pretty girl of Iranian descent. She looked into my eyes and said, “Hmmm, I think I would like to run some more tests.” Five hours later, three rounds of eye drops, a boring cycle of waiting in the waiting room and then having bright lights shone into my eyes, and a brief examination by her boss produced the verdict.

“I think you have RP, Retinitis Pigmentosa” she said. She showed me blown-up pictures of my eyeballs (I still have them and so included with this post) and pointed out the faint dots scattered around. She further explained that in view of my deafness, it may well be a condition called Usher’s syndrome, but she would have to refer me to another specialist for that diagnosis. I took the news pretty calmly, not least because it had been a long and very boring day and the whole thing felt rather surreal. But things were going to get a bit more amusing. “What are you doing on Saturday?” she suddenly asked. “Wow” I thought, “maybe this day is about to get better?” So I replied that yes I was free. “We have a group of trainee doctors and we like to have good subjects for them to practice diagnosing.” Noticing the odd expression on my face she paused. To which I explained to her that asking a man if he was free on Saturday usually meant something else. Her subsequent blush was the highlight of my day! She was a nice girl and yes I went along and got peered at by a group of trainee doctors. Some of them even got the diagnosis right!

It has to be said that not much changed for me. I went to see more specialists and the Usher diagnosis was confirmed. I got on with things, I had things to do, places to be and I wasn’t going to let Ushers stop that. I moved to Bristol and was still driving. It felt like a problem that could wait. The tipping point for me came about five years later when I started to get worried about the driving. For those who know Bristol, it is not a car friendly city and there are a lot of reckless cyclists and pedestrians seemingly bent on lemming like dashes into odd places. I realised I was missing things, important things and it started to feel like one day I was going to just not see someone with tragic consequences. So I referred myself to the eye specialist and the DVLA who came back with the devastating judgement that I would have to surrender my driving licence.

It was quite a shock, which seems silly really as I should have been expecting it. More than anything else, it was the perceived loss of independence that really got to me. I wanted to continue to be self sufficient, to be able to go anywhere I wanted and to do what I felt like doing without having to rely on others. It was how I lived my life, with the attitude that my disabilities were not going to stop me. In some ways that feeling never goes away, if I am having a bad day that feeling of not being as independent as I once was bubbles up. I have grown more comfortable with it over the years. I can even make the joke that others have to do the driving so I will have to do the drinking on their behalf. But that little spark of resistance is still there, couple that with my innate stubbornness and no doubt I do persist in doing things that others would give up. For example I still cycle to work whenever it is light enough. Risky? Undoubtedly, but I feel it is manageable and I absolutely do not want to give it up unless I have to. I feel that as an Usher sometimes it is vital to stubbornly hold onto the things that are important to you even if it does involve taking a bit of measured risk on the way. It is the only real way we have of showing that we will not let our disability rule us and we are still capable of having meaningful lives and having fun while we are at it.

So yes life has changed a lot, but that is inevitable for anyone, with or without a disability. I have to make some adjustments, but I find it is more a matter of planning ahead and thus avoiding putting myself in difficult situations. Work is sending me to Copenhagen for a couple of days next week. I am looking forward to it because I have never been there before, but I am going on my own and a northern European city in late November is going to get dark early which is a bit daunting. However, I have planned the route and I am informed that the Danes are superbly friendly and helpful. In fact, I even have a small bet with myself as to whether I can make it to the train at the airport while carrying my white stick without receiving an offer of help. Sometimes you just have to ignore caution and take a bit of risk to experience the best of life and people.

My advice to other Ushers is don’t let the fear, particularly of the dark, rule you. Try to avoid staying at home because facing that dark and noisy world is so daunting. You are probably not going to really enjoy clubbing, but there are still so many things you can do. You just have to want it bad enough and to be willing to plan well to make it happen. Failing all that, just cheerfully jump onto a friend or even a stranger for help. You can be amazed how positively responsive they can be.

Or as it says on the T-shirt – Partially deaf, partially sighted, totally Ushers!

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