Whispers in the Dark
It’s never been easy. Having to scan the floor in front of you when walking down the street. Having to pre-plan the route to the nearest toilet in a restaurant. Having to feel your way through an unfamiliar room in the pitch black. Having to hold onto your mate as they stroll through the club. Having to desperately apologise to someone after walking straight into them in the supermarket. Those of you with Retinitis Pigmentosa know exactly how it is. Those of you who don’t might only begin to imagine. What on earth is it like to see in front of you, and to your left, but not in the middle? Is it black? Is it blurry? It’s neither. It’s just not there. I ask you the same question: What is behind your head? Is it black? Is it blurry? No. It’s just not there. This is what it is like living with peripheral vision loss. “Oh, you’ve got vision loss? Have you thought about wearing glasses? Are you wearing contact lenses?”. I can’t blame people for being uneducated on the matter. How many people do you ever bump into with peripheral vision loss? It’s just not a common thing.
The eye is a complex thing. Most people know about the lens, the iris, the pupil and the retina. Maybe you know about the cornea, the macular and the optic nerve too. The majority of those who suffer from some degree of sight loss have a problem with their lens; It just isn’t the right shape. Some people are shortsighted, where they are able to see things close, but not far. Some people are farsighted, where they are able to see things far, but not close. Some people just have a lens that doesn’t allow them to see either. This is all stuff that can be corrected with glasses. Glasses simply alter the way the light rays enter your lens, so that the correct image ends up coming out on the other side. Most of you probably know that the image projected on your retina is actually upside down. It is the brain that ends up inverting this image, so we see things the right way around. It’s amazing what the brain can do. There was an experiment where some guy was asked to wear glasses that turned everything upside down for a few weeks. After just ten days he was able to fully adapt to this upsidedowness, and his brain had actually inverted the image back the right way up. The process of him removing the glasses caused him to start seeing upside down again. It then took another week or so for his brain to adjust back again. Similar things happen to those with sight loss. Someone born completely blind just has no possible comprehension of colour. People say things like “hot is red and cold is blue”, but what the hell does that mean to someone who has never seen a colour in their life? You cannot fully comprehend something you haven’t experienced; it is just a fact of life.
“What did you say?”, “Sorry, what was that?”, “Excuse me, I didn’t quite catch that”. No doubt that these are phrases that deaf people almost feel like they need to have recorded on their phone, so they don’t have to waste their breath repeating them every fifteen seconds during a conversation. Being sat there on a table, watching people throw their opinions about whilst moving your head back and forth trying to lipread everything being said is, let’s put it this way, bloody exhausting. Some people are easy to understand; they have a clear crisp voice and speak in a way that resembles the language that you have grown up understanding. Their lips move in a concise manner and they don’t have any lisps or a strong accent. Personally, I find women a lot easier to understand than men. Their tendency to have higher pitched voices really fills the gaps in my hearing loss. On the other hand, some people just cannot be understood, no matter how hard you try. Whether they have a strong accent, a really quiet voice, lips that barely move, or a massive beard that gets in the way, it is just a constant struggle. I’m sure other deaf people can relate, but is there ever a certain person in your social group or who you work with that you just hope you never have to have a conversation with? It’s not that you dislike them, but it is just the fact that you know it will be a constant struggle to have a one to one conversation with them.
It’s not just the social struggles. Being deaf comes with its other problems. Ever wondered what it is like to be woken up by a vibrating phone under your pillow? Trust me, I’d sooner have an elephant wake me up. Ever had to go into a hotel and work out how you are going to know the fire alarm is going off? Ever felt extremely isolated because your hearing aid stopped working or you had forgotten to put fresh batteries in your wallet? Ever had terrible earache, but needed to make the decision between sticking the hearing aid in and suffering, or pulling it out and not hearing? It does come with its benefits though; Being able to turn your hearing off is a huge bonus in some situations. Kid screaming on the bus. Off. Bloke playing his music too loud next to you on the plane. Off. Annoying conversation that you don’t want to hear. Off. Car alarm going off outside when you’re trying to get some sleep. Off.
What’s worse though? Would you rather be blind or deaf? It is a common question that goes around, especially during philosophical discussions with a bit of alcoholic influence. I love these sorts of discussions. It really takes people to a different level of respect for their senses. Some people would rather be blind because of their love of music and the birds singing in the morning. Some people would rather be deaf, so they would still be able to drive their car and see the stars at night. Whichever you choose, it will no doubt mean a significant part of your life will be diminished, or worse, destroyed completely. Some people don’t have that choice though. Some people must deal with both. Being completely blind and completely deaf at the same time is extremely rare, but those who have some degree of both hearing and sight loss are fighting a battle of two competing senses. Typically, one backs up the other. You may hear stories of blind people with supersonic hearing, or deaf people with eagle-eyed vision, but those with Usher Syndrome and other deafblind disabilities are neither of those. One sense doesn’t help the other. Rather, it makes the other even worse. So I think it is safe to say that you should make the most of the senses you have, as it is something that you might one day wake up without. It is our senses that make us who we are, and without them, we would live in a very bland world. But for those of us who are deafblind, I guess we must make the most of those whispers in the dark.
Please, Please, Please Consider All Our Needs
I was prompted to write a blog on reading this quote:
“For people without disabilities,technology makes life easier. For people with disabilities technology makes things possible” (IBM training manual 1991).
That quote was made 3 years before I was born and how true it is.
My life would be so different without technology, even in my lifetime it has gotten better and better and allows me to be the person I am.
Without hearing aids I would struggle so much more with communication, that is not to say I wouldn't be oral but life would have been far more difficult as I know it was for many older than myself.
I had analogue hearing aids to start with, friends my age who were profoundly deaf and did not find hearing aids useful were not implanted until 3 or 4 years old meaning they were a long way behind their hearing peers speech wise, some caught up and some didn’t.
Today children are bi laterally implanted often under a year old and that early access to speech is allowing them amazing communication skills, being implanted 2/3 years earlier makes a huge difference.
The earlier the access to sound the better.
Profoundly deaf children are now so much more inclusive than ever before. I really enjoy speaking in schools where they have deaf children and seeing how well they are doing with their up to date technology - the Cochlear Implant.
However, it makes me very sad that children and adults who need hearing aids often do not get the best available and have to get by.
I did okay with analogue and early digital hearing aids but I was one of the lucky ones, I had fantastic support from my family and local services, unfortunately this sort of support remains a post code lottery.
Now that I have the very latest in hearing aid technology, GN Resound's Linx2 I can see what so many are missing.
Anybody who wears hearing aids will tell you a new pair of hearing aids takes some getting used to, usually a couple of weeks or so and I was fully expecting this with my Linx2, however, these hearing aids took nearer 6 weeks to fully appreciate and it was really hard work. The reason for this was there was so much more for my brain to process, yes a whole lot of new sounds I had never experienced, it seemed my brain heard the new sounds before the old ones and I was in a state of confusion with it all for at least 2/3 weeks and it was a very tiring time, however, I slowly got used to the new sounds. The new sounds and different way of hearing has resulted in lots of compliments on the new quality in my voice.
Strange, I thought my voice was ok before but to be told it has improved has made me very happy.
The clarity of sound is amazing, I can decipher lyrics in songs rather than humming along and making up my own words.
I hear familiar voices differently and I can now rely on my ears to compensate for my blindness which really is a massive deal.
I have blogged about coping strategies and how the deaf can hear with their eyes, lipreading, facial gesture, body language, how some use sign language, a completely visual way of communicating and how I felt more deaf as my eyes began to fail when in actual fact my hearing was stable it just showed how reliant I was on visual clues - I was more deaf!
Usher Syndrome (deafblindness) has brought a whole lot of new challenges and some of them can be eased with the ability to rely on the awesome technology in the Linx2 which goes a way to compensating for failing vision with their ability to provide such clarity in hearing, to make listening less exhausting and the most amazing feature that blew me away “Spatial Awareness” now not only can I hear things I have never heard before, I now know where sound is coming from and can adjust settings myself based on my environment.
When you then add the amazing connectivity to smartphone and Applewatch I can now hear and speak confidently on the telephone, giving me the ability to communicate in a way I never thought possible.
Usher Syndrome type 2, the type I have is the most common of all Usher types and can be diagnosed at any age. Most have a hearing loss between mild, moderate or severe so tend to be hearing aid users and yet they do not receive the same consideration as those profoundly deaf, in fact there are areas in the UK where they have or are looking to save money by not providing NHS hearing aids to those with a mild loss or only funding one which is a complete nonsense.
Hearing aids, even the very best like mine are a fraction of the cost of cochlear implants.
Being able to hear is important to ALL deaf people irrespective of level of loss.
We need the best technology for all, it means allowing people like myself to be valuable and inclusive members of society and not isolated.
Blindness and technology is different for me, firstly I was not born blind, I had perfect vision until I was 11 years old, at least I did for sure in daytime, darkness has always been my enemy, I thought just because nobody liked the dark! Perhaps I was night blind back then!
Anyway it doesn't matter, I was brought up a very visual learner and for that reason a lot of what is expected of a “blind person” doesn't work for me as a deafblind person.
Firstly I was told I must learn braille and learn to touch type!
Braille didn't work for me as I could read text, even though the text has to be in a certain format for me to access it and I can only read a little at a time, why did I need Braille, perhaps a string to my bow at some stage but not a priority for me.
Touch typing was an interesting one because I could virtually do it already, I guess because I’d had use of a computer from an early age it wasn't really something I had to learn.
Having a macbook has enabled me to access the world since my Usher Syndrome diagnosis no extra software just awesome accessibility built in.
However, I have realised there is a lot of work to be done on accessibility and I guess understanding will come as those involved with accessibility become more aware of the different conditions and exactly who they are catering for.
Deaf people with hearing aids or cochlear implants can hear, how much they hear comes down to the individual, lots rely on captioning or subtitles to access information.
In my case I’d rather be asked what i can hear, then my needs can be accommodated properly.
Equally most blind people can see something, I have read somewhere that only 5% of blind people see nothing at all, again for me I’d like to be asked what I can see.
The assumption that all deaf people hear nothing and communicate with sign language and that all blind people use Braille or Voiceover is stereotyping and not acceptable.
Clearly nobody considers people with Usher Syndrome might not be able to use Braille and not hear to use voiceover or like myself not made the transition to aural learner in which case it would make absolutely no difference how much verbal describing any app does, Facebook are you listening?
You are not meeting the needs of the majority of blind or deafblind people who choose to read text they can modify themselves.
It is pleasing to hear Android users can finally access dynamic text, just cannot understand why the hold up for those of us using IOS, especially as it has been made available on Facebook messenger.
I have asked when the required changes are being made but no response to date, I’m hoping sooner rather than later.
I know lots of people with Usher Syndrome and our needs do differ however we all have one thing in common, we want to benefit from technology, it really does make so many things possible, it can enhance our lives, allow us inclusion and safety, give us the ability to communicate with others and to be active members of society.
Please, please, please consider all our needs..
Partially Deaf, Partially Sighted, Totally Usher!
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!