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Items filtered by date: June 2019

My name is Helen Colson. I’m 29 years old & live in Southport, Merseyside. I was diagnosed with Retinitis Pigmentosa and as I am moderate-severely deaf, Ushers Syndrome last year. 

It wasn’t easy getting my diagnosis after Specsavers were concerned about my visual field test results and referred me (via my then GP) to Heartlands Hospital in Birmingham as that’s where I was based at the time. 

After dilating my eyes and other tests, they initially wanted to write it off as jelly breaking off at the back of my eyes which is apparently common for someone in their late 20s but they were concerned about scarring on the back of my eyes and was told I’d be seen again December 2017. 

In November 2017 I went to do my visual fields testing again but didn’t see a consultant until July 2018. I weirdly don’t remember this appointment at all. Other upon informing them I was just about to move to Southport; I was asked to stay until my diagnosis was sorted. Two months later I had another visual fields test and the nurse was concerned as it was getting worse, so managed to get me to see a trainee. We had a chat and it was suggested I needed to go to two other hospitals in Birmingham for further testing as they couldn’t do an MRI scan. 

In October I went to the Midland Eye Clinic for electrodiaganostic testing which I got very anxious about as by now I was getting desperate for answers. I returned in November to see a different consultant who didn’t know why I was there and would later on send my diagnosis via letter. 

I felt like my world fell apart after reading that letter and jumping straight onto Google. I’d only ever heard of Ushers when Molly appeared on SeeHear about a decade before. 

I did eventually see my consultant again January 2019 & my care wasn’t transferred. But I am seeing Professor Black in Manchester next month. 

It’s been a very scary six months post diagnosis especially as I was left with no information or support initially and due to my pre-existing ambolyia in my left eye I didn’t want to go blind. I also had a massive breakdown but now I’m learning to rebuild my new life. I have a symbol cane & am learning to use my long cane. I am going through the process with Guide Dogs to get one. I’ve luckily found someone here who also has it herself. So we both really support one another. 

I’m also currently running my own page (Living with Ushers Syndrome) & am making a documentary about the condition as so many have no idea. 

I don’t know what the future holds but I’m trying to tackle it head on & won’t let it stop me from continuing to add to my many achievements.  

Hello Everyone! Would like to wish you all happy deaf blindness week! I’m so sorry that I haven’t posted in it feels like ages! But I’ve got soooo much to tell you all and I can’t wait to tell you everything. I think this blog is going to be a very long one so I think you will need a lot of time to read it, sit down with your favourite thing to drink and a few snacks and I just hope you are looking forward to reading it. The things I am going to tell you are, going to the hospital and getting tests done, getting an Apple Watch, more stuff about my cane and meeting others with usher syndrome such as Molly Watt so here it goes....

So let’s start from the beginning of these hectic months, so I went to the hospital to have an electro diagnostic test where I had my eyes numbed and I had wires put under my eyes to test my muscles and nerves in my eyes, not going to lie I was sooooo nervous because I had no idea what was going to happen and I kind of liked it that way but then I didn’t if that makes sense. Like I knew that if I didn’t really know I wasn’t going to freak out as much, honestly it wasn’t that bad! There was just no pain at all which was soooo good, the only bit that wasn’t very nice was the eye drops and then when the doctor put these sticky pads on my head he had to use some sticky glue for it to stick and it was gritty so it hurt when he was putting it on my head it was like a scratch but yeah it really wasn’t bad which was such a relief haha!!  My next appointment to go to my normal hospital is in August when I will find the results as much as I do want to know my results it’s not going to change anything, as you all know there is no cure, it’s still upsetting but there’s no point waiting for one to happen you’ve just got to keep going!! The people who have had eye drops will know what I mean haha, your eyes do just water!

Next up is about MY APPLE WATCH!!!! I haven’t wanted an Apple Watch just for the fun as you know I love technology haha! But now my eye site is getting worse I’ve found out that an AppleWatch is like the next best thing because it’s small for my peripheral vision and it vibrates to notify me when I’ve got a text or call. I can answer calls on it and I can hear it very well and it’s always with me, so it’s very handy! Another thing I’ve learnt is that it can tell you where to go if your on google maps, I had a go on the weekend it was really good, different taps tell you to go left or right and then when you have reached where you have to go. It is a very good gadget so anyone with usher syndrome I would definitely recommend it. I’ve only had it for about a month and it’s changed my life completely!!

So here goes the more emotional talk, as you all know my views on having a cane from a previous blog.  The lady has come in again and has ordered a cane for me, like really? I sooo don’t want one but then I’ve got to try! We were going to have a go with one she has brought in but because of exams being on we couldn’t which I was quite happy about haha! I just hate the fact that when I get the cane out I’ll be read as blind, I’ll become a book! I just don’t want to be seen as blind because I’m just scared that people will say stuff like omg she’s blind or oh yeah let’s trip her up she won’t see me or even laugh at me because I’m holding this cane. Just yeah I’m not really happy about it but at the end of the day it will tell others to stay away from me and to just mind out the way, if they don’t they will get hit haha! But yeah no one is alone, people do have canes and manage so I’m sure I will. Once I start training I may enjoy it which will be good haha!

The final thing I want to talk about is on the 22nd June there was an Usher Kids Day for children and families who are suffering with usher syndrome and need help and get to know others with usher syndrome. I’m not going to lie I really really didn’t want to go because I was like what difference is it going to make? But believe me it made me way stronger and I met some amazing people who I can now talked to about my condition and help me with any problems. I spent a whole day there and

everyone was so nice and so understanding and it was nice to know that I wasn’t alone. It was very emotional hearing other people’s stories and about how they felt but also how similar everyone’s symptoms were! It just brought a massive relief that I’m not the only ‘deaf blind’ person in the world. One of the best things that happened to me was meeting literally like my hero Molly Watt who also has Usher Syndrome, I don’t know how I would of coped these couple of months if it wasn’t for her

positivity! She is literally the best person ever in the world and just to meet my idol was sooo emotional! She was just soooo lovely and it was really nice to get to know her even more although I was in a right state haha how embarrassing!! 

 

 

Where does our story begin..... Alice (now 16) was born profoundly deaf. In Oct 17 at a routine eye test (Alice has always worn glasses) they picked up an issue all we were told is she had black spots in her retina it was nothing to worry about!! That she would be refered to the hospital.

On returning home I did what most of us do and used good old Google my heart sank I had a panic attack and didnt know who to turn to.

Fast forward to February after local appointments we had an appointment at Gosh where we were told Alice had rp and combined with her hearing impairment she has Usher1 syndrome (I knew as I had already been doing alot of research) just needed the formal diagnosis. Alice was registered sight impaired on that day and many appointments were booked. It was then on speaking with Paula the eclo we first heard of Molly Watt. On the train home both me and Alice watched videos and read articals by Molly it's then that we reached out. From day one Molly and Jane have been amazing.

Jane (Molly's Mum) has been a huge help to me as a mother who is that bit further ahead of Alice and I on the usher journey. The information she has given me has been invaluable to getting where I am today.

The Usher Road for us has been a very rocky one. School was awful they couldn't or wouldn't except Alice had issues because she used he residual vision so well despite 6 months after diagnoses being registered severely sight impaired. They even refused to allow her to use her cane in school!!! Jane guided me through many a minefield and eventually Alice got the support needed. As a result of her guidance pointing me in the right direction and letting me chew her ear off as well a many sleepless nights on my part fighting the local authority trubunal Alice now has a place at The Royal National College for the Blind.

Alice has a special bond with Molly and really looks up to her she is an amazing role model and advocate for Usher. We both follow one of the first things Molly said to us when we met her..... Focus on the can dos and not the can't dos. As a result Alice did complete her gcses including art. Also as a result of meeting Molly and Jane we now have so many new rp/usher friends (family) who I am now able to be there for if needed.

Molly and Jane thank you for the last 2.5 years helping both me and Alice learn and understand that anything is possible being deaf/blind having Ushers........ Exciting things are awaiting Alice now ❤️ #ushfam #rpfam #deaf/blind #inittogether

Tuesday, 25 June 2019 14:11

The evolution changing Usher Syndrome

Usher Syndrome is a strange thing, strangely it is evolving even though it hasn’t changed. 

It remains a condition without a cure. There are variations in level of deafness, in level of blindness, in chosen mode of communication, in chosen method of mobility aid(s).

We all have daily challenges living with deafness and progressive blindness but it doesn’t mean we are incapable of living fulfilled lives.

Since my diagnosis and acceptance of my condition I have learnt lots of things not just about the condition but also about attitudes towards it.

Sadly there is still ignorance surrounding it and also far too much negativity.

Usher Syndrome is a challenge for all living with it, however the challenges vary from person to person. 

Communication is an interesting one, age is definitely, in my opinion a big factor. 

It is wrong to assume all with a profound hearing loss communicate using sign language.  At 24 years old I have met lots of people with usher worldwide and my observation is that the older generation of all usher types with varying levels of deafness are more likely to use sign language to communicate, but there are some younger people still using sign language, that said some do communicate orally too.  There must always be consideration of the varying communication types. 

The younger generation, our millennials, are much more likely to be oral, though again some do sign.  I believe the reason for the change is because hearing aid technology has never been better.  Cochlear implants have made an enormous positive difference along with the complete evolution of hearing aids in my lifetime from analogue to digital and more recently the smart hearing aid - yes, assistive technology wins the day. 

It is actually technology that is evolving, not Usher Syndrome and we need to embrace it.  To really embrace it there needs to be more understanding of needs and what assistive technology is available and the biggest ask is we need funding for real enablement.

Ever considered a pair of smart hearing aids would cost less then the equivalent of somebody being unemployed for a year and that is without considering the health and wellbeing of a person able to be an active part of society, to be included is priceless in my book.

Neither cochlear implant nor hearing aids are a cure for deafness but an amazing aid for listening, hearing and accessing communication and information, but that is only the beginning.

Did you know the *smart hearing aids I wear have bluetooth connectivity to a plethora of mainstream technology.  What this means is that sound is streamed directly from my iPhone, applewatch and to my ears, as a result I can now hear and use a telephone which might not sound much to many but when you consider how often help is still only available via the telephone, it shouldn’t be that way but it is.  Using a telephone is also very enabling in the workplace.

Being deafblind and able to hear clearly brings access to the many apps that amongst others Microsoft have developed for the blind that can only be accessed aurally, for instance I can point my phone at an object, person, environment and the app (Seeing AI) will describe it to me.  This enables me to be more independent to not be fearful of not having somebody to ask when I am out and about, on that note I should mention the importance of directional sound for somebody deafblind.  Since wearing smart hearing aids I have benefitted enormously, I can now not only hear a sound, be it speech, a siren, a sound of danger or an alert of any kind I can turn to that sound and act accordingly.  I can now rely on my ears to compensate for my eyes, for me this has been the greatest thing I have experienced since losing my sight.

I always thought I had pretty good speech and I did but since wearing smart hearing aids my speech has improved. I hear speech differently, tones and clarity, a sort of warmness I hadn’t experienced before and has made so much difference. 

I no longer feel the isolation I used to in what was a pretty silent dark world.  Doesn’t everybody deserve that? 

For your information:

These two youtube pieces demonstrate the change in my speech - check me out at age 14, excuse dodgy hair and glasses! https://www.youtube.com/watch?v=z8tXf36Qx6E 

then compare now, thankfully dodgy hair and glasses gone too https://www.youtube.com/watch?v=Hq6rRQTIoqM - don’t we want this for everybody?

 

*Smart hearing aids worn LiNXQuattro by GN Hearing

I regularly champion assistive technology, it has changed my life beyond recognition and I want that for all living with Usher Syndrome who can and would without a doubt benefit.

Sunday, 23 June 2019 19:32

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. 

 

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