Hi folks I hope you are all well and keeping your spirits up.
I thought I’d write an update on my experience wearing Linx Quattro hearing aids from Gn Resound for just over a year. Donated by the Molly Watt Trust in collaboration with GN Hearing and The Hearing Clinic, Glasgow.
I was the first person to be asked to take part in a very important and necessary project to acknowledge the real hearing aid need, over and above deafness alone for those living with deafblindness. I have Usher Syndrome type 3 which is incredibly challenging.
I am now living with a severe to profound hearing loss and around just 3 degrees of central vision, no peripheral vision and total night blindness.
Usher Type 3 is different from both Type 1 and Type 2 and the rarest, it really hits home later in life.
I went through my teenage years ok but noticed something with my vision was wrong at around 21 years old. I’m sure you’ll appreciate I only see through my eyes and presumed nobody sees in the dark, I didn’t have any idea I couldn’t see because I had a condition causing night blindness!
My deafness came later.
I remember at about 28 years old I applied for a very good job at a nuclear plant working in the turbine hall . There was over 800 applicants and I was told the job was mine, however, after a medical I was told I had failed due to my hearing loss. You could’ve knocked me down with a feather!
So back to reality it was one of the many kicks in the teeth that people with Usher often deal with!
I progressed with life, had to work really hard even set up a couple of businesses along the way but at the age of 45 I reluctantly had to admit defeat. By now my eye sight so bad I couldn’t even find my way to the bus stop on a winters night!
I applied for a guide dog and got the result to my genetic testing which took a while but came back that I have Usher Syndrome Type 3, I was at this time told I needed a hearing check up! My hearing quickly went down hill from mild to moderate to severe now I’m tipping severe to profound.
To date there is nothing that can enhance my blindness accept to use assistive technology where I can, however options for the deaf or blind don’t always fit for deafblind people, all too often we have to muddle through either as a blind person person with deafness of a deaf person living blindness rather than seeing the bigger picture!
I’ve found in my experience I need to continuously adjust and try to stay ahead of the curve just to live my life. I’ve gone from cane to guide dog to specialised wrap around glasses to multiple hearing aid types.
So why is the very best available access to sound so important to somebody deafblind like myself?
Enablement to access sound, not just to hear but to hear so well you can rely on your hearing to keep you safe and inclusive.
Struggling to get by isn’t ok when there is tech to make life so much more doable.
I feel very privileged to be the first person chosen as part of an important and, excuse the pun ‘eye opening’ project being run by the Molly Watt Trust with the support of GN Hearing and in my case The Hearing Clinic, Glasgow.
The project is to demonstrate the additional challenges of people deafblind over and above deaf or blind and the absolute need for us to access the very best in hearing aid technology.
Molly Watt Trust collaborated with GN Hearing and Chris Stone at The Hearing Clinic to enable me to be fitted with Resound LinxQuattro smart hearing aids.
The day I was fitted with my hearing aids I learnt more about my hearing than I had ever learnt in my years of wearing hearing aids and to say I was blown away by my initial experiences of sound really is an understatement and here I an a year on and life has never sounded or been better even through this awful pandemic.
I have blogged about my experiences but been quiet as little happening with the many lockdowns that have been in place however what I have done is take the time to reflect on what smart hearing gives me.
I can now hear traffic when I’m out with my guide dog, I hear 360 degrees around me which when you consider I only have 3 degrees of central vision. To say my hearing compensates for my blindness, I think the numbers say it all!
I feel safe, my confidence and ability to get around hugely enhanced.
Throughout lockdown I’ve been out every day rain hail or shine on many walks, fully appreciating the noises of nature, the different birds singing. It’s also the simple things too like listening to songs or being able to hear a mobile phone conversation.
I am one of many people who really need the very best in smart hearing aid technology which is why they need to be made available to the deafblind community.
Unfortunately at this time Resound Linx Quattro are not available on the NHS which is madness when considering the positives they bring to my relatively small community
Yes they appear expensive initially however when you look further ahead - enablement to get out and about without fear of danger, when you’re able to work, access telephone calls, be inclusive, live your life and not feel the isolation often attached to Usher Syndrome and all the problems attached to it of which mental health is often a big part. When you consider these things are these hearing aids really so expensive? I think they would save huge amounts in financial support for many. Financial independence is beneficial to all.
Over the years I have spoken with numerous audiologists who see best available hearing aid tech as the best option for me as they will enhance my hearing for as long as is possible, and in doing so compensate a little for my blindness until the possibility that I may require cochlear implants, something I hope doesn’t become necessary, however should it become my only option the NHS would fund this at a cost exceeding £50k plus and yet won’t provide top quality hearing aids at a fraction of the cost - WHY?
Please help us on our quest if we don’t shout out we will not be heard!
If you want to help or make a donation so someone else could get the amazing benefit of these wonderful hearing aids which do change life’s take it from me as I know, then please contact www.molly-watt-trust.org .
Together we will change life’s for the better.
For now I’m thankful to the Molly Watt Trust, GN Hearing and The Hearing Clinic and hopeful Molly Watt Trust can continue to work with GN Hearing and help others like myself.
Well it’s the year of 2020 everyone!
It certainly has been a time of reflection for me, as a result I wanted to share my last decade in a blog that might provide hope for those living with progressive conditions such as mine. As hard as it can feel there is a future, it will be challenging but hard work and determination can see you through.
I have always hated New Years, I’m that emotional sobber in the corner as midnight strikes!
For me the concept of time is quite terrifying, to be frank I have always tried hard to not look too far forward nor back! However it’s this time of year where you’re forced to think about these things.
And then there’s the people talking about the past DECADE as we walk into the next.. well my mind has been having an inevitable real field day thinking about all those years...
10 years ago, I was 15, had been registered blind a year and it was not long before I was put on the waiting list for a Guidedog. I was at boarding school, where I was tormented for being different - I couldn’t sign British Sign Language, so therefore I was “a sad excuse of a deaf person,” I didn’t look blind so therefore I was “pretending,” I had both pupils and teachers grind me further into the ground daily. All I wanted was to be like everyone else, to have no worries besides my GCSEs - not the worry of not being able to actually see enough to read them let alone sit the exams and pass.
At 15 I was put on extremely strong medication to try and rectify a secondary eye condition (Cystoid Macular Oedema,) in the past decade I was on these twice for between 6-8 months. Both times I went from an average size 8/10 to a size 4/6. My mental distress was at the brink and I’d spend most nights crying in pain. The medication never worked. I even had injections and eye drops to try and save my failing central vision - none of which worked. All this while I was at a school who to be quite honest, didn’t want me there.
I had 5 degrees vision left. (Thankfully it’s been stable since.)
Fast forward a few years, I lost friends I gained friends I found the college who restored my faith in humanity... I found *Molly* again. I have Strode college in Egham Surrey to thank for that. I’ll always be eternally grateful for the teachers, the support and of course the life long friends I made there.
Leaving college I proved the little doubtful molly I had inside me that I WAS worthy of succeeding, I even got into university. Sadly university wasn’t to be even after my tireless battle with the authorities to gain the support I absolutely deserved.
At 20 I got a job at Apple where I worked as a specialist and these guys, I’ll always thank for seeing my true value. I found my niche - technology! The ever impactful tech that had gotten me that far and I hadn’t actively realised it until then.
My confidence built up and I built a field of contacts, I bought my first applewatch and wrote a blog that went viral and literally changed my life.
After the applewatch blog I made contact with GN Hearing who's smart hearing aid technology would go on to enhance my life. GN produced the first hearing aids completely compatible with applewatch and iphone meaning I had the a pretty enabling awesome accessibility toolkit. My confidence was high and as a reult I left apple to persue a self employed journey in accessibility, web and app design and usability.
My first dear Guidedog Unis who had come into my life when I was 16 years old was forced to retire early, saying goodbye was to date one of the hardest things I’ve had to do. Thankfully her forever home is with a good family friend and she is now loving a happy retirement.
My second guidedog the beuatiful little Bella aka Welly trotted into my life and has been instrumental in my life since. Thanks to that poppet, I can earn a living and do so safely and with genuine happiness having my little best friend by my side at all times.
I have been able to work with massive companies like Apple (HQ,) LinkedIn, NHS Digital, Spotify, ASOS - the list goes on.
I started working with the amazing guys at Sigma - they too, have been amazing in contributing to my growth in independence, knowledge and confidence.
I have travelled the world, travelled europe delivering keynote presentations and meeting amazing people. Making memories ?
And here I am, I am the person I’ve grown into today and well, I'm pretty proud of myself.
Though things as always remain challenging, the past couple of years have been steady upward progress for me.
In 2019 I proved to myself that I could get even *better,* and with my Molly Watt Talks journey have done incredibly well - if I may say so myself! Also as a person, I’ve grown so so much.
I won’t finish saying it’s all been perfect - it’s been awfully hard at times, I still battle with my mental health and spend some days in bed crying at the thought of leaving my house.
That’s life though, and never be afraid to sit a day out here and there - it’s not easy being human, learn to give yourself a break and to not feel bad. One thing I learned just in the past year.
My wish list for this next year/decade is to grow, to advocate for others, to travel, to work and meet more awsome people, buy my own home and to see others living with my Usher Syndrome thrive.
So before I sign off I want to thank YOU all for still being here - I see you! Not literally obviously, I am blind (heh) you’re so very appreciated.
May we all continue to grow, and learn. Here’s to the next 10 years...
Do you have a year plan, a ten year plan or a wish list? If you do please consider sharing, write us a blog we can share and lets make these things happen.
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
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.
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.
Dr Mariya Moosajee is a Consultant Ophthalmologist at Moorfields Eye Hospital and Great Ormond Street Hospital for Children in London. She specialises in genetic eye disease such as Usher syndrome. She leads an active research team at UCL Institute of Ophthalmology, London, where she is undertaking several projects related to Usher syndrome in the hope to develop a potential treatment that may be applicable in the future for patients and their families. Here is a summary of her projects:
1. Developing small molecule drugs that can treat a subset of Usher syndrome patients
Approximately 30% of genetic mutations in Usher syndrome are due to an abnormal stop signal being introduced in the gene (this is called a nonsense mutation). When your cells try to read your gene, if it encounters this premature stop signal it will stop making the required protein, and this loss of function leads to Usher syndrome. There are now a group of drugs that can bind to your protein-making machinery in your cells and override the abnormal stop signals leading to the formation of normal full length functioning protein. This can restore between 20-25% levels of normal protein and this may be enough to halt or slow the retinal degeneration seen in Usher syndrome. This work has been published in the scientific literature and we are now trying to translate this therapy to patients. To learn more, please visit: https://vimeo.com/199657263. We are trying to raise the funding to move into a clinical trial. Please watch this space, for updates on our progress. If you are keen to check what type of mutation you have causing your usher syndrome, please send Dr Moosajee an email (contact details below) with your genetic report so she can advise you on whether this treatment would be suitable for you.
2. Developing a non-viral gene therapy for Usher syndrome type 2 caused by USH2A mutations
Small molecule drugs only works on a subset of patients with a particular mutation. The gene USH2A is the commonest cause of Usher syndrome type 2 and contributes to the majority of Usher syndrome cases overall. Mutations in this gene can also cause retinitis pigmentosa without any hearing problems. The gene is very large and extends over 19,000 kilobases (or letters of the genetic code). Unfortunately, conventional viral gene therapy vectors can only accommodate genes that are smaller than 9000 kilobases. So we need to find an alternative form of gene delivery for USH2A. My team is developing a non-viral gene delivery system which can hold large genes, including USH2A, and testing its safety and effectivity in Usher disease models. This includes (i) a zebrafish model with an ush2a genetic change generated through gene editing, and (ii) human retinal models from patients own skin samples, which are reprogrammed to stem cells and then differentiated into early eye cups growing in a dish. To learn more about this stem cell technology watch this animation: https://vimeo.com/227432768. This project is still in the preclinical phase and will last 3 years. We will keep you updated on the progress.
3. Natural history clinical studies to understand disease progression and identify reliable outcome measures for future clinical trials
At Moorfields Eye Hospital we have a state-of-the-art Clinical Research Facility with high-tech imaging equipment that enables us to study the detailed clinical features of Usher syndrome and monitor disease progression. My team are making advances in our understanding of the relationship between the genetic diagnosis and the clinical features, providing insights into disease mechanisms and potential therapeutic targets. We are also trying to establish the clinical tests which can measure a true change in the condition over a fixed period of time, so when we move to clinical trials, we will know what measures portray an accurate response to treatment.
Mariya works closely with Prof Andrew Webster and Dr Adam Dubis at Moorfields Eye Hospital and Dr Richard Harbottle at the German Cancer Research Center, Heidelberg. Here is a joint summary of ongoing research that may be helpful for Usher patients: https://vimeo.com/238404158.
For more information, please visit www.mariyamoosajee.com, follow her on twitter @MariyaMoosajee or on her facebook page DrMariyaMoosajeeLab (https://www.facebook.com/DrMariyaMoosajeeLab/).
On Saturday 16 September 2017, Usher Syndrome Awareness Day, I was very humbled to join in on an accessibility workshop hosted by Molly Watt from Molly Watt Trust and Chris Bush from SIGMA, held at the Thames Riviera Hotel in Maidenhead Berkshire. It really was a ‘blind date’ for me as I had only recently met Molly and prior to meeting her I had no idea what Usher Syndrome was. Usher Syndrome is very much an invisible condition, I was pleasantly surprised by a room filled with over 30 people that had travelled from as far as Scotland and Leeds all making their introductions and it really wasn’t until I saw the flurry of furry hounds with their High Vis harnesses on that I would have noticed anything different about this group of guests at the hotel.
On meeting Molly for the first time in Starbucks, on the surface, there wasn’t anything about her that alerted me to a condition of almost total blindness and deafness, until I spotted ‘Bella - the wonder dog’ sitting on the floor beside her wearing the High Vis paraphernalia. Usher Syndrome is a condition which affects both hearing and vision with the main symptoms being hearing loss and an eye disorder called retinitis pigmentosa or RP and as a vibrant and energetic mainstream millennial, Molly has not allowed her condition to stifle her modern day way of life. Although she is able to communicate using sign language Molly prefers to converse orally, (and boy can she talk for England) as well as maintain an active and strong presence across social media and on top of all that a whizz when it comes to state of the art digital technology - she is even able to maintain her own Digital Hearing Aids from an App on her iPhone reducing those nightmarish visits to the audiologist. A truly model citizen for the connected home industry.
Being an active millennial, Molly has also ensured that (aside from fashion) she is up to date with modern technology and has even managed to find her way into some high profile manufacturers who are developing state of the art technology including some for the Deafblind community. I guess it’s not without doubt that together with her determination Molly has been fortunate to have been selected as a model ‘guinea pig’ for some of this really cool and expensive kit, including #Applewatch, #iPhone, #ReSound Digital Hearing Aids #LiNX3D, #Ring Door Bell, #Philips Hue Lighting etc.
Molly had wanted to be a primary school, but was let down by the system, her university failing to provide access to her course, she therefore made the decision to move into the field of accessibility and enablement.
Molly is comfortable standing in the front of a room and captivating her audience, not to mention she has a great sense of humour too. This confidence and after writing a blog about how the applewatch transformed her life that went viral, also afforded her the opportunity of being invited to Apple HQ in Cupertino twice to provide her insight into the world of the deafblind and the needs of those living with Usher Syndrome along with how she utilises her #AppleWatch to communicate and navigate safely through society on a daily basis as well as in her home & family life - mum keeps a close ‘watch’ over her through technology.
Over the last decade, technology, smartphones, tablets and apps in particular have in many ways changed the way people live their lives on a daily basis and even made the world a smaller place. For many though, the arrival of this emerging technology can be very daunting, that is until you understand the benefits of how to extract the best use out of it to your advantage, regardless of abilities, everyone uses their devices in a different and personal manner. Who better to demonstrate some key advantages of these products, highlighting the important things as well as some useful tips too, than Molly herself. Impressed at how these products have impacted her way of living and with her tenacious entrepreneurial spirit, she took the initiative of organising a hands on accessibility and usability workshop in order to share her experiences with other members of the Usher community so that they too can enjoy the benefits of exploring these new arrivals.
Commanding the attention of an audience of around 30 (and their canine partners) is no mean feat for any speaker, however Molly and Chris managed this with ease and the audience soon became engrossed with the content and couldn’t wait to get hands on with exploring their own devices.
Not having previously had the need to explore the accessibility features on my mobile phone, I was suitably impressed by some of the tips that Molly and Chris shared with her group (Screen Reader vs Speak Screen, voiceover, inverting colours, zoom/zoom region, swiping with two or three fingers, double and triple tapping the home button -who knew all these options were available etc) that even I myself, a sighted and hearing individual have adapted to using some of these techniques to make my access that much more comfortable for myself including the ‘show controller’, zoom window and nightmode on twitter now that I have reached the age where I have to rely on reading glasses to see anything on my screens.
About me:- Passionate about the Digital Landscape and emerging assistive technology that is taking the connected smart home and city by storm, it is through my profession in proximity mobile marketing and my basic understanding of the Deaf community that I have identified a unique and niche new form of digital communication using a combination of this emerging technology and a mobile application as part of a #SmartCity Infrastructure that will transform the lives of millions of individuals offering them a more independent and inclusive way of living. I have started a project to develop this revolutionary new digital communication channel dedicated (but not exclusive) to the Deaf, Hard of Hearing and Sight Loss community and it was during my research into this project that Molly and I connected. In my experience of proximity mobile marketing where creative agencies are continuously looking at emerging tech and innovative ways in which to engage brands with their audiences in the physical world there is a lot that they can learn from people who have restrictions in life with how to overcome challenges.
I would like to leave you with this quote - Employees with disabilities drive innovation. Disability creates a constraint, and embracing constraints spurs inventive solutions - Haben Girma and please do consider that Molly-Watt-Trust is an independent Charity - Molly Watt Trust, registered UK charity 1154853 and would benefit greatly via any donations, please be generous.
I would like to take this opportunity to thank Molly, Chris, Jane and Andy for being such wonderful hosts, great cakes by the way, Thames Riviera Hotel for being so accommodating with the group and their guide dogs and most importantly for all who supported Molly by attending the workshop. There were some wonderful raffle prize giveaways including the Ring Doorbell and Philips Hue starter kit. Let’s hope that there will be loads more to come and I would highly recommend the services of Molly-Jane Watt as a keynote speaker or gadget guru for accessibility, however please do bear in mind she is an independent Charity and cannot survive on freebies.
applewatch, #Apple, #Ring, #ReSound,
Author - Frank Viljoen, Director of MOOHBE a proximity mobile marketing specialist consultant working on a revolutionary digital communication channel for but not exclusive to the Deaf, Hearing and Sight loss community.
We are celebrating 3rd Usher Syndrome Day with two day’s of fun and learning
Friday 15 & 16 September 2017
Thames Riviera Hotel
The Friday evening will begin at 7pm with an Amy Winehouse Evening full details https://thamesriviera.com/events/adele-returns-2-2-2/
There will also be a raffle.
For those interested in attending both events and who would like to stay at this beautiful hotel set on the River Thames we have been provided preferential rates, book early to aviod disappointment. To take advantage of these rates please contact the hotel direct 01628 674057 and mention the Molly Watt Trust.
Saturday morning will be free and starting at 10.30am.
We are offering a relaxed get together, a free assistive tech workshop, learn how to get the very best from your handheld devices, iOS or android - session run by our own Molly Watt and Sigma’s Head of Experience Design Chris Bush.
This is an amazing opportunity.
Chris and Molly run this workshop to and with many professionals in design and technology around the country and will be running it in San Francisco later this year.
All with Usher Syndrome /RP or parents/carers of those with these conditions would benefit hugely from this workshop.
Coffee, tea and cakes will be available.
There will be two speakers (to confirm).
The whole session should wrap up around 1-1.30pm after which you can enjoy the beautiful area, Boulters Lock, Cliveden or nearby Windsor with all its history and attractions.
The nearest train station is Maidenhead, it is a 15/20 minute walk from the station. There is a taxi rank at the station or please contact us for alternate taxi numbers.
We met a lovely lady from Northern Ireland at a Molly Watt Trust ‘In it Together’ event in Edinburgh in October 2015, she has been a hearing aid user since she was 4 years old and was managing very well.
At that particular event we were very fortunate to have a presentation from GN ReSound’s Graham Roberts who was able to share with us the many impressive hearing aids and products available from the supplier.
That presentation together with Molly’s in depth and ongoing testimony of her GN ReSound LiNX² and how they have enhanced and enabled her life led to an influx of enquiries about how and why these enabling smart aids are not available to those living with Usher Syndrome (deafblindness), particularly as they offer far more than most hearing aids provided by the NHS.
It is fair to say everybody at that event, particularly those using hearing aids where very interested in the enablement smart hearing aids can provide.
Molly coped with standard Phonak hearing aids provided by the NHS until she was 20 years old, however, from 14 years old when she became registered blind, deafblind she struggled and the reason for this in the main was because of her blindness.
For 6 years Molly insisted her hearing had deteriorated even though test after test showed her hearing, thankfully remained stable.
In actual fact what had happened was her ability to lipread, to use facial expression and body language had gone along with her sight, her ability to access sound and communicate had dwindled, demonstrating just how much the deaf rely on their eyes to hear. In other words she could no longer ‘fill in the gaps’ that her eyes had enabled.
It is a fact that the deaf hear with their eyes.
GN ReSound LiNX² provide a hearing experience those who have experienced really benefit from. They provide an incredible clarity of hearing, they enable directional sound meaning that whilst Molly is blind she can now turn towards a sound, something she could not do with her NHS provided Phonak hearing aids, she can also identify not just where sounds are coming from but most importantly she knows the sounds of danger - just imagine how important that is to safety for the deafblind.
These hearing aids have incredible bluetooth connectivity to iPhone and applewatch, also android devices giving the ability to many deafblind to access aurally things they could only dream of previously. Using a telephone as a telephone for the first time ever is most certainly a huge positive.
Not only can they be adjusted be independently adjusted but enable a very personal hearing experience and in so doing bring great confidence and independence.
Our friend from North Ireland was one of many desperate to have access to GN ReSound LiNX² and indeed has asked her NHS audiology department, their response was somewhat bizarre - an appointment with the Cochlear Implant Clinic!
Cochlear implant is amazing technology for the deaf, however, not only is it not for everybody it is also incredibly intrusive, expensive and most importantly not what the patient wants why not consider more enabling hearing aids which happen to be a fraction of the cost.
Figures suggest the cost of 1 cochlear implant would be close to the cost of 10 pairs of best quality smart hearing aids!
There are lots of people who have been hearing aid wearers a long time and like Molly have gotten on very well with them but now need more as Usher Syndrome/ blindness looms close.
GN ReSound have a variety of hearing aids including the ENZO2 for those with a profound hearing loss and whilst they are the creme de la creme of smart hearing aid technology their cost in comparison to cochlear implant is very reasonable.
Cochlear implant involves surgery, it is considered that deafblind people should have two implants to enable directional sound for safety and rightly so, directional hearing is a must for the deafblind, safety is imperative for all, whatever hearing aids worn.
Most get one implant at a time so often two surgeries after each substantial aftercare and rehab at huge cost.
I’m not saying this shouldn't be the case, of course it should where appropriate, however not every deafblind person wants such invasive surgery preferring an alternate option.
Patients should always have a say in their care as it tends to be them who are the real professionals and who will have done the research based on their condition.
I know there is a long and in depth assessment for cochlear implant and so there should be but surely every patient’s individual requirements should be considered?
My friend in Northern Ireland was told there is a 3 year waiting list is this acceptable? Why not consider best hearing aid options?
We continuously hear that our NHS remains financially fragile, that it needs more funding but very rarely do we hear about solutions, about genuine savings that do not include reducing staff - surely this scenario has to be seriously considered
I would like to say our friend in Northern Ireland’s scenario is unique but sadly it is not!
I recently visited the ATOS London Offices, for an appointment set up by my Mum, Jane.
We were there to meet with Dr McKillop to discuss varying issues and negative experiences I and many others have endured at PIP assessments and as a result of inappropriate awards for many of us living with Usher Syndrome or Retinitis Pigmentosa (RP).
The meeting was set for 2:30pm purposely so I could travel to London outside of rush hour. Mobility for the deafblind/blind is stressful anytime of the day but particularly in busy periods.
I had been involved with with the original pilot assessment for PIP, as a result I was not overly concerned about my assessment, unlike many who not only find filling out the application form stressful and inappropriate for people with sensory impairment and need assistance completing it.
I am lucky my Mum has always taken care of forms for me and as capable as I am I dread the day I have to do form filling alone.
I shouldn't feel that way, with real understanding, using the assistive technology available and good lines of communication there has to be a far more appropriate way of doing things that do not cause so much distress.
Finding a better way is something I would like to work on with whoever would like to work with me.
Planning the journey into London was much easier with my Mum as she was able to drive us both to a tube station. The tube is much easier to use than the mainline train, however, it isn't easy.
On this occasion, Mum decided to drive and park at Hounslow West tube station, so we could take the Piccadilly line, followed by the Victoria line to Warren Street station and then a short walk to Triton Square. This was the most direct route with minimal changes underground.
The reason Mum drove into Hounslow was as a result of thinking ahead to avoid crowds, anxiety and darkness. Though the meeting started at 2:30pm travelling into London at any time is stressful for a DeafBlind person.
My guidedog is partially retired and whilst Guidedogs for the Blind have told me I could wait up to 18 months for a new guidedog life cannot go on hold for this amount of time so I have to make the most of the resources I have, in other words to ask for help when I need to and to get from a to b using my cane. I am not as comfortable or confident with my cane outside of my comfort zone which would just be my local area.
Whilst I am in this situation I have to arrange my work and transport around assistance from others as my work takes me all over. I’m very lucky to have a very supportive family and network of friends happy to help when they can but for me asking for help isn't ideal but something I have to deal with.
On this occasion I had to use my red and white cane (red and white striped cane indicates user deafblind) and thankfully had the assistance of my Mum to make the day less stressful.
Forward planning is always a must so with Mum it meant a half hour/forty minute drive to Hounslow West tube station to take roughly an hour tube ride to central London for the meeting at 2.30pm.
Then allow an hour, possibly two for the meeting, hope to miss rush hour on the tube back to Hounslow, knowing that on reaching Hounslow West and the car it would be getting dark this time of year. At least this way I could sit in the car with my eyes closed (rested) after a challenging journey.
I should point out that Retinitis Pigmenosa (RP) which is the blindness part of Usher Syndrome is tunnel vision and often results in complete blindness in the dark, bearing in mind through winter months the days are long and often dark so very challenging. winter also brings the brightest sunshine which also causes unbearable glare on very delicate eyes so also blinding.
Though the journey was carefully arranged in a cautious attempt to reduce as much anxiety and stress to myself as is possible this doesn’t ever totally eliminate stress. I would hang onto Mum and whilst I am trusting the cane to detect dangers, obstacles and depth perception I still naturally scan around me in an attempt to use any visual clue that helps me navigate safely.
Scanning is exhausting with 5 degrees in one eye, plus the ongoing light changes the underground brings additional stress to the eyes because of the darkness (night blindness) and sensitivity to light and changing light conditions.
At best it is a horrendous headache and worse a total nightmare.
I know you could think stop scanning right? Or maybe even close your eyes and rely on the cane and allow Mum to guide me!
Much easier said than done, especially with progressive conditions like retinitis pigmentsosa as though registered blind I was born fully sighted so closing my eyes does not come easy, scanning is very much a part of the coping strategy even though it is horrendously exhausting.
I explained to Dr McKillop that what I get from scanning is useful as in, when I look at a face I can only see a small portion of it, perhaps an eye and nose or a mouth, for me being deafblind I would scan the face to draw up a picture of the face as a whole in my mind and then concentrate on looking at the mouth for lipreading which is another part of coping strategy but as I’m sure you can imagine truly exhausting. With my new hearing aids I now benefit from being able to locate sounds, I often look to where I hear the sounds to better hear this could be anything, a person, a car, train, siren, lots of environmental sounds that help keep me safe.
My head will naturally turn to where I hear sound and without thinking my eyes are open and scanning to detect the sounds - all strategies developed to cope in a world of sight and sound.
Ticket machines at tube stations are not easily accessible and tube travel not free for people not living in London. There was nobody in the tube station to help me purchase tickets so again thankfully my Mum was able to do this for me.
Once on the tube, and seated I then use my iPhone to zoom in on the underground maps to check how many stops there are before changing tube (I looked prior on ‘Tube Tamer,’ app) I find myself closing my eyes to rest, RP eyes are incredibly sensitive and closing them when I can is helpful. It is a lot less stressful sitting next to somebody I know, I rest my arm so it is touching my Mum’s, I feel safer then and can relax a little.
Once I know exactly where we are going, who I am with, how many stops I feel a little reassured.
Reassured enough to not feel or watch the motion of the tube going from tunnel to outdoors to tunnel, light to dark, dark to light, as this is very disorientating. I avoid this as much as possible as my eyes struggle to adjust in inconsistent lighting, lighting conditions.
I discovered a few years back I could not look out the window of a moving car door without feeling a little motion sick, my eyes will often feel they are bouncing side to side, and having my eyeballs feel they move around so quickly without moving my head hurts.
I can no longer just peer out a window and watch cars drive past, just like I can't sit in a moving train or tube and watch the outdoors in a moving vehicle. As time has gone on I have realised that to avoid feeling unwell or any onset of vertigo before I have stood up, I will often just close my eyes. Having my new hearing aids go some way to reassure me of my surroundings, listening for the tannoy for any crucial messages, but of course on this day I had Mum.
Having Mum relieves a lot of stress.
Between underground lines we had to travel up escalators, if I had my guidedog it would have been the need for stairs or lifts as she was not escalator trained and in the past this has been a source of worry for me as although I do not live in London I do travel to London for work fairly regularly and once again I would have to find out which tube stations are accessible for Unis and I!
The majority of the journey was on the Piccadilly line then a series of tunnels, escalators and lifts to the Victoria line, onto another tube, this time only for 2 stops.
Thankfully Mum guided me to where the lifts were to avoid more crowds. My cane tactfully establishes where steps are, and at the top and bottom of steps there are bobbly grids that I could also feel through the cane to indicate the start and end of a set of steps.
Signage underground is everywhere, and up close I can see however without walking close enough to see or using my iPhone to magnify I couldn’t access this material.
Thankfully on this day I had my cane and Mum.
Navigating up more escalators, where I would hold on to the railings to keep me upright without feeling dizzy with all the light changes going from underground to ‘overground.’ Before you know it we have to find our tickets and tackle crowds to get through the barriers. There are often underground staff lurking by these gates to assist, on this occasion Mum walked us to a wider barrier /seen as an accessible barrier where a member of staff stood, however he shook his head and pointed to the other side where the other accessible gate was placed, I only knew he had pointed because my Mum told me, I couldn’t see his hands, his whole face just the smallest part of him! Apparently we had gone to the ‘wrong side,’ how is somebody blind supposed to decipher this?
Again, thanks to Mum she guided me to the ‘correct,’ gate and pulled me through the crowds.
Being outside in the bitter cold was refreshing after being underground for 50 minutes however it was a new challenge.
The hustle and bustle on the pavements and to navigate to the London offices of ATOS where our meeting was taking place.
On this particular day it was cloudy but bright.
I had delicate eyes from a headache the previous day the bright clouds didn’t help. The clouds reflect glare and my sensitive eyes do not cope well, even with protective eyewear it is challenging because, of course I am blind in the dark! Catch 22!
I feel the meeting went very well and was very useful to us all - we all always have something to learn from others.
I was particularly impressed that Dr McKillop considered my travel back on public transport and that he suggested we left before the real build up and madness of rush hour in London.
I was able to share with Dr McKillop a great deal about the challenges both the Usher Syndrome and RP community meet with on being assessed often by people who are not fully aware of the very real daily challenges we face, often because of how we present ourselves and how ‘capable’ we can appear and of course many of us are very capable but not without support and I mean the right support, coping strategies and understanding things the current PIP application form does not take into consideration.
I want to help, to educate and raise awareness of these hidden conditions to those carrying out PIP assessments and also enable people like myself to not fear these assessments which to date are to many very daunting, frightening in some cases - just the thought of an appointment often causes untold anxiety and distress and I understand why when so many are not awarded correctly and then have the added trauma of an appeal, some are even too afraid to apply and struggle on without financial assistance.
I am informed that in the case of Usher Syndrome alone 68% of people who go to appeal win their appeals which is a very high number, of the other 32% most cannot face an appeal and therefore miss out which is very concerning.
After the meeting wrapped up we headed back out into the cold. My eyes had to adjust from the meeting room, the reception and to the outdoors all lighting was totally inconsistent - these things are all very relevant to people like myself and in understanding RP these things must be taken into account.
It took a while for me to adjust and although we left before rush hour the sun was going down, winter sun is far brighter than summer sun and can actually be very painful on ‘RP eyes’ - more light adjustments. Sunglasses can only do so much and of course the darker they are the more blind I become!
At this point I am really pleased to be hanging onto Mum, she will be my guide on the way home - not only am I out of my comfort zone, I am anxious and in an environment I am not used to. This is also very relevant as I live in a small town, nowhere near as busy as a big city.
It was busier, creeping towards rush hour!
Not surprisingly the men and women sat in ‘priority,’ seats weren’t keen on giving up their seats. My vertigo was already playing up from all the light changes and now being underground in a moving tube, all I could do was hang on, lean against my Mum and close my eyes.
Thankfully although it was busy we did arrive back in Hounslow in good time.
Walking out of Hounslow West tube station at around 5:30pm it was dark. I am fully blind in the dark. Mum guided me to the car and helped me in. I sat in the front and folded my cane away, ‘until the next time’.
I felt both relieved and relaxed to be in the car with my Mum, also a huge sense of achievement at what we had done.
However once we hit the motorway, listening to some Motown, my eyes began to throb. The car lights and street lights, glare all around me me.
Looking straight ahead all I could see was blur and bright red lights everywhere and even in the more lit areas I’d glance towards my Mum who just looked a blur so distorted is my sight in these conditions, it really is very disorientating and then motion sickness kicks in.
Once again it was time to close my eyes and just let my ears do the listening, to the music and to Mum’s voice, Mum’s reassuring voice I know so well.
What would I do without Mum?
Truth be told, I have to do it without Mum sometimes, and I can - and do, however the stress levels are ten times worse.
As I demonstrated at the Offices of ATOS I manage because of the various tools and assistive technology I have access to, none of which are cheap to buy.
My Resound LiNX² hearing aids connect me to the Apple ecosystem - iPhone, applewatch, iPad and MacBook enable me to have a life and need to be replaced as and when necessary.
These items of equipment are in no way ‘flashy items’ but essential equipment to give me access to the things most can take for granted.
Mobility is the one thing these items cannot directly assist with however they do help with navigation and the enablement to use certain apps that enable when I am out alone with my cane or preferably my guidedog (hopefully my new match will not be long) we can get from a to b as safely as is possible.
This vlog was made to demonstrate the NEED for enabling assistive technology for those who need it:
So PIP is Personal Independence Payment, I’m sure you will see my ‘independence’ comes at a substantial cost and whilst I have a very serious and progressive condition I wish to live my life as positively as is possible and with appropriate support from the state, nothing more and nothing less.
We all deserve that consideration.
This vlog was put together in part on my way into London that day and thereafter using my ipadpro to demonstrate and simulate my trip.
I would say the visual field here is greater than mine but gives an idea of the challenges people with Usher Syndrome and RP deal with: