An interview with Molly Watt: Putting Usher Syndrome on the map

For this post, I wanted to share Molly Watt’s story. I first came across Molly in 2015, after I read her Apple Watch post. I had also just received my Apple Watch and was curious about other people’s experiences. What’s different about Molly, is that she has Usher syndrome, which is a rare genetic disorder caused by a mutation in any one of at least 11 genes resulting in a combination of hearing loss and visual impairment, and is a leading cause of deafblindness. Usher syndrome is the most common cause of congenital deafblindness (the elderly is the biggest group). Usher syndrome is incurable at present. Usher syndrome hasn’t held Molly back, she’s even set up her own charity, the Molly Watt Trust and much more. When reading each of her subsequent blog posts. her writing was creative, courageous and candid, and that resonated with me. In fact, it resonated so strongly with me, I decided to visit Molly in her home town of Maidenhead, England to interview her. It’s a longer interview than what I would normally post, but we have so much to learn from Molly (and others like her), that I was compelled to include as much as possible from her answers. Listening to Molly was also a powerful reminder, that we often focus so much on ‘empowering’ or ‘activating’ or ‘engaging’ patients themselves, that we ignore the patient’s family and friends who play a very critical role. I feel there are so many voices currently not heard, do we need to change the way we listen?

The image below is a 360 image from my interview with Molly. 

Post from RICOH THETA. - Spherical Image - RICOH THETA

1. You were recently had a meeting at Apple's HQ in America to share your views on accessibility. Can you tell us more about how you ended up there?
The main thing was the Apple watch blog post that I wrote, and through the charity, I discuss how we can access things through tech. I have been an Apple user since my diagnosis 10 years ago, I had a Mac eventually in education to access exam papers. So when the Apple watch came out, I was unsure what it could offer, and I bought one out of curiosity, and thought I would probably return it. Accessibility has been at the cornerstone of my life since the diagnosis, and we got my website set up after Xmas 2014, and my mum encouraged me to blog, so I did.

This was the first personal blog post wrote. I think the timing of it was shortly after the launch of the Apple watch, there was a lot of bad press saying it was a toy, but I found from the perspective of sensory impairment, it opened a lot of doors for me to be more independent, I never missed a phone call because of the prominent haptics, the digital touch features were really beneficial socially when out with friends, maps was a big feature for me, navigating from A to B with the watch. When I’m out I’d rather have my phone in my bag, as it’s much safer for me, and the watch enables me to do that. My post generated quite a lot of positive reviews about the Apple watch. All of that is how after a few months, Philip W. Schiller, the senior vice president of worldwide marketing at Apple retweeted me and my website crashed due to so many hits. From there onwards, a lot of people contacted the trust. We have to remember that a lot of people can’t afford the watch, many with Usher syndrome are shuffling between jobs.

Apple had reached out to the trust to speak with me, and since we were going on a family holiday to California, they said, well come and visit us. They were genuinely interested in hearing my story, and to understand how technology can enable accessibility much more in the future. As a family, we travel as much as possible, because my sight may completely go at any time. I wrote a post about my trip to California.

2. Many people laugh at products such as the Apple watch calling it a toy or not seeing any value in using it, but it has been of value in your life. What can be done to get people looking at all possible uses of new technology?
I wasn't sure about the Apple watch, I couldn't really understand what it would do for me over the iPhone that I have relied on for years. My decision to purchase it was last minute really as a couple of my friends were getting one. It's great my friends did as I might not have got one myself as we were able to explore the features of the watch together. However, for me after a little fiddling around with my insight into my real need for accessibility I was able to really put it to the test. I believe people give the Apple watch a bit of a hard time because they don't use it in the way somebody like myself does. I have learnt how to use it to make a real difference to my life, and it’s a brilliant piece of equipment I have come to rely on. I guess because I rely on technology I have become an expert in my own way of accessing it!

3. You've got your own charity and you've spoken at places such as the Houses of Parliament and Harvard Medical School. When you were younger, did you envisage you would reach these heights?
I had no idea. I think my own struggles have made me feel passionate about making a difference, to raise awareness of ability as much as disability, share my experiences good and bad and demonstrate the importance of accessible, assistive technology. I am definitely not the person I was since being diagnosed with Usher syndrome.  

Being deaf is very different, it is not rare.  It is however challenging and there needs to be support and assistive technology.  In the area I live, support of the deaf was excellent. Usher Syndrome diagnosis brought confusion and inexperience of supporting somebody with the condition particularly in school - my education became a nightmare as I couldn't access the curriculum without modification and nobody knew what they were doing.

At my real time of need the only people I could rely on were my parents who continued to battle for me even though they also did not really understand what I was going through. I definitely get my determination and drive from them.

I've been speaking since I was 14 and making awareness videos.  It was my way of telling people what I was going through, how I felt and what I felt I needed by way of support. That's how it all began and as the years have gone by I have found my work public speaking a very useful skill and a way of reaching the larger audience with the many messages I have.

4. When it comes to accessibility and new technology, what's missing? What are the 3 top inventions that you'd like to see come in the next few years?
This is a hard question as I'm not an expert on what is possible. I believe people in design, design of everything can be improved by the inclusion of accessibility from day one. The obvious things like all websites need to be completely accessible. I rely on this sort of thing, picking up a book isn't an option. Things like hotels often terrible design, decor, carpets & wallpapers clashing and the poorest lighting. Most public places are difficult.

I cannot wait for the driverless car to be available to people like me, I'm sad I'll never experience driving but excited to think this technology is on the horizon.

5. For those living with Usher Syndrome, do they feel like their wants & needs are being heard. If not, what could we do to be better listeners?
Definitely not, there is a lack of understanding and awareness.  Usher syndrome is the most common cause of congenital deafblindness and few are experienced in dealing with it hence few get what they need. Life is a constant battle.

I'm sad that people with Usher Syndrome struggle to be understood and often live isolated lives.
Many do not work, do not socialise, and do not have access to enabling technology to allow them access to social media and if they did, they need help in learning to how to use the technology. Some use sign language which again can be isolating and can cause difficulty getting employment as communication support is often needed and hard to access as cuts to Access to Work continue. I think professionals should encourage people like myself to be vocal about their needs and to listen and take onboard their thoughts and feelings. All too often people tried to speak for me and it is not acceptable. Encouragement from the point of diagnosis is important.  

I'm fortunate my parents have always encouraged me to speak up.

6. I understand you've faced many challenges when dealing with the NHS, schools and charities/support groups, can you tell us a bit more about what happened? 
I'll answer this one at a time:

The NHS were good with my deafness diagnosis when I was little and up to my Usher diagnosis, thereafter it has been a different story. Sadly, audiologists who are often the first point of contact either know of the condition but have not treated anybody with it or worse, know nothing about it. Either way it is not helpful to the patient and needs to change. It is the same with ophthalmologists, who know about eye conditions but not much about deafblindness.  Whilst conditions are rare, there has to be professionalism in dealing with all conditions. An example of not having a decent understanding is my NHS audiologist who has known me since I was very young and has monitored my hearing with regular tests the results of which are followed up in writing. It would be great if I could read those results, which were completely inaccessible until I pointed it out that they were completely unaware of my accessibility issues. Not a thought about how I am able to access information in font 10/12 on white paper and black text!

Equally I have sat at Moorfields Eye Hospital and during the appointment, was spoken to whilst a Professor looked at his computer screen - everybody knows deaf people need to see faces to lipread and for facial expressions, even those of us with very little sight. These things should be obvious!

My experience of a mainstream school was excellent whilst I was deaf, there was great support.
Again after my Usher Syndrome diagnosis there was a lot of confusion, I was given the support of a VI teacher as well as my teacher of the deaf, and neither had supported somebody like myself.
A multi sensory teacher had to be "bought" in from a charity and yes she understood the condition but with one visit a term to educate those supporting me and myself things did not go the way they should have. This resulted in me struggling to deal with what was happening to me, I felt a burden and looked to move schools, my biggest mistake ever.

I thought going to a private school for the deaf, who were familiar with my condition I'd be with people like myself! I couldn't have been more wrong. The deaf kids were cruel, questioned my deafness as I have good speech, questioned my blindness as I appeared to see. The staff were just as bad. I boarded initially and spent hours in my dorm as I physically couldn't get from dorm to dining hall in the dark, nobody noticed or cared. Teachers didn't modify my reading material and if they did it would be on A3 paper making me feel very different. I struggled for 2 years trying to deal with my failing sight, being in denial as it often seemed easier to be that way surrounded by deaf kids telling me I was fine - it was hell.

It was made worse when I got my guidedog who did enable me to get from A to B safely then I was denied access to all social areas as my need to get from A to B was not as important as the need for a younger boy with a dog allergy to move freely around the school. 

I left with depression and a nervous breakdown at 17 years old.

That school knew all there was about Usher syndrome - they knew little, I was treated very badly.

Sense is the main deafblind charity, they cover/support all types of deafblindness, including deafblind with additional issues from the very young to the very old and everything in between and they are great at campaigning however I do feel people with Usher Syndrome often miss out and that’s why we set up the Molly Watt Trust.

My family travel to the USA to find out information about Usher Syndrome, there has not in the 10 years I have been diagnosed an Usher specific conference yet several for other types of deafblindness even though Usher Syndrome is the most common cause of congenital deafblindness.
Sense does a great job but there is little for those with Usher syndrome. Being an ambassador I'm always happy to help/work alongside them on any Usher projects. I am an Ambassador for Sense and happy to do what I can when I can to promote awareness of Usher syndrome, something I do as part of the work I do with the companies I have worked with. I have spoken for several charities including RP Fighting Blindness and also Berkshire Vision. I often feel on the outside looking in, I don't fit in the deaf community or the blind community and yet I feel I'm a part of both along with the Usher community and society in general.

Belonging somewhere is important to us all.

7. You wanted genetic testing, but encountered resistance from the system. Why did they think it was a bad idea for you to have genetic testing?
I wanted genetic testing when I was 15 years old, back in 2009. I had studied genetics a little at school and I wanted to know exactly who I am. My parents asked at Moorfields Eye Hospital in London the next time we were there and we were told ‘NO’ because of funding and because there is no cure for my condition. I remember feeling very upset and my parents following up the request for genetic testing with my GP.  Thankfully he understood the need and arranged for me to see a geneticist from John Radcliffe hospital in Oxford. My geneticist was brilliant (Edward Blair), he explained things in full and even provided a history lesson on where Usher syndrome came from. Some 6 months later I was told I have Usher syndrome type 2a. The importance of knowing is essential should the chance to trial anything become an option in the future. If there is any clinical testing of that gene in the future I can decide if I'd like to be involved. Being told ‘NO’ makes you feel you are a lost cause which just escalates the isolation this condition brings. Everything is a battle with this condition.

Something else to be considered is the benefits system. I have been assessed more times than I can say. Sadly people think deafblind, no hearing, no sight and no speech.  When they see me they are often very shocked and then don't believe I have any disability. On one occasion I arrived for an assessment (ATOS) and was told by the doctor he had googled Usher syndrome the night before!  He did not have a clue what I deal with on a daily basis.

8. When it comes to innovation in technology, and in particular around accessibility, what is your long term dream? 
I'd like people with disabilities to be considered from day one.  I'd like those with rare disabilities like mine to have access to all equipment they need and to be taught how to use it. I’d like them to have access to transport and benefits to enable them to work.

I think developers of everything need to understand the unique needs of all. For them to realise disabilities are not black and white. Sensory impairments are not two colours. Some with Usher are profoundly deaf (usually type 1's), the older generation might not have used any hearing aids so rely on sign language (BSL) and later tactile signing as their vision deteriorates - their communication skills and needs differ to the younger generation who have (parents chose) cochlear implants hence access to sound young and oral. My generation in the main, wear hearing aids and are oral. This is in my opinion a huge positive to accessing our world. However those who sign must always be considered regarding accessibility. And being blind is extremely rarely total darkness. There are many grey areas that are not often considered. 

In an ideal world I'd like to work/consult with developers around the world working on accessibility for all.  I'd like to be a part of moving forward with assistive technology. I believe if technology works for people like myself it will work for the older generation who's eyes and ears start to fail them as they grow older and this is very important with our ageing population.

9. Do you think there are other people like yourself around the world? Have you built your own network or is that something still to come?
I know of a few people doing similar to what I do. I have built a network which continues to grow, I am quite well known for my work around the world something I have been doing since I was 15. There is definitely more work to do and lots more to come. I hope that one day having Usher syndrome can just open up unique doors for every individual, rather than the progressive isolation and depression lack of access and awareness can give.

10. Who has inspired you the most in your life, and why?
My parents, particularly my mum and my grandparents.  They have always encouraged, supported and fought for me and I have learnt so much from them.
My mum always told my brothers and my younger sister we could be anything we wanted.  At that time she didn't realise what was around the corner for me but she still believed I would make some-thing of myself and I will, one way or another!
Before I could speak (at age 6), my Nannie, Pat, would sit me down and we'd make cards, paint and create for hours. We'd do jigsaw puzzles and watch Disney videos. My creative streaks definitely arose from those days. I was born creative and to this day use those skills. My children's books have frog characters, my Nan loved frogs. She inspired me.

11. If people want to work with you, what would they need to be offering to get your attention?
Opportunities to speak, to motivate, to innovate, to consult, to make a difference, to be heard.
My passion is accessible assistive technology and educating others.

12. If others wanted to follow in your footsteps, what would your advice be to them?
I'd encourage others to think about what is important to them, how to use their unique skill set to make a difference. Work hard and be passionate about your cause. Plus of course, never be afraid to speak up. Find ways to express yourself, in that process you eventually find yourself and also the confidence to help others.

[Disclosure: I have no commercial ties with the individuals or organisations mentioned above]

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Finding a signal within the noise

Apple's new iPhones and their new watch. That's what dominated the technology headlines last week. More firms are planning to enter the smartwatch space. In health & social care, what value do these smart watches actually add? The Apple watch claims to be able to track our activity levels, with new fitness and workout apps. Sounds great for those who want to monitor their activity or monitor their workouts in the gym or whilst jogging. I wasn't blown away by the keynote and I began to wonder how useful are these health & fitness features for someone whose job involves sitting for the entire working day? Bus drivers, taxi drivers, truck drivers? How useful is the Apple watch for someone confined to a wheelchair? Add a price of $349 for the watch and that you need your iPhone nearby the watch to use all the functions, and I wouldn't blame you for concluding that the reality of the Apple watch doesn't match the hype. Has it been designed by the 'worried well' for the 'worried well'? Are the primary beneficiaries those of us with above average income/education, who are digital savvy and already engaged with our own health? What value Apple's watch will add to health & social care? Only time will tell. 

Technology for Parkinson's disease

For me, what's remarkable, is that last week, there were three advancements in the development of technology for Parkinson's disease (PD) that didn't grab the headlines the way the Apple watch did. The main symptoms of PD are tremor, rigidity and slowness of movement, and it's estimated that between 7-10 million people around the world are living with PD. There are no lab tests to diagnose PD, no cure, and we don't know what causes PD. 

The first advancement was at the British Science Festival, where Dr Max Little gave an update on his work for the Parkinsons Voice Initiative. He'd already developed the technology to test for symptoms using voice recordings alone, more in his TED talk. He's gone one step further now, by using smartphones to track how the disease progresses in those that have PD, collecting data on voice, location & movement every 20 microseconds. He is now conducting larger trials to evaluate the technology, and he has said, "This new kind of remote data analysis will help patients monitor their conditions on a minute-by-minute basis from the comfort of their own homes".

We often hear talk about bringing the hospital into the home, and it's encouraging to see someone actively working on that. What I find particularly fascinating is Dr Little is interested in developing a tool "that could potentially provide specific feedback to people on symptoms that matter to them". The research has been going on for 8 years, and it reminds me that every problem in health & social care can't be solved with a weekend hackathon.

The second advancement is an activity tracker from Australia called the Parkinson's KinetiGraph Data Logger that allows automated reporting of a PD patient's movements. It's just been approved by the FDA in the USA. The device is prescribed to a patient, who wears it on their wrist, and it can collect data for 10 days, after which it's downloaded by the physician. This wearable technology offers physicians the ability to know much more about changes in a PD patient's movements outside of the doctor's office. The device even vibrates and reminds patients to take their Levodopa medication, and records data on when the patient took the medication. Remember that in my testing of the new Android Wear smart watches, an app already exists that allows me to get medication reminders on the watch, and to record when I have taken or skipped my dose. For many in the world of health, wearable technology is just a buzzword with little or no value, and I can understand their perspective. It's easy to dismiss this emerging area of computing, but dismissing it entirely might be foolish.

The third advancement was that Google X bought Lift Labs, joining the Life Science division. What do Lift Labs make? Their product, Liftware, is a spoon that vibrates to stablise tremors, which makes it much easier for people with PD to eat. The founder of Lift Labs, Anupam Pathak, "sees the technology being extended to other everyday objects, as well as extending the diagnostic capabilities to be able to monitor tremors over time." Google X is the part of Google that runs their most ambitious projects. Many of the people I meet who are experimenting with new technology in health & social care, are driven because of personal experience or a family member's experience. The mother of Sergey Brin (one of Google's co-founders) has PD, so I find this acquisition of Lift Labs most fascinating. Once again, time will tell what they bring out in the future. You can see more in the video below.

Finding the signal

Finding the signal within the noise of Digital Health is increasingly a challenge. The three advancements I listed above could both improve the quality of life for PD patients as well as enable a deeper understanding of this disease. They are not just cool technologies, but designed to solve real problems associated with different aspects of PD. 

Now, Apple's watch might offer extra features once it's launched, but for now, it's health features leave people wondering, "Is that it?". What problems does it solve that existing technology does not? 

How do we evaluate and test emerging technologies in Digital Health? This fledgling industry is evolving so rapidly that many organisations I speak to are struggling to keep up with the pace of change. How do we determine what offers sustainable value? One of the steps that seems to be missed is about understanding needs. Do we truly understand what doctors, nurses and other frontline staff want? Do we truly understand what patients want? Do we truly understand what the ordinary consumer wants?

Is the Digital Health industry at times trapped in the dogmatic perspective that the system is always wrong, and startups are always right? 

On top of that, do we actually have a genuine understanding of the underlying problems facing us in health & social care? I meet so many organisations that believe by making services 'Digital', everything will be transformed. I challenge that mindset. Have you stopped to examine whether the underlying process or service is flawed? Merely 'digitising' an existing process or service isn't going to help in the long term. It makes your process looks shiny and modern, but it's fundamentally still a flawed process. 

This week, it was announced that two US hospitals are working with Apple to trial the use of the new HealthKit platform with patients that have diabetes and other chronic diseases. Apple have built up amazing levels of trust with consumers around the globe by delivering a simple but great user experience. Could they do the same with health & social care? Many of us are excited by the possibility that companies with no track record in health, such as Apple, could transform aspects of healthcare delivery, but it's too early to tell. As Dan Diamond points out, an unsustainable hype cycle built up for the Apple watch, and another is building up in advance of the iPhone 6 release this Friday with regard to what the new iPhone can do for our health. 

In a thought provoking article by Darius Tahrir, which questioned the benefits of Digital Health, Julia Adler-Milstein, a professor in the School of Information at the University of Michigan said “There’s a risk of backlash against the technology: we were promised all these benefits, and we’re not seeing them”.

Evidence based medicine is the bedrock of modern healthcare systems, so how can we practice evidence based Digital Health? What evidence do we need to help us find the signal within the noise? Is the health of the Digital Health industry at risk if we don't take steps to generate, evaluate and share the evidence?

[Disclosure: I have no commercial ties with the individuals and organisations mentioned in this post]

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