“Life is either a daring adventure or nothing at all” – Living with Usher Syndrome (Type 2) – Sue Scharf

Article category: Events .
June 27, 2022
In the words of Helen Keller - Life’s an adventure or nothing at all - this is my motto

Picture the scene: it is an overcast, cold late Autumn afternoon. You’re sitting round the dining table in a cozy cheerful home with a few good friends, enjoying wine, tea and sumptuous eats. It is Book Club day – a monthly event much anticipated – as it’s a time to switch off from work, household chores and life’s dramas – and just enjoy the stories of everyone’s day-to-day lives, spiced up with loads of humour and good vibes. Then load shedding happens – the light goes, yet the banter and fun continues, despite the setting sun and pervading darkness. For you though, the enjoyment dissipates, as the faces of those around you start to disappear, the sound of chatter is merely a sound, no words are “heard”, and you can’t even see where your bag is. An all-to-familiar knot forms in your stomach as what started out as such a pleasant afternoon, rapidly turns into a nightmare, where even the thought of standing up becomes scary as you know you’ll knock something over, bump into something or someone. The friends are so helpful, sensitive and caring – especially as you announce that you must leave the happy party, desperate to get home to a familiar environment, where you know where everything is, and most importantly don’t have to concentrate on what is being said and by whom…

This is the reality of what Ushers Syndrome is.

I am 58 years old and have Ushers Syndrome, a genetic condition whereby one loses one’s hearing gradually (in my case since birth) and then a few decades on, one develops a condition called retinitis pigmentosa – whereby the retina behind the eye dystrophies gradually, causing initial night blindness and loss of peripheral vision (RP is also known as tunnel vision), ultimately resulting in full blindness.

One might think, but isn’t the loss of hearing bad enough? But hey, what is the definition of “bad”? I adjusted to this disability just fine, wearing hearing aids since age 4, lip-reading since before that, leading a fairly normal life of main stream schooling and university, pursuing a career that I love, being blessed with 2 amazing kids (now successful young adults) and incredible supportive friends.

Frustratingly, hearing aids have their limitations, amplifying only those frequencies that still exist. As such speech recognition is the biggest challenge in this journey: I typically hear a couple of words a person speaks, and my brain fills in the gaps of those words not heard. This produces some rather hilarious or awkward outcomes, where I “heard” the exact opposite of what was being said. Me: how is your Mom? Friend: She sadly passed away last year (heard as “she’s staying with us this year”). Me: That is wonderful! Friend: bewildered look… Me (now awkward and embarrassed): please repeat what you said…  

When the RP started in my early 30s and significantly worsened in my 50s, was when life became a LOT more challenging! You see, I need to lipread to “hear” – and if the light is poor (as it is at night or in a dark room or when it is overcast) OR too bright, my eyes cannot process the light and visibility is severely diminished), I cannot lipread – and so I cannot hear. In addition, with tunnel vision, similar to looking down a vuvuzela from the narrow end, the “field” of vison close up is so narrow that one cannot see things right in front of you. E.g., an open cupboard door (trust me, I have a couple of scars from walking into doors!), a wine glass (I have sent many flying in my life!), etc. And yet the field increases the further one is away from an object/view. I see and appreciate beautiful views – so long as the sun is shining!

Whilst these challenges frustrate me, I am constantly reminded of the wonderful things that have happened in my life BECAUSE I have Ushers! I have met wonderful people: I have a truly special friend who also has Ushers – she has enriched my life in so many ways! I have met others who are either fully blind or are also partially sighted, and we have experienced an instant connection and developed wonderful, supportive and caring relationships. These friends who have disabilities are the ones I can laugh the most with: we unashamedly share stories and anecdotes concerning disability-related experiences with great hilarity and humour. I have a guide dog, April, who is my guide and companion in life. Adventures with her have given me totally new perspectives on life and people in general. My list wouldn’t be complete if I didn’t add in the guide dog trainers from SA Guide Dogs: wonderful, incredible, dedicated, deeply caring people, who through their HARD work in training not only dogs but the humans who receive these dogs, enable blind/partially sighted people to lead an independent life. It has been a privilege to both receive a dog, and to work and learn alongside these trainers from Guide Dogs. These are just some of the people I have met and experiences I have had thanks to my Ushers. There are countless more – all enriching my life in ways not even imagined.

Medical science being what it is: fast moving and dynamic, has a “treatment” for full hearing loss: a cochlear implant, which supplies ALL the frequencies, giving back full hearing AND speech recognition. I am hoping to receive a bilateral CI in the not too far distant future – ensuring that I will be able to hear a full conversation clearly – even from behind a mask! And, in case of total sight loss, the need for lipreading should disappear.

While there is no cure yet for RP, I truly believe that there will be one (or at least a medical intervention that will halt the progression of RP) in the not too far distant future. In the meantime, I will embrace the words of my ophthalmologist of “learn to accept and live with low vision”, and the guidance of my low vision optometrist who constantly teaches me new tips (like “expand the field (of vison) – hold the object/device further away – and increase the font!”). Experts like these who are full of optimism, whilst being realistic (never giving false hope), and friends and family who support, listen, encourage and motivate me – these are the people who keep me motivated and inspired. This journey is not lonely, so long as one does not isolate oneself!

In the words of Helen Keller, “Life’s either a daring adventure or nothing at all” – this my motto – and whilst there are incredible frustrations and limitations, there are an equal measure of special times, incredible adventures, and incredible people alongside me. These are what makes life so worth the while – a life in which I hope in some small way to inspire and encourage others.

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