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Making the best of Best Disease

It started with occasional double vision as I approached the end of my teens in the early sixties. After a round of visits to opticians and eye clinics my mother took me to see an eye specialist at a London hospital. I tried not to grimace as the eye specialist ground his ophthalmoscope ever deeper into my eye sockets. Mr Trevor Roper, the specialist, applied drops to my both my eyes, and we waited 20 minutes for them to dilate sufficiently.

After examining my dilated pupils, he explained that my mother and her family’s eye history were the source of my problem. I knew my maternal great grandmother was totally blind and that one of my aunts was born with only one eye, but my mother could see fine, so why me? Patiently he explained that this inherited disease, then called Dominant Macular Cysts, was very rare, little was understood about it and there was no treatment or cure. His reassurance that it progressed slowly, seldom leading to total sight loss was far from comforting, but it did provide me with a good argument with which to convince my dad that a future as an accountant, despite how good at maths I was, was not the best choice. In that sense it was a bit of a result!

Moving forward with my life

The double vision ended after a week, then two years later, reaching 21, I abandoned my accountancy articles and sought a more interesting and exciting career. The mid sixties were good times in London with plenty of career opportunities and prospects. My worries about future eye problems slid into the background as my eyesight remained undiminished in any way. In fact, it stayed that way for many years.

During those years I climbed the corporate ladder, bought my home, enjoyed a full social, cultural, and sporting life, travelled extensively for work and leisure with hardly a thought about my diagnosis. My annual visits to the eye clinic regularly confirmed that both eyes were fine and that my Best Disease, as it is now known, was stable. Such an inappropriate name for such a disease I have always thought, shouldn’t it be called Worst Disease? The name comes from mutations in the BEST1 gene, which produces the protein Bestrophin-1, but who the hell named them?

Changes in vision

In the 1990s things started to change. I was in my fifties and had started to wear spectacles for reading and figure work. However, my vision continued to relentlessly deteriorate. My central vision almost disappeared and then as my peripheral vision faltered and began to fail, work became impossible. Nearing my sixtieth birthday I finally had to accept my situation and take early retirement.

So, at 60 I found myself, due to a rare inherited disease, entering Shakespeare’s sixth age still with my hair and teeth, spectacles on nose and without eyes! Six decades of living in the light ending in a permanent black cloud and realisation that this was my new role and I had to learn the part.

Life as a blind person

My new life as a blind person started with being registered as blind, getting my BD8 form (predecessor of the current Certificate of Severe Vision Impairment forms), from Moorfields and then registering with my local council. The shock and emotional turmoil of that period and those events still resonate with me and help me relate to those currently facing this life situation.

Now, almost twenty years later, I can look back with considerable surprise and satisfaction at the life that grew out of that personal crisis. A new life only possible with the support of my long-term partner, who stood by me throughout, and the multitude of amazing and supportive organisations, charities and official bodies set up to assist our community. Impaired or lost sight cannot be replaced, but there is so much that can be done to minimise the impact, overcome many of the barriers and provide alternative ways of continuing to live an enjoyable and interesting life. But first you need to want to take up the challenge and make the best of things, in my case make the best of living with Best Disease!

New beginnings

Not wanting to retire yet I got help from my local Job Centre to apply for a course at the Royal National College for the Blind in Hereford to get me to a reasonable level of IT proficiency. That led to a job opportunity with Scope, as they set up a team to provide disability awareness training to commercial companies. I then found a position with City University in the Health and Sciences School as their Business Development Manager, before finally retiring aged 67.

All of this was made possible by the support of my local Rehabilitation Officer and her mobility training, my DLA benefit (Disability Living Allowance), the Access to Work scheme, of receiving a wonderful Guide Dog and so much help from my employers and many volunteers throughout. Plus, the vital and extraordinary advances in technology such as speech software for computers, tablets and smart phones, audio books and now smart speakers and home assistants. Sometimes I feel that the future has arrived already in my voice activated environment!

Retirement

Retired for ten years now I find my life full, busy with adult learning sessions for drama, singing and creative writing. It is wonderful how much is available if you just research and ask. My local gym makes adjustments to help me exercise and cinemas and theatres provide audio description services for almost every film or show. Museums and galleries have special events for blind and partially sighted guests and theatres provide touch tours to go on stage and even meet the performers: sighted friends fight to join me if my partner is not available!

Would I prefer not to have lost my sight? Of course, but has it been worth making the best of my new life as a blind person? Absolutely!

By Robin Davies

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