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Tuesday, 02 December 2014 00:00

Down the Loo or in the Nettles!

Down the loo or in the nettles at the end of the garden: from the age of 3 my top two destinations to hurlmy detested hearing aids. 

Painfully shy, diagnosed profoundly deaf and devoid of much speech, I took every opportunity in venting my fury at having to wear such instruments. I mean, have you SEEN those things we had to wear back in the 70s? Worn on a harness with huge wires travelling from chest to ears that shouted ‘Look at me, look at me!’.  Not to mention the shocking level and quality of noise that was funneled into my ears.

Of course I have many, many happy memories of early childhood, but those that are sharpest in my mind are those of isolation, confusion and excrutiating shyness.

Nursery: I had absolutely no clue of what was going on most of the time.

Early school: desperately wanting to blend in but oh, the agonising, public shame of having to walk out of the class in front of everybody’s stares for my speech therapy, and then everybody’s questions afterwards. One classmate asking me ‘why was I death’?

My desperation to be a normal part of society was all-consuming and gave me a determination (some would say pigheaded stubbornness) to prove to everybody I was just as good as anybody, and better. With the steady improvement in hearing aids and relentless (and I mean, relentless!) determination of my mum to fight against the system and open up my world, I slowly but steadily started catching up with my contemporaries.  

By the time I was in to my second year at a private girls’ school (small class sizes and fewer teachers to remember my needs was, fortunately for me, a huge help), I was academically successful, ending up with 10 O’ Levels and 3 A’ Levels, was in many of the sports teams, played badminton for Surrey and had a decent circle of friends. With long hair to cover my ears and a good speaking voice, nobody would ever know I was deaf.

Now I turn to another memory: this time me at the age of 15, in my bed not being able to sleep. Praying: please God, please God don’t let me go blind.  Which is weird because I was not, at this point, suffering any loss is sight as far as I knew, it was that my bedroom seemed darker than usual and I couldn’t pick out things in the room I was sure I could before.

Although I didn’t know it at the time, the slow decay inside my eyes had started. My badminton started suffering. I struggled to see at parties. I couldn’t see the same as my friends in the cinema, I became ‘clumsy’.

But it wasn’t until I was 22 that I had any inkling of what was happening. I was having Sunday lunch with my boyfriend’s family one weekend when I complained about my poor night vision. My boyfriend and his father, who were both doctors, fetched an opthalmoscope, spent ages taking turns peering into my eyes and looking up stuff in their medical books, In the end the words Retinitis Pigmentosa came out, and there it was, my bombshell. Delivered to me post roast pork and apple crumble in a sunny living room in leafy Northamptonshire.

The formal diagnosis when it came whilst sitting opposite Professor Bird at Moorfield’s Eye Hospital, was of course utterly devastating for me and my family. The unbearable unfairness of it, the overwhelming fear of my new future was terrible.  Consumed with numbing grief, resentment was at the surface of everything. Why me? I had already beaten a profound hearing loss so why this as well when I need sight so much more than other people?

My parents supported me and loved me, but I knew they felt an enormous sense of guilt about passing on such genes. I didn’t have enough in me to deal with their feelings of guilt and sadness. My parents always fixed stuff, it was frightening to see them and everyone else become powerless.

I was offered no effective, appropriate support for someone of my age and stage of RP. The literature, containing pictures and stories of guide dogs and canes and people twice my age, made me feel ten times worse as I was nowhere near that stage and I threw everything away I soon as it came in the post. 

Completely lost, this was the beginning of my new mountain to climb.

‘Down the loo or in the nettles’ is to be continued.

Monday, 24 November 2014 00:00

Falling Into The Darkness

This was another day in my life when Ushers Syndrome had well and truly won.

I’d had my shop for three years after struggling with the demons in my head after taking my redundancy. I’d started a shop selling wallpapers and soft furnishings, I’d had a huge build up of emotions. I’d ran the shop for three years, but Ushers was taking over.

It was becoming harder and I was now on the waiting list for a guide dog.

The shop landlord wanted me to commit to another three year lease and also to increase my rent. The time had come to sell up and ship out. My heart was heavy, anybody that knows me will be sure to tell you I don’t do things half heartedly, either all or nothing as if i have a massive point to prove. Turns out all my Usher / RP friends feel the same, as if they struggle for acceptance in the normal world. But what is the normal world?

Back to the Thursday 13th of June 2013, I’d managed to sell most of my stock and had a date to be out of the premises, it was in the two weeks time on a Saturday. I’d managed to sell off lots of stock, but had to get rid of the fittings shelvings etc, this had all been made up in the shop and was too big to take out of the front door. I’d sold some of the display boxes I’d had made for my wallpaper. I’d sold two lots of twenty boxes (these were pretty heavy and made from MDF).

My shop closed at 17:30, so i made arrangements for the display boxes to be collected after that- as you can imagine, middle of June it was a lovely night and quite dazzling on my damaged eyes. I told the guy to bring help but he didn’t and if the truth be told, I didn’t feel comfortable about the whole thing from the word go. The only way out was to slide them along the floor, out the rear fire exit door, then lower them off the flat roof the the van below (which was ten and a half feet). My partner and her father arrived. They watched on the ground as me and the other guy slid them along the roof tied ropes around them, then proceeded to lower them down. It wasn’t easy but we did it.

Thank goddness I thought, took one step back and fell ten and a half feet, I hadn’t seen the hole in the corner. It was a rectangle about eight feet long by three feet wide, but someone had bricked up the wall. So not only had I fallene, but I had no way out!

When I fell I guess I was very lucky, as I had landed on my feet and quickly pulled myself together, but when I fell I didn’t feel right,  I’d hit the floor with my hand and elbowed myself in the ribs. Also my glasses had fell off I searched about all the time, all the time I could hear people screaming my name, asking if I was ok. Then as I tried to pick up my glasses with my right hand I felt pain, I’d never felt pain like this in a very long time. I looked again, my right hand had in fact snapped off the end of my arm. I felt physically chlaustrophobic, how the hell can i get out? I thought. I didn’t want to panic anybody, I yelled “I’m ok, I’ve just broken my wrist I think”. Even though I knew I’d done more than just break it. There was lots of panic on the other side of the wall, as they decided to go and get ladders. I should of dialled 999, but I guess it was the Usher /RP that stopped me!

I went for the hard option rather than embarass myself. It felt like an eternity, all the time I was trying to stay awake and talk to my partner’s father on the other side of the wall, I’d even managed to call up A&E and book myself in.

Eventually, the ladders arrived along with a squad of helpers. One thing I didn’t want or need was someone to remind me about RP/Ushers and how I shouldn’t have even attempted this job in the first place. The ladders were now lowered and I was up the ladder before anybody got one foot on the rung, then the ladders were dropped over the other side of the wall. This time they demanded someone went before me, by this time they’d all seen my hand and how yellow i was becoming. They took forever to get me down. I jumped in the car only to be met by a hundred questions. I knew it was bad, because I couldn’t even put the seatbelt on and my partner wanted to throw up. It was a long, very long couple of miles to the hospital. I even called A&E again: “Hi it’s Colin, we are on our way”.

We arrived in A&E only to be met with a glowing, friendly smile and a wheelchair. It was Mark, a guy who used to work for me many years ago. He was brilliant. He took me to A&E and got me x-rayed and scanned, he then told me it wasn’t looking good and would have to reset, but there was splinters. So now I had everybody off the ward as an audience. He injected my hand twice, held my shoulders, and popped my wrist back on the end of my arm. Next I got a million questions about the incident and what did I say? I fell over the back door step, I guess I did this to hide the embarassment of RP Ushers.

I was on very large doses of pain killers and liquid morphine and very very tired. I was admitted to the ward and constantly fussed over by everybody. To be honest I hate that, especially as I kept thinking this is your own bloody fault, and that damned Usher Syndrome got me again!

The medical staff were great. They talked in detail about me but I was with the fairies on morphine, only to wake up two days later to find they couldn’t operate on me in Dumfries, but wanted the top man in Scotland to do it: Mr. Hamed at Gartnavel, Glasgow. They offered me a taxi, but I declined. I arranged my mother and father to take me up. Now I faced a further five days in a single room with Ushers, I was more concerned about that than my hand. I was met by the great man himself, a young guy and a good sense of humour. I was gowned up and ready to go, he said “ It says you fell over the back door step. Now tell me what really happened?”. So I did,  I explained about my vision. He promised to try his best, as long as I stayed off roofs and tried hard on my physio.

After a couple of days, he came to see me. He said “You’re a lucky boy, you have 12 titanium pins, but I don’t think you’ll ever get full movement”. Before I left he sent me to physio. They told me five exercises ten times each, three times a day and stay off work for at least six weeks. So off I went back home.

The next day I was at work I had to wind things up, it was very hard. But I did it. I also kept doing my exercises all day. Finally the day came to empty the shop, it was a huge relief and felt right at the time. As if someone was telling me to behave, enough is enough. So I continued my exercises and went back to Mr. Hamed after six weeks he said “how are you doing?” He just laughed. “No more roofs for you. Your wrist is about three inches wide, your neck is half an inch, you’re one lucky boy”. He then laid the backs of his hands on his desk and said “You’ll never ever get this movement back”. I did the same and he was shocked. I told him “You made a promise and I made you one”. I guess I was so mad at the situation, I felt I owed him for wasting his time and being such a fool.

Well I guess the moral of the story is don’t be too proud to let others help, sometimes it is better to just walk away. Life is too precious.