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.

"Mum I can't see the whiteboard at school properly"

Me: "Are you sitting close enough to the board?"

"I always sit near the board and the teacher, always have to  otherwise I cannot lipread and miss out in class".

Me: "I think you need an eye test, maybe you need reading glasses"

"I'm also getting terrible headaches".

An eye test was arranged for both daughters that coming weekend.

Yes, both daughters as the younger daughter always felt she was missing out on the never ending appointments Molly already had because of her deafness - she now realises just how fortunate she is I should add.

Arriving at the local opticians at 9.30am that December morning both girls quite excited we waited for their names to be called out.

Lily went first, she was very excited and was thrilled to be told she needed mild reading glasses.

Molly was next, her test seemed to go on and on and on and twice I was asked if other staff members could look at her eyes as she was displaying something out of the ordinary.

I began to feel very anxious, coincidently I'd had my own eyes tested for the first time just a week or two before so I was suspecting something wasn't right.

However, Lily was skipping around with excitement that she needed glasses!

By the time the optician had finished his investigations and spoken with several staff members my husband had got to town from football with the boys.

The girls were asked to choose their frames with another member of staff as Molly too needed mild reading glasses whilst Andy and I were asked into the consulting room with the optician.

The door closed behind us and then came the hammer blow which was to change our lives forever.

"Do you mind if I ask about Molly's hearing as we noticed she wears hearing aids?"

Molly was born severely deaf, picked up at 18 months after lots of fighting to get her tested properly as her speech was not developing - we had been told Molly's deafness was a "technical blip" it is nerve damage and therefore she would need hearing aids all her life, we had dealt with that.

The optician explained that he had recognised RP (Retinitus Pigmentosa) when looking into Molly's eyes, a progressive retinal condition, that it looks slightly different in a patient with Usher Syndrome.

Usher Syndrome is deafness and RP and there are several types.  "Your daughter is the only patient I have ever seen with, what I suspect to be Usher Syndrome, which is why I asked if my colleagues could see, it is very rare and some opticians might never ever see it".

Then those words I'll never forget "Don't be alarmed but your daughter must see an eye specialist as soon as possible".

On leaving the consultation room I was in shock, I could see my two beautiful daughters trying on glasses, excited and happy, just how they'd always been and all I wanted to do was cry - it's true it hadn't been confirmed but as much as I didn't want to believe it, I just knew not only did I have much to learn to support my daughter but that life would never be the same - I was in shock.

That news came on 11 December 2006, two weeks before Christmas.

Google came into its own, I read and researched, read and researched and read and researched from that day until 2 January 2007 when we had our first visit to Moorfield Eye Hospital in London and saw the amazing Professor Tony Moore who confirmed Usher Syndrome and most likely to be type 2, this was later confirmed through genetic testing much later on.

I made it very clear at Moorfield that I did not want Molly told of her diagnosis. I felt at only 12 years old breaking this bombshell to her should come from her parents.

How I told Molly is another story......

My name is Anum, I am 20 years old, soon to be 21.

Some of you probably know that I have Usher Syndrome as we went to the same school.

Where can I start?

I was born in Africa, Kenya, some of you couldn't believe that I was born there but I was.  

I was born hearing, then when I was about 8 months old, I got meningitis. My family wasn't expecting that to happen and struggled with what to do about me because I was only 8 months old.

We went to the hospital for a few checks up. The doctors told my parents that I was fully deaf and there was no going back. So my father who is currently in Kenya made a huge sacrifice to send me away to England to have a better life, better education and a better future.

So I moved to England when I was about 1 year old with my mum, my brother and sister while my father stayed living in Africa, working hard as a doctor for his whole life. We still do go to Kenya and visit him in the summer of course, sometimes it can be hard because of the distance! Anyways we moved to England and I started a hearing impaired primary school and while there I had some problems, with falling down, banging in something, dropping objects and unable to find things!

My teaching assistant noticed something wasn't right while I was doing games, I kept falling over, being very clumsy and unable to find the ball. After this happened she informed my mother about it. My family noticed as well at home and got concerned. So they decided to take me Moorfield eye hospital for a check up.

To be honest I don't even remember going to that hospital for the first time because I was only about 9 or 10 years old. They told my mum that I have Retinitis Pigmentosa which is the blind part of the Usher Syndrome. This made my life really difficult because my deafness and blindness were linked together.

I was aware I had Usher Syndrome when I was 10 years old. I struggled so much, struggled to see in the dark, worried what other people would say, got so frustrated when I trip over, bump into people, even though I do say sorry to them but they gave a bad attitude back which is dreadful, I get so upset when it makes my life hard but I won't let Usher Syndrome take my life over. I am always try to be strong.

My family and close friends understood it and supported me through it. They also help me in the dark as their guide. I really do appreciate it because I do need some support!

I hated going to hospital for my eyes check up, it was horrible and dreadful. I hated the eye tests and eye drops. Even worst I hate hearing not very good news from the doctors which upsets me. Sometimes when I go to the eye hospital for my check it takes hours, then at the end there is bad news.

I get really frustrated and wonder what is the point of going there because there ain't gonna be any miracle or cure to make my eyes better but what can I do?

What has happened has happened, no one is perfect and we have to be happy with what we got and what we can achieve.

I went to a boarding school for 8 years and met few people who got ushers. They probably felt similar to me.  I was glad that I wasn't the only one.

At first I used to let my ushers bother me,  stop myself from going out with my friends, used to stop myself from being social, used to stay indoors but then  I realised that it was making my life miserable so I decided to do what I wanted to do. I go out a lot, have a good social life.

I go clubbing which involves the dark with lots of flashing lights and I have support from my friends and family who keep their eye out for me and always make sure I am ok.

You mustn't let your ushers stop you from anything. Be happy and do what you want to do and what you always wanted to do.

There are days I feel so down but I try to do lots of things to keep busy and avoid being negative. I used to be negative but now I am not. Good days and bad.

For those who have Usher Syndrome, you are not alone, try not to let it bother you. Sometimes I get dreadful headaches, eyes aching, feeling dizzy, flashes, tired and weak. Sometimes I get very clumsy and fall over or trip over and my friends would laugh at me and I would laugh at myself  because I don't let it bother me! I am glad I am not alone and totally understand you guys feeling! BE STRONG AND DON'T LET YOUR USHERS BOTHER YOU even though it's a horrible condition! Keep smiling