Retinitis Pigmentosa (RP) has always been something that I've denied but this year as I turned 39 it became clear I couldn't deny it anymore.
I was 7 years old when the news came that there was RP in my family. My sister was the first of us to be officially diagnosed it was the early 1980's and there was very little information around then she recently told me that back then she was informed by the specialist at Moorfields that there was no cure or treatment, she would most likely go blind and not to have any children! However, she now lives in San Francisco with her husband and two amazing children Lauren and James both in their early twenties and both unfortunately have RP.
My memories of that appointment are vague all I remember is having eye drops that made me unable to see for a few hours which I remember was scary especially at 7 years old. From there on I didn't think about it, RP was just an occasional conversation piece that we had an uncurable eye disease in the family that might make me blind but probably not until I was very old!
I continued to live my life and was lucky enough to have an amazing one with many extreme highs and lows enough to write a pretty good book but that's for another time. I travelled the world working as a singer beginning my career as a Bluecoat at Pontin's and eventually becoming a touring cabaret act performing at holiday parks and hotels all over Europe. My travels eventually took me to Glasgow where I began to settle and build a new life. RP was still very much at the back of my mind even though I knew I was struggling with going from light to dark places, by this time I was in my mid twenties. I was building a career in car sales was in a relationship and the years began to fly by without much thought of my eyes.
By the time I reached my 30's my thoughts turned to becoming a father, I never entertained the idea before, nothing to do with RP just down to having too much fun working in entertainment. On November 4th 2007 my life changed when Ellie was born, I took to being a dad as naturally as I take to the stage and as the tattoo says on my right forearm "It's like a song I've never heard I've never sung but know the words" a line from one of my favourite songs which describes becoming a father.
The bond between Ellie and I was so strong unfortunately the same couldn't be said for the one between myself and her mum and the relationship came to an end by the time Ellie turned 3.
At that time I had gone back to singing again driving all over the uk performing as a cabaret again. I made the hardest decision of my life to move back to manchester to be near family and to give up singing again so I could regularly make the long drive to bring Ellie down from Glasgow. By this time even though my eyes would struggle in darkened rooms I could still drive with no problem. As hard as it was trying to start again being so far away from my little girl I knew it was something I had to do, I had a gut feeling that it would eventually work out and that's when I met Amy.
Amy came in to my life and brought with her the promise of a love filled life I thought I'd never find. Amy had two boys Harvey and Louie, Ellie and I slipped into their world like it was just meant to be.
Within 2 years together I had gone back to car sales trying to build our life we were blessed with the arrival of the cutest baby boy you will ever see Austin, he arrived in June 2013 and on Xmas day that year I asked Amy to marry me and she said yes!
We then started to plan our wedding for New Year's Eve 2014. The following year everything changed!
By May this year my eye started to get worse and a check up at the local opticians confirmed my worst most denied fear my RP had started to rapidly progress and my driving days were over. My first thoughts were of Ellie and how was I going to be able to see my little girl? My second was what was I going to tell work?
I told work straight away and they let me go on the spot as I could no longer continue my role without a driving licence.
My little families world began to unravel, I went to the specialist who confirmed me as legally blind and gave no other answers. I then went to the job centre and asked for help. From there on it has been a constant battle to try and get the help we so desperately needed but were plunged into extreme poverty falling behind with bills and just finding it extremely hard to survive due to the horrendous waiting times for PIP etc never mind not being able to see Ellie due to the cost of collecting her from Glasgow.
As the months have passed this year my eyesight has rapidly declined to the point where I have now started long cane training and am on the list for a guide dog to try and get back the independence I have lost as I now suffer with anxiety due to my vision loss my confidence has all but gone and I very rarely go out alone.
The things that have kept me going is getting married to the love of my life on New Year's Eve as planned and meeting an amazing friend in Simon who I met through one of the facebook RP support sites on the advice of my sister in San Francisco.
Simon has Usher Syndrome Type 2 and it was obvious from the start we had so much in common other than our crap eyes. We shared a love of music and the same sense of humour. He is my best man for the wedding. When simon and I became friends he invited me to come round to his house as he had a mini recording studio so I could sing and let off some steam. Here began the start of a little confidence returning and the idea for my RP project.
Simon and I had held a couple of informal meetings with other RPers and that's where we met Colin who also has Usher Syndrome Type 3, a very special person who was equally passionate about connecting those who felt alone.
As time and eye sight went by I became more vocal and passionate about the injustices visually impaired/blind people were going through all over the world and that's where "Stand By Me RP" was born.
I was asked to perform at a meeting for people with RP and Ushers in the north and decided to created a theme song for us that I could share worldwide, I decided to use "Stand by me" http://youtu.be/qxFdmqZoceo because everyone knows it and then I re-wrote the lyrics around the fears a lot of us have. Through the support of the VI community and the amazing people who have responded to the video and continue to keep sharing it the message has reached place and people I never thought I would reach.
Molly and her incredible family and Joanne Milne who's story reduced me to tears in seconds in fact I have written a song about Usher Syndrome for Jo which we will unveil together in the near future. All of a sudden my future looks good again I am determined to take the disease that has stripped me of my confidence and use it with my passion for music to help as many as I can whilst in turn helping me find me again. Amy and I are stronger than ever and our wedding is only weeks away and even the recent trip to the specialist who confirmed that my central vision is deteriorating fast could not dampen my new found spirit. I will begin 2015 a married man and plan to fight to raise awareness for RP and Ushers with your help of course
I will at every opportunity try to raise awareness of RP, Usher Syndrome and Guidedogs.
As anyone who suffers with Ushers will know it is a trying disease which has a profound effect on not only the sufferer but those around them. All too often this is the frightened parents coping with the prospect of watching the child they love struggling to cope with a world that doesn’t really know or understand their illness. It is the children who have to realise that their Mum or Dad are “different” and can’t see them grow up or hear their words. There is one group however that very few people mention or consider and that is the partner of an Usher sufferer.
These people share the hardships of life not through having to because of hereditary but through love and caring. These people feel the pain of the sufferer but also feel their own pain. These people fight each day to survive for themselves and their partner. I know I am one of them.
I think to understand my life you need to know the full story. It may take a while so sit down and make yourself comfortable.
It all started one beautiful August afternoon (13/8/1991 at 2 pm to be precise). I was being a Good Samaritan and helping a teacher find the area office. There I saw a beautiful lady in a flowery sundress that captured my heart immediately. To look at her you would never know she has Usher’s and if her colleague (a mutual friend) hadn’t said I wouldn’t know of her disability. The next day I telephoned her and arranged a date. She was very open about her condition although at this point we didn’t call it Usher’s but RP.
I quickly fell in love with this beautiful lady and it wasn’t long before we started dating. I guess I realised the true extent of her illness till we had been going out a fortnight. She came to stay over and we went to the pub for the evening. As it was summer we left in bright sunshine and everything was easy. By the time closing time came it was dark. We walked home and Nicole clung on for dear life. In fact her grip was so tight it hurt and she stumbled a few times. Being a new relationship and a gentleman I didn’t say anything until we got home and asked if she was ok. She explained that she couldn’t really see anything during the walk. It didn’t change the way I felt about her but it made me think.
I took my wife to meet the parents and the rest of my family (I lived away) the following week. They fell in love with her instantly but didn’t fully understand her illness until darkness fell. They lived in a flat which had dark outside stairs. We had to climb them followed by my mum and dad and brother. As we climbed I told her “step, step, step, level”. Later my brother asked what the instructions were for so we explained. Still we didn’t know about Ushers..we thought she just had RP!!
Our love grew and a year later we married. I loved her with all my heart just as I do now but neither of us knew what lay ahead of us. We struggled to start a family and had IVF. We never thought it the day would come but the little blue line finally materialised. We headed to the docs for confirmation. At the end of the joyful visit the doctor asked Nicole “so what is Ushers Syndrome?” We looked non- plus at each other as this was the first time we had really heard of it. The doctor just shrugged and told us not to worry about it until after the baby was born.
Nicole had a real hard pregnancy. High blood pressure and pre-eclampsia prevailed throughout and I nearly lost her on the operating table but we came through and we had our first daughter. A few weeks later we went back to the doctors as we wanted to know about how Nicole’s disease would affect our new baby. The doctor sent us to a genetic counselling. It was at this meeting that our world started to fall apart. I will tell more in part 2 of my story