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Sunday, 09 November 2014 00:00

Young and Living with Usher Syndrome



Hey my name is Jodie, and I am any normal eighteen year old except having to cope with this cruel condition too!
So here’s my story.. I had no clue what RP or Usher syndrome was until I joined secondary school, and had a friend in my year who was diagnosed with it, I learnt bits from her just by asking her questions about how she coped and what it was like. I knew she struggled in the dark a lot because she always asked someone to guide her around the school. I always thought to myself how brave she was coping this condition at such a young age and that I was the ‘lucky’ one that I didn’t have it. She sadly left by the end of year seven and then forgotten about Usher as I got on with my life. The Halloween night came by and I was out trick or treating with my friends and my sister, then there was a low brick wall with a street lamp above it, I just happened to walk straight into it and tripped over and bumped my head, of course my friends laughed and my mum got bit concerned about it because anybody would have seen that. Weeks went by, I began to bump into things or trip over the objects and I chose to ignore it and thought I was just being clumsy child. My mum wasn’t having it so she took me to opticians to have my eye tested, I didn’t really know what was going on and just thought I needed glasses. But until the lady said that I could be diagnosed with Usher syndrome and needed to transfer me to the hospital for further tests. I just sat on the chair froze and thought to myself this is impossible? I was only 13 years old .. My mum went hysterical and burst into tears, so we both walked out the opticians and told my dad and my sister the news, my dad fought back his tears and tried to stay strong for me while my sister started to cry. They already knew little bit about the ushers because I told them about my old friend I had in my school. 
When we got home, my mum researched more on the computer about the ushers, and I used to get really angry at her and telling her to stop looking it up and that I ‘don’t’ have it, I guess I was in denial at that time believing that the lady at the opticians had got it wrong. Then it was the day I had to go to John Radcliffe Hospital in Oxford, my hands was shaking and my thoughts were all over the place but I had to stay strong for my parents. When we saw the doctor, she tested my eyes with field vision tests and I remember the first time I had eye drops in, I hated it! Then the consultant came in and started explaining about the Usher syndrome and gave my parents books and leaflets. I just sat on that chair just blanking everything out and thinking this must be a terrible dream. I went home later on that day, and I knew I had to tell my friends at school, so I started to tell my close friends that I have Ushers syndrome and they were very supportive and already knew little about the condition. So I was little more relaxed around them because they treated me like normal and only ask if I needed help to get about in the dark. I used to be so stubborn and say ‘No’ I can cope with this all by myself and pushed my friends away when they asked if I needed help to get around in the dark, as I walked in the dark by myself, I struggled so much and got so terrified because I didn’t want to walk into something and get hurt. So I ended up telling my friends to help me get around in the dark and they understood that I wanted to be independent teenager but only needed extra help. 
Now my family and friends are all aware of my condition and learnt that they don’t need to go over the top about it and just take one day at a time, I still go to hospital appointments and yet I still moan about going or I didn’t want to do the tests and I still get frustrated when I trip over things and bump into something but that’s something I have to live with I guess? I had no one to talk to about my usher similar to my age until I met this lovely girl Molly who also had the condition. I just remember the first time inboxing her on Facebook and asking how to open up to people about my worries and I kept bottling things up inside me because I didn’t want to upset anybody and wanted them to think that I coped fine. She replied back and she was so calm and reassured me that it wasn’t good holding things in and it’s better to let people know how you’re really feeling. So I took that advice and opened my worries to my close friends and boyfriend and they were really supportive, especially my boyfriend took his time to research the condition and try to understand that I needed little support at night times and not to wave the sides when I can’t see very well, he even goes to the hospital appointments and coped my complaining about the eye tests that I had to do. He was there all along just by holding my hand and making sure everything is ok alongside by my family too.
You should never let usher stop you doing things you want to do in life, even though it may be hard but try and achieve it with extra support, I am very glad that Molly had set up the website and blogs where I can happily read people’s stories and feel a lot better about it when I know I’m not alone in this cruel condition. So I would like to say great big thank you to Molly and her family for taking their time to do this amazing website/charity to get more people to be aware of the condition. Lastly I like to say big thank you to my family for being so supportive and being there when I need it.

Monday, 03 November 2014 00:00

Glendalough Trail - 1 November 2014

Well, here I am with some amazing friends and family( I'm the short one with red hair!!) getting ready to take part in my very first charity event here in Ireland to raise funds for the Fighting Blindness.ie.

It was an amazing but extremely tough challenge in the Glendalough Valley in the mountains in Co. Wicklow, especially as it was my first time partaking in such an event. Admittedly, I'm so delighted with myself for completing it and not even the sore legs can take the smile off my face. It was also a very important event for me personally, as while fundraising I decided it was time to reveal to everyone that I am losing my eyesight and that it is due to Usher Syndrome type 2. Only some of my close friends and close family knew beforehand, and even though they had given me enormous support and still continue to do so, it was time to make others aware. I wasn't diagnosed with Retintis Pigmentosa until March 2013, a few weeks before my 34th birthday. It was with complete and utter shock to learn of this diagnosis and I don't think i could ever forget that day, how could anyone really? After diagnosing me with RP, it suggested that it could be due to Usher Syndrome and I was urged to find out more about this condition. I left the ophthalmologist's office having heard his words but not comprehending what he actually meant. It was only when I got home and like everyone does, I 'Googled' it all. That's when it hit me, I'm going blind. When he spoke of my eyesight getting worse and worse but hopefully I might have good central vision for another 10/15 years, that's what he meant, I'm going blind. He talked of the possibility of gene therapy which looks promising but probably not for another 15/20 years, he meant I'm going blind and there's no cure at all. It was really difficult to believe that I was having to deal with going blind when I was already deaf. Kept thinking was this 'supposed god' having a laugh? Reading up on Usher Syndrome, I knew straightaway that it was what I had and more than likely it was type 2. It really was like reading about myself. Genetic testing later showed that I do indeed have Usher syndrome type 2 (USH2a). It was awful though, having to tell my parents that the hearing loss was in fact genetic (previously thought it was due to an infection) and that I am also going blind now. I know they did blame themselves for awhile but I'm hoping that with me being open and honest with others about it, it will show them I will be just fine. I came across Molly Watt during my first Google search on Usher Syndrome and kept thinking wow, fair play to her for all that she was achieving and not letting it hold her back. I really admired this girl and I still do!! I also came across Mark Dunning and the Usher Coalition, Megan Kennedy and many others, and the positivity and faith they had but yet being totally honest with how hard it can be was admirable. They all gave me the strength to believe that I can still achieve things in life despite it getting harder to do so. After a year and half of coming to terms with the diagnosis, I realised I didn't want to hide it anymore. I did hide my hearing impairment and I had left it to others to guess that I was so. But hiding going blind... no, that would be so draining and completely unnecessary. So I put up on Facebook the link to my fundraising page where I explained briefly what I had and how it was affecting me. It really worked out well as I had a lots of people talking about it and it really raised the profile of Usher Syndrome in my area which I'm delighted with.. Also by raising awareness of the condition I was hoping to get support and understanding in return, and that is happening. The amount of support I got was overwhelming! There is still some way to go though with raising awareness and making others understand how tough it is. But sure it's only the start of my journey with Usher and I intend to make it a posiitve one even if it does get a bit bumpy along the way.

Friday, 24 October 2014 01:00

Me, My Wife Claire and Usher Syndrome

When my wife and I discovered that she had Usher Syndrome, my first thoughts were confusion, what was Usher Syndrome, what does it mean and why has she got it?
It felt so cruel hearing all this that we both broke down neither of us knew what to say or do, I just knew I had to be there for her and be strong for her. 
 
Together we started the long journey to discover all we could with numerous Opthamologist and Gentics appointments, sometimes there was so much to take in and we would come away heartbroken. 
 
I felt so relived that it wasn't just "Clumsy Claire" and that there was a reason behind it all, as a few years back i had noticed little things but wanted to see what the doctors had to say. 
I find Claire inspirational and I'm glad she found other Usher friends that she no longer feels alone and isolated, she's no longer a hermit she's become my "Happy Claire" again and I believe MWT has done that for her seeing how Molly copes with the world has taught Claire a lot. 
 
Claire now has a guide dog which made me feel Proud of her but also upset as it made it all so real and I realised it's something Claire has to live with everyday, it's easy for me to forget that she stuggles so much but with Minnie her Guide Dog beside her I know she will be fine.
 
Claire has her confidence back and along with it her independence I am now able to work full time feeling safe in the knowledge that Claire is getting on well with life now.
Friday, 24 October 2014 01:00

Hi "I am Claire"

Hi I'm Claire Cooper and I have Usher Syndrome type 2, I've been partially deaf since birth with 2 digital hearing aids but never considered myself deaf as I've always been oral and never needed BSL. I had help where needed throughout school and college. 
 
When I left college I began a career in the hospitality industry working in bars and clubs I was living a normal teenage life. But then when I hit 20 I began to notice the spotlights at work were becoming an issue among many other things like bumping into lampposts and falling off curbs etc, and soon became known as "Clumsy Claire".
 
At 21 I got married and only a month later we got the answer as to why I had been so clumsy.... Usher Syndrome
 
Well that took the wind out of my sails and really knocked our honeymoon period into a downward spiral, I couldn't see the light So became depressed and questioned my marriage as I didn't want my husband to become a carer, it felt like I had been given this life sentence and wondered what I'd done so wrong to deserve this. I learnt the prognoses and thought my life was over. 
 
My husband was amazing he said he would stick by me no matter what, that he would always be there.
My parents and family took it hard with self blame as its a genetic condition but they too said they would support me through everything I did. 
 
I also found Molly and Jane Watt before MWT was born.  They were the first people I discovered with a link to Usher, when I discovered my diagnoses I'd never heard of it and felt alone so to find these amazing people really showed me the light, even though Molly is younger than me she was so positive and was achieving so much, her mum is such a fighter too helping so many with Usher Syndrome overcome obstacles if something isn't right then Jane is there without question. 
These people are amazing and have helped me through so much they don't even realise, without them I don't think I'd be where I am today.
 
Which is a Mother to a Beautiful 3 year old girl, we decided to move our life plans forward so I could see my child grow up while I still have sight. I'm also a Guide Dog owner, my dog Minnie has been another little life changer and has firmly put our lives back on track. 
I also enrolled myself into Open University to start studying again which without Jane and Molly's help I don't think I would have ever considered again. 
 
Usher Syndrome may have been the worse news I've ever had to hear and also the biggest life changer but it's made me who I am today thanks to the amazing people around me I've become a fighter and a person who has Usher and proud!
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