If you are looking for me, chances are I will be travelling to far flung places, horse riding, hiking, rock climbing, scuba diving or attempting to ski. Or I could be telling a funny story about the time I sat on stranger, tripped over a dead person, stumbled into a pig carcass, wandered into a crime scene or got my leg stuck in a long drop toilet. Otherwise I could be at work, doing my bit to help vulnerable people.
I look normal to most people but have already lost over 90% of my vision due to Retinitis Pigmentosa (RP). The most significant effects are night blindness and tunnel vision. I can’t see distances nor anything to the sides. Sunlight hurts and I can rarely see much without strong contrast. I have very little depth perception which makes walking down stairs difficult. The text I read needs to be enlarged. I also have macular oedema, which distorts my vision even further, like opening your eyes in water. My right eye is all but destroyed from this pesky intruder.
Diagnosis and the darkness
I was diagnosed with RP in my early 20’s and it was a significant shock for all of us. There is no family history and no one knew that I couldn’t see. Not even me. On the bright side, at least it explained my lack of coordination and terrible driving. My mum still turns pale when she thinks of her experience in trying to teach me the rules of the road.
Following the news, I explored various blindness organisations and searched for support groups. While I found few, at the time they seemed to be for the ‘really blind’ people and I didn’t fit. I couldn’t relate and was quite confronted by it all. It was a lot to deal with as a young person and I was quite distressed. I felt anger, frustration, isolation and fear. I couldn’t talk to my parents as they were overwhelmed with guilt and sadness, and while they tried, my friends couldn’t really understand. The shock of being diagnosed soon gave way to denial. Total vision loss seemed so far away and so I ignored it for a while and went back to being ‘normal’.
But after a while, things started to get harder. I struggled with social interactions and had to give up driving. I found it difficult and embarrassing to ask for help. I hated asking people to give me a lift and help me to get to the toilet. I would have panic attacks and get anxious in unfamiliar places or when I wasn’t sure how to get home. On several occasions, I cried far more tears at parties than was socially acceptable.
Eventually I sought out counselling to help me cope with the grief and loss. This helped immensely. And while I got myself back on track, I didn’t realise it would be an ongoing process. Having a degenerative condition means that your period of adjustment is continual. It is normal to experience highs and lows depending on life circumstance and it can often take time to adjust.
Emotions attached to deteriorating vision loss, however gradual, can sneak up on you when you least expect it. You may go to do something you have always done and be shocked that you can no longer see something. You may go for a check-up and be told you have lost several more degrees than you had expected. Some of the most difficult times can be when you reach important life stages or big decisions such as pursuing a relationship, starting a family, changing jobs or buying a house. These times were always incredibly stressful for me as I felt the added weight, complication and uncertainty of my vision loss. Yet, it has all turned out OK so far.
In my 20's I was terrified of losing my friends or not finding anyone to love me. In my 30's I struggled with the decision of whether I wanted to have children, and if I did, I wondered if it was ethical? I worried about my career and what my future earning capacity would be like. Could I manage a mortgage if I was blind? These are all big things for anyone to deal with and in hindsight I wished I had been more open and talked about them. Instead I struggled through several bouts of depression feeling totally isolated and hopeless.
Sunny side of the street
In those dark moments I couldn’t even begin to imagine the life I have now. I didn’t think it was ever going to be possible. I am now in my 40’s with a wonderful life partner, supportive family and incredible network of friends. I am university educated, well-travelled and gainfully employed.
It has taken me a very long time to acknowledge that things are better and more manageable if I put my pride and shame aside and ask for help. It hasn’t meant trading my independence, rather just accepting that there is often more than one way to do something. Over the years I have learned to adapt and cope through discovering things I enjoyed doing. I love music and so took up partner dancing for fun. I learned to use touch and musicality to be a better follower. While some of my partners still cop a few elbows to the face, I am certainly getting better.
Tickle my funny bone
I have often travelled places by myself which is a significant challenge, a great accomplishment and lends itself to a few funny stories. Like the time I stepped off the edge of the Inca trail in Macchu Picchu, fell off the stage dancing burlesque in London, got on the wrong bus in Mexico, got lost in a blizzard in Nepal and hugged strangers thinking they were my relatives. I try to keep a sense of humour about my life and experiences and it has become my way of surviving.
Another strategy was working hard to develop a good social network and learn how to talk about the challenges. My friends have been my saviours, and despite my fears none have dumped me for being a drag. Some years ago, I was lucky enough to be one of Australia’s Youth Ambassadors and attended the Retina International Congress in Germany. I met young people from all over the world and we looked at ways to support others just like us. Sharing stories, connecting and being inspired by what other people are doing literally changed my life.
While being handed the diagnosis of eventual blindness can feel like Armageddon sometimes, it generally isn’t. The world is full of burgeoning technology, advances in science, incredible opportunities and adventures to be a part of. Sure, it will be difficult and frustrating at times. But you can get through it, and live the most fulfilling life you can imagine.
My top tips for managing vision loss
• Be kind to yourself and others. Don’t be afraid to be vulnerable and open about how you are feeling.
• Reach out for help when you need it. Help others. You might surprise yourself with what you can do.
• Connect with interesting people of all ages. It is one of the most effective ways of building up a support network you can draw on.
• Don’t put yourself down or ever think that you aren’t worthy of grabbing whatever dream you have in mind to accomplish.
• Fill your pockets with tips and tricks to make life easier for yourself. And don’t be afraid to use them. There are so many life hacks available and so there is no need to do it the hard way.
• Embrace what it means to be you. All of us are individuals yet we can feel immense pressure to conform to what is ‘normal’. Don’t get caught up in comparing yourself to others or trapped by what it is you think you ‘should’ be doing. Take the time to explore what means something to your growth, development and happiness.
Laugh. At yourself. At life. Find the humour and use it as a shield on those days that are tough.
I have autosomal dominant Retinitis Pigmentosa. My grandfather, father and brother also have it. However, until my father was diagnosed at 45 years of age, we didn’t realise that both he and my grandfather had it.
My father was still driving when he found out. He is now into 70’s, still independent and has useful vision of around 30%. He has two sons from his first marriage, one of whom was diagnosed with RP in his late 20s. My brother’s rate of vision loss has been faster and the proportion he can now see is probably around the same as our father. I also have a sister from my father’s second marriage. She and I are twins. She does not have RP.
I was diagnosed in my early 30’s following the birth of my first son. Seven years and three kids later, I have stopped driving of my own accord. Even though I can still legally drive, my blind spots are in the way and I don’t want to take any chances. I have what is called sectoral RP, so currently a few sectors on the sides of my vision are affected. The rest is still pretty good. My night vision is also ok. I have to be up during the night a lot for the baby and I manage mostly without turning on the lights, using only the small glow from the baby monitor.
I work in front of a computer a lot and usually struggle with glare, especially in the office where they have a very bright set up for the lights. I no longer go to concerts or movies as it’s very painful for my eyes. I don’t enjoy going to crowded spaces, the beach or swimming pool as I am not able to watch the children properly and I panic. I am socially active, running a few social groups in my area and am also in the school committee. It is frustrating to switch from being very independent to being reliant on other people, but this is something I have to get used to.
There aren’t too many people I can discuss my situation with as those who hear about it react in such a strange way. As if I’m an invalid, or don’t know what I’m talking about, or don’t try hard enough to cure myself. It is also harder to tell people that know me from my “normal” days. I find it is easier to reveal to people I meet now as they don’t know me in any other state. I have basically adjusted to the idea of having RP and have accepted it. It was not an easy path to take to acceptance but I have figured that out of everything else I could have received in terms of illnesses this is not the worst. It doesn’t hurt. I won’t die from it. Chances are pretty good that I will keep some useful vision over the years. I can still be independent.
I really hope for a treatment, if not a cure. It makes it easier to cope when I read about the progress of the clinical trials.
What advice would you give to your younger self?
Ask for help. I had too much pride in being independent.
Try to be comfortable with asking so you can get the support you need. Whether it’s a note taker for school or something to help with your employment just ask.
Having a degenerative condition means that things change over time and you need to learn to adjust along the way.
As an athlete I am constantly asking my teammates and guides for help. If you can be ok with asking for what you need at the time it can help you to feel more confident. I would also tell myself to be ok with having a disability. And be accepting of what might be coming.
What helped you cope and adjust?
When I was diagnosed, social media didn’t exist and it wasn’t as easy as it is now to find out about the support available. It was a big relief for me when one day I came across the blog of someone who had written about their experience of having a vision impairment. It was like they had described everything that was in my head. I showed my mum and sister and they cried.Things also really changed for me when I went to England and met with five other young people with eye disease as part of a small gathering of the Foundation for Fighting Blindnesss UK RP Community. It was there that I started to realise what I could do. It was a tipping point for me and the start of my life in many ways. I gained the confidence to talk about what it was like to have a disability. Speaking with them helped to break down the barriers in my own mind. It also helped me to feel like I had a place in the community and society more broadly.
How do you feel about your diagnosis?
Disability has spurred me to do things I never thought were possible. It is important to know and remember that whatever your diagnosis that the future is not that bad. I have gone on to do things I never thought I would like public speaking and being an elite athlete.
I have developed the confidence and the attitude to think about what I can do. Now I think “ Why can’t I do that?”. Once I got back into sport I realised anything was possible. I started to look out for opportunities and understood what my purpose and direction was. I never believed that I would find something to do that I was so passionate about. I went from drinking with my mates to setting my sights on the Paralympics.
What about relationships?
I have developed a great network of friends through the sports community and that has contributed to developing my confidence.
I didn’t think I would ever actually be able to have a ‘normal’ relationship with someone. However, it is possible. You may think that you can’t have them because you have a disability but that is not the case. You can have a house and kids and relationships just like everyone else.
My dad Arthur is a determined, fiercely independent, unassuming, kind and generous man. He has never been defined by his RP blindness nor seen it as a disability. Diagnosed in his early 20s, he’d just assumed he was clumsy. He did his National Service, worked in a bank, and then worked as a Chartered Surveyor until he was in his 60s. He didn’t stop driving himself until around 40 years old.
By now he probably has less than 3 degrees of vision. But he is fitter, and more active than most 88 year olds that I can think of. Last week he walked 2 ½ miles over the fields and stiles in Cheshire with my brother. Most days he walks ½ mile to the supermarket to buy the groceries on his own. In 2016 he travelled with my 84 year old stepmother to Russia. They spent three nights on the Trans Siberian Railway, stopping off along the way, and loving every minute. Recently he travelled alone across London on public transport to go to a funeral.
He only uses his cane when there are lots of pedestrians. He tells me when it is safe to cross the road.
My dad is my inspiration. He has never complained about his eyesight that I remember, and has never used it as an excuse not to do things. It may take him longer to accomplish what he wants to do, he may have many bruises on his shins, he may annoy people by bumping into them, but nothing has never stopped him. His tenacity and love of life is outstanding. I aspire to be half the person he is