I’ve agonized over writing this confession (for the lack of a better word) for months. I’ve decided just to come out with it.
Many of you know that I was born profoundly deaf and received a cochlear implant when I was six.
What many of you don’t know is that in the same year, I was diagnosed with Usher Syndrome, which is hearing loss coupled with retinitis pigmentosa (RP)(.[1] It’s the leading cause of deafblindness. (Insert Helen Keller jokes here.)
Yep. That means that I’m losing my sight in addition to having a major hearing loss.
Why the last year and half was the worst time in my life.
Two years ago, my RP wasn’t terrible. I had some peripheral vision losses, but I compensated quite well. It was more of an annoyance than an impairment. As strange as it may sound, I didn’t think about it much.
Then about a year and half ago, I developed a blurriness and distortion in my central vision. I went to various doctors and none of them could explain it. They had never seen anything like it before. I was so afraid that it was my RP taking a turn for the worse.
A top specialist in Boston confirmed my fears. The damage was irreversible and related to my RP, albeit an unusual development, but not unheard of. There was no going back to my old life.
The worst thing about it was that it happened suddenly and unexpectedly.
Over the course of eight months, my central vision went from 20/40 to 20/200, uncorrectable with lenses. I can’t do a lot of things that I could do even a year ago. I can’t read regular print. I can’t see distance almost at all. All straight lines look wavy to me. I had to give up watching movies because I can’t read the captions fast enough.
Usher Syndrome is very unpredictable. Some people have good central vision well into their fifties. Others lose nearly all of their hearing and sight by thirty. Perhaps I was foolish and naive, but I was always convinced that I’d be one of the luckier ones, that I’d keep my sight well into my forties. I was wrong.
How does it feel to be losing your sight as a deaf person?
It’s really, really complicated and devastating, especially as a deaf person. It’s taking away something that you depend on the most in your every day life.
On one hand, it requires a tremendous change to your lifestyle. Now that I can’t read regular print or navigate the world as easily, I had to put on my big girl pants and adapt. I’m learning braille and how to use a white cane. I interact with objects differently now. Since my vision is unreliable, touch has become my most dependable sense. That, of course, means that my world is shrinking into the space that I can reach.
On the other hand, it also changes your identity, inside and outside. People look at and treat you differently when you have a white cane. To throw the hearing impairment into the mix only makes things worse. People don’t think that I can do anything anymore, even think. It doesn’t seem to matter what I’ve done before in life. To many people, I’m at best, a pity case, and at worst, a drain on society. Quite frankly, it’s a depressing feeling.
Perceptions aside, I can feel myself changing, both for the better and for the worse. Now, I’m more attuned to the sight that I still have and noticing more. I may not see much, but I treasure what I do see. In many ways, I’m more sanguine about a lot of things because when your choices dwindle down to single digits, you learn how to stop bitching and moaning about the little things (well, mostly).
The changes, however, aren’t all positive. As my world recedes into touches, smells and tastes, I can feel myself withdrawing. It’s becoming harder and harder for me to go out and socialize with people who don’t know what to do with me. I’m already a natural introvert and I’m truly scared that it’ll turn me into a hermit. Despite (and perhaps because of it) my introversion, I love interacting with people.
Why am I telling you this?
On the Internet, I could pretend to be sighted and hearing, so why am I not doing that? It’d be easier in many ways.
I’m not doing this for pity. In fact, I’d rather you not share your pity. I don’t need or want it.
I’ve kept my condition a secret for a long time. I saw it as a weakness, something that I needed to hide. In a demented way, I wanted to defeat, beat it somehow.
I’m sick of the shame and secrecy. Only now do I realize that it’s not something that I need to beat (or is even possible). It just is. It’s a part of me now, like it or not.
I promised myself a long time ago that when it happened, I would deal with it. I feel like the best way for me to come to terms with it completely and wholly (I’m almost there, I think) is to be as open and honest about it as possible.
I’m hoping that by being open and honest, I can reconnect with the world and prevent myself from closing wholly into myself. If I can give others insight into my experiences, it’d be reward enough for this admittedly painful time in my life.
So, if you have any questions, consider me open for business (unfortunately, I can’t provide 24-hour service).
P.S. From the bottom of my heart, I apologize for not telling you sooner. I felt terrible about it, deceptive, even.
First published on Quora here: http://www.quora.com/Cristina-Hartmann/Posts/A-Confession-of-Sort
Christina Hartmann is a writer who decided to give up a law career for the unexplored waters of writing. She strings words together and hopes for the best, saying: “Time will tell whether I’m an idiot or not.” She is currently finishing up the final touches on her first novel (The Secret Value of Zero), a YA sci-fi novel about genetic engineering and utilitarianism. You can follow her on Twitter as @cmmhartmann.
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Andrea Arnett
April 10, 2013
I admire Christina for her decision to tell us about her Usher Syndrome. I was not aware that Usher is the leading cause of deafblindness. I had assumed it was Rubella, which caused my partial blindness. It must be harrowing to face a decline in the senses like this. I am fortunate in that my vision has not altered really since childhood, and I am now 53. My close vision is deteriorating, making it difficult to read fine print, but this might be due to ageing. My distance vision is very stable. I have nystagmus also. Meniere’s disease has taken away my hearing in my right ear but I can still cope well in everyday situations. People are usually surprised when they find out about the extent of my vision and hearing loss. I hope Christina will have the courage to continue living in the everyday world and stay a part of the community. The internet is a great leveler – we are out there in cyberspace and need not be judged on our disabilities, colour, gender, looks etc, as it should be in real life. Supportive friends are great to have, whether in person or online. My mother was a founder of the Victorian Deaf blind and Rubella Children’s Association, which is called Able Australia these days, as back in 1958 it was difficult to get support and sharing with other parents was a great help.
Paul Redfern
April 10, 2013
Wow – that took some guts! Far from pitying you, I felt admiration (and not in a patronising way either) to say: “Look – this is how it is for me”. And I learned something.
I just hope there are enough of us who will treat you with some respect and not assume that you have suddenly become helpless and incapable of anything.
Em Aris
April 11, 2013
What an eloquent and honest explanation of your journey so far. You show that you are one who has the spirit of an optimist and optimists adapt to their circumstances in positive and creative ways as you will. ‘Thank you for sharing’ as they say.
innocentsofoppression
April 11, 2013
Christina – you are NOT alone here.
I went through a similar experience as you did – my vision took a sharp dip 15 years ago and I was forced to retire from work at the age of 31, but I still continue to write books and write/direct short films for television. I just don’t let my Usher stop me from what I love doing.
You may be interested in UsherLife, a social networking and resource website that I founded and coordinate. It’s a highly valuable place for Ushers to go to virtually as well as socially. Visit http://www.usherlife.co.uk and there is a Facebook group (if you are in Facebook) which has over 300 members from all of the UK and around the world – it’s the premier social netowkring group for Ushers of all types. You are most welcome to join us and we’re a real nice bunch of Ushers that shares news, knowledge and experience. UsherLife is run completely voluntary by me and a few others.
Very good article, I will post the link into the FB group for others to comment.
Nick
Carrie Ann Golden
October 18, 2013
I also have Usher Syndrome…all I can say is Thank You! Thank you for sharing this with us. I don’t feel quite so alone now 🙂
Dan Brinkman
November 22, 2013
Good post, thanks for writing about your experiences with Usher Syndrome, Christina.
As a man with Usher Syndrome, Type II, I too have challenges to overcome (limited hearing, no night vision, peripheral vision issues, at times social awkwardness stemming from these sensory limitations, fear for the future) and am comforted to know that I am not alone. I suspect there are lot more people out there who have for one reason or another tried to hide their condition, and hope they will likewise find the courage to let others know of their struggles, challenges, and at times their joys.
A lot of my career aspirations and life goals have been derailed in the wake of my diagnosis but I am still finding ways to enjoy life, new mountains to climb, so to speak. Reading your post makes me want to speak with others, to network more, to help others, and be helped by others, with the burden of hearing and vision impairment. God bless you for your courage.
Dan
vwgirl1978
December 6, 2013
Thank you for sharing. My son is 14 and was just diagnosed with Ushers and already is in the most severe stages of it. He was so well compensated that he fooled everyone to think he had perfect vision including the doctors. This has bee tough on everyone except him because he still feels like he sees normal. I want him to get in touch with others in similar situations. I wish there was something I could do to fix it or make it better. I’m praying for some type of technology that will help bring it back, similar to cochlear implants.
Lewis Campbell Jr.
June 14, 2016
I received a comment from Christina earlier today, that I had made an error in responding to her rather than to the person she was commenting on. She writes so well, that I was interested in reading more of what she writes. I was enjoying the list and her book on Amazon. Though I just started reading it, its well worth the time, just for the lyrical way she writes.
I didn’t really think much about it, not knowing anything about her. Coming across one of the many men and woman I read about or talk with every week, together helping each other gain a bit of wisdom, a bit of strength to get through our days at times.
We all confront challenges, some worse than others. I suppose as a avid book reader and budding writer like Christine her story resonates a bit more for me, as losing some of my own hearing and having lost the perfect sight I used to have from my injuries has made the tale a bit more poignant I suppose than it might have been even a couple of years ago.
We can sympathize with others who have problems, physical or otherwise. Some will be flippant and say…”it could be worse, she still has her mind” True, and a strong healthy mind it is, yet it does not speak to the emotions she and others go through in the transition of the loss. Myself included.
I think if I had a conversation with Christine, I would like to say as I did with my daughter; when she was four and having some major health problems at the time, that the challenges we face are common to us all. If its not one thing at one time, its another at another time. And yes my four year old daughter looked at me and said “huh.”
Smiling as she did. She knew I tend to think out loud and work through my thoughts, and learned to ignore most of what I said until I was able to say to her at her level what I needed to say. It’s was the last conversation I ever had with her; the way she was then, the way she is now. She’s in her twenties now, and mentally the same happy four year old mind she had back then. Learned to walk, though talking seems to be something she will never do. Yet her manners, her way of smiling, she’s still there, regardless the bodies damage that makes it difficult at times to connect with us.
I laughed at the time; knowing I study and think over a problem until it hits me in the face with the reality of its existence. In essence the words I gave her were those words people in our lives who love us always give. That we are here for you, even if it’s just a good word for the day, a good thought, a “keep working at it” in the marathon’s of our days when things get a bit more tough than normal. Whatever normal we find ourselves in at any given moment.
I loved Christine’s story of her Father’s passing, yet now I see in her words here, the same type of strength she showed as her “Father’s Daughter.”
Thanks for sharing