Cristina Hartmann: A confession of sorts (on having Usher Syndrome)

Posted on April 10, 2013

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:

Screen shot 2013-04-09 at 19.00.59Christina 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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