My Deaf/Blind Story (Usher Syndrome)

This is just a breakdown of my hearing and vision loss. Not a story about my entire life, I promise.

Hearing loss

I was born with a hearing-impairment. Fortunately, my parents weren't willing to just let some doctors pat them on the head, tell them they were imagining things as young parents and send them on their way. Instead, they requested a hearing test when I was just a baby. They discovered I have a moderate to severe hearing loss in various frequencies. In other words, in low tones (think bass or fog horns) I'm only mildly affected. High tones (speech, whistles, or squeaks) I am severely to profoundly hearing-impaired. I started wearing hearing-aids when I was just under a year old, and was fitted for my second one around three years old. I've been wearing hearing-aids since. Please keep in mind that at the time I was born, it was recommended for children with my level of hearing-impairment be sent to a deaf school full-time. As in, live in this school, when I was just a kid. My parents bucked the trend, ignored the doctor who told them I would never amount to much because I would never be able to talk well, would never do well in school, would need special care my entire life, blah blah blah. My mom thoughtfully sent him a copy of my straight-A report card (from a public school in a regular classroom). Go mom & dad:-] I owe quite a bit to my parents already just for what they had done up to this point. But then they worked hard, paid for many years of private speech lessons and worked with me at home constantly. They are incredibly loving and were determined to give me the best life possible by giving me the tools to succeed in the real world. They never told me I couldn't do anything because of my hearing loss, so I never knew I shouldn't be able to do things. I had no idea it was supposed to slow me down. Oops. Instead, I went to public school, had many friends, played in different sports, worked hard, earned scholarships to put myself through college, was married, worked as a paralegal, had a kid, got divorced, moved halfway across the country, and did pretty much anything else I wanted/needed to do. I still speak well, though I do have to be careful with my enunciation at times. When I'm tired its especially pronounced, but most people have no idea I'm hearing-impaired. I don't really care if they know or not as I don't hide it at all, but it is just one part of me.

Vision loss

My vision was perfect as a child. No one had any idea that I had this disease, instead they all thought I just had an unusual form of hearing loss. When I was around nine or ten years old, I noticed it was harder to see at night. Not enough to be a huge difference, but enough that I can remember it now as an adult looking back in time. As I got older, I found it getting harder and harder to see at night and became even clumsier than normal (believe me, that's saying something. Grace is NOT my middle name. Ahem. ) During my fourth grade year, it was discovered that I, just like the rest of my family, was very near-sighted. Enter the awful, ugly, brown, bifocal glasses. In fourth grade!! Again, thanks mom & dad. Naw, I'm not scarred from that...why do you ask? During a routine annual visit to the optometrist when I was 15 years old, my mom mentioned to the doctor my complaints of not seeing well at night and my clumsiness. He examined me, sat back and said, “Hunh, I've read about this before. I'll be right back!” He came back into the room with a book and showed it to my mom. Then he referred us to an eye specialist up in St. Louis. Hmm. I didn't really know what was going on, and I think my parents were in shock. After some crazy testing (remind me to tell you about the tests sometime, if you're interested. Very gnarly.) at the eye specialist’s office, the specialist sat us down and informed us I had a very rare disease called Retinitis Pigmentosa (RP), the eye disease portion of Usher Syndrome. He told me I was likely to go completely blind someday. He then pulled out a pair of hideously ugly sunglasses and told me the only thing they knew was that sunlight and glare was painful for people with my eye condition so I should be sure to wear wraparound sunglasses all the time while outside. Um. What??

I found out that I needed a thousand times more light than the average individual to see in low light or at night. I also found out that I had small blind spots in my lower peripheral vision. Ahhh, that explains why I kept taking out my poor younger siblings when I walked across the living room. Fortunately, I was still cleared to learn to drive because it had not impacted my regular vision yet. I went on to get my drivers' license and was able to drive safely for about ten years. I did start to notice little things that were harder for me, but for the most part it wasn't a drastic change in my day to day life yet. During my junior year of college, I was finally restricted from driving at night but was still cleared to drive during the day. While driving, I went annually to have my peripheral field checked. I refused to ever be responsible for causing an accident just because I didn't want someone to tell me to stop driving. I'm not going to lie, the day I found out I had to stop driving sucked. I still remember it vividly, and I still miss driving. I would love to just jump into a car right now and go for a long drive. Hell, I'd love to just drive myself to the grocery store. I miss just running simple errands. However, it is what it is and I just have to move on. I've accepted a lot out of necessity, but I'm not going to ever lie to you and say that I enjoy not being able to drive. It sucks. There, how's that for honest?

When I was in my mid-20's, I discovered I had cataracts in both eyes. I had them removed in December of 2005. Now that's an interesting experience...I didn't have mine removed the normal way, instead they used nerve blocks to protect my remaining healthy cells. Hello drool city. Really, not one of my more attractive moments. Nothing like having your doctor come up to you in the recovery room excited to introduce you to a fellow doctor because you're a unique case, and you can't feel the side of your face. I was shaking his hand with my right hand and holding up my cheek with my left hand:-]

As I went through my 20's, more of my peripheral field and my night vision went. I learned how to use a white cane, aka blind cane but only really needed it at night and in large crowds. Then, all of a sudden, I lost an enormous chunk of my remaining vision within a very short period of time. I went from being able to adapt and it not really affecting my daily life, to it being an enormous pain in the ass within a short time period. By this point, I was living in an apartment in downtown Tampa, Florida, had just had my baby girl, and was suddenly a single parent.  I was taking my kiddo to work with me full-time while working full-time (amazing boss, thank you Mardi!). Yeah, stress might have played a tiny part in it. Ahem. I eventually moved back to Missouri, lived with my parents for eight months, regrouped, then moved into a little house with my little girl. We lived in that little house for six years, during which time I gradually started using my cane full-time. Last year, September of 2014, I bought my first home (rented up to now) and we now live in a little home within walking distance of my tadpole's school. I am down to about five degrees of usable peripheral field at the moment, and it has remained somewhat steady for the past couple of years. Translation: I have tunnel vision. If you were to wear a mardi gras mask or hold up your hands shaped like c's to your eyes you might get an idea of what its like.  Its really hard to describe, actually.  I'm also considered to be completely nightblind at this point.  My color vision acuity has become affected as well, so I struggle with differentiating between purple and brown or pink and orange, etc.  Side note: don't ever ask a kid you just grounded to help you figure out which socks are black or blue.  Just saying. 

I am hoping I won't progress too much further, but while I hope for the best I'm preparing for the worst. Please don't think I'm saying losing all of my vision would be the end of the world, it wouldn't. However, I don't want to be caught unprepared either. I took a correspondence course in beginner braille, and while I am taking a break from it right now, I hope to get back into it at some point. Even if I never need it, it would be helpful to give my eyes a break at times, and it has already come in handy in the elevator or dark restaurants. You ever notice the restroom signs have braille on them? That's a good thing, trust me:-]

I'm extremely fortunate because in my type of Usher Syndrome the hearing loss remains steady. In the other types, people lose both their hearing and their vision. I struggle at times, but its nice to be able to rely on my hearing when possible.

I'm not sure how to talk about my hearing and vision loss without sounding either overly clinical or overly personal. I hope I struck a balance today. Regardless, this is the story about my hearing and vision loss, not the entire story about me. You'll just have to get to know me over time to see that, while this affects me enormously, it doesn't completely define me. Just happens to be a facet of my life. I did oversimplify some areas. If you want to know more or want a clarification, just ask me:-] Thanks for reading!