Am I? Legally Blind?
Well, no, not yet technically, but there is a possibility of being blind later in life. I know a lot of older people begin to lose their vision due to age but I not only have a higher chance of going blind, it could be a lot sooner rather than later.
What is it?
Usher’s Syndrome according to Genetics Home Reference, “is a condition characterized by partial or total hearing loss and vision loss that worsens over time. The hearing loss is classified as sensorineural, which means that it is caused by abnormalities of the inner ear. The loss of vision is caused by an eye disease called retinitis pigmentosa (RP), which affects the layer of light-sensitive tissue at the back of the eye (the retina). Vision loss occurs as the light-sensing cells of the retina gradually deteriorate. Night vision loss begins first, followed by blind spots that develop in the side (peripheral) vision. Over time, these blind spots enlarge and merge to produce tunnel vision. In some cases, vision is further impaired by clouding of the lens of the eye (cataracts). However, many people with retinitis pigmentosa retain some central vision throughout their lives.”
There are different types of Usher’s Syndrome and different gene mutations associated with each. I won’t go into each type here because that would take a long time to go through, but you can go to Genetics Home Reference, linked above, and I’m sure they will have more information on it.
How Did I Find Out?
It usually starts in the late teens or early adult years. For me, it started around my senior year in high school, 2010. I didn’t know this until four years later though. I was playing intramural sports at NAU with some friends and I was starting to notice that I was having a hard time seeing at night. Usually, the fields I had played on were well lit, but at NAU, the fields they used weren’t lit well enough for me. So I decided to see someone about it.
I saw a retinal specialist in Phoenix, Arizona. After two hours of testing and trying different things, he ultimately came out and said, “You have Usher’s Syndrome, there’s no cure, and you will be going blind.” As you can imagine, I didn’t take the news lightly. I was angry. I was angry at how the doctor described it so bluntly, I was angry with life, and I was just in denial.
Obviously, I went to another doctor for a second opinion. He had a much nicer bedside manner and wasn’t so blunt. In fact, he gave me a sense of hope. Not that I wouldn’t have anything, but that I’d be okay, no matter the diagnosis. December of 2018, literally three weeks ago, I got the results of a genetic testing that was done. They can confirm with partial certainty that I have Usher’s Syndrome, but they can’t give me any more information than that.
Here’s why, generally, geneticists try to find at minimum two genetic markers that confirm a condition. Each marker explains a little bit more about the situation than others. For example, the gene mutation they found in me, is an Usher’s Syndrome related marker, but they could not find the second. The one they found explains that I have Usher’s but they couldn’t find the mutation that says whether it will be fast or slow. So now I know I have Usher’s but I don’t know if and/or when it will ultimately get worse.
My family and I have known about my hearing impairment since I was three, but since I never had issues seeing except to read, doctors never thought to have me tested for anything else.
What Can I See?
Well, this answer ultimately depends on the situation! Am I inside or outside? Is it day time or nighttime? How much lighting is there where I am? Many different factors affect how well I can see at any given moment. Let’s start with day time. Where I work, I am constantly in and out of meetings with professors, department chairs, and others. Let’s assume that you’re all sitting at a round table that seats eight people. So the person sitting directly across from you is maybe five or six feet away, or two meters. IF that person is talking and I am looking at them, I can see their face but everything an inch or two away from the face is blurry. Not dark, just so blurry that I can’t really see unless I look in the spot. What I see is where my central vision is looking. Refer to the picture below.
The picture shows the degree field of vision that is still very clear to me. I can read, write, and get around. Instead of the rest of my vision being non-existent or dark, it is just very, very blurry. For example, looking at my computer screen to write this, I cannot see the sides of the desk I’m writing on and it’s a really small desk.
At night, my vision is worse obviously. Refer back to the picture above, that is very realistic representation of what I see at night, minus the faces. If the lighting is poor and any visible light is behind a person that’s standing in front of me. I won’t see their face. Getting around at night in crowded places, with poor light is so stressful for me that I would rather a person to guide me or to avoid the situation all together. I have used a cane, but those really only work best during the day and when you know the area you’re in. If you are in an unfamiliar place, it can be scary and stressful because you have to strain your eyes and focus really hard on where you are, that you miss everything around you. With good lighting and in an area that’s not crowded, I can generally get around on my own.
That is the basic idea of what my vision is like. It’s not pretty and at times it’s very upsetting, but it’s part of my life. Although, I know this is coming, it’s not here just yet and that means I’m going to continue working towards my goals and do my best to be happy with whatever comes. 🙂