“You know, we sometimes forget you’re blind,” my grandmother matter-of-factly stated in response to a situation that inevitably happens between me and every sighted person I spend any amount of time with. The exact event varies, but always involves me not seeing something that is right in front of me. In this particular instance she had left me a cup of coffee on the counter, which I only discovered after nearly knocking it into the sink when I set a second cup by it to reach for the sugar bowl. I must have looked a bit dumbfounded because she continued: “You get around so well most of the time we just don’t think about it.”
I remember it gave us both a good laugh, but for a good part of my life that rather mundane exchange with my grandmother would have bothered me because it seemed like I constantly had to defend my own inability to see.1 This was especially difficult when I was younger because I’ve always seen the way that I do and so I had nothing to compare it to. I knew I was colorblind, but it held as much meaning to me as being legally blind. I once heard my father tell someone that my vision was like watching “an old black-and-white television.” It was the first time that people seemed appeased by a description of my blindness and so for the longest time it was the explanation I used. Until I actually watched an old black-and-white television and realized this description was wholly inaccurate. I could tell the difference between what I was seeing and how things appeared on the television screen where the world was portrayed as monotone and I found it hard to distinguish things like clothing and furniture from the background. Whereas in reality things were vibrant and full of contrast. Sometime after I had a discussion with my father about this and, at least for me, it was the moment of realization that there was possibly no way sighted people would truly understand how I saw things.
I was born with the retinal condition achromatopsia. You probably have never heard of it outside of the few mentions on this blog because it is incredibly rare, affecting roughly 1 in 33,000 people.2 Nowadays there are genetic tests that can aid in diagnosing achromatopsia, but when I was younger the tests were far more complicated. There were signs, of course. As an infant the main one was that I had nystagmus, which is an involuntary movement of the eye and is a common sign of vision loss in children. However, it can occur all on its own and it can even improve with age. My parents were predictably freaked out about it, but their fears were basically brushed aside by doctors. I can’t really fault them on this because these were the same doctors that told my parents my allergy to cow’s milk was a heat rash and so mostly I’m glad my parents didn’t succeed in freezing me to death before that was cleared up.3
Anyway, the years passed and as my father put rather succinctly, “We just thought you were clumsy.” He explained that I could “obviously see stuff” but then I seemed to deliberately walk into the coffee table on almost a daily basis. In his defense, he did cart me around to pretty much every doctor on the planet, though, to this day I’m still not sure if it was my increasingly quirky behavior that spurred him on or his lack of faith in military doctors after the heat rash debacle.
It was through this that I became familiar with colorblindness tests like the example of the Ishihara Color Test to the left. I can only assume that lack of understanding about achromatopsia contributed to how often these color perception tests were administered. Otherwise, someone probably would have bothered to explain to me that as I possess absolutely no ability to perceive color it was literally impossible for me to see anything beyond that first plate’s number 12.4 Instead I perused these circles with a same devotion many give to Magic Eye images.5 I forget exactly how I came to understand that I was completely colorblind, but I can assure you that it had nothing to do with these tests.
The other main feature of achromatopsia is that it causes severe light sensitivity. The type of achromatopsia I have is classified as “complete rod monochromacy” and means that the only functioning part of my retina are the rods. Whereas cones function best in bright light, rods saturate at higher light levels. It’s basically the concept of being dazzled by high-beams, except that in my case I don’t have cones to pick up on the visual slack and so instead I’m completely blinded by light. You know how most people loathe going to the dentist? Well that’s basically how I feel about the eye doctor. Part of this is because having my eyes dilated is basically like taking the entire sun and shoving it straight into my eyeball and it seems to take about twice the normal amount of time for the solution’s effects to wear off. But mostly it’s because I have very distinct memories of being subjected to electroretinogram tests. ERGs test the eye’s reaction to natural stimuli, which sounds innocuous when you put it like that. In practice it was more traumatizing and involved sticking me in a dark box and having lights shined straight into my eye for an hour. Really puts that teeth cleaning into perspective now, doesn’t it?
My childhood nightmares aside6 there is one last defining characteristic of achromatopsia: it is incredibly stable. In fact it is so stable that aside from a change to bifocals at about ten, I’m wearing nearly the exact same prescription in my eyeglasses right this moment as I did in the first pair I was given at four. My eye doctor finds this one of the most fascinating things about achromatopsia. Second only to the fact that the retina of an achromat does not appear any different than that of a fully-functioning one. He and I have very different feelings on what is fascinating, but then I wasn’t the one who went to college so I could look in people’s eyes all day.
What’s more, as I’ve detailed before, if I’m in an optimal light situation I can read regular print without the aid of a magnifier. Much like the coffee table, this has caused no small amount of confusion for people throughout my life. It’s difficult to understand unless you fully grasp the anatomy of the eye. In the simplest terms, it’s basically cones that handle distance vision and give clarity and crispness to your sight. Without them, there isn’t so much an absence of these but a substantial drop in both. Meaning that things I can see are in sharp detail, but they need to be relatively — sometimes extremely — close. A person with 20/20 vision looking out at the horizon on a clear day can see for quite a ways, but at some point things start to grow indistinct and they can’t quite make them out. In my case, that line where things grow indistinct is very dependent on the amount of light, but sometimes it’s hardly six inches away. Which is why trying to explain what I can see in terms of visual acuity is like describing a cell phone to Alexander Graham Bell. It sort of gets the point across, but it’s not really the same thing at all.
Recent events have sparked a lot of chatter about the different viewpoints between people who are born blind and those who lose their sight later in life. It’s a topic I’ve given a lot of thought to over the years and actually wrote a paper on for a Psychology class in college. In a lot of ways the two are almost different disabilities because of the different psychological effects they present.7 Where this comes into the sharpest relief for me is in understanding color perception. Being able to perceive color is an ability, but actually identifying colors is something that is taught to you. Our understanding of this has grown lately due to scientific studies that prove animals such as dogs, cats and birds can perceive color and can be taught to appreciate them. Learning colors is one of the very first things we teach our children and because of that knowledge a person with color perception would consider my vision an absence of color, but it’s really not. Unfortunately, there’s no way to really bridge this gap because you can’t really unlearn colors and my understanding of vision would change drastically were I suddenly able to perceive them. When I think about it I can understand how frustrated people in my life must have been in trying to understand my vision. Me? I find the whole thing thoroughly fascinating.
1. Though it’s more accurate to say that my elementary through high school years were spent feeling disgusted and ashamed of being blind. But that’s a whole other post.
2. It should be noted that statistics on achromatopsia have not been updated since the 1960s, but using the United States July 2011 census results that works out to less than 9,500 people in a country of an estimated 21.5 million people with “trouble seeing.” I’ve been seeing the same eye doctor, a low vision specialist, since I was four and he’s only ever had one other achromatopsia patient. And aside from people I’ve met via the Internet and through a support group (that was more accurately a list of pen pals), I’ve met one other achromat in my life and it was at a school for the blind.
3. I’m really not kidding about the freezing me to death thing because they had me naked in my crib with fans blowing on me and the thermostat set to 55°. I ended up being properly diagnosed on the fly by a pediatrician on a bus who took one glance at me in my mother’s arms and bluntly told my parents: “That baby’s allergic to milk.”
4. The first plate is a control plate that everyone should see regardless of the level/type of color blindness. I can kind of see the 15 in the sixth plate, but only because I know that it’s supposed to be a 15. The other plates are 8, 29, 5, and 3. If you have red-green color blindness you’ll see different numbers for those five plates.
5. No, I can’t see those either.
6. ERGs and colorblindness tests featured heavily in a recurring nightmare I had as a child. Honestly, I try not to think about it.
7. It’s worth noting that there is something of a controversy over this. Essentially it boils down to the two being different because one is a “worse” type of blindness. Yeah, I don’t understand it either.