Tag: Best’s disease

In my ongoing efforts to document and communicate my experiences with a degenerative eye disease, I want to add to the list I started in my last blog post.

My Quirky Vision, a list continued

• When I go to pick up my daughter from basketball practice, even when I’m wearing glasses, I can’t pick her out from across the gym.
• Watching my daughter’s basketball games and my son’s swim meets is very difficult and exhausting. I can follow most of it, but it’s all a bit blurry.
• In general, I avoid eye contact with people; I’m afraid that I should recognize them, but I won’t be able to. I feel awkward looking at them too long, trying to figure it out. Of course, this awkwardness is exacerbated by my introversion.
• I also avoid eye contact because looking directly at people’s faces is difficult. They’re out of focus, which tires my brain as it tries to make sense of what I’m seeing.

Words like “fuzzy” or “blurry” or “out of focus” don’t seem to quite capture what my vision feels like to me. I should read some more accounts of other people’s vision problems. It might help me to express my own experiences more effectively.

This weekend I came across a blog about running and low vision. The author/blogger has Stargardt’s, which is similar but a bit more extreme than Best’s, which is what I have. In 2012, as part of an effort to educate others on her experiences as legally blind, she tweeted about her blind moments or, her “brief, or sometimes not so brief, lapse, of sight,” using #blindmoments. She also wrote about it on her blog: Blind Moments

Her effort to document these moments has inspired me to try and document my own quirky vision moments*. I’m starting a list in this post. When I can figure out where I want to put it, I’ll turn it into a page.

*After reading through the below list, I’m not sure if these count as “moments.” Maybe quirky vision examples?

List! My Quirky Vision

• I can still read out loud, but it takes a lot longer and I often trip over words.
• Reading cursive ranges from difficult to nearly impossible.
• I can hardly (if at all) see anything that has low contrast.
• I can rarely catch a ball.
• I don’t always see the cursor and can spend several frustrating (and frantic) minutes searching for it on the computer screen.
• I usually severely under fill glasses because I can’t see the top of the liquid filling up in the glass. Or, I overfill and spill.
• When I’m waiting at a stoplight, I sometimes lose sight of the light, even when I’m trying to keep focused on it.
• Quite often, I can’t read the credits for a tv show, especially credits that move very fast.
• I miss out on a lot of what is going on in tv shows because of how fast the action is happening.
• I rarely get sight gags in tv shows (like “modern family”) because I don’t see them.
• It takes a tremendous effort for me to read big letters, like the white ones in this image:

In fact, to read these letters, I had to cup my hands over the top of my eyes and very slowly read the words. It was very difficult.

Recently I was diagnosed with Best Disease, or vitelliform macular degeneration. Named after the doctor who first presented on it, Dr. Friedrich Best, it’s a very rare eye disease that affects your central vision. Very little is known about it and there’s no cure. It’s hereditary and dominant but its effects vary so widely, from hardly any impact on your vision to becoming legally blind, that most families are unaware that they are carrying it or that they have a 50% chance of passing it on to their kids.

For me, so far at age 42, the effects are manageable and minimal, but very inconsistent. Overall, I can still see fairly well. Before my diagnosis, I didn’t even wear glasses, except for when I’m driving. I will now. Bifocals, in fact. But, everything is always a little blurry. Sometimes I can see things very clearly. Other times, I can’t see things if they first appear in my central field of vision. I don’t mean that they’re too fuzzy or too hard to see. I mean, they just aren’t there. To me, they don’t exist. I have difficulty focusing on images or seeing things when there is too little contrast or not enough light. I fail colorblind tests. I can’t track the cursor on the computer screen. I have trouble making out the details in people’s faces. And I can still read, but it’s much harder to read out loud without hesitating over words.

Having Best disease kind of sucks, but not really. I’ve already been adjusting to limited vision for at least 20 years now, so it’s a big relief to finally know why bikers who I hadn’t seen even though I was very carefully looking would suddenly appear in my peripheral vision or why I would become so overwhelmed in the grocery store trying to find that one item on the shelf or why I freaked out when I had to try and read the menu board at a fast food place or why I would hate driving to new houses because I could never see the house numbers or street signs as quickly as I needed to or why images on a billboard just didn’t make sense as images or why I never seemed to be able to recognize the faces of people that I had encountered regularly, but might not talk to that often. I wasn’t losing my mind. I didn’t have some serious neurological problem. I just had a freaky eye thing, where the cones and rods in my central vision resemble scrambled eggs and everything looks just a little fuzzy.

Yes, Best disease is a big deal. Yes, there is no cure. Yes, it will most likely mean that one day I won’t be able to read without a magnifying glass or that I might not be able to see people’s faces because my central vision is reduced to a gray, blank, fuzziness or that I could trip and fall on the stairs because I can’t see the step. And it has already meant that I didn’t swim in my first ever open water swim race, the one that I signed up for way back in January and was very excited about doing but had to gut-wrenchingly drop out of at the last minute because there was too much fog and I wasn’t sure that I’d be able to see the buoys to stay on course, which were green and I probably wouldn’t have seen them even if was a perfectly clear day because Best in some ways mimics red-green deficiency so I have a lot of trouble seeing green.

But, Best disease isn’t that bad and it has it’s benefits. At least for me. So, I’m starting a list, one that I hope to add onto as I encounter new reasons, for why Best disease is the best disease.

Here’s a reason that I discovered last Friday when my daughter came home form school and told me that she couldn’t eat the sandwich I had made her because it had mold on it:

I don’t have to make my daughter’s lunch for school anymore!

Because of my quirky vision, I couldn’t see the mold on the bread. And I might not see it again. While she was impressively relaxed about it, moldy sandwiches will not work for my daughter, who is a self-proclaimed “germaphobe”. Since she knows that I can’t guarantee that I will see that mold, I’m off the hook! Free from the toil of making sandwiches day after day after day. Free from having to figure out if I’ve put the proper amount of peanut butter on it or correctly guessed the jelly du jour or if I should use the ham that we just bought because she had to have it or the turkey that she likes all the time, except for when she doesn’t. I can live with that.