What is is like to see with Stargardt’s
If you know me and have spent any time with me, you’ve probably noticed that my vision isn’t very good. Whether I’m holding a piece of paper or my phone up close to my face or sitting inches away from my computer monitor and still using the magnification, it is pretty obvious that I have a vision impairment. Many people ask questions about my vision such as, “can you get stronger glasses?”, “is everything just blurry?”, “how did you get it?” and more. So this blog post is meant to answer some of those questions and help others understand what it is like to have this vision impairment.
What is Stargardt’s?
Stargardt Macular Distrophy, or Stargardt’s Disease, is an inherited form of macular degeneration. The macula is the central region of your retina. So, as the name implies, macular degeneration is the deterioration of that central area, thus causing central vision loss. Symptoms (decreased vision) usually manifest during teenage years, but in some cases people are diagnosed even younger or much later in life. I started losing my vision and was diagnosed around age 17. Stargardt’s doesn’t make a person totally blind. Vision usually deteriorates to visual acuity of somewhere between 20/200, which is legally blind, and 20/800. The rate of vision loss varies, but can be as quick as just a few months to go from 20/20 to 20/400.
Could Stronger Glasses Help?
I wish! The eye is like an old film camera. The cornea of the eye is like the lens of the camera. It is responsible for focusing on the image and letting just the right amount of light in so that the image isn’t too bright or dark. The retina is like the film of the camera. It captures the actual image to be processed. So, if your lens (or cornea) is bad or defective and you can no longer focus properly, you can fix it with a new lens (glasses or contacts). But If your film is damaged (retina), then it doesn’t matter how good of a lens you have, your image is not going to come out clearly. Since Stargardt’s affects the retina, there’s nothing that can be done for it right now.
Is Everything Blurry?
In general, everything is a little blurry and then there’s one big empty blind spot in the middle. But the blind spot isn’t just a black or gray dot. The brain tries its best to fill in the gaps with what it thinks is there. Did you ever do the thing as a kid where you draw a line with a gap and with an X on it to find your blind spot, and see how your brain fills it in with a continuous line? (Here’s an online version of that) It is like that but on a much larger scale.
There are some websites and mobile apps that try to simulate what it is like to see with Stargardt’s or macular degeneration in general. But most of them show big black or gray spots for the blind spot. Some show it more of a donut shaped blind spot, which I’ve never experienced. So, using my limited Photoshop skills, I’ve created some images to approximate how I see the world.
In this illustration you can see how the central vision is gone and the brain tries to fill in the gaps with what might be there. In this case, since there is, a white wall. You can also see that it is easy to make things disappear, like the plant.
Here a half a person disappears, filled in with the background color, which is blue. Pretty strange, but neat at the same time.
Sitting in a car, it is sometimes fun to see if I can move my blind spot around to make entire cars disappear!
Talking to people up close can still be challenging. Trying to look directly at their face puts the blind spot directly over them, making it difficult to see facial expressions, etc. So it is not uncommon for those of us with Stargardts to actually not look directly at you while talking to you. Instead we look off to the side of your face so that we can (sort of) see you., which, as this next picture shows, is still kind of blurry.
So even with this strategy, if you are more than 5-7 feet from me, I couldn’t tell you if your eyes are opened or closed.
Trying to Read
Reading print is tough, especially with small font sizes. Even if the font is larger, reading is slow as my eyes struggle to focus on each new word while also trying to look around the big blind spot. So unfortunately, I don’t read as much as I would like to. When possible, I prefer to read things digitally on the computer where it is easy to zoom or enlarge the text size.
One trick I do for smaller print such as cooking instructions or medicine bottle instructions is to take a picture of it with my phone and then zoom in on the picture to read it.
Some people ask me if the words just look too blurry to read. I don’t consider it blurry, just too small to read. For example, if you were to try to read this text from across the room, say 15-20 ft away you probably wouldn’t describe the text as blurry, just too small to read from that distance. That’s how it is for me from just 2 feet away.
So, I actually do wear glasses. They get my vision from 20/400 to about 20/80 or 20/100, which is borderline the limit for being able to drive. Ohio, and many other states, offer what is called “bioptic driving” for those that are just short of the visual acuity requirement for driving. It is basically driving with a little binocular mounted to your glasses, which you use to peak through every once in a while to read a street sign or see the color of a traffic light, etc. You have to go through a training program and pass a test with it. So, I do drive. I try to only stick to roads that I’m pretty familiar with and I try not to drive at night or in the rain unless absolutely necessary. While driving, I try to constantly be looking around in different directions in front of me to make sure my blind spots aren’t making me miss anything. Unfortunately, most people with Stargardt’s cannot see well enough to be anywhere close to being able to drive. I’m lucky. I know that.
Any Treatments Available?
Currently there is no treatment of any kind. The retina is too fragile to perform surgeries on it without risk of tearing or otherwise damaging it. However, technology and research have come a long way over the last decade. There is a lot of research going on currently with things like stem cell treatments and gene therapy that are looking promising and have shown positive results in early clinical trials. I believe there will be a treatment in the next 5-10 years.
Will my kids have it?
Stargardt’s is hereditary, meaning it is passed down from your parents. The only way to know for sure is with genetic testing. Rather than testing each child, we just had my wife tested to see if she is a carrier. She’s not. So there’s no chance my kids will have it; but, they will be carriers as it is a recessive gene.
This question comes up a lot from those that have Stargardt’s. To explain how it all works would require going into a little bit of a genetics lesson from high school biology, which I’ll save for another blog post later. (UPDATE Jan 3, 2018: I did create such a blog post – How did I get Stargardt’s? Will my kids have it?)
How Does it Affect My Life?
Overall, life is good. I’m generally a positive person and I try not to let my vision hold me back. I’m lucky enough to still be driving, which helps tremendously. I love my job – working on a computer all day is ideal for me with the ability to zoom/magnify stuff. I can still play sports like racquetball and even coach my kids Little League baseball teams. Some of the things that are different or annoying or bother me to varying degrees are:
- Unless I know you really well and can identify you by your shape, how you walk, or the sound of your voice, I probably can’t tell who you are until you are within about 10 feet of me.
- Even then, if I don’t know you too well and haven’t had many close-up conversations with you that would let me see and get to know what your face looks like, I probably won’t recognize you. I hope you’ll forgive me.
- When it comes to watching TV, I usually have to choose between snuggling up with my wife on the couch or sitting up close to the TV so that I can actually see what’s going on. That sucks.
- Trying to spot my kids among a group of other kids, like at a playground or daycare, is impossible unless I’ve memorized what color clothes they are wearing and look for that.
- I usually can’t read the menus at restaurants, so many times I simply ask the person waiting on me what is popular or what their favorite thing on the menu is. This usually works out great because I like pretty much anything and am always open to trying something new.
- I coach my kids’ Little League baseball teams and have to memorize who is playing each position or the jersey numbers of the kids because other than that they pretty much all look the same to me unless I’m standing right next to them.
- I have to use binoculars for things that most people wouldn’t, like watching my kids play a soccer game, or watching a concert or sporting event from the good seats.
- It is tough or impossible to read a story to my kids at night unless the font is large.
- If I’m playing a sport with a small ball, like squash, racquetball, golf, or even baseball, I will often lose sight of the ball in my blind spot, especially if the ball is moving fast. So I try not to be too competitive at that stuff since I know I’m at a disadvantage.
- Movies with subtitles are worthless, even in a theater, unless someone sitting next to me reads them to me.
- I love to draw. But drawing now is a struggle sometimes, especially if I’m trying to see or draw some finer details.
- Smart watches sound cool, but I could never use one with vision this bad.
- Speaking of watches, digital clocks are much harder to read than analog clocks.
All in all, Stargardts does have its challenges, but by keeping a positive attitude, utilizing technology, and staying optimistic about the current research going on, I feel like I live a pretty normal and happy life.