“How did you get it?”, “Can you get stronger glasses?”, “Is everything blurry?” These are just a handful of questions Keith Ruston’s asked about living with a sight condition. Here, Keith explains what it’s actually like living with Stargardt’s.
Whether I’m holding a piece of paper right up to my face or sitting inches away from my computer monitor and still using the magnification, it’s pretty obvious I have a visual impairment. I started losing my vision and was diagnosed with Stargardt disease around age 17 years old.
What is Stargardt’s?
Stargardt disease is an inherited form of macular degeneration. The macula is the central region of your retina. As the name implies, macular degeneration is the deterioration of the macula, causing central vision loss.
Stargardt’s doesn’t make a person totally blind. Vision usually deteriorates to visual acuity of around 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.
Can stronger glasses help?
I wish! Eyes are like an old film camera. The cornea of the eye is the lens, it focuses on the image and lets in just the right amount of light so the image isn’t too bright or dark. The retina is the film of the camera, it captures the actual image for processing. If your lens (or cornea) is defective and you can no longer focus properly, you can fix it with a new lens (glasses or contacts). If your film is damaged (retina), it doesn’t matter how good of a lens you have, your image isn’t going to come out clearly. Since Stargardt’s affects the retina, there’s currently nothing that can be done.
Generally everything’s a little blurry with a big empty blind spot in the middle. The blind spot isn’t just a black or grey dot, the brain tries its best to fill in the gaps with what it thinks is there.
Using my limited Photoshop skills, I’ve created some images to try to show how I see the world.
The first image shows a sighted person's view of a woman stood in front of a blue door. In the second image, simulating the view of someone with Stargardt's, half the woman disappears, filled in with the background colour, which is blue. Pretty strange, but neat at the same time!
Talking to people up close can be challenging, if you look directly at their face this puts the blind spot directly over them, making it difficult to see facial expressions. It’s not uncommon for people with Stargardt’s to not look directly at you, instead we look off to the side of your face so we can (sort of) see you, which, can still be kind of blurry.
The first image shows a sighted person's view sat talking to a friend. In the second image, simulating the view of someone with Stargardt's, the person's face blurs so you're not able to read their facial expressions.
Can you read?
Reading print is tough, especially with small font sizes. Even if the font’s larger, reading is slow as my eyes struggle to focus on each new word while also trying to look around the big blind spot. I don’t read as much as I’d like to but when possible I prefer to read things on the computer where it’s easy to zoom or enlarge the text size.
Some people ask if the words are too blurry to read, they’re not blurry, just too small. Reading for me is like if a sighted person tried reading this text from across the room, you probably wouldn’t describe the text as blurry, just too small to read from that distance.
One trick 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.
Can you drive?
So, I do drive. My vision goes from 20/400 to about 20/80 or 20/100 when I wear glasses, which is borderline the limit for driving. Ohio and many other states in America offer bioptic driving for people just short of the visual acuity requirement for driving. It’s basically driving with a little binocular mounted to your glasses, which you use to peak through to read a street sign or see the colour of a traffic light. You have to go through a training program and pass a test with it. I tend to stick to roads I’m familiar with and try not to drive at night or in the rain unless absolutely necessary. While driving, I constantly look around in different directions in front of me to make sure I’m not missing anything because of my blind spots. I’m really lucky; most people with Stargardt’s can’t see well enough to be anywhere close to driving.
Are there any treatments available?
Currently there’s no treatment. The retina’s too fragile to perform surgeries on without risk of tearing or damaging it. However, technology and research have come a long way over the last decade. There’s a lot of research going on such as stem cell and gene therapy that have shown positive results in early clinical trials. I believe there’ll be a treatment in the next five to 10 years.
Will my kids have it?
Stargardt’s is hereditary, meaning it’s passed down from your parents. The only way to know for sure is with genetic testing. Rather than testing each child, we tested my wife to see if she’s a carrier. She’s not, so my kids don’t have it but they’ll be carriers as it’s a recessive gene. I’ve written a more detailed blog post in answer to this question.
How does it affect my life?
Overall, life’s good. I’m generally a positive person and 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. But here are some things that are different, 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’re until you’re 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 help me get to know what your face looks like, I probably won’t recognise you. Please forgive me!
When watching TV I 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, perhaps at a playground is impossible unless I’ve memorised what colour clothes they’re wearing.
I usually can’t read menus at restaurants, mostly I simply the waiter or waitress what’s popular or their favourite. This usually works out great because I like most things and trying something new.
It’s tough or impossible to read a story to my kids at night unless the font is large.
Movies with subtitles are worthless, unless someone sitting next to me reads them to me.
I love to draw but drawing is sometimes a struggle, especially if I’m trying to see or draw 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, Stargardt’s does have its challenges, but by keeping a positive attitude, utilising technology, and staying optimistic about the current research going on, I live a pretty normal and happy life.
Source: This article originally appeared on Keith Ruston’s blog and has been republished with his permission.