As you can read in the „About Me“section, I have been suffering from Stargardt’s disease since I was five years old. This is manifested by the gradual degeneration of rods and cones on the retina, mostly in its middle, so people with Stargardt’s disease lose central vision. The peripheral one should be preserved, but there are cases where degeneration also affects this site.
Some theory first
Stargardt’s disease is a disease transmitted genetically. It is caused by mutations in the ABCA4 gene, which encodes a protein involved in the transport of used parts of the photoreceptors. Mutations affect the processing of vitamin A, resulting in accumulation of the toxic dimer of vitamin A. As a result, retinal pigment epithelium and photoreceptors die. It is therefore important to avoid vitamin A dietary supplements, but some ophthalmologists are not aware of this and recommend these products.
Stargardt’s can be different for everyone and can be diagnosed at a different age. An important factor is also the type of a genetic mutation or mutations. It is a progressive disease, which means that the condition worsens with age. Someone’s eyesight stabilizes at a relatively good stage, but in some cases it can end up with practical blindness, very rarely the total blindness has been detected but normally it shouldn’t lead to it. My case can be considered as one of the worse ones.
How do I see?
The question I hear the most. The answer, however, is not at all simple and for someone with eagle eyes, it is usually hard to imagine. Therefore, I decided to write this article, in which I will try my best to describe how I actually see.
All of you probably remember from physics or biology the explanation of the so-called blind spot that each of us has at the place on the retina where the optic nerve passes into the eyeball. In my case it looks like I have several of these blind spots and even much bigger .. That sounds scary, but in fact it is not at all. I do not see the spots on their own. The brain is so clever and adaptable that it creates and replaces the blind spots by creating some “fictional” filling.
Let’s say I’m looking at a white table that has a mug, a cellphone, and a pencil on it. Thanks to peripheral vision, I can see the table in front of me and perhaps a mug that stands on the left in the field of vision. But the cellphone and pencil that lies in the middle can be no longer spotted by my eye, so the brain thinks there is nothing else on the table. However, as soon as I move my head to the right, these items suddenly appear as a magic trick, which however makes the mug disappear instead. Then no wonder that sometimes when I want to read a message on my phone and reach for it, I pour out a mug that “wasn’t “ there at all before.
One of the biggest drawbacks of those blind spots is that I basically can’t read a continuous sentence. It happens to me exactly the same thing as in the previous case. If the text is large enough, I can catch the first letter of the word, but not the rest, so to read something, I have to keep moving my head back and forth and concentrate terribly, and after about ten seconds my eyes start to hurt. For those reasons, it means reading is simply not possible.
When I look at the computer screen, I still see with peripheral vision that something is written on the screen but I cannot read what. Instead of reading with my eyes, I have been using a screen reader for a while, which reads the text on the screen for me, and now even Braille
I lost the ability to recognize details such as people’s faces, small things that someone holds in their hands, or how many fingers someone shows quite early. This means that I see people as silhouettes and recognize them in good lighting by height, clothing, nowadays mainly by their voices. For example I had a huge problem whenever meeting large number of people such as at school, where I constantly mistook one schoolmate for another and yes, it was not a large problem for me to say Hi to a teacher 🙂
Sometimes I get into quite funny situations when I try to pick up a piece of clothing that has fallen on the floor, bend and touch the sleeping cat instead of a T-shirt or take a TV remote control from the table instead of chocolate.
A person with Stargardt’s will probably also have a problem with orientation already from the beginning. The main issues are signs on buildings, stairs, bollards and columns. In my case, even people going in the opposite direction. Because when such a person, let alone street lamp gets into a blind spot in my eye and stays there too long, broken nose is born :-).
Another unfortunate thing is the number of busses and trams, traffic lights, and especially the crossing of the road – I do not want to mention what would happen if a car got into the blind spot … Therefore, the white cane is priceless in all situations. I also use orientation training, where an experienced person will teach you routes according to landmarks to various specific places.
Stargardt’s disease is associated with photophobia. It had been avoiding me for a long time, but it has been showing up lately too. What bothers me is the direct sun, so I can’t do without dark sunglasses. Hats and caps also help a lot.
Inside, it is not comfortable to sit in a dark room opposite to windows and other light sources such as computers and phones, so I always set their brightness to a minimum and I prefer a black background. Although other people with Stargardt’s disease will probably argue with me in this regard, I personally see much better in the dimness than under strong lighting.
Probably the last drawback is the problem with color recognition. Although it is not so dramatic, at least yet, it still causes inconvenience. I am mainly confused by colors of the same shade so I might have problem recognizing black, brown, dark blue, dark green and dark purple. Another thing is then a combination between multiple colors of the same shade for example in a picture or book. Seeing something green written on a blue background is absolutely impossible.
As I have mentioned before, everyone can get affected differently. If you have your own experience, feel free to shatre them with us.