A New Hope
With the help of my very supportive parents, we found that one of the world’s leading doctors in genetic eye diseases—a man named Dr. Stephen Tsang—was located a mere half hour away at Columbia Medical Center. We paid him a visit, and he and his staff confirmed it was definitively RP. They were actually able to give us a great rundown on WTF Retinitis Pigmentosa is.
What Is Retinitis Pigmentosa?
Retinitis Pigmentosa is a rare genetic degenerative eye disease that affects roughly one in five thousand Americans. It progressively diminishes peripheral vision over decades, eventually leading to tunnel vision or, in some cases, legal blindness. Total blindness is very rare.
It’s estimated that 1.5 million people in the world suffer from RP. The age of onset varies widely from person to person, but I first started noticing symptoms at the ripe old age of 34.
In essence, RP stems from specific genetic mutations that impair photoreceptor function and survival. This means my retinas are slowly dying, starting with the rods. The rods pick up light and help us see levels of luminosity or brightness. They’re located on the periphery of the eye and are crucial for helping us see in the dark. This is why the first two symptoms of RP are night blindness and loss of peripheral vision.
Other symptoms of RP include low vision, migraines, eyestrain, sensitivity to light, slow transitions from bright to dark and vice versa, and the worst one of all: a strobing at the edges of my vision that is there all the time, day or night, eyes open or closed.
Dr. Tsang explained that this was what happens as my retinas die off. I have a backstage ticket to the death of my vision, and I’d like to give it back. The best way I can describe this phenomenon is that it’s the visual representation of panic.
I included a video of my cat Miso suckling on his blankie and making biscuits. What you’re observing here is the frame rate of the iPhone slo-mo camera not syncing with the on/off strobing of the LED lighting in the room, which is imperceptible to the naked eye.
What it captures is what RP looks like to me. Not in slow motion, but that strange, pulsating, strobing effect is always there.
I can also see this pattern, in the next video, within the chaos. Yes, it’s a ceiling fan. That’s the best way to represent it. Mix the two together in a blender and add the occasional odd spots, difficulty seeing in the dark, and sensitivity to the sun, and you can begin to imagine what I see.
At each visit, Dr. Tsang makes me do multiple tests in machines that look like sci-fi torture devices. One, called an electroretinograph, involves attaching electrodes to my eyes to measure their response to light as I look at a panel of flashing lights. There’s also a visual field test where I put my head in a white dome. I’m told to look forward, and every time I see an LED flash, I push a button. Based on my response, they create an image that represents my field of vision in each eye. There are also multiple photos taken of my retinas that show the progress of my degradation.
The good news is that Dr. Tsang told me I have a very mild case of RP and that I have 20-30 years of usable vision. Whatever that means.
