What does retinitis pigmentosa look like?

The first symptom of RP is usually trouble adapting to the dark, and if a person does not mention it, careful questions about dusk and dimly lit places often bring out a history that goes back to childhood or the teenage years ¹. Dave Steele, a poet with RP, describes it as "struggling from light to dark places" and says that in his early twenties "it was a very, very slight adjustment and one that I didn't actually at the time put down to RP. I just adjusted, took a couple of seconds and got on with things" ².

Maggie, who has Usher syndrome type 1 (RP with deafness), puts the dusk experience this way: "The moment the sun hits the horizon, details disappear, and it feels like I've got sunglasses on anyway" ³. She also describes the paradox many people with RP live with: "light sensitivity paired with night blindness", so that on a bright day the choice is to squint or to wear sunglasses that make it too dark to see ³. Bright car headlights are a particular problem because the eyes struggle to readjust to the darkness afterwards ⁴.

This is the part families find hardest to believe. Side vision in RP is lost first in the mid-periphery, roughly 20 to 25 degrees out from the center, as a ring of blind spots, and this early loss "is rarely recognized by the affected individual and is usually not a presenting symptom" ¹. Children with it "may be misconstrued as 'clumsy' before constriction of visual fields (i.e., 'tunnel vision') is detected" ¹.

The reason is that the brain papers over the gaps. Dave Steele explains: "when you walk into a room, your brain subconsciously scans what's in the room and often it fills in the blanks for what your eyes can't see. Sometimes people who are visually impaired feel like they can see more than they actually can until they're actually physically tested" ². He describes his own field test: "I had to hold this button and every time a light went off in my peripheral vision, I had to click the button to signify that I'd seen it. The test started and I just sat there holding the button and not clicking it" ². Afterwards, he says, "it was almost like all of a sudden the veil came down and I started to realize that I couldn't see as much as I thought I could" ².

So when your child says "I can see fine", they are telling the truth as they experience it. The missing pieces do not announce themselves.

Over years the ring of blind spots widens outward and inward until a small central area remains, which is the "tunnel" people talk about ¹. Laura Lawson Visconti, who writes about living with RP, has about 24 or 25 degrees of vision left compared with roughly 155 degrees for a normally sighted person: "To put it plainly, I see in tunnel vision" ⁵.

What is inside the tunnel is often sharp. Laura writes: "The central vision I do have left is very sharp and healthy, so unlike macular degeneration or other retinal diseases, I do not suffer from blurry vision" ⁵. That is why a child with RP can read a book or a phone perfectly well and still walk into a door frame. Central sharpness is usually preserved until the late stages ¹.

What is outside the tunnel is the question everyone asks. Laura answers it directly: "Many people ask me if I see black in the periphery where I've experienced vision loss and the answer is that I do not; I simply do not see anything, same as the boundaries beyond a normal sighted person's range of vision" ⁵. Not everyone describes it the same way. Hannah, who has a cone-rod dystrophy with later side-vision loss, describes discovering that a lamp had been switched on beside her: "I realized that I had completely, it was black, it was completely black on my left side... There's nothing there" ⁶. Both are honest descriptions. What they share is that nothing in the lost area draws attention to itself.

In practice this means your child does not see things that appear from the side. Laura: "I do not see objects or people that unexpectedly pop up in my path, like a waiter approaching my table at a restaurant, or someone offering a handshake" ⁵. Rebecca Alexander, who has Usher syndrome type 3, remembers that as a teenager "I often tripped over the dogs who were below me if they were lying on the floor or coffee tables; my worst vision was right below me" ⁷. People with RP compensate by moving their eyes constantly. Laura again: "I scan a lot and my brain fills in the information that my eyes miss" ⁵.