The question of “How are your eyes?” pops up from time to time at a social gathering with friends or a holiday meal with extended family. I don’t find this question rude or intrusive, but I’m never quite sure how to answer. Especially when the question often seems to come out of nowhere – not even closely related to the last topic of conversation. Is the question being asked as a polite “How are you?” to which a “Fine” or “Okay” is expected. Or is the questioner hoping for a detailed description of my last trip to the optholmologist? Did they see me accidentally dip my finger in the salsa bowl, thus prompting them to wonder how much more vision I’ve’ lost since they saw me last?
My typical response goes something like, “Well, RP is like getting older – it happens so slowly over time that you don’t notice the changes on a day to day basis. Yes, my eyes are worse than they were 5 years ago, but I can’t exactly define how worse.” The questioner typically changes the subject as abruptly as they started it, leaving me to wonder if I’d given a clear enough answer.
A recent trip to a retina specialist at the Casey Eye Institute provides an updated answer to the “how are your eyes” question for those interested in details.
My visual fields remain at less than 5 degrees with the outer periphery still in tact, a common trait in CRB1 (the specific gene mutation of RP that Joy and I have). For those confused by “degrees”, here’s some context: A fully sighted person has 180 degree of vision. My retina specialist pointed out that the outer periphery of retained vision is very useful for navigating. It was interesting for me to learn that this is a common trait of CRB1 because I have noticed that when I discuss vision loss with other people who also have RP, not everyone has the outer periphery of retained vision. My central vision hovers at 20/60 in both eyes, and can not be corrected above that. At my last appointment 3 years ago, it was still at 20/50, so this is slightly worse. My eyes are still very light sensitive, and I still have night blindness.
The biggest change I’ve noticed from my last eye appointment is how much less I care about the results of my exam. While I would prefer for my vision not to decrease further, I know that the condition of my eyes does not determine the path of my life. Reading the eye chart used to cause me anxiety because I wanted to get all the letters correct. It felt like a test that I was constantly failing, no matter how hard I tried.
There was a new test at this appointment that I’m guessing would have really upset me a few years back, but somewhat amused me at this appointment. It was a booklet filled with pictures comprised of little dots that make up various shapes using different colors. While I was able to point to a specific dot and tell the color, I was not able to pick out the various shapes. A fully functioning eye is able to pick out the various shapes in each picture. The doctor’s assistance administering the test seemed somewhat surprised that I could not pick out the shapes, and eagerly gave me little hints to help me “pass” the test. It truly did not surprise or bother me that I could not decipher the “hidden” shapes, and I felt no pressure to pass other than the fact that I think it would have brightened this man’s day. He was super kind, and I found it endearing that he cared so much about this test. But to me, I see how I see. The results of an eye examine does not change how my eyes function.
My retina specialist, Dr. Pennesi, is smart. The kind of smart that leaves no doubt that he knows what he’s talking about. He started seriously geeking out over the photos of my retina. For me, it was like watching a detailed slideshow of a neighbor’s summer vacation. I was kind of interested at first, but started nodding off by the 8th photo. There were these white spindly lines that he found particularly intriguing, to the point he asked if I could stay an extra 30 minutes to have additional photos taken with a new camera that could gather even more photos of these white spindly sensations. He said the results of the additional photos wouldn’t necessarily benefit me in any way, but that they would be useful to him in his work, so I was happy to oblige.
And of course the part of my appointment that attracts the most curiosity is the research for a cure. Dr. Pennesi was once again very knowledgeable in this area. The most promising treatment for CRB1 is currently Optogenetics. I really loved his iPhone analogy. He explained that the current clinical trials are like the brick sized 1980s cell phone, and that for someone like me who still has that residual central vision of 20/60 and ability to read print, he would not recommend any clinical trials until the science has progressed from the 1980s brick cell phone to the iPhone 2 or 3. The amount of time it will take to make the jump from big old cell phone optogenetics to sleek modern fancy iPhone optogentics is unknown.
For anyone who would like to see the research move along at a rapid pace, there is a specific organization dedicated solely to finding a cure for the CRB! form of RP. It was started by a group of parents, as this form involves childhood onset and therefore affects many children. Visit www.crb1.org for more info!
So that’s how my eyes are doing. Perhaps the next time I’m asked this question, I’ll invite the questioner to read this blog post.