Me and RP (Part II)

Shortly after I graduated from Gonzaga, I started doing a lot of Internet research on eye conditions in an attempt to explain my clumsiness - that's when I stumbled across an eerily fitting description of the condition known as retinitis pigmentosa (RP). Mostly, I was doing this research to assuage my curiosity - I don't think I truly believed something was "wrong" with me at the time. You see, with RP, it's hard to really gauge the vision loss. As it turns out, my peripheral and night vision have been slowly deteriorating throughout my life, so there has never really been an "AHA!" kind of moment where I could 'put my finger' on any noticeable change in my vision.

I first brought up the possibility of having RP with my parents, who rightly pointed out that it seemed odd that I wouldn't have been diagnosed in the 15+ years I had been seeing an eye doctor. However, we all agreed it made sense to raise the question in my next eye appointment. Several months later, I dropped in to get a new prescription at a Pearle Vision in Northgate mall with my buddy Rock. I posed the question to the optometrist who pulled out his scope to examine my eyes more closely. He quickly saw some of the telltale physical traits of RP: "mottling of the retinal pigment epithelium with black bone-spicule pigmentation" (as described on Wikipedia). He followed up with a computer-aided photograph of my eyes that more clearly showed the characteristics of RP and referred me to the University of Washington (UW) eye clinic for a follow-up appointment. Over the next year, my family and I visited doctors at UW and Oregon Health & Science University (OHSU) who confirmed that I had X-linked RP, in addition to my mother and brother.

The symptoms of RP vary significantly from person-to-person. In my case (and my brother is similar), I have a working field of vision of approximately 25-30 degrees (90 degrees is "normal"). At night, my vision gets much worse to the point where I have significant trouble navigating at all (e.g., I am essentially night blind). One of the most critical elements of the condition is its impact on my independence, namely my ability to drive a car (I touch on this point in Part I of this blog). I am currently still legal to drive, but do so on a very limited basis and no longer drive at night. My initial diagnosis came about six years ago and it's been a rollercoaster of a journey since. There have certainly been some times where I've been left anxious, scared or depressed at the possiblity of losing my vision, but those times are relatively few and far between. Most importantly, I've got an amazingly supportive, loving wife, an incredible family support structure and some of the greatest friends a guy could ask for.

This past weekend, my family, Cara's family, and some friends all took part in the Oregon VisionWalk in Portland, OR, as a fundraiser for the efforts of the Foundation Fighting Blindness (team picture is below and Cara and I will post more on that later). I am also taking part in a clinical trial for a supplement that may help to preserve the retina and delay the slow degenerative vision loss. I'm also optimistic for several of the research projects ongoing with the FFB and it's affiliates, including gene therapy and retina transplants.


I could blog all night about my thoughts, analyzing, and experiences (both frustrating and uplifting) related to RP. But, this blog has already gotten pretty long. Before I end, I want to thank my beautiful wife, incredible family, and wonderful friends for supporting me, loving me and of course...for driving me around! Y'all are amazing!

Me and RP (Part I)

As many of you may know I have retinitis pigmentosa (RP). RP is a genetic eye condition that leads to gradual vision loss, primarily affecting peripheral and night-vision (e.g. low-light and bright-light conditions). As I get ready to participate in the Oregon VisionWalk this coming weekend, I wanted to (1) describe an experience I had several years ago (this blog - Part I) which involves my vision (or lack thereof); and (2) share my personal thoughts and feelings about living with RP (next blog - Part II).

Without further ado, here's Part I (I apologize for the length in advance):

I've got many friends and family members who would agree that being a passenger with me behind the wheel is a nervy experience. I've always felt like a bit of a clumsy oaf; bumping into things, tripping over my own feet, struggling to navigate at night, these were all regular occurrences in my life. But, I didn't think much of it until I had a nervy driving experience all to my own. Until now, this is an experience that I have not shared with many people...

I was driving home alone from birthday gift shopping at the Super Mall in Auburn, WA and after a wrong turn, I ended up in an area I wasn't familiar with. Stopped at a red light, I noticed that the road crossed four railroad tracks ahead. The light turned green and the car next to me peeled off (high school kids out being crazy), while I slowly accelerated through the intersection and towards the railroad crossing(s). Something didn't feel right to me as the car began to pick up speed; a feeling that was reinforced by an ominous train whistle that pierced the air. I looked in my rear view mirror as I approached the first set of railroad tracks and noticed the cars behind me had stopped at a second traffic light that I had previously failed to notice; a light that was red to keep the tracks clear for the oncoming train. "Thunk-thunk". My car crossed the first set of tracks and I began to panic as I approached the second set of four railroad tracks. My first reaction was to hit the brakes as it began to dawn on me that I was playing chicken with a train. Train versus Corolla? Train wins and it's not pretty. But where was the train?

A second whistle pierced my thoughts (and if it's one thing I can do, it's analyze...probably to a fault) and I fought off the urge to stop; afterall, it's not wise to stop on railroad tracks. "Thunk-thunk." I cleared the second crossing and was coming up to the third crossing when it dawned on me that I needed to stop looking in my rearview mirror at the stopped traffic and start trying to figure out where this looming train was. I frantically scanned left and right as my front tires ("Thunk") and then the rear tires ("-thunk") cleared crossing #3. Another whistle. It felt like the train was in the passenger seat next to me. The earth rumbled from its proximity. But where was it? Why couldn't I see the train?

My eyes darted left and right as I approached the last of the four crossings. I silently pleaded for my eyes to locate the train, but my vision didn't seem to work in my panicked state. Left and right. Everything seemed to move in slow motion. Left and right. I was mere feet from the final set of tracks at this point. Left. Right...THERE. There was the train. Just as my front bumper crossed the track, I had finally spotted the train off to my right through the front windshield. It was close and quickly getting closer, dwarfing my Corolla as I crossed the tracks. I could see the engineer and we locked eyes. I'm pretty sure he was thinking, "what the $#%@ are you doing?!" I couldn't take my eyes off that looming train. That incredulous engineer. What was I doing? Time seemed to stop.

"Thunk-thunk." My back tires cleared the tracks and snapped me back into the moment. I was clear of the railroad tracks. I looked at my rearview mirror and just beyond my rear bumper, I saw nothing but the steel side of the massive engine car. The train had missed me by no more than a foot. Eyes back to the road ahead of me, I approached another intersection and another red light. I stopped. Those crazy high school kids were idling next to me. They had seen the train and decided to be crazy and cross the tracks anyways. The driver rolled down his window and gave me an enthusiastic thumbs up. A thumbs up? They must of figured I had seen it too and had "out-crazied" them with my unintended game of chicken. The light turned green and the high schoolers peeled off. As they did, a passenger leaned out of the back window and yelled, "That was crazy!"

With these words echoing in my head, I slowly proceeded through the intersection and pulled over onto the shoulder. I put the car in park and instantly began to shake uncontrollably. That was crazy. I had just been inches away from a moving train. What's worse is that I didn't see the signal light OR the train until it was almost too late. My shaking turned to sobbing and I sat there on the side of the road shaking and crying until I had no more shaking or crying left in me. I then took a deep breath, collected myself and slowly pulled back onto the road to make my way home.