by JIM KNIPFEL
June 11, 2017
It Runs in the Family
The annual family reunion on my momís side is always a big deal because itís, well, a big deal. She was one of ten children. Many of her siblings went on to have a few too many kids of their own, those kids in turn did the same thing, and so on. As a result the annual reunion attracts upwards of two hundred people, most of whom Iíve never met and would never recognize if they stopped me on the street and told me their names.
††††††††††† The last family reunion I attended was in 2003, when it was held in Green Bay. The hoo-hah took place at Bay Beach, a popular local park with picnic areas, gazebos, a go-kart track, a small roller coaster, a bunch of kiddie rides and, despite the name and despite being situated right on the bay, no beach to speak of.
††††††††††† Iím not sure how this happened, but at one point during that long August afternoon, I found myself sitting at a picnic table with my Uncle Tom and a few cousins. It took a few minutes before it dawned on me that everyone sitting at that table had retinitis pigmentosa. What finally triggered the revelation was noting most of the conversation seemed to revolve around adaptive technology and dealing with Social Security. How we all found our way to that particular table Iíll never know.
††††††††††† Up to that moment, I had no idea there were so many blindos on my momís side of the family. There must have been six or seven of us. Another cousin who was hanging around the table claimed she had a mild dose of RP too, but no one ever believed anything she said. My Uncle Tom, who knew full well what the game was a long time back, had been the one who correctly diagnosed me fifteen years before any professional eye doctors got around to it.
††††††††††† ďI donít go to bars anymore,Ē he said at one point as we were sitting there drinking Pabst. ďTheyíre too loud, and if I canít hear, I canít see.Ē It took a long time before I realized how deeply profound that statement was.
††††††††††† Later in the afternoon, though he hadnít found his way to the blindo table, the son of still another cousin tracked me down. He was about seventeen, had just been diagnosed with RP, and was wondering if I could give him some idea what to expect. He was also hoping I might be able to offer some helpful advice on becoming a professional writer. Yes, well, he was a bright kid anyway, so I told him what I could about RP, but let that whole ďwriterĒ thing slide. No need to depress the youngster any more than necessary in the middle of a big party.
††††††††††† Without getting into the complex genetics of how RP is passed along, letís just say a blindo population like that, even in a group that large, wasnít exactly the luck of the draw. Still. When I mentioned just how many of us there were on that one side of the family, my assorted eye doctors always got very excited. Although it made sense in genetic terms, numbers that large remained a rarity, I guess.
††††††††††† If I was worth a damn, at this point Iíd slip off into a bunch of claptrap about the significance of having so many blindos in one family, the secret and gentle bond we all share, and all the deep spiritual lessons weíve learned from one another. Fortunately for all of us Iím just a cheap hack, and have no interest in doing any such thing.
††††††††††† Now limp ahead some thirteen years, from that afternoon in Bay Beach to a second-floor apartment in Bay Ridge, Brooklyn. Morgan had been reading up on the current state of RP research. Over the decades things had become much more complicated. When I was officially diagnosed in my mid-twenties, Iíd been told I had plain, boring old Classical RP. Now it was unclear if such a designation even existed anymore. Researchers had concluded it wasnít simply one gene mutation that resulted in RP. No, there were dozens, even hundreds of combinations that cause the retina to implode that way, and in fact there were now close to two hundred-fifty specific variants of the disease, each with a different name and each caused by some unique mutation. Imagine that!
††††††††††† Just for fun, we thought it might be neat to find out what specific mutation I was carrying, and what unique subsidiary of the RP conglomerate had afflicted me. It might make for an interesting tidbit to drop into conversation.
††††††††††† There were all those commercial genome companies out there now which, for a fee, would offer a complete breakdown of my DNA, right? Just send them a cheek scraping and a check, and a week later theyíd call with my new proper scientific classification.
††††††††††† Of course it wasnít quite that simple. Most of these companies were pretty shady, and even if they were all legit, they might be able to tell me if I had any Congolese blood in me, but they were pretty fucking useless when it came to identifying real medical conditions in terms of the genetic blueprint. There was a better tack we figured, one that not only would give us the results we were looking for, but might even get me paid in the process.
††††††††††† I dropped a line to my ophthalmologistóa woman I liked a bunch though I hadnít seen her in yearsóasking if she knew of any research studies going on around the city that might pay me the big big bucks for the privilege of analyzing my warped and unique genetic makeup.
††††††††††† A couple of days later, she sent me some links to sites listing assorted government-funded research studies in need of subjects. Most werenít in the city, most had nothing to do with RP, and those few that did were looking for a specific cross-section of subjects that didnít include me. Morgan and I glanced at the listings briefly, figured it was kind of a useless pain in the ass, and forgot about the whole idea. It had only been a whim anyway. Who really cared what kind of genetic mutation I had if the results were the same?
††††††††††† A few months passed without me thinking about any of this. Then out of the blue I heard from my ophthalmologist again. The details were a little murky, but it sounded like she was involved in a research project of her own here in the city in which I might play a part. Over the years and quite by accident Iíd been the subject of a few research papers about RP, and though I never got a dime for any of it, I was hoping this time would be different,
††††††††††† As I mentioned, sheíd always been very intrigued by the fact so many members of my extended family had the disease. The first thing I had to do, she told me, was send her a list of the names of all my relatives with RP, and how they were related to each other.
††††††††††† Sure thing, I thought. No sweat. So I began compiling a list. Letís see, there was me, of course, and Uncle Tom, who was my momís younger brother. Then I stopped.
††††††††††† Thinking back to that day when we all sat around that picnic table at the reunion, I realized I had no idea who any of those others were. They were cousins, sure, and Iíd played with them when I was seven, but I could not remember any of their fucking names. Worse, I couldnít remember which aunt and uncle they belonged to. I mean, I knew Tomís son had a case as severe as my own, which was a start, but what the hell was his name? Otto? Bill? Randy? By 2003 heíd grown into a really big guy with a heavy beard, but I couldnít very well pass that along to my doctor. ďSo then there was this big guy with a beard, and another fat one with glasses, and, um.Ē
††††††††††† It was all kind of pathetic. So I called my mom. Not wanting to come right out and admit I didnít know who most of my cousins were, I just asked if she could give me the names of everyone in the family who had RP. Well, bless heróeighty-one years old and still as sharp as they come, she rattled the list off the top of her head.
††††††††††† Turns out there were seven of us: me, Uncle Tom, Tomís son, and a brother and sister from another aunt. That seventeen-year-old (who hadnít subsequently shot himself in the head as Iíd thought for some reason) was the son of another cousin. The son of yet another cousin has likewise recently been diagnosed with RP as well. They just keep coming.
††††††††††† I put all the information into as coherent a form as I could without resorting to flow charts, and sent it off to the ophthalmologist. I have no damn clue where any of this will lead or if it will result in anything at all, but it might be interesting. Or not.
††††††††††† Sad thing is, after gathering all this information about my cousins and passing it along to my doctor, I still wouldnít recognize any of them if they stopped me on the street. Of course given theyíre all blind too, itís not something Iím going to worry about too much.
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