PXE Vision: By and For PXEers
by Pat Manson
I´ve been asked to write a column for the PXE International website to address some of the issues that PXEers and their families face. This will be part of the overall effort that Chris Vocke, Assistant Director of PXE International, is leading to revamp the website to include more useful information and to make it more user-friendly. So within the next few weeks, PXE Vision: By and for PXEers will begin to appear on the website. There, I hope to cover on a semi-regular basis (one or two columns a month) a wide range of topics — discovering you have PXE, coping with vision loss, PXE & your family, disability, privacy, etc. I´ll be writing on these various subjects with two complementary goals: 1) to pass on some information I picked up in my professional life, and 2) to look at that information as a PXEer.
Before I get too far, I should tell you a little about myself. I have PXE, pretty bad in fact. Like many with PXE, I was diagnosed years ago, at age 23. I´m now 51, live in Richmond, Virginia, USA, and am married with two sons, 18 and 22. Neither has PXE. I have 20/250 in my better eye, so I´m legally blind. After two-plus years of rapid deterioration, my vision has been stable for a year. I have a few mild skin lesions. My GI system is okay so far. My blood pressure and cholesterol are both a little high, but untreated to date. I´m watching them. I also have reflux and am hyperextensible with numerous resulting joint problems, conditions probably not caused by PXE. Despite all this, until I was 48, apart from fairly serious, but garden-variety, myopia (near-sightedness), I was fine . . . I thought.
I was a corporate lawyer, an “inside” or “in-house” attorney, meaning I was actually a company employee, rather than working for a law firm. I´d had a good career so far and was at my professional peak. My last two positions had been as a corporate officer and the general counsel (chief legal officer) of two large companies—Hamilton Beach/Proctor-Silex, Inc. (small household appliances) and Albemarle Corporation (NYSE chemical manufacturer). Before that, I´d been a lawyer for several pharmaceutical companies in New Jersey and Virginia. Going back further, I´d been a lawyer for a large New Orleans law firm, and before law school I taught English. In my legal career, I´d handled a number of matters relating to employee privacy, disability in the work place, insurance and drug research and approvals. These proved helpful when my vision suddenly began to deteriorate, and I had to make some critical decisions in a short amount of time. I wanted to maintain my career in the worst way and nearly killed myself (and my wife Gerlinde and the son still at home) trying. After two-plus years, I had to throw in the towel. I helped my employer hire and train my replacement, then went on long-term disability. And that´s where I´ve been since January 1st of 2004.
Like many “sick” people, I find that I like to talk about my condition, perhaps even too much. I suppose we need to talk about it. So that´s what I´ll do in the column. But I promise that PXE Vision will be about us--PXEers, not just me. In a short 39 months, I went from a healthy and successful professional to a stay-at-home dad on disability, a surprising and perilous journey to be sure, but one that many PXEers must take in one form or another. It isn´t a trip meant for sissies, but it isn´t voluntary. As all PXEers know, it starts with incredulity, ignorance and fear and progresses to knowledge and understanding. But it´s a passage not without humor, and it may even provide glimpses of self-discovery. My plan is for us to take that journey together in the column, stopping along the way to explore the topics above plus others. I could just summarize each topic one at a time in textbook fashion, but I´d rather look at them from our peculiar vantage point—as a PXEer. I hope you´ll tune in. Otherwise, my wife and sons will have to listen to me read my entries aloud—haltingly—over and over. I´ll talk with you soon.