Thursday, March 8, 2012

Why E.J. Scott Runs

E. J. Scott, CHM's "Running Man"

There are posts which evolve of their own accord as they are written.

There are posts which go places I never imagined them going prior to having completed them.

Lastly, there are rare posts like this one - those which I don't know how exactly to begin, neither do I know how they will ultimately end.

Well, I guess I do know how to begin - the trouble is in the process of 'the starting'.  

When all else fails, go with the basics: 

  1. Post a photo, and, 
  2. Free-style some random verbiage as the thing begins to breathe on its own

But as for the ending...

I just don't know.

Nope. I can't tell you yet. I honestly don't know how this one ends.

By way of your introduction to E. J. Scott, our aforementioned "Running Man", let's begin with a few of the things which I DO know:

  • I have never met E. J. Scott
  • I have never met E.J.'s girlfriend, Deborah Ann Woll, the actress who portrays Jessica Hamby on HBO's "True Blood" series
  • E. J.'s Occupation, according to his website, is listed as: "Being awesome, but it pays terrible"
  • E. J. Scott and I have much in common (as we are both 'awesome', although honestly, when it comes to 'being awesome' - he 'owns' me)
E. J. Scott has a rare retinal degenerative condition known as 'Choroideremia'. How rare? At last count, there are fewer than 6,000 Americans afflicted with this particular genetic-pie-in-the-face-from-God.

You'll never hear about Choroideremia (a.k.a.: 'CHM') on the evening news because it doesn't affect enough people (I believe there are at LEAST 6,000 people who would argue with this last statement, but in the end, it is what it is - this particular genetic defect is 'Honest Politician' rare).

This is one of those 'Good News / Bad News' situations.  You most likely don't know what Choroideremia is, nor what it does.  The good news is that if you don't know WHAT it is, it most likely does not affect you. 

Conversely, E. J. knows all too well what Choroideremia is. So do I. We both have become acquainted with the condition differently, but as he has experienced the progression of this visual condition personally, I have seen it through the eyes of others. Six of my favorite eyes in the world.

There is another major difference between E. J. and me:

E. J. wages his war against CHM publicly. 

In 2012, he plans to run in twelve marathons to raise money to find a treatment, cure, or perhaps another 'miracle' to stop the progression of CHM in himself, as well as 5,999 of his closest friends. 

As I sit on my rump typing this, E. J. runs.   With two marathons started and finished - he has ten more scheduled through the remainder of this year.  Not only is E. J. running these road races on behalf of all those with CHM, but he's also running them...  Blindfolded.

At 36 years old E. J. is legally blind.  He's used a cane to augment the peripheral vision he's lost over the years since being diagnosed about a decade ago. He got the idea to wear the blindfold to both call attention to CHM as well as to protect his remaining eyesight. Bright sunlight will hasten the progression of CHM; so when he jogs through the throngs of other runners on race day - he's the only one who runs in the dark.

E. J.'s younger brother and his seven-year old nephew also have been diagnosed with CHM.  Typically, when CHM 'hits', it comes after your family like your family is the trailer park and the CHM is the angry gray tornado hungry for corrugated metal.  It takes what it wants and leaves you wondering "Why us / me?".     

In an article I found on the
Austin Statesman, E. J. provided some insight into his experiences with CHM and 2012's 'marathon' of Marathons to raise money to find a treatment:

Times when he bumped into people or didn't see someone started to make sense. And, he said, it's scary. Especially to watch his family members follow suit.

"I'm running for them," he said.

Putting one foot in front of the other amid thousands of people running alongside him can be confusing, he said.

Listening to someone instruct his every move gets annoying. He's stumbled before, and the noise alone is overwhelming.

At the finish line of the first marathon he ran, in Chicago in 2010, he was exhausted but triumphant.

He has 10 more marathons looming and $120,000 to raise. But his family will be on the sidelines. And he hates this disease.

"That's why I do everything," he said.

"I'm running for them", he said in Austin, Texas.  Reading the statement above shamed me.  After all, what have I done lately to support the effort to add Choroideremia to the list of 'Cured Illnesses'?  Other than monthly matched contributions to the Choroideremia Research Foundation I make monthly at work...  In a word, nothing.  

I wait for a miracle to happen, a cure to be found, wait for smiling Angels to descend from Heaven as I sleep, nudge me awake and tell me, "It's okay, we've fixed them - no more need to worry - it's all good.  They're okay now.  All that praying you did over the past eighteen years?  It WORKED.  He heard you..."

As of this morning the only nudge I received was the one from the loopy little rescue dog as she bolted up onto and across the bed to throw herself down next to (in truth, 'across') my legs at 5:15 this morning.  This would have been about five minutes prior to beginning this post.  

Maybe she WAS my nudge?  They say that "God works in mysterious ways" but Sandy?  Seriously?  A dog who recycles her food?  

I dunno... 

But what I DO know is that I'm writing this post for a reason.  It is not political.  It is personal and it is time-sensitive.  It is a request for support made on the behalf of roughly 6,000 Americans and for the families of all those affected by CHM.  

If you can afford to help E. J. Scott fund his cause for the elimination of Choroideremia and its 'definitional removal' from the Medical Encyclopedia, I would be eternally (as in, 'For all time') grateful.  

I'm delighted to report that HUGE progress has been made over the past two years.  Researchers are using available funding to develop a workable gene-therapy treatment for this disease AND successful outcomes from this research will extend into other eye-related illnesses.  Several months' ago clinical trials began in the U.K. and are (hopefully) coming soon to the U.S. and Canada.

I'd try to explain how it all works, but being NOT a medical guy, I'd just mess it up and you'd probably get mad at me.  Instead of my own rambling and incoherent thoughts, I'm embedding a video here of Dr. Jean Bennett, researcher at the University of Pennsylvania, so she can overview what's up with her research and its implications for those affected by this, and other retinal degenerative diseases:


E. J. Scott is not only running against the miles involved in associated marathons he's in, he's running against time itself.  

He's running for the hope of a cure and he's running with my family's hopes riding firmly on his back. 

If you can help support his efforts to raise money against CHM, please take a moment to sponsor his race for vision by visiting his site at:   


Via the homepage of the Choroideremia Research Foundation's site at:  CRF 

On the CRF site you'll find a video from E. J.'s recent appearance on the Dr. Drew show and get a chance to meet the man behind the blindfold.

My profound thanks to E. J. and his efforts to put a stake-through-the-heart of this condition.  

If you can assist financially (or by even getting the word out about CHM fund-raising via the forwarding of this post) in searching for a treatment for Choroideremia - please accept my thanks in advance.

Thanks for your time - make it a great day.

Mike Kane

alias:  'Moos'

alias:  'Dad' to Ben, Jon, and Tim

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