Please click here to order your copy of Worth the Ride: My Journey with Duchenne Muscular Dystrophy. All proceeds go to Parent Project Muscular Dystrophy (PPMD)

Thursday, July 12, 2007

PPMD Day 1: Part of the Family


Part 1 of Winheld's World coverage of Parent Project Muscular Dystrophy’s (PPMD) 2007 Annual Conference, July 12-15, in Philadelphia.

It's a family that no one chooses to join, but when your child is diagnosed with Duchenne or Becker (considered a less severe form of Duchenne) muscular dystrophy, you automatically become a part of it. That was one of PPMD Founding President Pat Furlong's key messages in her opening address on this, the first day of the conference. Pictured (at left) are me and Frank, my nurse.

It was a message that certainly resonated with me. For years, I tried to ignore that "family." I didn't want to be a part of it at all; I just wanted to live my life. But you can't leave this family (just like the mafia). Sooner or later, it pulls you back in! For me, it was all of the medical stuff I've been through in the past five years that brought me back. It made me realize just how fortunate I am to be here and that I should use my good fortune to make life better for others with my disease.

Honestly, a lot of what was covered today was over my head, but I'm supposed to be an intelligent person (though that is debatable), so I gave it my best effort to follow the proceedings. One thing I did understand was the excellent analogy offered by Dr. Steve Wilton of the University of Western Australia (gotta love that Aussie accent) presented an analogy, likening the lack or shortage of the dystrophin protein in the muscles of someone with Duchenne's or Becker, to a tractor with a faulty shock absorber. As the doctor explained, the tractor might work for a little while with the bad shock absorber, but eventually it will break down. But how do you do remedy the problem? Do you try, for example, to repair the shock absorber? Do you try to replace it with something else? This is the dilemma that scientists face in their quest for a cure.

Dr. Richard Finkel of Children's Hospital of Philadelphia (who also happens to be my neurologist) spoke about how doctors prove the benefit of a potential treatment. For example, walking 30 feet in a given amount of time may not mean much in one's daily life. But if a treatment improves a child's ability to accomplish that task, that may be one way of measuring that treatment's effectiveness.

One important point I took away from today is that researchers are not looking for a single treatment, as many people might think. That's because the genetic mutation in those with Duchenne's isn't the same from one person to the next. In some people, material is missing, whereas in others, the order is wrong. So there may be a need for four or five treatments, each tailored to certain portions of the Duchenne's population.

Last but not least on this day, I nearly killed myself (and my nurse) boarding the train on the way home! You see, getting into the passenger car requires me to make a sharp turn, while at the same time trying to get over the threshold. Well, as I made that turn, I must have turned too widely because I got stuck, dislodging my arm rest. Frank put down my bag and helped me get across the threshold. He then turned around to pick up the bag. At the same time, I inadvertently knocked the left arm rest into my right hand, throwing the wheelchair into reverse at full speed -- right out of the passenger car and into Frank! Then, as we tried to get me back in, the train started moving. It was quite embarrassing, to say the least. Better luck tomorrow...

2 comments:

djb said...

Hello,

I met you at the conference today and want to thank you and your fellow presenters for letting us in to the normalcy of your hardly average lives. You guys are great and a model for just the way I'd like to deal with my life and any adveristy that might come into it.
thanks for the pep talk - and thanks for the blog.


Deb Brown

JB said...

SEPTA has some trains maybe just for R1 that are like French doors that open in the middle. I don't know why they can't have them on all trains. I don't know if you have to make a sharp turn to get into your seat.

It's like when I went into Temple U bathrooms with you that were difficult to get into with those narrow halls and right-angle turns. I wouldn't have known what it was like to get through them with a wheelchair otherwise.

Do the people who design these things do it without consulting people in wheelchairs?

P.S. Next time you're in Doylestown, check out the crossing signal traffic lights they've installed. You have to manually push to cross one way, but not the other, on some four-ways. These lights don't always work. I can't wait for some blind person to be there by themselves and have no idea what is going on, or not be able to cross because the lights don't work, so they will sue. I especially like the one near the train station. That one has a big black metal box that juts into the sidewalk. It's at a perfect height to be in the way. You probably couldn't navigate it in a wheelchair. That's a lawsuit waiting to happen -- or does that fall under sovereign immunity?

JB