Defibrillator Dude

Defibrillator Dude

Since starting this blogging thing three years ago, I have met some very interesting people. Scott Sands certainly qualifies as one. Some of you reading this already know Scott, who is 43 and also blogs about living with Duchenne muscular dystrophy at Scott Sands Alive. I have learned a lot from him, from fighting for the care I need to learning how to look fashionable with a tracheostomy. Plus, it gives me hope to know that you can live into your 40s with DMD at a time when, sadly, guys with DMD are typically only living into their mid-20s at most. Scott has helped me through some tough times, usually by giving me a (virtual) kick in the ass! He also wrote a very nice review of my book.

If you haven't paid a visit, Scott Sands Alive is a must read. People think I'm funny, but Scott is hilarious. He also pulls no punches about this life that he and I and countless others live. It is a life that was in jeopardy for Scott this week, when he experienced some frightening cardiac symptoms.

As I can tell you first-hand, you can be the toughest S.O.B. in the world (and Scott is) but when it's your heart, it's a scary thing! The good news is that Scott is now an official Defibrillator Dude, having undergone successful surgery this morning to implant a defibrillator in his chest. Sounds like he will be back to blogging very soon.

Welcome to the club, my friend. May your heartbeat be regular and your shocks be few!

Fancy Footwork

Fancy Footwork

After trying on pair after pair of new shoes in recent weeks, I finally found what I was looking for (at left). Getting new shoes is no big deal for most people, it is a bit of a challenging for many of us with DMD, whose feet have turned inward as a result of the disease. My old pair had grown molded to the shape of my feet over the years, but they were at least 15 years old and falling apart at the seams, as you can clearly see below.

I was pretty pessimistic that I would ever find anything as comfortable as my old shoes. While being unable to wear shoes isn't the end of the world, it would have been one more thing this disease has taken from me. Why let that happen if I could help it? With my nurse's assistance, I found a pair of wide-width shoes a couple sizes larger than my actual size (it's not as if I'm going to walk in them) and voila, my feet slid right inside. It was love at first, um, feel. I bought them and wore them out of store.

With any luck, my new shoes and I will both make it another 15 years!

PPMD Day 2: Meet and Eat

PPMD Day 2: Meet and Eat

Part 2 of Winheld's World coverage of Parent Project Muscular Dystrophy’s (PPMD) 2008 Annual Conference, July 17-20, in Philadelphia.

One thing I have found about attending conferences is that you do a lot of eating! I certainly did plenty of that today. In between, I met many people (here I with Donna, of Austin, Texas, a big fan of my book) and participated in some very important panels.

First, I was part of a group of medical professionals and patients that discussed issues relevant to the adult population with DMD. Respiratory and cardiac considerations were the focus of the discussion -- I told the group that "I have had pretty much every intervention that exists" -- although we also talked about social issues. The discussion went so well that the hour-long session nearly stretched another full hour.

Personally, I was amazed at how far treatment of the disease has come. Now, some doctors are putting their patients with DMD on the same cardiac drugs that I credit with keeping me alive -- much earlier. Non-invasive ventilation (NIV) has also become much more commonplace. Do I wish that I had such interventions in the years prior to my entering adulthood? Perhaps. But there is so much that medical professionals have learned in recent years that was not known when I was coming along. Even so, I have been able to live as long as I have lived because the doctors treating me did what they knew how to do at the time to save my life -- and it worked.

A few hours later, I participated in a panel of adults with DMD that met with a doctor from the U.S. Food and Drug Administration (FDA) to discuss our views of risks/benefits and desired outcomes of potential clinical trials for DMD interventions. For those of us in the later stages of the disease, being able to keep walking is obviously not the desired outcome, as we have been non-ambulatory for years. So our views are different from those of the parents of younger children with DMD.

The fact that someone from the FDA wanted to meet with adults with the disease is a significant development. For far too long, it seemed that the adult population with DMD was being all but ignored. I found it to be a very emotional meeting because we had a chance to say what has been on our minds for years. I concluded my remarks by telling the doctor the story of how I listened to a father of a young child with DMD at last year's conference plead for help so that his child would not end up in a wheelchair as if that was the worst thing in the world. "Well, what about us? If there's anyone who should be desperate, it would be us. We are much closer to the end of the line and just want to live our lives."

By the end of the meeting, I was completely spent -- and hungry. But first, I met with the other adults on the panel on which I will be speaking tomorrow afternoon to prepare our remarks. The truth of the matter was that we did not need a whole lot of prep time. We have some sharp minds in the group (except for me!) and we're going to do a great job tomorrow.

My long day came to its conclusion with a nice relaxing meal at a nearby Italian
establishment with my wicked funny pal from New Hampshire, Pat Moeschen; (from left) his girlfriend, Ashley; my nurse, Frank; and Pat's mother, Helen . I don't know where the guy gets it from, but even after a long day, Pat still had his usual sick sense of humor!

Exciting Times

Exciting Times

Some things are truly worth the wait. Today, the New York Times story about DMD research and treatment for which I was interviewed several weeks ago finally ran. The article and video can be found here. I thought it was a great piece that highlighted the fact that without a cure for Duchenne's, doctors are now focusing on managing the disease, "making better use of available therapies to eke out longer lives for their patients." There is no doubt that it is this philosophy that has been responsible for keeping me around.

Naturally, I was most impressed with the video segment that ran with the story because it featured me! Just like the print article about me that ran in the Philadelphia Inquirer on Monday, the story was not overly dramatic, but offered an honest account of my life. Not only did it address the medical issues that I face, but it also focused on some of what I've been able to accomplish in spite of my disease.

Still, I would be remiss if I did not raise an objection to the part of the article that described how one boy, whose ability to walk appeared gone forever, regained that ability. While this is a wonderful thing, I think that parents often get too carried away with the fact that their son is going to be a wheelchair. Obviously, no one wants to be in a wheelchair. But the fact of the matter is that you can accomplish much in the wheelchair and I think it's important that parents of children with DMD, one of the audiences targeted by this blog, understand.

Today's world is becoming more and more accessible. And let me tell you, when I started using my wheelchair, it was a tremendous relief. Sure, I was able to walk before that point, but it certainly wasn't easy. I was terribly unsteady on my feet, constantly afraid of falling.

My point here is that, yes, we need to cure all aspects of this disease. But let's not forget that being unable to walk will not kill you, but the pulmonary, cardiac, and nutritional aspects of the disease will.

Perhaps with stories like the one that appeared in the New York Times today, hopefully one day soon, we won't have to talk about any such aspects of DMD because there will be an effective treatment for the disease.

Genetically Speaking II

Genetically Speaking II

How many times can I show a picture of me talking to a class? I decided to change things up and have the genetic counseling students I spoke to today surround me in the above photo. I don't know if you noticed that they're all of the female persuasion. I did, though I didn't happen to get any phone numbers!

Still, they were nice enough to listen to me for an ENTIRE HOUR. Prior to my appearance, the students learned about Duchenne's from one of the doctors at The Children's Hospital of Philadelphia. My job was to share the more personal side of the disease. I used a PowerPoint presentation containing photographs at various points in my life.

But as this was a class on genetic counseling, I also shared my thoughts about genetic testing and pregnancy termination. I took some heat from a few readers for my comments on this to last year's class. I told the students that I would personally not want to bring a child into this world knowing he would have DMD. That doesn't mean I'm rejecting my life or the lives other guys with the disease. There was no genetic testing when many of us came along. Once you're here, I believe you must live your life to the fullest. But nobody wants to have Duchenne's. I would not knowingly want to subject my child to it.

That's just my opinion, though I would think there are others out there who feel the same way. So criticize me if you will, but please respect my opinion as I'll respect yours.

Darius Goes... to Your Home

Darius Goes... to Your Home

Several months ago, I wrote about "Darius Goes West," an uplifting documentary film (see trailer, at left) that follows the cross-country journey of Darius Weems, a then 15-year-old with Duchenne's, with the assistance of a group of loyal friends. You can ready my previous entry here.


Now, after screenings across the country, the film is coming to DVD, just in time for the holidays. To order a copy, please click here.

We all have a part to play in the fight against Duchenne muscular dystrophy, whether it's Darius with this film or me, with my book about my life with the disease. So, just as I will soon be asking you to purchase my book, I encourage you to purchase a copy of "Darius Goes West." Proceeds from its sales go to Charley's Fund, an organization started by the parents of young boy with DMD that is dedicated solely to funding a cure or treatment for the disease.