As the title says, September is CMT Awareness Month. It seems like every month is awareness month for some horrible disease. I can’t (and won’t) write about them all. But this one is special. CMT (or Charcot-Marie-Tooth Disease) is, according to CMTAUSA.org
“the most commonly inherited peripheral neuropathy and is found worldwide among all races and ethnic groups. Discovered in 1886 by three physicians, Jean-Martin Charcot, Pierre Marie, and Howard Henry Tooth, CMT affects an estimated 2.6 million people.”
Before I suggest you support research for the cure for this disease, I’d like to tell you a story. My story.
When I was about five or six years old, I was diagnosed with CMT. It wasn’t a difficult diagnosis for the doctors to make. My father has it. Many of my aunts and uncles on my father’s side have it. And the type we have is not something that tends to lurk within you, rarely showing its face. It begins to affect you when you are young, when your muscles are developing, and your bones are malleable, causing atrophy and deformation. Left unchecked, or in particularly bad cases, it can confine the child (or a the adult if it is late-onset) to a wheelchair for the rest of their lives.
A week after my eighth birthday, I had major surgery on both feet to correct issues that would have eventually caused me to have club feet if not treated. I spent six weeks in a wheelchair with massive plaster casts from the tips of my toes to the bottom of my knees. These surgeries involved muscle and tendon transplants, and bordered on the experimental. I was told (though I can’t say this is true), that I was the second CMT patient in the world to have those surgeries done. The surgery was a huge success. I went from constantly stumbling and falling down, to being able to walk and run (although slowly). I played baseball. I cross-country skied. I did need follow-up surgeries when I was 12 and 16 to correct further issues with my feet and toes, and then had another pair of surgeries last year to deal with my toes yet again, but that first surgery was miraculous.
If you look at me today, standing still, you probably wouldn’t know that I have CMT. But as I begin to move around, you might notice a few things. I tend to lift my knees higher than most people when I walk. The muscles in my feet and ankles have atrophied so that my foot “drops” when I walk. If I don’t lift my legs like that, I’ll stub my toes into the ground and trip over my own feet. The high leg-lift quickly fatigues my leg muscles. To reduce the fatigue, I normally wear carbon-fiber braces for my feet when walking around a lot. If you see me in shorts (without the braces) you’d also notice my ankles are abnormally skinny. My calves look like inverted champagne bottles.
You’d also, after a while, notice my thumbs don’t do a lot. I have about one pound of grip force with both thumbs. A normal adult is capable of around 25-30 pounds of grip force. I tend to pick up small objects between my index finger and the back of the first knuckle on my thumb. It doesn’t always work, but I’ve learned to adapt. Handshakes are somewhat awkward as well, as my thumb tends to be in the way when extending for a greeting. I also have problems holding pens or pencils, especially if they are skinny or slippery. I rarely write more than my name these days. I type everything.
You’ll also notice that my balance is a little shaky. You won’t see me walking the high-steel of a construction site. Heck, you won’t even see me on a balance beam two feet off the ground. My muscles just don’t respond at the rate needed to keep my balance in critical situations.
I’m sure there are other parts of me that don’t work exactly right because of the CMT, but I don’t notice them on a daily basis. I’ve adapted, and, to a certain extent, overcome this disability. Rarely, as a teenager, or a young adult, did I hide behind my disease. In fact, I think I often pushed myself harder because of it. I didn’t want to admit that I had a disability. I played a lot of intramural sports in college—basketball, soccer, softball, flag football. I didn’t play them particularly well, and many times I had to deal with a lot of heckling from friends and from other players who just thought I was clumsy or just plain sucked at sports. I did suck at sports, but I was playing, and that fact exceeded all of the expectations of the doctors who first saw me when I was 8 years old.
As an adult though, I did begin to slow down. You just don’t play a lot of team sports as an adult (unless you are a professional athlete). But I still golfed, bicycled, swam and hiked, played pool and video games (which is actually therapeutic for my hands). It wasn’t the CMT that brought those activities to a screeching halt. It was having twins 5 years ago—twins who, thankfully, do not have CMT. Of course, now that I’m not as physically active, it is harder to stay in shape. CMT patients have to work harder to maintain their physical condition, and I’ve been slacking there for the last few years (for good reason for the last year and a half or so). But as soon as I can start working out again, I will be.
CMT has limited me. I do not dispute that. But it has also shaped me. I spent a lot of time on computers as a child while recovering from my surgeries, and I now make a good living working with computers. I read a lot of books as a child because I didn’t play hockey in the winter like all my other friends did. Now I write novels. I don’t think I would appreciate stretches of good health as much as I do had I been blessed with it all my life.
What does the future hold for people with CMT? There currently is no cure, though there are doctors and researchers around the world working diligently to find one. Much is known about the genetics of CMT, and every day, they get closer to finding ways to make CMT a disease from the past. In fact as early as 1990, I took part in genealogical studies which began to trace the genetic mutations that cause the neuropathy. The Charcot-Marie-Tooth Association (CMTA) is at the forefront of leading and funding this research, and is an organization I donate to annually. For about a year, back in the late 1990’s, I was even on the CMTA’s National Board of Directors. I once raised $5000 for CMT Research by riding in the Rocky Mountain Cycling Club’s Denver to Aspen Classic, a 200 miles in a day bicycle ride over 4 9000 foot passes. I wasn’t able to finish the ride—it is a brutal undertaking after all, and I just didn’t have it in me that day.
I don’t know if the cure for CMT, when it is found, will ever benefit me personally. Perhaps there will be a drug therapy that I can take that will halt CMT’s progress in my body, or perhaps even reverse it. I’d love for someone to invent a glove that I can slip onto my hand that will augment the strength or nerve impulses my thumbs to allow me to function more normally. I could become a bionic man. Look out, Steve Austin.
But by continuing to fund the CMTA, and the research it does, I hope that this generation of CMT patients is the last generation of CMT patients. Like polio and smallpox, perhaps thirty years from now, we’ll only see pictures of patients in historical archives. The scientific world can move on and tackle the next horrible disease.
To get there, the work needs to continue. If you are looking for a cause to support this year, please consider the CMTA. Perhaps it will be your donation that puts them over the top in the search for the cure.
Got this picture from fellow author CC Humphreys today. It’s the new cover for the US Edition of his excellent novel, A Place Called Armageddon. I reviewed the UK Edition last year. If you like historical fiction, this book is a must read.
If you look really closely at the back cover, (double-click to enlarge) you’ll see a familiar name. It’s my first appearance in a book like this, and I have to say, it’s pretty damn awesome.
Also, if you are in Seattle on September 23 at 2:30 PM, stop by the Elliot Bay Book Company to hear Chris read from his book, and, I assume, sign copies of his books. Chris is a fantastic speaker, and is well worth the time go and see.
Fresh from our little backyard garden this afternoon.
I think we’ll be eating a lot of salads this week.
It’s old, and the clothes are, well, old. But damn, this is guitar at its best.
In a previous blog post, I wrote about how watching all the TED Talks on NetFlix changed what I thought were the most important issues we, as a global community, had to deal with. This is the first in a series of blog posts I’m calling “FutureView” covering those topics in a little more detail.
Unless you’ve been hidden under a rock somewhere for the last ten years, you’ve heard all about climate change and global warming. There are still, unfortunately, portions of society who deny that this is really happening (though those portions are growing smaller). And, even more unfortunately, there are even larger portions of the society who deny that humans have been responsible for this change. The world is a big place, right? There’s no way we puny little humans could have an impact on a global scale.
To that view, I say, “Codswallop!’” I’m not going to spend any time going into the science of what we have done to this planet. The science is sound. The research has been done and all the reputable scientists agree. If you disagree at this point, you’re either defending the position because your job demands that you do, or you’re a complete nutter. Climate change is happening, and humans have caused a great deal of it. It’s time to own up to our crimes, pay the piper, and start fixing this mess. We’ve been a greedy, destructive pestilence upon the land, and if we don’t do something, and do something soon, we’re in big, big trouble,
So what do we do? Where do we start? I suggest starting locally—with your own habits. There are hundreds of thousands of ideas out there. Much can be accomplished by just changing our daily routines. Substitute re-usable or biodegradable containers for disposable plastic bags when you pack lunches. Require that the next vehicle you buy at least double the fuel mileage your last one got, or better yet, go plug-in hybrid. Put up solar panels on your roof. Encourage your company to allow for more work from home if possible to get more cars off the road. Buy organic food. Reduce your consumption of meat. Pass along other, simple, environmentally friendly tips to your friends and neighbors. Keep an open mind, and always try to do better today than yesterday.
Actions taken locally will help on the global scale. But there are more global efforts that I’d like to see really take hold. From watching the TED talks, I learned just how important our oceans are as a source of food and as a global climate management system. We’ve chewed through 90% of the large fish in the oceans—the tuna, the cod, the sharks, the whales. Our fishing methods have left thousands of square miles of previously productive ocean bottoms as nothing more than a muddy wasteland. We’ve poisoned the world’s populations of sea birds, turtles and reef fish with all the garbage we’ve dumped in the oceans. The carbon dioxide we’ve spewed from our exhaust pipes, smoke stacks and industrial farms has acidified the oceans close to a tipping point that will soon cause the remaining fish will die from lack of oxygen, and cause disruption of the major currents which redistribute warm and cool waters (and hence nutrients) around the planet. We’re this close to a complete disaster.
Yet there are things that can, and should, be done by the world community, and done immediately. Here are a few, in no particular order.
- Create massive ‘reserve’ areas in each ocean where no commercial fishing, no polluting (i.e. no drilling, no bilge dumping), no heavy ship traffic is allowed. We need a minimum of 25% of the oceans to be in such reserves, and they need to be in the areas where the spawning / breeding is taking place. This includes the mangrove shallows along the coasts, and critical reefs wherever they may be. Where reserves have been previously created, incredible improvements in the fish population has occurred within 5-10 years. With a worldwide, and immediate, effort to create these reserves, the fish populations could be on their way back within 15 years, and restored within 25.
- One of the single best commercial ideas I saw during the TED talks was the the one given by Mike Biddle on ways to completely recycle existing plastic. He’s turned this concept into a company called MBAPolymers. If this approach works, at least one of these recycling processing mills should be created in every country around the world, and near every big city. By recycling all the plastic we have already created, we could dramatically reduce the need to drill for more oil to feed our plastic monster, and clean up both our land and our seas. We need to make the recycling industry a priority in our communities. During World War II, the children of the US went door-to-door, and scoured every abandoned lot for scrap metal. Can you imagine a day where the children of the world searched for bits of plastic instead?
- Use the might of the navies of the world to enforce a strict “No-shark fishing” policy worldwide. Sharks are much-maligned, but critical members of the ocean ecosystems. Perhaps the most critical. Millions of sharks are killed each year for their fins to add to bowls of soup or as part of Asian remedies. This is an insane practice that is decimating the oceans, and must stop. We need a global push to vilify societies that persist in this barbaric practice, and to begin serious economic sanctions against countries who do not act.
- Use those same navies (and remote, autonomous aircraft launched from their decks) to patrol the oceans, hunting for ghost nets (the thousands of miles of castaway fishing nets that kill millions of fish) and large garbage gyers. If a spy satellite can read the license plate on a moving vehicle, surely those same satellites can be used to locate/track these nets and this garbage. We have spent trillions of dollars worldwide building armies and navies intent on destruction. But it seems their work is already done for them. The world is already on the brink of destruction. Why not use those people, those skills, that organizational structure, that technology, to save the world, instead of killing it? Fiscally, and environmentally, this just makes sense.
- The limiting of some types of fishing will undoubtedly cause hard times for those fishermen who have previously supported their families through fishing. We must give them an economic alternative to those activities. Why don’t we put them to work cleaning up the oceans? Have them scour the coastlines for garbage and bring it back for processing (perhaps at a recycling plant from #2 above). Have the bigger boats make trips out to the Pacific Garbage Gyer to begin the process of cleaning that up. Of course, the particulates there are small, and new technology must be found to make this possible, but there has to be a way. We have to find a way. Providing incentive to begin this clean up will spur this type of research. For those who say this is not economically feasible, understand that a) we already pour billions of dollars into underperforming fisheries to subsidize the livelihood of these fishermen, b) cleaning up the oceans while simultaneously cutting back even further on fishing for a few years will allow the fish stocks to recover even more quickly. This is a win-win. My concerns with this idea have only to do with the environmental economics of having fossil-fuel propelled boats out on the ocean creating more pollution than they are cleaning up, and that with this kind of money at stake, there will be those who will work the system to claim funds that don’t actually help the oceans (i.e. turning in garbage from on-land instead of spending the time at sea, truly cleaning up).
Perhaps these views are too simplistic. Maybe I’m just dreaming. But at the end of my dream, we emerge from this ecological nightmare, united as a global population to restore the planet to where it should be, so that our children, and our children’s children, can live in a clean world where their dreams are not encumbered by their parent’s legacy. My dream starts locally, with small changes I can make to make my community cleaner and better, and ends when we all live in a sustainable world.
Six months ago, when I was diagnosed with Guillain-Barre Syndrome, I quickly discovered I would be spending a lot of time in front of the television. There just isn’t much else you can do while you are recovering. But I couldn’t just sit there and watch “Talk TV”. I’m a strong Type-A personality. I had to do something productive. So I set my sights on my NetFlix queue.
First, I plowed through all the movies which had accumulated over the years that my wife had no interest in watching. That took a month or two. I then began watching documentaries, like Ken Burns’ series The Civil War and Baseball, and some others as well. You know, the documentaries you have on your queue that sound like you should watch them, but you know you’ll never have the time. Well, I had the time, and I watched.
Soon, however, the queue of movies began to dwindle, and, as I got back to working part-time, my ability to watch two hour shows diminished as well. Somewhere along the way, I 
discovered that NetFlix had a huge collection of TEDTalks available on line. I began to watch those.
Now anyone who really knows me, knows that I can be a little obsessive-compulsive when it comes to completing things. Back before my kids were born, I played World Of Warcraft, and I became an epic leveler. I couldn’t leave quests incomplete, and I had to do them all. I leveled 9 characters up to level 60 (which was the top level at the time), and spent god-knows-how-many-hours doing it. That stopped when my wife was 7 months pregnant with the twins, and I really haven’t thought too much about it since. But I digress.
When I began watching the TED talks, it appeared as though it would take me a couple of weeks. No problem. I still had lots of time. But what I didn’t know was that NetFlix would continue to release more and more collections of TedTalks. I think I started with 15 collections on my Instant Watch queue at the beginning. As they added more to NetFlix, I added them to my queue. Today, there are 40 collections online, for a grand total of 540 talks. I have seen 539 of them. The only one that I knowingly missed, was one that was completely in Spanish (which I don’t speak), with no subtitles. I’ll go back and watch it if someone wants to be picky about it. Each talk is somewhere between 4 and 29 minutes long, though the majority are between 16 and 21 minutes in length. I have no idea how many total minutes of TED I have seen, but several days worth at least.
So those are the numbers. It’s a lot of watching. And I’m sure, at any moment, NetFlix will add more, and I will no longer be done. Them’s the breaks. But at this moment, I have finished. I don’t remember each one of them, and not every one of them was mind-blowing, but a huge proportion of them were amazing, and some of them were life-altering.
Life altering? Yes. Truly. Before I started watching these talks, I had a view of the world acquired through 40 years of living and working in Western Society, heavily influenced by modern media, a Roman-Catholic education, a B. Sc. in Physics, and 18 years in “Corporate America” (and Corporate Canada). I had fairly strong beliefs in what I thought was important in life, and what was worth discussing, working on and working towards.
And that showed by my selection of which TED talks I watched first. I wanted to see the ones on Space and Technology, cool inventions and adventurous stuff. And those were all pretty good. But as I watched those, I found myself pushing some of the other collections of talks towards the end of the list, as I apparently valued them less, or thought I would find them less interesting.
But it was those collections that have made the most profound impact on my way of thinking about the world, and my life. As I began to learn more about climate change, the importance of the oceans, the effect of truly bringing women into the leadership of society, and the differences between religion and compassion, I began to re-evaluate what I viewed as important. It altered my “Future View”—how I see what is possible, both good and bad, and what needs to happen to steer things more towards the former and away from the latter of those two outcomes.
I can’t cover everything I learned in this one blog entry. But I can tell you what I now believe to be the most important issues we face on this planet. In future blog posts, I’m going to go into more detail about these issues and my views on them. But for now, here is the list. It’s not an exclusive list, and over time, I expect it will change slightly.
1a. Climate Change / Preservation of the Oceans
1b. Women’s Rights and Equality
3. Providing empowering technology to the poor
4. Education
5. De-politicizing Religion
6. Space Exploration
The topics are huge and diverse, but so were the talks. Of course, my original views still present strongly in these subjects. Space exploration will always be very important to me. Luckily, a number of the other things that I think are very important fall into the Climate Change discussion, so I will cover those there.
Not only did these TED talks influence what I think about and how I think about them, they’re already influencing what I do, and how I do things. I pack my kid’s lunch differently to have lower impact on the environment. I vote differently to try to enable more women to lead. I evangelize about the positive effects of early childhood education. Furthermore, I expect that at some point these thoughts will carry over into my career and what I am going to do with the rest of my life. But that’s a whole other story, and one that is not yet even outlined.
At this point, I am going to end my quest to watch every TED talk as it appears on NetFlix. Spending too much time on any one area is never good. Diversity of thought is critical in this day and age, and the TED talks do tend to lean towards the progressive side of the house. It also takes up a lot of time, and I hope to soon be able to spend more time doing, and less time watching.
Regardless of where you currently sit in the liberal-conservative discussion, you can learn a lot by watching these talks. And when you’re done, tell people about what you saw. Because, as they say at TED, these are definitely Ideas Worth Spreading.
I first heard about “Ticket To Ride” via Wil Wheaton’s wonderful show, Table Top. (see video below). Wil and his gang looked to be having so much fun playing this game, that I had to have it. We ordered it last week and got it on Friday.
The kids (who are 5 1/2 years old) couldn’t wait to play it. We worried they were perhaps a bit young, and at first the game was a bit over their heads. We left all the cards face up, and my wife and I helped them with their strategy quite a bit. But by the end of the first game—which took about 3 hours spread over two days—they pretty much had grasped all the major concepts. My son had grasped them so well, he kicked my ass, and I finished last.
We all had a great time playing it, and I highly recommend it. I can’t wait to play it again. It would probably play a bit faster with slightly older kids so you don’t have to try to play three hands at once, but it is still fun for kids who are tired of simple games like Uno. This would also absolutely be fantastic to play with 4-5 adults. Now we just have to find some that want to play it with us.
Joe Beernink 