research

Therm-a-Rest Founder Offers Unforgettable Gifts to CMT Community

bestfootforwardweb3 Comments

James Lea

It was in the winter of 2012 that we met the elderly man sitting in a bulky hospital-style wheelchair, covered from shoulder to toe with a plush blanket. Jeana Sweeney and I had flown to Seattle, WA to speak at a local CMTA branch meeting. We had just begun the presentation when a bright and lively young woman entered, pushing an old man to the front of a fully-packed room.

He put “Jim” on his nametag. Jim listened intently to our talk, not missing a beat. He even asked a few questions with his quivering, shallow voice, which betrayed an inability to completely catch his breath while speaking. He must have been around 90 years old, 100 even! And, he was obviously affected by decades of living in a body with CMT, but he forged onward.

At the end of the meeting, once  Jeana had finished her pep talk about CMT awareness and fundraising, a few people hung out to ask questions, socialize and munch on home-baked sweets!  While I was speaking to the branch leader, stuffing brownies into my mouth, I spied Jeana who was making a most definite beeline for Jim and his caretaker. Hmmmmmmmmmm…

45 minutes had passed and I was more than ready to hit the road and make our way back to the airport. Most people had left, but Jeana was still chatting away with Jim and his caretaker. As I was packing up my stuff, I overheard Jeana ask, “Have you ever thought about getting involved or giving to the CMTA?”

“OMG, really?” I thought as I struggled with the zipper on the projector pouch. “Really? Tell me she didn’t. Did she just ask that elderly man we do not even know for money? Seriously, we have to have some boundaries here. She’s great at fundraising and all, but taking advantage of the elderly is not right!” I made a mental note to talk to her as soon as we were out of earshot.

On the way back to the airport, I expressed my concern. Jeana just started laughing. “You only heard like one tiny part of our conversation, Elizabeth. And I was not taking advantage of anyone! I can’t believe you would even think that of me!” she added with a serious scowl.

Long story short, Jim or James Lea is known for his invention of the Therm-a-Rest mattress. If you do any outdoor activity or camping, you’ve probably heard of Therm-a-Rest! Throughout the conversation, he willingly mentioned his philanthropic activities and admitted that he wanted to help people with CMT. Having been diagnosed with CMT early on in his life, he wanted to give back to a cause close to his heart. But first, he had to do some homework.

thermarest

He asked Jeana if she would be willing to go back to Seattle and spend a couple of days with him to talk about the CMTA, its mission, and financials. More than anything, he wanted to get to know Jeana better, to understand her values, her work, and her purpose.

Jeana ended up spending 3 full days with James, from 9am-7pm  with James, who was still very busy with his various business ventures. Later, I called James and interviewed him. Here is the article I wrote for the CMTA Report:

James Lea is one of the original founders of Cascade Designs and the developer of Therm-a-Rest, the world’s first self-inflating camping mattress. Born in Tacoma, Washington, on October 22, 1920, this spritely nonagenarian shows few signs of slowing down any time soon. When asked what he does in his free time, Jim was quick to respond, “What free time? I am very busy!” When he is not working in his office, he tries to remain as active as possible. He enjoys being on the lake and working on boats, always trying to improve their function. Jim also put a lot of emphasis on taking care of himself by eating right and maintaining good lifestyle habits. Once in a while, he might even play his ukulele, which is increasingly difficult due to his CMT. Yes, Jim Lea has CMT, as did his siblings, father, and grandfather, who got around using two canes.

Jim was in his thirties when he first noticed signs of the disorder, “When I walked, it felt as though my socks were bunching up under my feet.” It was not until he was in his late 50s that neurologists from the University of Washington officially diagnosed him with Charcot-Marie-Tooth. As a successful engineer and businessman, Jim has managed his CMT over the years. “Truthfully, I just try to ignore it, work around my difficulties, go ahead with my day and do the best that I can, every day.”

 

Jim Lea “poses” for a picture. Later he admitted, “This picture was a hoax! I never rock climber in my life. Look at the shoes I am wearing!”

 

 

 

 

 

Forever inquisitive, Jim has also done quite a bit of research on CMT to better understand treatments, therapies, and current CMT research. Choosing to accept his CMT as an undeniable presence in his life, Jim never shied away from telling others about the heritable disorder passed on from generation to generation in his family. In fact, just last year at his 90th birthday party, he took it upon himself to spread awareness of CMT by passing out brochures and educating his guests about the disorder. How I admire James Lea and his positive, upbeat attitude! Despite the fact that CMT is affecting his breathing, his hands, his feet, and body, never once did I hear him complain or lament about his struggles. Moreover, encouraged by recent progress in the CMTA’s STAR initiative, he does hope that treatments are forthcoming for our younger generations.

His message to younger people with the disorder is to “stay strong, accept the condition, and find alternative ways to achieve your goals and dreams.” Not missing a beat, Jim also hopes that scientists will hurry up and find a way to create … another Jim Lea, at 65, who could do some of the many things he still wants to do in this lifetime! At 91 years old, Jim Lea is one of the most positive and upbeat souls I have ever had the opportunity to meet. His never-give-up attitude, inquisitive mind, and perseverance are most admirable, making him the legend and role-model he is today.

Jeana passed all his tests with flying colors, as did the CMTA.  Jeana and Jim created a true friendship which grew and blossomed right up until the day he passed on December 20, 2016. When talking on the phone was no longer possible due to hearing loss, Jeana sent him cards and letters, always making sure to include one of her daughter Rylee’s drawings.

Jeana Sweeney and her beloved Rylee.

James Lea gave a large part of his estate to the CMTA after his passing as he not only believed in the work of the CMTA but also in one of the Association’s most well-known, hardworking and genuinely caring CMT advocates – Jeana Sweeney. Jim will remain in our hearts forever, and his investments into the CMTA will help many for a very, very long time.

 

12 Compelling Reasons I’m Talking About CMT This September

bestfootforwardweb1 Comment

Top Twelve Reasons I’m Talking About CMT during Awareness Month

 

12) Confused Faces-When I tell people my son has Charcot-Marie-Tooth disease, I get looks like this:

Let’s stop the nonsense. I’m looking for reactions of recognition, like Dr. House’s below:

11) Dentures? Besides having had too many cavities, crowns, and pulled teeth, there is absolutely nothing wrong with my choppers. Yohan has beautiful teeth and healthy gums, too.  Dr. Howard Henry Tooth discovered this progressive neuromuscular disease at just about the same time as the French neurologists,  Dr.  Charcot, and his disciple,  Pierre Marie. So now we are stuck with Charcot-Marie-Tooth or CMT.

10. Eponyms. Jean-Marie Charcot is known as the father of modern neurology. And, he made sure no one would ever forget his legacy. Why? He was generous enough to share his last name with a host of other diseases he unraveled:

An external file that holds a picture, illustration, etc. Object name is 46fig1.jpg

 

Here is the short list of the master’s eponyms:

 

Sounds a bit narcissistic, don’t you think?

9) But, It Could Be SO Much Worse! 

I stop my complaining when I remember that the name COULD have been definitely much worse.  Why? One of my good doctor friends, a most reliable source, explained that Dr. Nikolaus Friedreich, as in the neuromuscular disease Friedrich’s Ataxia,  also wanted credit for the discovery of CMT, but the message of his discovery did not reach the authorities in time….something to do with an unannounced closing of government offices. So the Grand Poo-Bahs did not receive the important Carrion Pigeon or telegraph messages. Bummer for Nikolaus and hurrah for people with CMT everywhere! We don’t have Charcot-Marie-Tooth Freiderich disease (CMTF), but rather just CMT.

Thank God for small miracles.

8. Let everyone know that CMT does not stand for:

Country Music Television

Childen’s Musical Theater

Certified Massage Therapist

7.  Pronunciation: Give others ways to remember the name, pronounce like:

\(ˌ)shär-ˌkō-mə-ˌrē-ˈtüth-\

or

or

6. . Shark’s Teeth Convention? Once, I wanted to book a large room for a CMTA conference. The short discussion went something like this: “Hi. I need to book a meeting room for the Charcot-Marie-Tooth Association.”. “Okay, let me see. What is the date of your Shark Tooth meeting?

Carcharias_taurus_teeth (1)

Enough said. Ugh.

5. Rent a Costume or Let Your Pets Do The Dirty Work!

If you feel awkward about starting the CMT discussion, you and your animals can dress up like a shark – it’s fun and you are bound to get s few questions! Or, just paint your horse with non-toxic paint. People will ask…..trust me.

apple

4. Braces – Tell people you wear braces and when they look at your mouth, lift up your pant leg and flash them with your one-of-a-kind ankle-foot orthoses (AFOs).

 

 

 

 

 

 

 

 

3. Genius!

Wow your friends with your knowledge of medical jargon. Use CMT-related words like:

peripheral neuropathy, autosomal dominant, hereditary motor and sensory neuropathy, dorsiflexion, plantar flexion, pes cavus, myelin, axons, exome sequencing, orthosis, etc……

They’ll be stunned by your brilliance.

2. Participate! The CMTA makes it fun to celebrate and talk about CMT during CMT awareness month.  We have an entire interactive site dedicated to CMT awareness: https://www.cmtausa.org/community-powered-awareness-month-2018/

Or just send around the How Much Do You Know About CMT Quiz? 

Click here to take the quiz: https://bestfoot4wardblog.com/2018/09/02/its-cmt-awareness-month-how-much-do-you-know-about-cmt/

Be the expert on questions they can’t answer. Teach a friend and make good use of your never-ending CMT knowledge!

 

1) Acceptance-Whatever you do, talk about your CMT. Share your experiences with people who might not know about CMT. Most people want to know and CMT Awareness Month provides a platform to share resources and stories in an effort to shed light on this progressive neuromuscular disease, its symptoms, its effects.   Knowledge is power. Go forth and tell someone!

 

CMT: So Much More Than Hands and Feet!

bestfootforwardweb3 Comments
Yohan gets Rylee’s mind off her knee pain.

 

“What’s up with the knee brace?” I asked 12-year-old Rylee, who was visiting from Pennsylvania for a couple of days. Head down, in a whisper, she told me, “My knee keeps popping out of joint. It hurts really bad when that happens. ” Just the thought of having my patella on the side of my leg for any extended amount of time makes me cringe. Yuk!

 

I will not stand for this. Will you?

Helping Yo with his drops!

“Ugh. Can you put these drops in my eyes?” Yohan groaned. He had just had eye surgery and needed drops every hour, on the hour. I almost said, “Buddy, you can do that yourself.”  And then I remembered his tremor and weak grip strength.  “I tried,” he grimaced, with water streaming off his cheeks, “but I missed!”

I will not stand for this. Will you?

Bethany and Elizabeth

I dread the thought of Bethany coming down with a cold because coughing takes energy and congestion robs her of the precious air needed to breathe effectively. Did you know that CMT can affect the nerves leading to the respiratory muscles? For some with CMT, the thoughtless act of breathing freely becomes a forced, challenging and anxiety-provoking undertaking, which may necessitate frequent visits to the ER. Not fun. Not fun at all.

I will not stand for this. Will you?

So, you see, CMT is much more than a funky way of walking, foot drop, high arches, and leg braces. CMT may also causes cramps, falls, balance difficulties, cold extremities, nerve, muscle and joint pain, curled hands and toes, altered reflexes, extreme fatigue, sleep apnea, hearing loss, etc.  In short, CMT is a bitch!

I will not stand for this. Will you?

The most amazing thing about CMT is that we can fight it. Unlike many diseases, the genes that cause many types of CMT are known. The CMTA’s scientists are working tirelessly to stop or slow down the progression of CMT, and they are making headway. We’ve accomplished so much since 2008:

 

Highlights

• Clinical planning has started. Working together with the NIH and the MDA, we are developing our clinical infrastructure: new centers, more clinicians, patient information and history over time.
• We are developing ways to measure disease progression in people with CMT.
• We are starting to use cellular and animal models of CMT to help pharmaceutical companies test their drugs quickly.
• We are partnering with pharmaceutical companies and labs to apply breakthrough genetic therapies (like gene therapy, RNAi, CRISPR) to CMT.
• We will continue to expand our program to cover more forms of CMT.

Join me to raise the funds to bring a drug to market and rid the world of CMT. Big goal? Well, maybe, but why not dream big? We’ve got to start somewhere.

WE will not stand for this.

Imagine what we can do TOGETHER! Did you know funds are what stand in the way between us and a treatment for CMT? Not scientific understanding. Not resources. Not clinics. Not patients. MONEY. I certainly don’t have all the money needed to bring a drug to market, but with your help and the help of others, the money can be raised to support scientific research. It’s doable.

Chris Ouellette-The organizer of the Cycle 4 CMT

Want to help? Our family has put all its efforts into Vermont’s Cycle (And Walk!) 4 CMT event and thanks to friends and family like you, we’ve raised close to $650,000 over the past 4 years. That’s not chump change, friends.

We are now preparing for the 5th Annual Cycle (and Walk!) 4 CMT event, to be held in Charlotte, VT on Sunday, August 26. Every dollar counts. Any donation is valued. Spread the word.

To join our efforts to build a better tomorrow, check out our website: www.cycle4cmt.com and donate a little or a lot.

 

Here is how to start changing lives:

1) Register for the Cycle (and Walk!) event and/or,
2) Fundraise! Tell others why this cause is important to you and/or,
3) Spread the word and register a friend and/or
4) Sponsor a rider, a walker or the event itself and/or
5) Sign up for the after-party.

Thank you for offering a promising future to Yohan, Rylee, Bethany and the 2.8 million + people around the world with this debilitating disease.

Never Give up or Give In. Just GIVE!

 

9 Surefire Ways to Feel More Positive

bestfootforwardweb7 Comments

Everyone gets down from time to time. When I am not in a good place, I try to think about something positive and uplifting. Sometimes, well okay, often,  my mind brings me to unusual places, which make me laugh. Let me share 9 of my thoughts with you. Do any resonate?

You are a miracle.
  • You are all winners. I often find myself saying, ” I never win anything.” Then, I thought about this statistic: According to scientists, it is estimated that we have a 1 in 400 trillion chance of being born. 1 in 400,000,000,000! So, let’s say that there are currently about 7.5 billion people living on the earth right now.  Let’s create  553 planet earths, each with 7.5 billion people.  Now, you have to randomly choose just 1 person from any of the 553 planets.  That person is you! That’s how incredibly lucky you are to be alive today. Crazy, right? We are all winners in the lottery of life.

 

  • Freedom rocks. There is so much beauty in the world and most of us have the ability to enjoy the air, sky, animals, friends, etc. I often think of those in prison, in solitary confinement or people who live in countries where freedom is severely restricted. Take a minute to take in and appreciate the tiniest of detail in your surroundings. Then celebrate your freedom by dancing, throwing your arms up, kissing a loved one. Why? Because you can.

    Appreciate the beauty all around you.

 

  • Positivity. The way you interpret life events will definitely influence your feelings because thoughts create emotion. As master of your thoughts, you can put a positive spin on whatever happens to you, which will improve how you feel on a regular basis. And, if you end up in a slump, remember that you will not ALWAYS be in that same exact frame of mind. Laughter, joy, and happiness are not far away because feelings are temporary and fleeting. The way you feel will inevitably change.

 

  • I’m a modern girl. I am so happy I live in modern times, and not in the Middle Ages. Life does not look so fun back then. Serfs under the feudal system worked long and hard, doing back-breaking work all day long.  Women spent their days spinning wool and making clothes (seriously, can you see me successfully making clothes on a spindle all day? They’d throw me to the pigs). Only  50% of the people lived until 35, which is the average life expectancy of the time. There was no electricity and families lived in one room huts where 1/3 of the space was fenced off for the animals. The hearth in the middle of the room gave off heat, but the air was always smoke-filled. You get the idea.

    Daily life in the Middle Ages

 

  • Cave Girl? No thanks! I’m also glad I was not born a cave girl. With my horrible vision, I would have been TOAST very early on. By the time I was in 2nd grade, my teacher figured out I was very nearsighted. Today, I am considered legally blind without correction. So when I play out my pre-historic times life, I get this scenario, “That pile of leaves looks fun! One, two, three-JUMP!” Then, in midair, I freeze, “Oh snap! The pile of leaves just made a growling noise that sounds just like a sabretooth cat. Good-bye cruel world.”
Sabertooth Cat
  • The internet is amazing! Information 24/7 at your fingertips. And Skype helps connect to people far away, in the US and way beyond. Today, it’s so much easier to research a topic from home instead of going to the library, using the Dewey Decimal system to find a book on a topic of interest.Now, video call a friend or a relative….just make sure you are wearing clothes to be seen in, at least from the waist up!

 

  • This photo makes me nostalgic, but I prefer the internet any day!
  • Changing your first name is totally legal. Seriously, there are some parents who did not think straight when naming their child. Here are a few very unfortunate names I’ve found while browsing the awesome internet:
  • Jack Goff
  • Dick Smalley
  • Ben Dover
  • Donald Duck
  • Phat Ho
  • Preserved Fish (Thanks, Shirley!)

 

 

 

 

 

And, there are many, many more horrible names out there. Many of these names are found on prison warrants for arrest.  Well, what did you think would happen when giving your kids such embarrassing monikers?

  • Evolution is Cool For example, giants do not exist. Bare with me on this one. I always wonder how many ants I’ve unknowingly killed. To an ant, I am a huge monster, with big, murderous feet. I am so thankful I am not a tiny ant, and even more grateful that giants exist only in folklore. Can you imagine if we had to be worried about being crushed by giants roaming the earth? That would totally suck.  And on that same note, I must confess that I am happy the dinosaurs are extinct. I just could not deal with knowing that at any time, I might come across an enormously fast lizard with little tiny hands reaching out to devour me for lunch.

    Giants are fake.
  • CMT Sucks!  (Joyce Steinkamp, thanks for the lollipop idea!)CMT stands for Charcot-Marie-Tooth disease-a progressive disease of he the peripheral nerves. Know if you have CMT, there is much more information, research, progress and awareness of this very common, but rare disease than ever before in history. And our researchers are working day and night to provide treatments to stop CMT. Bottom line – hope is real. But, believing without acting will not get us over the goal line.  So, if you really want to feel good about yourself, get involved in a charitable cause like the CMTA by volunteering, donating, publicizing, joining igive.com or amazonsmile.com (choose CMTA as your charity of choice). Be part of the solution – cmtausa.org

Jeana Sweeney, this image is for you. xoxo