Katerina is a bright young college student living with Charcot-Marie-Tooth disease. Officially diagnosed at 17 years old, her symptoms progressed rapidly, leaving her little time to adapt to a physical disability, unrelenting fatigue, chronic pain, leg braces, and a wheelchair/scooter.
Although her CMT symptoms pose many limitations, she’s a fighter and continues to go to college, dance, and play a big part in the CMTA’s new young adult community – Compass. She also is a talented writer, aiming to encourage and inspire others who live with chronic illness, pain, and fatigue.
Listen to her podcast, subscribe to her blog. She’s a gift to our community! xoxo
Yohan and me at the VT Cycle 4 CMT in 2019
The Article
“He has what?” I asked when the neurologist mentioned something about sharks and a
tooth. Dr. Sum, the pediatric neurologist was using words like nerves, genes, muscle
atrophy, and progression. I wasn’t able to quite grasp what he was trying to convey, but it
did not sound good. Something was amiss with my 7-year-old son, Yohan, and now this
ailment had a name – CMT or Charcot-Marie-Tooth disease. When he told us this disease
was incurable and progressive, I completely lost it.
Before he left the room, he gave me a card, “Here is the website for the Charcot-Marie-
Tooth Association (CMTA). Call them for a packet of information. In the meantime,
continue with physical therapy, and occupational therapy. I’ll see Yohan in a year unless
something else crops up.” And that was that!
Neither my husband nor I tested positive for CMT, so why is it that my only child had a
heritable genetic mutation causing a life-changing neuromuscular disease? What did I do
wrong? How will we deal with this as individuals and as a family? What does the future
hold?
I would ask myself these and many other questions over and over again, trying to
understand, striving to make sense of why an innocent child, my only child, had to endure
such challenges so early on in life. Initially, I experienced grief in all its stages—denial,
anger, bargaining, depression, and acceptance—and just when I think acceptance
remained strong in my heart, I had setbacks, reverting to anger and sliding down the
ladder once more.
A World Shattered in a Million Pieces
My world shattered into millions of pieces that day, and I never thought we’d be able to
pick up all the scattered bits to rebuild our dreams, our hopes, and our wishes for Yohan.
I quickly learned this reassessment would not be a one-time project, but a repetitive task
taking time, effort, and a lot of soul searching.
Seeing a child struggling with pain, braces, physical limitations, and obvious differences
made me unspeakably sad. My maternal instincts told me to protect, shelter, cajole and
especially do something—anything—to make the world a friendlier, more secure place
for him. The more his self-esteem plummeted and self-confidence lessened, the more I
would try to make his life easier in any way possible.
Yet, kids are resilient and smart. On some level, Yohan felt my fears and reflected them
back by becoming more and more anxious, less focused, and simply put, a very unhappy
child. Something had to give.
What Else Could We Do?
Lightening his load did not seem to be the answer, and neither did catering to his every
need. In retrospect, I was allowing him to be more dependent on me for everything, and
his teachers in school commented on his lack of autonomy and self-motivation.
My husband and I thought long and hard about what was playing out before our eyes and
decided to get some help from a therapist who counsels families on raising children with
medical issues. Intuitively, we knew what measures needed to be taken, but implementing change is hard
and we desperately needed someone to guide us. Slowly but surely, we stopped treating him as different
and let him experience the world on his terms. For me, this was probably the hardest, but most essential
job I had as a parent of a child with special needs.
Tools for Independence
Working together, my husband and I learned how to provide Yohan with the tools needed
to be independent, self-sufficient, tenacious, and optimistic. After numerous
discussions and a lot of trial and error, we got on the same page and worked as a family
towards common goals. My husband started bringing Yohan on camping trips, desert
excursions, and kayaking adventures, treating him like every other kid on the trip.
I changed my mindset, letting him blow off steam on the way home from school, and
listened without judging by creating a safe space for him to open up and talk. Sure, I still
tended to stray at times, fretting over hypothetical possibilities, living much too far in the
future, and being obsessed with “what ifs”—but a shift was taking hold, and overall, life
became more manageable and much more fun.
When all is said and done, Yohan was not the top athlete in his class, so we had the
opportunity to do things a little differently, creating a life full of enriching and rewarding
experiences. Over the years, our motto has been, “Let’s Make it Happen.” We follow our
dreams, live in the moment, cultivate new experiences and live our best lives possible.
Yohan became an expert archer, was scuba-dive certified, visited the Galapagos Islands,
volunteered many hours to CMTA, and graduated from a first-class University and
Graduate school. He is now working in the field of HR for a local start-up company and
enjoying his success.
Yohan is all grown up now!
Encouragement For Parents
If your child/children have CMT, life can still be enjoyable and fulfilling. There is no one
road map to raising a child with CMT, but here are a couple of key concepts I often share:
-Accept (eventually) the CMT diagnosis – it’s the first step.
Talk about CMT with family and friends; don’t hide it.
Help your children describe what CMT is, in their own words, if asked.
Let your children know it will all be okay because it will.
Embrace challenges and praise your children for doing their best.
Create a safe space for your children to talk about frustrations and anger.
Let go and let them live their lives to the fullest, with autonomy and independence.
Laugh heartily and often. Laughter really is the best medicine.
Involve yourself with the CMTA. We have so many resources for parents and kids alike.
Camp Footprint, the CMTA’s sleep-away summer camp for kids with CMT changes lives. Our volunteers make us shine. Get involved and meet forever friends who understand. Neither your children nor you should deal with this alone. We are better together.
I could not be prouder of Yohan. He’s kind, empathic, funny, and engaging. He rarely complains about his CMT, and lives with the knowledge that every day is a blessing, He has a supportive extended family and friends who love him for his authentic self. If there is just one gift with which I wish to leave him, it is the knowledge that he can achieve his heart’s desire. He just has to believe!
– Elizabeth Ouellette
CMTA Board Member, Elizabeth Ouellette
I’ve been volunteering for CMTA for the past 20 years. Here are a few of my most cherished achievements: I created a school-based program, Teaching Kids About CMT, built the national CMTA Branch network, initiated CMT Awareness week, co-founded the Cycle 4 CMT and co-launched the CMTA’s official podcast CMT 4 Me Podcast with my brother, Chris, who is also on the CMTA Board of Directors.
Eleven years ago, late CMTUS founder, Gretchen Glick and I talked about starting the first ever CMT Awareness Week. The 2 organizations (CMTA and CMTUS) worked collaboratively to get the word out about CMT. At the CMTA, I had posters created, wrote email blasts, and asked our branch leaders to spread awareness through groups across the country.
This first CMT awareness week was so successful, that the following year, we dedicated an entire month to CMT awareness, and somehow, Gretchen got American radio host, Shadoe Stevens, to do a PSA about CMT. Listen to this 29-second clip from 2012:
Eleven years later, it is still hard to explain CMT. I read Kenny Raymond’s latest blog post where he addressed this issue, and he brilliantly defines some of the challenges of explaining what CMT is. Thanks, Kenny B. Raymond for putting your thoughts on paper for reflection. How do you explain CMT? Before you answer, read Kenny’s article and then, come up with your elevator speech and share it in the comments. I’d love to hear your ideas!
Kenny Raymond at Cycle 4 CMT, VT
We All Know the Drill
Exploring the Burgeoning Question: “What is CMT?”
by Kenneth Raymond
“Why are you wearing shin guards? You play soccer?”
“What’s wrong with your hands?”
“What’s wrong with your legs?”
Etc., Etc., Etc.
We all know the drill. The answer to the seemingly never-ending questions involves those three lovely letters, C-M-T. And then, the proverbial follow-up, whether it’s a random person in public or even a healthcare provider, requires us to dig deep in hopes of giving them a straight-to-the-point answer that’ll leave them knowing just enough about our disease to remember the name should they hear it again, all the while hoping we give them enough information to know it’s not a tooth disease and that it has nothing to do with sharks.
What is CMT?
“What is CMT?” I’m always trying to improve on how I answer this question. I can easily rattle off some quick factoids, such as CMT is a heterogeneous group of inheritable peripheral polyneuropathies whose name comes from the three doctors who first described it in 1886: Drs. Charcot, Marie, and Tooth; and this name, CMT, has since become an umbrella term that refers to many different inheritable sensory and/or motor neuropathies. Quick and to the point, right? This doesn’t say much about what the disease is though.
Medically, CMT is a genetically caused neuromuscular disease—neuro because peripheral nerve, muscular because the disease in the peripheral nerves causes symptoms in muscles. Genetically caused because each subtype is caused by a mutation in any one of many different genes.
Medically, CMT is an inheritable multisystem neuromuscular peripheral polyneuropathy. Inheritable because each of the genetic mutations that cause CMT are inheritable. Peripheral because CMT is a disease of the peripheral nerves. Polyneuropathy because CMT affects more than one peripheral nerve at a time (poly), as opposed to only one peripheral nerve (mononeuropathy). Neuropathy because peripheral nerve disease. Then, multisystem because CMT can affect hearing, vision, breathing, genitourinary, and much more, in addition to feet/legs/hands.
Statistically, CMT is the most commonly inherited neuromuscular disease nobody has ever heard of. This one is a weird dichotomy unto itself. CMT is a rare disease by every statistical and modeling measure. At the same time, when it comes to inheritable neuromuscular diseases, in totality, CMT is the most commonly inherited. In this context, common and rare can peacefully coexist even if it seems like they shouldn’t.
These above are just a select few examples of how CMT can be described. All of these descriptions are fine and dandy, but not only are these difficult to remember, firing off any of them to Jane Q. Public tends to render confusion about a disease they’ve never heard of. Is there a viable solution—a grand unifying answer, so to speak?
The Elevator Speech
I’m often asked to give my “45-second elevator speech” on what CMT is. My response typically hits several talking points and is usually along the lines of “CMT stands for Charcot-Marie-Tooth disease and is a rare inheritable neuromuscular peripheral neuropathy named after the three doctors who first described it more than 130 years ago. Although rare by definition, affecting only 1 in every 2,500 people, and totaling about 3 million people worldwide, CMT is the most commonly inherited peripheral nervous system disease. CMT causes the peripheral nerves to stop working correctly; and this leads to muscle weakness and atrophy, joint changes, difficulty with walking, and hand issues. Some who have CMT have breathing issues, hearing impairment, vision problems, bladder issues, and GI issues. The disease progressively worsens over one’s lifetime, there is currently no treatment, the disease can’t be cured, and it affects everybody very differently from one another.” Sometimes, people will ask follow-up questions. Other times, we part ways with only a, “whoa,” and maybe they’ll recognize the name the next time they hear it.
My “elevator speech” has been a go-to for many years, having evolved only slightly since my initial CMT diagnosis. It’s very easy for me to throw it out there anytime I’m asked. Does it say enough about what CMT is as a whole that it could be adopted by anybody who needs a quick go-to description? Until a week ago, I thought it did and I thought it could. What changed?
Bicyclists as a Catalyst
Kate Lair and Kenny Raymond
For the first time, I attended the Charcot-Marie-Tooth Association’s Cycle 4 CMT fundraising event held annually the last weekend of August in Charlotte, Vermont. This event is huge and people from all over the place, not just locals, attend and/or participate. I met and spoke with many CMTers. Some CMTers were cycling participants riding a treacherous 40-mile course through the western Vermont mountains even though there were shorter less-challenging routes. Some CMTers were there to participate in non-cycling activities. Some were event volunteers. Some were parents who do not have CMT, but their child does (or children do). Some were members of the CMTA leadership and social media teams.
Some of the CMTers at Cycle 4 CMT used wheelchairs, canes, walkers, etc. Some CMTers wore leg braces. Some CMTers had breathing issues. Some CMTers had severely twisted and deformed feet. Some CMTers had hearing loss. Some CMTers had speech impairment. Some CMTers had <fill in the blank>. I’m confident there were many hidden symptoms that went unmentioned and unnoticed. Without a doubt, it was the most diverse single-source representation of what CMT is that I have experienced in-person. As I spoke with CMTers and as I looked around, it became apparent that my go-to elevator speech is grossly inadequate and under-represents what CMT is.
It’s well established that CMT can and does affect everybody differently, and even within the same family. CMT can cause many things. Not everybody who has CMT will experience all symptoms of CMT. The mix of symptoms, the severity of individual symptoms, the rate of disease progression, and the overall disease severity can be quite different for every CMTer. What one CMTer experiences cannot be used to gauge or to predict what the disease will be for the next CMTer, regardless of subtype. It’s one thing for me to read it, and another for me to witness these concepts firsthand.
Is There a Solution for the Question?
What is CMT? The answer to that question, as it turns out, is quite different for every CMTer. My CMT is different than somebody else’s CMT. CMT, for me, looks quite different than does CMT for another. CMT, for me, is twisted, contorted, crooked feet that have led to tendons tearing requiring corrective reconstruction surgery of my right foot (and upcoming surgery for my left foot). CMT, for me, is weakened hands that easily cramp, a knee that used to dislocate before corrective surgery, bilateral hearing loss, unrelenting fatigue, chronic whole-body pain, progressively weakening upper leg muscles, spine changes (kyphoscoliosis), premature degenerative joint changes, speech/vocal difficulties, and for me, CMT is breathing issues. For another CMTer, CMT is wheelchair dependency, is an inability to hold and use a pen or pencil, is 24/7 mechanical ventilation via tracheostomy, and is total deafness. Yet, for another, CMT is none of these things, or a is a combination of these.
What is CMT? True to CMT, there isn’t a one-size-fits-all answer. The answer to the proverbial question is unique to the CMTer who is asked. The answer is even unique to the healthcare provider and to the scientific investigator. There are wrong answers to the question, such as a suggestion that CMT is an autoimmune disease. While CMT might share symptoms with some autoimmune diseases, such as Multiple Sclerosis (MS) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) for example, CMT is decidedly not an autoimmune disease. Nonetheless, the answer to the burgeoning question is dependent on what CMT looks like for the one who’s giving the answer.
If you were to line up ten random CMTers—somebody who has CMT or somebody whose loved one has CMT, and ask each, “what is CMT?” each of the ten answers are likely going to be very different from one another. The differences are not born of inaccuracy or of a misunderstanding of their disease. The differences instead come from how differently CMT looks for each individual and from how each person individually experiences CMT.
What is CMT? For me, CMT is a cruel and often debilitating neuromuscular disease that looks very different from person-to-person. For me, what was once an easy answer to a complex question, or what was a complex answer to an easy question, has become exponentially more difficult to answer. As a CMTer, it’s easy to answer the question by simply describing what CMT looks like for me. As an advocate, however, I’ve learned my “45-second elevator speech,” while it gives a lot of information, is a disservice to the CMT community as a whole. The solution? I resolve to do better. I have to do better. I will do better.
About the Author
Kenneth Raymond was first diagnosed clinically with CMT1 in late 2002, at the age of 29. He was genetically confirmed to have CMT1A a year later. Kenneth has since devoted his life to studying, researching, and learning all things CMT, with an emphasis on the genetics of CMT as they relate to everyday CMTers. As a member of the Charcot-Marie-Tooth Association’s Advisory Board, Kenneth serves as a CMT genetics expert, a CMT-related respiratory impairment expert, and as a CMT advocate who is committed to raising CMT awareness through fact-based information rooted in the latest understandings of CMT. Kenneth has just published,
Charcot-Marie-Tooth Disease Gene and Subtype Discovery: The Complete Bibliography – Fall 2022 Release: A Desk Reference for the Everyday CMTer, the Practicing Clinician, and the CMT Researcher Paperback which is available on Amazon. Kenny also has an excellent website and blog to check out: expertsincmt.com
Kenneth Raymond is an administrator of several Facebook groups. He helps people with their CMT questions, especially those related to breathing and genetics. The CMTA is fortunate to have such a well-versed, well-educated Advisory Board member willing to work tirelessly for the CMTA community!
Comin’ at you! What do you get when you mix chaotic creativity with organized comedy? You get the #1 CMT Podcast available today: CMT 4 Me. Despite being polar opposites, this brother-sister team brings it all together in an exciting and informative series focused on all aspects of CMT. Meet Chris and LizO (Chris and Elizabeth Ouellette), dedicated siblings on a mission to magnify the voices of individuals with CMT, share their challenges and success stories, and raise awareness of Charcot-Marie-Tooth disease.
Highlights
As kids, they learned homelessness was not someone else’s problem.
Giving back is so much more than pizza and donuts for Halloween.
Chris: One, two, three. Hello, everyone. This is Chris and Liz O.
LizO: We’re a brother/sister team.
Chris: And, on behalf of the Charcot-Marie-Tooth Association, AKA CMTA, we are coming at you.
LizO: Coming at you not from coast to coast, from the East Coast. We’re both in Vermont, and I’m here for a couple more days.
Chris: Nice. We’re at our camp in beautiful Colchester, Vermont on the beach of Lake Champlain.
View From Camp on Lake Champlain
LizO: Yeah, it’s nice.
Chris: So, Liz O, guess what?
LizO: What?
Chris: This is another fantastic episode of our famous podcast named what?
LizO: CMT, the number four, me, CMT4Me.
Chris: That’s right. What is this podcast all about? It is a comprehensive podcast covering all aspects of CMT, the voice of individuals living with CMT, their challenges, and more importantly, their inspirational stories. We will also cover research updates, fundraising, and interviews with the CMTA community, such as board members, branch leaders, CMTA leaders, but overall, an opportunity to spread awareness through the eyes of those with CMT. So, Liz O, I’m pretty excited about today’s podcast. Totally different, right? This podcast is going to focus on what?
LizO: Me and you. Who are we? Why are we doing this? Why are we even doing the CMT4Me Podcast? What is our relationship? What are we all about?
Chris: That is right, folks.
LizO: Should we start?
Chris: Yeah, that is right. There is a lot there, and we’ve been thinking about this for quite some time. We really wanted our listeners to understand a little bit more about Liz O and I and our past, our history, why we’re engaged in the CMTA, as Liz O said, why we do this podcast. What I’m hoping is if you folks have questions, you’re able to send those in, and we can answer them on future podcasts. So, where do we start, Liz O?
LizO: Well, do you want to start by saying how I got involved in the CMTA?
Chris: No, I don’t want to start there at all.
LizO: Okay, well then why’d you just ask me?
Chris: Just because I love to ask you a question and then totally, we don’t do it. So, I was thinking … Let’s start a little bit, right? So, I’d like to go back to kind of our upbringing. We were both born here in Vermont.
LizO: Oh, way back.
Chris: We’re going to go way back to the beginning. Just so our listeners know, I am currently … Actually, I’m going to be 56.
LizO: Oh, now we’re talking about you. What the hell? I just started, me, and then you’re like, “Well, I’m going to be.” It’s not about you. It’s about us.
Chris: Right, so this is the Chris Podcast, and you won’t learn much about Liz O, but you’re going to learn a lot about me. So, it’s perfect. I’m right in my element, right in my spot.
LizO: I should have never agreed to this podcast.
Chris : I know, I know.
LizO: Okay, let’s get going. Come on.
Chris: All right, let’s do it. Go ahead.
LizO: No, go ahead. You have this great idea.
Chris: No, it just made me think, right? I’ll be 56 in July. We were born in beautiful Burlington, Vermont. In reflection, it really made me think, what’s the story? And, what’s the connection? One thing about our upbringing, and I think it really ties into the fact that we’re engaged with the CMTA. We’re engaged with the CMT community. And, we’ll touch base upon our fundraiser, Cycle4CMT, going into its ninth year. But, really there’s a pretty good background in terms of why we are engaged and I think why we give back and where that really came from, right? You see where I’m going with this, Liz O?
LizO: Yeah, I do. I do.
Chris: And, what are some of your thoughts on why I think we have this foundation in terms of giving back to the community in a number of different ways over the years? Where’d that come from?
LizO: Yeah, I think the spirit of volunteerism, it came from … I don’t even know if that’s a word, but-
Chris: I was just going to say, “Is that a word?”
LizO Cool. It’s volunteerism. Anyway, it is today. And, it goes back to our mom who has always been very generous, giving, and not just with her family, but with people she doesn’t even know. This is an example, and I know you have many, many more, but at Halloween, everybody loved our house at Halloween, because she just didn’t give a Reese’s Peanut Butter Cup or a quarter. She would order Domino’s pizza, and everybody would just come and chow down pizza, go out. It was just so much fun, and people remember that to this day.
Chris: I still have friends of mine that are in their 50s, that they go, “Oh, my gosh. How’s your mom doing? I remember those Halloween nights.” And, she also would take the popcorn machine from, at that time, the candy store that our folks owned down on Church Street and be giving out popcorn as well. But, people loved that. Then, I remember we used to have to go out and pick up all the Domino’s Pizza boxes all around the street, because we learned the entire Burlington area. But, it was pretty cool. But, that was her thought, right? Doing something different, right? And, what more could she do? Like you said, there’s so many stories. I think about if you and I ever went with our mom, Bev, to Church Street downtown, one thing is everybody would run up to her. Everybody knew her, right? And, she was always trying to help people, and I remember-
LizO: Not only people that we knew from school, but she knew a lot of the homeless. She knew a lot of the people that didn’t have a lot of money, because that was her focus. She wanted to help this community in Burlington, right?
Chris : Yeah.
LizO: She was always out there.
Chris: That was a full time job.
LizO: Yeah, and she has a degree in psychology, and she would talk to these people. But, for us growing up, I hated it at first. Looking back, I didn’t like it. I didn’t hate it, but it was just, where do we have to have Thanksgiving, at St. Jude’s? This is a rooming house she had with people that didn’t have tons of money. It was Section 8 or whatever. And, we had Thanksgiving, but we learned to accept people of all kinds. It doesn’t matter about their financial background, right?
Chris: Yeah, yeah, and I think it was also her way of telling us, A, be fortunate you have a family, right? And, back to that Thanksgiving is about giving back. So, as a family, we will together give back to the community. And, I do. I remember that. I was like, “Oh, my God.” I remember this Thanksgiving, and she decided that, and we’re around all these 80, 90 year old people. What fun, though? She was so humorous. She dumped all these apples in this big container filled with water, and the elderly folks were bobbing for apples. She looks at me, she’s like, “Your turn.” I’m like, “There is no way I’m sticking my mouth in that water.”
LizO: I remember that.
Chris: But, it was great. It was adding fun, and the people loved it.
LizO: Mom is a lot of fun.
Chris: So, I agree with you. She is a lot of fun, but I remember it was that reflection of leaving and being like, “Though I didn’t want to go, that was a good day.”
LizO: It was.
LizO: Right, I am too, and I think life, honestly, and I know this was instilled in us early on, but I think life is about giving back. It’s about giving back to the community, and I take a lot of pride in giving back. Just to volunteer and to help a group of people is a gift in itself. It just makes you feel good, and you know that you can make a difference, right?
Chris : Totally.
LizO: That’s where the CMTA comes in.
Chris: Yeah, and I think talking about that is the CMTA and just thinking about Yohan and his diagnosis and really how you personally have been involved with CMT and the CMTA for how many years now?
LizO So, it will be 20 years in December of 2022.
2012 – Joined Board of Directors, CMTA
Chris: And, folks, keep that in mind. That is 20-plus years total volunteering. When I say volunteering with Liz O, this isn’t five hours a week. This is 40, 50 hours a week, weekends, never complains, totally engaged. And, this is what her life has become. I will speak on behalf of those with CMT and people in the CMTA, so fortunate to have someone like that driving to advance research, help find a cure, spread awareness. And, the stories go on and on of what she’s done, but that’s pretty in-cresible. In-cresible? You like that, I just made that word up, in-cresible.
LizO: Just go with it.
Chris: I love it. Hey, instead of incredible, that’s in-cresible. Sweet, okay, I got that down. But, anyways, very impressive. That’s giving back.
LizO: So, when Yohan was born, we had no idea. We don’t have CMT in our family, and so he started exhibiting signs early on just with light sensitivity and walking on his toes. At seven, he was diagnosed, and as parents, we didn’t know what Charcot-Marie-Tooth was. We had never heard of it, and there were very few, few, few resources at the time. They gave us the address of the Charcot-Marie-Tooth Association. At that time, we had just moved from France to California, and I decided to go back and get a degree in psychology.
LizO: When I was done with that degree, we learned that Yohan has CMT. So, I’m like, “How can I use this degree?” It was sort of just meant to be. So, I just got involved, and there were two people working at the CMTA 20 years ago. We knew a little bit about 1A and other types, but nothing to the extent. We didn’t have the resources at all of what we have today. So, we started building the branch network that we have now and awareness month. It was so fun at that time, because we didn’t have much. With Jeana Sweeney, we just built out the whole organization to all these fantastic resources and met so many people. So, I do this for Yohan, but I just feel like the CMT community is my family.
Chris: Okay, that’s what it is. Yeah, no, it’s pretty impressive, pretty impressive when you think back. I come back to Yohan’s diagnosis and/or my first involvement with CMT, and I actually do recall. I think it was on a phone call where you said, “Hey, Yohan and CMT,” and my first comment was, “What do you mean? What’s CMT? What do you mean there’s no cure? You know where science is. There has to be a cure, and what does this mean for him?” And, it really took me, I think, quite a while to understand what CMT was and/or how that may affect Yohan. Obviously, you guys are in California, so I didn’t see you all the time. All I could hear are some stories about wearing braces and potential surgeries in the future.
Serial Casted -short-term success
Post Surgery – Foot reconstruction surgery
Chris: Then, you guys would come to Vermont, and he was very young. You really couldn’t notice very many symptoms being expressed, but also knowing that there was this underlying kind of diagnosis that you guys were trying to navigate through and also trying to explain to the family. So, it was really … Then, the fact, I think for me was every summer coming to Vermont, and he would just … Every year, he’s older and older. Then, I could start over a period of time seeing some of these subtle or slight changes from one year to the next. That brings up the components of, at a young age, hey, let’s go for this walk. Hey, let’s go for this hike. Oh, my God. You’ve really got to come in the winter and try skiing or snowboarding. That’ll be cool.
Skiing was a cherished moment in time.
Chris: And, he engaged in those items. Then, I could see over a period of time, it was like, well, I really don’t necessarily have an interest in doing that. Maybe that was his way to express some of the challenges, though I knew he would want to do it. I could just see that over a period of time, based on his symptoms, that got me a lot more closer to what CMT is and really grasping what this disorder is. I would think it’s probably challenging when you talk to someone who doesn’t know anyone with CMT or what that is. It might be hard for them to make that connection to really what it is.
LizO: It a little challenging, because I will say, “My son has this neuromuscular disorder,” and then they see him, and he looks fine. What are you talking about? You’re totally the making this up, but let’s just go through some of the symptoms that people with CMT have. Chronic pain, burning nerve pain, no feeling, because it affects the sensory nerve, so no feeling in your lower legs or your feet, in your hands. Your muscles start to atrophy, because the nerves don’t work anymore, so people get claw hands, claw toes. They have tremors. It can affect your back, scoliosis, kyphosis. Some people are affected with their vocal cords, their hearing. More people than I thought are affected by their breathing. People need surgeries and the feet don’t fit into the high …
Some of the many CMT symptoms
LizO They either have really high arch feet or totally flat feet, and the toes curl. It’s a challenge, and the biggest challenge when Yohan was first diagnosed, they thought he had cerebral palsy. So, I’m like, “Okay, it’s very mild. It’s not progressive.” Then, they came back with a CMT diagnosis, and it’s a progressive disease. So, it’s not going to get better. It’s not getting better. There are treatments like exercise or physical therapy or occupational therapy or surgery, but it’s going to keep getting worse until we find a cure. So, that’s what I’m all about, trying to find research and spread awareness and increase the resources we have. We’re doing a great job in that, but I’m still frustrated that we don’t have anything to stop the progression. But, we’ll get there.
Chris: I think what also hit me really hard in terms of what CMT is, is this was years ago, I think probably in 2014 or so. I think that’s when I joined the board of the CMTA, and I went to a conference in Boston. That was my first conference. So, I’d been kind of exposed to what CMT is with Yohan, not really engaged with anyone else that had CMT and would read stories, positive and negative, et cetera. But, I’ll tell you, going to this conference in Boston, and you were there, and I can totally remember walking out of there that I now have a much better appreciation and understanding of CMT. A lot of things, if you’re not engaged with CMT, I remember the first thing I did. I meet someone over at a table getting coffee, and I go to forcefully do the American awesome-
LizO: Handshake.
Chris: Manly handshake, and I grabbed that individual’s wrist, and I’m serious, I felt like I broke their wrist. They just couldn’t really return that handshake that I was so accustomed to and brought up doing in terms of a standard greeting. Then, I looked around the room, and I could see mothers or daughters together in wheelchairs or people having difficulty walking, et cetera. It was really, really mind blowing. I think that was really the turning point for me, that not only through our family and Yohan, but really starting to think about what else can I do to give back to this community? And, that really prompted a lot of different actions, further involvement for me in the board, more engaged, I think, with Yohan, and as we’ll talk about is that leaning into which I’ll say, our ninth annual coming up in August Cycle4CMT fundraiser that I’m really proud of that’s raised over $1.7 million for research. That story in itself, which leads to a number of different topics, is between you and I, is how that fundraiser got started, right?
LizO Right, and before you go into that, I just want to say I’m looking at that picture in back of you, and I see you and Yohan. Yohan was here in Vermont, and I believe there’s always a silver lining in every situation, any situation that’s tough. The silver lining is that you and Yohan have forged such a strong relationship. He thinks the world of you, and I can’t thank you enough for getting involved, because we felt pretty alone in this disease. For you to come out and spend so much time and energy and get the word out and talk about it and understand it means the world to me. But, it also means the world to Yohan, and I can’t thank you enough. Really, he was here, and you guys went to breakfast. He goes, “I think I just want to be with Funcle Chris,” fun uncle. And, you guys just laughing in sync, it just means the world to me. So, thank you for being involved, and I wish more families would get involved in the lives of others with CMT.
Chris and Funcle Chris 2022
Chris: I do. I love him. He’s so awesome. We have such a great relationship, a lot of respect for him. I can’t imagine not doing some of these things. I think too that that relationship with Yohan and the connection to CMT has also prompted my dedication to CMT, not only for him, but as I’m more engaged in the community, with the three plus million people worldwide, and really trying to think of how we can reach those individuals, how we can spread awareness, and what more we can do any way possible. And, the key is funding research, finding a cure for this disorder at some point in time. I do agree with you, Liz O. I think at some point, we will get there. And, you and I were talking about it this morning. The CMTA is a relatively small association. I think we’ve committed well over $20 million in research to date, but it takes so much money, right? It takes so much money just to get to clinical trials, and then the failure rate of clinical trials is very high. But-
LizO: I think the statistics were it takes between 400 million and one billion dollars to bring a drug to market. But, that doesn’t mean the CMTA has to put that much in. We have really attracted pharmaceutical companies and biotech companies. There are more people today working on CMT than ever before, and it will only keep increasing. So, there’s a lot of hope if we can get them to get more involved or we have something very promising. They can take that and bring it to market, because they have the big dollars, right?
Chris Exactly, and going back, Liz O, too, I do want to talk about the inception of the Cycle4CMT fundraising.
LizO: Oh, yeah, let’s talk about that Bahamas trip.
Chris: Bahamas trip, and folks, listeners, we’ll touch base a little bit upon Liz O and I in terms of our relationship, but what we would do as a family I thought is very important is to travel at least once or twice a year, which I don’t say that lightly, very fortunate that we were able to do that. And, I definitely do not take that for granted.
LiizO Can I say something?
Chris Yeah.
LizO I just want to say that your kids had to follow a strict schedule for school, but we would just take Yohan out of school, because early on, we decided that seeing the world is educational, and we would do as many trips as we could. Because, honestly at the time, and we still don’t know what his capabilities will be in the future. And, I’m really, really glad we did that. And, we did take him out of school. Sometimes, he had homework and everything on vacation, but what he learned and the experiences he had, it’s just incredible, and we’ll never get those back. So, I’m so fortunate to be able to have done that with him, really.
On an unofficial school vacation, Yohan got scuba certified!
Chris: Yeah, it’s cool. And, the fact is right when we would travel, Yohan, he’d do a lot of things. Then, as I stated earlier in this podcast, I would notice he would be able to do less items. He would still have a great time on vacation, but our focus turned a little bit to what was he capable of doing. And, it hit me, and it was 2014. We were in The Bahamas, me, you, Yohan, my wife, Mia, our son, Warren, and daughter, Lila. And, we are sitting there in a Starbucks having coffee, and I was just looking at Yohan, because his feet were all scraped as he was walking around the pool. His water shoes, he had-
LizO: Well, the day before, because I’m so scattered, I got there a day before you and left the day earlier, because I messed up the schedule and the days.
Chris: I forgot that.
LizO: So, that first day we got there, I’m like, “Let’s go in the pool.” And, we had these water shoes. Since he has pinky toes up in claw, we put holes in the pinky toe, and he was out there. He was in the pool and the lazy river, walking all around, and he got out. Since he has no feeling in his feet initially, he looked down. His toes were raw. He had scraped all the skin off his toes, and then the pain set in. That set us up for a limited, limiting vacation, and it was awful.
Chris: Yeah, yeah, no, I remember that. So, we’re sitting there having coffee, and I remember I had just started cycling at that point. I started chatting with you and with everyone there, I said, “What more?” It was going in my mind actually, before I expressed that. I was thinking, what can we be doing, right? I don’t want to just sit on the sidelines. There has to be something we could do. Thank God, you were very knowledgeable because you were engaged with the CMTA and talking about research. I started to think, hey, maybe we put on a cycling fundraiser. And, I have never done any fundraising. I think maybe you were engaged in some fundraising.
LizO: Oh, yeah, I did.
Chris: Maybe it was through branches and things like that, but I was totally new, new to biking. And, I remember saying, “Well.” You asked me what my thought is. I was like, “I don’t know. I just want to tell maybe 10 of my friends, and we’ll try to raise some money, and I’ll go down [inaudible 00:25:59] Vermont and sit on the beach afterwards, and we’ll cook some hamburgers and have a beer or so. Maybe we’ll raise a couple hundred dollars, and I can start there. Then, with the sister/brother team, with your energy, and I think that translated to my energy where, well, that’s not enough, right? What can we do? And, behold, we launched the first Cycle4CMT event in October. It was October that first year in 2014 in Vermont. It was cold.
LizO: It was cold.
Chris: But, it was cool, because we had probably 80 plus participants, and that event was more kind of family and friends oriented. I went out and I posted some cycling routes, and I had these little tiny signs on the side of the road that had arrows to go right or left. I didn’t realize, well, you’re probably at times going downhill, could be going 25, 30 miles an hour. Maybe you won’t. And, people didn’t even see the signs. Everybody went off course.
LizO: Remember, Kevin Thibodeau, he left, and we didn’t even know where he was. And, he came back miles later, not in shape or anything.
Chris: Totally.
LizO: But, you know what’s great is everybody was laughing about it.
LizO: Everybody understood. That’s great. There were two people with CMT, and fast forward, if you go to the eighth annual, we have a lot of people with CMT attending, walking, cycling. It’s really become quite big, and we have an event this year in Wisconsin. Then, we have, well, not an event. We have a ride in Wisconsin, and we have a ride in San Francisco and people doing their own thing. So, it’s really expanding, and it’s really exciting.
1st Annual Cycle 4 CMT
Chris: They were.
Cycle 4 CMT 1st Annual
.
Chris: Yeah, and it’s cool to reflect back, thinking, sitting in the coffee shop, talking about a fundraiser, and where we are today. As I reference, that has just grown. The attendance, we usually at the signature event in Vermont, which is now always the Sunday prior, the week prior to Labor Day, we usually have about 200 plus participants. There are new faces every year. There are more people coming that have CMT. People go out for a ride. We have a great breakfast. We have a full catered meal. We have live music. We enjoy some local craft beer and cider. We always try to bring in researchers, our leading scientists to talk about where we are in the research front. The cool thing is then we just have a great silent auction. That’s very powerful here in Vermont, right? The community is so engaged. Probably, we’ve had at times 100 silent auction items, ranging from people donating skis, to hiking shoes, to biking jerseys, to gift certificates, to hotels, and you name it. That’s a fun, but not an easy feat to obtain those silent auction items.
Paul Kang gives a speech in Juliana’s honor
Juliana Snow lost her life to CMT at age 5
LizO: No, the Cycle4CMT is really a lot of work, and every year after the event, we’re just like, “Should we do this next year? It’s taking so much time.” And, what I remember is you have people like Paul Kang and Stephen Lee coming from Washington and Connecticut and talking about little Juliana who died at five years old from CMT. It’s very rare to die from CMT, but it happens. Stephen flew all the way here, and then we had the interns, Emily and Erin.
Emmily and Erin
Chris: They’re awesome.
LizO: They both have CMT, and they’re so, so motivational and inspirational and just great young women. Then, when we go, “Oh, my gosh, we’re so tired.” You want to tell the story of Riley, Riley who came up to you?
Chris: Oh, boy, I’m serious, folks. I’m sure a number of you have done fundraising. It’s not an easy feat, and I’m not saying that to give Liz O and I credit at all. Like Liz O said, every year, we’re like, “Okay, that was the last event.”
LizO: That’s the last one.
Chris: “That was the last one. We’re totally exhausted.” I know when everyone leaves the event on Sunday, we’re laying on the grass, and now we still have to take everything down. No one can speak. We’re totally tired. But, to me, that’s part of it, right? You have to have some blood, sweat, and tears. These things shouldn’t be easy to begin with, but Riley, who’s so awesome, and-
Riley
LizO: He’s from Vermont, Essex.
Chris: He’s from Vermont, and how old is he now, Liz O?
LizO: I don’t know. He must be 13, 14.
Chris: Yeah, so Riley, and I don’t know how this got out there, but he at the time, maybe he was eight or nine. And, I’m talking to someone at the event, and Riley pulls on my shirt, and he says to me-
LizO: And, wait, wait, wait. So, Riley’s pretty affected by CMT.
Chris: He is.
LizO: He’s a CMT type four, so that’s two genes causing CMT.
Chris: Yeah, he’s in a wheelchair.
LizO: Now, he is is.
Chris: Or, now. He was wearing braces, I think at the time, needed assistance with walking, and made it over to me, and pulled on my shirt. And, I looked down at this little, little boy with his glasses and big eyes. He said, I think he called me Chris, which was great, “Chris, someone said that this might be the last year that you” … Sorry, folks. This is my emotional point. He said, “I heard you might not do this event anymore.” And, I looked at him in the eyes, and I could see his condition. I just was like, “Riley, this will not be our last event.” It just showed me how important that event was to him and to others. I think that has been not only with Yohan, but the CMT community and folks like Riley, that has driven that kind of tiredness and not wanting to do the event into passion and dedication to continue to move forward, and even though we don’t have a cure today, to stay positive.
Riley Ashe and Yohan at Camp Footprint
Chris: When you can see someone with CMT really have the opportunity to enjoy that event, and as I always say to Liz O, that event, as I reference, is not feedback for Liz O and I in terms of what we do. That event is for people with CMT, and that is why we do it. It’s their environment. It’s their voice. It’s giving them updates on research and doing whatever we can to spread awareness and help raise the necessary money so we can continue on this research path. So, that was inspirational, and it’s very interesting. It’s been probably five years, and every time that story comes up, I just start crying.
LizO: Well, and then Riley spoke at one of the events, and hopefully, he’ll be at the event this year. He probably will. His mom [inaudible 00:33:04] is a good friend. The other thing is he attend attends Camp Footprint, and that’s been life changing for him, but I just remember talking about … He didn’t want to lose the ability to walk, and he has. And, that’s the progressive nature of CMT, and we need to stop CMT. We Need to stop the progression.
Chris: The other thing that hits me at these events as well is the positive energy.
LizO: So fun.
Chris: CMT can have a major impact, however I just find such a positive environment with those that do have CMT. It gives you encouragement and strength to continue to try to fundraise and find a cure, because it’s just such an awesome community. You don’t find people that are sitting there, looking for sympathy. They are talking about what they have accomplished and what they can do. Some folks say, “Hey, if I had a chance” … I don’t know if I would say … It’s hard, right? Some folks would be like, “I don’t know if I’d say. Obviously, I wouldn’t want CMT, but CMT has really made me into this incredible person.” And, that is very heartfelt, warming, and touching to me, to hear those stories.
Chris: So, it’s a great environment. And, folks, this is definitely a pitch for the Cycle4CMT event as well. If you have a chance to get to that signature Vermont event, you’ll be blown away by it. It is beautiful. It’s a great environment. It is a lot of fun. It’s a great cause. On top of that, as Liz O said, there are rides going on throughout the country. You can go to the Cycle4CMT.com website, learn all about the event. But, again that event is for you, and if you can get out there and help fundraise and spread awareness, that’s our goal, and we’d love to have you.
LizO: And, this comes back to what I was thinking. You said people are so positive, and we have such a great group of people who Cycle4CMT. But, I think part of that is talking about giving back, instead of sitting there and waiting for somebody else to do it. Get involved in any way you can, so you’re part of progress. You’re going to be part of our solution. You, your money, even if it’s just a little bit, if everybody just gave a little bit, we’d probably have a cure by now. So, honestly, I just think I can’t sit by and watch Yohan progress or my friends, Bethany, progress, or Jeana, or people I’ve come to love, and Kenny B. I can’t do that. I have to be involved, and all these people are involved, and it feels good to give back. And, it feels good to see progress when we do.
Chris: Yeah, yeah, totally. So, Liz O, you’re talking, and I’m just thinking about our relationship, right? We don’t see each other that frequently, maybe a couple weeks each year. Hopefully, that’ll continue to grow as we-
LizO: You’re too busy. You’re too busy. Every time I call, you’re like, “Yeah, okay.” And, I’m like, “Hey, so” … I’ve got to go. Bye. Got to go. Bye. Got to go. Bye.
Chris: But, you’re busy as well, and it’s interesting. It is a good team. You and I are really two totally different people, but there are a lot of common characteristics as well. I’m going to just give a little bit of feedback, folks, to get you the details of Liz O. And, I would say number one, lot of fun, right? Always laughing, very, very intelligent, very well spoken. We’ve already talked about how she gives back, but on the side, she’s totally scattered, totally scattered. I am always like, “She makes it through the day. She does. I don’t know how she does.
Chris: And, here’s a great example. What does she do yesterday? Comes down to camp. She’s like, “I am going swimming.” I’m like, “Perfect. Go swimming.” So, she puts her bathing suit on. I’m doing something on my iPad, and she comes back out of the lake, and she’s like, “I can’t see anything. I can’t see anything.” I’m like, “What?” She goes, “Did I just jump in the lake with my glasses on?” I’m like, “I don’t know. I don’t know.” So, I’m like, “Here we go.” So, I go out in the lake. I can see these footprints in the sand. I go way out there, and sure enough, there-
LizO: I’m trying to help, and I can’t see anything.
Chris: Yeah, she can’t see anything anyways.
LizO: I’m stirring up all the sand.
Chris: Yeah, so there are glasses on the bottom of the lake, which I recover for her and continue to move on. But, that is not a surprise. There are probably five to 10 of those items that go on through the day. Can’t find her car keys. Can’t find her phone.
LizO: Never.
ChrisO: She goes on a trip with Gilles, who is our chairman of the CMTA, and what does she do? She loses the car keys in the desert, and he’s out horseback riding. She’s going for a hike, and so thank God, she posted her walk on this app called Strava, and Gilles followed it on Strava and found the rental keys. But, my point is she makes it through in that type of environment, and she’s not scattered on other things. But, those type of things, which aren’t real important to her, you know what I mean? There’s other things, and she gets through it. And, I am a little bit different. I’m not saying I’m not scattered, but I’m a little bit different.
LizO: No, no, no. You’re very different. Listen to this. (singing). I can’t even remember the tune now. (singing).
Chris: M.A.S.H.?
LizO: No, maybe it is. But, I meant The Odd Couple. We’re sort of like-
Chris: Oh, that is The Odd Couple.
LizO: Oscar and Felix.
Chris is Felix – Lizo is Oscar
Chris: Good point.
LizO: I’m Oscar. You’re Felix.
Chris: Totally.
LizO: So, you’re very organized. You’re very driven. And, you are very structured, and I am not. I am driven. That’s what we have in common, but you’ve always been like that, though. I just remember waking up in the morning, and you were nine. What nine year old looks outside of the window and goes, “Oh, my God, the grass needs to be mowed.” And, you get out there, maybe 10. You’re mowing the grass, and I’m like, “What grass? Who cares? Have it weeds.” It’s so interesting, and I remember I was kind of nervous about spending the night at camp here with you, because-
ChrisO: Well, I was nervous as well.
LizO: I know if I have any crumbs on the counter, he’s going to freak. I don’t know if I’m doing the dishes right. I’m just trying to respect your space. The other day, and I felt bad about this afterwards, but I don’t think of these things. I get a box. We’re over at your house. You have white furniture, which I think is stupid.
Chris: She gets the box out of the garage, folks, my garage that has been … It’s all dusty and things like that.
LizO: It’s not dirty.
Chris: What does she do? She’s like, “Can I have use this box to ship items?” I’m like, “Sure.” So, then what proceeds to happen?
LizO: Then, I take the box and put it on the white furniture and start packing the box. He goes, “Could you please take that box off the furniture?” I’m like, “Why?” I had no idea. It didn’t even dawn on me that the box was dirty or had dust on it. Then, a little aggressively, I kind of rubbed the box into the couch.
Chris: Yeah, that was very nice.
LizO: No, that wasn’t. It was just like it annoyed me, but I apologized later. I should just taken it off, but I don’t think of these things. We grew up just having a blast and jumping in mud puddles. What did you tell me earlier about the bathtub?
Chris: Oh, yeah. No, it was great. I think about growing up, right? And, this comes back to mom. Mom was very structured, very committed, high driving, tons of energy. Even today, the energy is off the charts compared to us. Education was really important to her, the whole aspect of giving back, realizing what you have, and just throwing that in there. Not to digress, but it just made me think, even at Christmas time, I remember as a little kid being in our station wagon, and mom would go buy a couple bikes or something and toys. And, we would drive through the old north end in Burlington, which was somewhat of a depressed area.
Chris: She would see a child or a family and stop, and we’d get out and give them these gifts. But, yeah, very, very structured, but also flexible. It made me think, she rarely got mad. I remember with our brother, Anthony, when you’re younger, you’re taking baths together, and him and I are just always creative. The bathtub is filled, and we’re pretending we’re on a ship, and we’re sliding down the bath, and water is overflowing out of the tub and going on the floor. Mom’s downstairs, and water’s coming through the ceiling and just, oh, okay, great. They’re having fun. Can you guys stop that?
LizO: They’re having a good time.
Chris: I grab blankets, and then I’d be sliding down the stairs and rip the carpet and whatever and smash into the wall. They’re just like, “Oh, the kids are having fun again.” So, that was pretty cool, right?
LizO: But, I think it was. It was, and we just had a lot of freedom. But, our personalities are very different and very alike. I think we’re very complementary. And, you make me laugh, too. You’re funny, so I like that.
Chris: Oh, well, same. Listen, I guess, folks, it’s a great relationship. Liz O, love you very much.
Liz Ouellette: Same back to you.
Chris: It’s cool, and it’s really cool to have the opportunity, these things, whether it’s the fundraiser, Yohan, the CMTA, has really, I think, also kept us connected.
LizO: Absolutely.
Chris: And, at times I wonder, I think, boy, if we didn’t have that, will we still be connected? And, there’s part of me that says, “I know we would. I know we would.”
LizO: We would.
Chris: Because, there’s that sense of family and appreciation for one another and Yohan and Gilles. You love our kids, et cetera, so that family aspect is big.
LizO: It’s huge.
Chris : And, I think you said it well. I’m proud of that. I do think we have a good example of how a family can come together and strive to overcome a number of hurdles, specifically as related to CMT, right? And, there’s a lot more power with more-
LizO: That’s right, it’s not just me and you. Our parents, our siblings, our community, our family, everybody is involved.
Chris: Yeah, it’s cool.
LizO: They don’t even hesitate.
Chris: That’s good. I hope mom’s listening, because we need her to make a big donation this year.
LizO: Yeah, I know. This is the reason we’re mentioning her. No, I’m just kidding.
Chris: Get out your checkbook, mumsy,
LizO: Mama.
Chris: So, Liz O, let’s talk a little bit about this podcast.
LizO: So this is an idea you had six years ago.
Chris: Years ago.
LizO: Yeah, and people were starting podcasts. We should do a podcast. At that time, I’m like, “How do you even do a podcast?”
Chris. I don’t know.
LizO: Now, everybody has a podcast, but you had this idea, and the board of directors actually supports us 100%, love the podcast. They love the podcast. And, thanks to Mark, it’s pretty easy. And, I love doing it with you. And, we have interviewed some people that are just amazing. Every single person, and so the CMT4Me podcast, and you came up with the name, which you’re very creative also.
Chris: And, CMT4Me. And, again, it’s another … And, keep in mind, don’t just push yourself aside on this one. This is collaborative. That’s what’s cool, and you’re just making me think, whether it’s back in Bahamas. I’m like, “I’d like to do a fundraiser,” but working with you continues that creative. And, where do we go? Because, you don’t want to do anything small, right?
LizO: No, I can’t.
Chris: You’re like, “Okay.”
LizO: It’s either 100% or zero.
Chris: Exactly.
LizO: That’s a problem, but that’s the way I am.
Chris: Exactly, so we work well together. That was really again thinking about, with my experience with CMT individuals is that, how do we give more individuals with CMT the platform and voice? As our intro says, it’s really their inspirational stories. How can we get more people with CMT connected to one another so they feel they have support? It goes to the same thing with the fundraiser. It’s a platform, and this podcast is a platform for individuals to express themselves, tell their stories.
Chris: It’s been cool, because there’s been some people that have listened to the podcast that then have reached out to another individual who we interviewed. Or, they’re new to CMT, and now they have resources. So, I feel really good about that. One thing I think we work hard on too, and someone made me think about this, was you don’t always want to just focus on the negative, right? Oh, here’s all the negative things going on. No, we’re realistic, and we talk about the facts, but there are so many great stories about overcoming challenges and sharing information. On top of that, the big goal is spread awareness, right?
LizO: That’s right.
Chris: And, we’ve got to spread awareness. That, I think, ties into how we can raise more money for research, if we have more and more people engaged.
LizO: So, I was just blown away yesterday, and I actually took a video of you soliciting merchandise or a gift certificate from a restaurant. You are such a natural. You just go in there, and I just watch you. Usually, this is our tactic. We walk in a store. I go shopping and buy something. Then, Chris starts talking to the owner about the cycle event, what CMT is, and tries to get a gift certificate while I’m checking out. Usually, the answer is yes, but you’re so talented at it. You just have no inhibition.
Chris and LizO at Cycle 4 CMT 2021
Chris: Wow, that’s cool. Thanks for that feedback. But, you participate as well, and it is a strategy. I’m like, “Liz O, you go buy something, because if you buy something, then how can they turn us down?” So, it’s great.
LizO: Then, if I don’t see anything, you’re like, “Well, I like this. I like that.
Chris: Right, so I usually walk away after her visit, multiple pairs of pants, shorts-
LizO: Shoes.
Chris Shirts, shoes, you name it. It’s awesome.
LizO: It works.
Chris But, it is, it’s fun, and it’s interesting. I always look at the faces of someone who we’re trying to solicit, and you go, “CMT,” and they’re like, “Okay.” And, then you keep going. This individual yesterday who finally came around, and you learn-
LizO I didn’t think he was going to.
Chris: I didn’t either, but then you learn-
LizO He was clearly like, “Whatever, whatever.”
Chris : You learn, things come up as you keep talking to people. What did he say? I said, “Do you bike?” And, he’s like, “Well, no, I have a motorcycle out there.” Then, that’s like, “Oh, well, I used to ride motorcycles. I had a Honda Shadow 500. Oh, that’s a great bike.”
LizO: So good at making those connections.
Chris: So, you make these connections. Then, you talk about, which I think is important, it’s that statistic of … I always forget. What is it, one in 2,500 or 2,800 have CMT? And, relate that back to say Burlington, Vermont or Vermont, population of 647,000, right? So, when you say, “Hey, we were born in Burlington. We’re native Vermonters, and by the way, you may not know it, but there’s over 200 people in our community that have this incurable disorder at this point.” They start to think, and I find a lot of times, after those discussions, it’s hard for them to say no. And, I don’t feel that they feel the obligation, but I think they understand. And, it’s that passion. And, Vermont is a really community-driven state that is always looking at ways to give back. That also makes it a little bit easier, but then people feel connected, right? You’ve got to bring them in terms of how they can help us towards our mission.
LizO: And, what’s really unique here is the community is important. The community feel, and community comes together when you’re in need. Vermont and the surrounding areas are just great for that. I just miss that. I miss it a lot.
Chris: Yeah. Yeah. Well, it’s good. It’s good. We’re on a good path, folks. Liz O, are we coming to the close of this podcast? I think we kind of covered our topic.
LizO: Yeah, we’re probably just talking. I don’t even know how long we’ve been talking.
Chris: People are probably like, “Oh, my God, will these guys shut up?”
LizO: Oh, my God, are they going to stop talking?
Chris: Right. But, folks, listen. For those of you listening to this podcast, this is our opportunity. We’re not going to do our standard close, but to really thank you. Thank you for listening. Back to Liz O’s comment about doing your part as well, if you can tell folks about this podcast, if you can direct them to the Cycle4CMT fundraiser or to the CMTAUSA.org website, we need all hands on deck here.
LizO: Back to the ship in the bathtub reference.
Chris: I know, I know.
LizO: All hands on deck.
Chris: And, Liz O, maybe some of our listeners can help us if we spread this right. What’s one of our goals that we’d love to do someday, is kind of-
LizO: Oh, my gosh, we would love to be-
Chris: When you think of the news, and what would we love to do? It’d be sweet.
LizO: I’m so sick of hearing negative things. It’s all negative. So, I see this-
Chris: The news? You mean in the news?
LizO: Yeah. Yeah, everything is just negative and worrying and worrisome. Let’s get a feel good story like us helping the CMT community to find a cure for this disease and talk about all the people that do such incredible things, despite the limitations that CMT imposes. Wouldn’t it be great, Good Morning America? Or, there’s so many, so, so many programs that we would love to be on.
Chris: Right, CBS News. Maybe we could be interviewed by someone who has a podcast now.
LizO: That’s right.
Chris: That has a greater reach.
LizO: Guys, we want to go national here. We want to go international, actually. We’d like to go international.
Chris: That’d be cool.
LizO: Get the word out about CMT. We work with people internationally, so let’s get this on. Let’s get going.
Chris: Yeah, let’s do it.
LizO: Do it.
Chris: Yeah. Liz O, have you heard of those Sprinter vans?
LizO: No.
Chris: No, okay. Well, they’re these cool vans you can put your bike in. People are buying these things now and throwing their skis in there and bikes and whatever. But, it made me think, wouldn’t it be kind of cool at some point where we could have as your background as the CMT4Me podcast logo on the side of this Sprinter van, and we tour the country and go to these areas and interview people with CMT. Wouldn’t that be sweet? That’d be fun.
LizO: Yeah, it would be fun, and we’d meet so many people. Now, that’s a story. Now, you’re cooking.
Chris: What’s it? Who’s the-
LizO: Alan Jackson?
Chris: No, no. Yeah, yeah, that’s Alan Jackson.
LizO: CMT came out, and I’m like, “Chris and Gilles, you guys have to bike there. You have to bike there, and we can make a story.” Chris was like, “I’m not biking there.”
Chris: No, it made me think of Al Roker, right? He goes on the road sometimes and travels in this van and does the weather in all these different areas of the country. We could do the podcasts in all these different areas of the country.
LizO: Yeah, let’s do it. You have to just stop working.
Chris: Yeah, okay, that sounds great. I hope people from-
LizO: Hey, talking about the podcast-
Chris: I hope people from where I work are not listening to this. No, just kidding.
LizO: So, about the podcast, if you want to leave a review, and we’d love to have your review, Apple Podcast has a place for that.
Chris: Awesome.
LizO: Yeah, so I just wanted to throw that out there, and it’s available on Spotify, Apple, all the major podcast outlets. You can hear this podcast, CMT4Me.
Chris: Awesome. All right, Liz O, time to go. That’s a wrap, sis.
LizO: All right, thanks, everyone for listening.
Chris: Yeah, thank you.
LizO: Cycle4CMT.com or CMTAUSA.org. If you have an interesting story, let us know, info@CMTAUSA.org, info@CMTAUSA.org. And, watch us on YouTube. We’re live.
The Cycle (and Walk!) 4 CMT event at the Old Lantern in Charlotte, VT on Sunday, August 26 will be upon us before you know it! Please act now to reserve your spot to cycle, walk and/or attend the unforgettable after-party!
Whether you are attending the event in person or participating virtually, here are your top 4 action items for you to complete right now:
The Cycle (and Walk or Roll!) 4 CMT event is so much more than a bike ride or walk around Charlotte, VT. It is a way to give hope to families and their children all around the world for a medicinal treatment to stop the progression of CMT.
I asked our friend Riley Ashe from Vermont, who never misses the event, to talk about what this event means to him. Here is what he said (get Kleenex out before pressing play):
My brother George’s heartfelt thoughts (grab yet another kleenex):
After-Party
Event After-Party
Rumor has it that we throw the best after-party of any non-profit event for miles around! Riders, walkers and party attendees are going to have a blast again this year.
After the morning activities, plan to relax in the beautiful setting at the Old Lantern in Charlotte, VT.
Quench your thirst with VT brewed beer, enjoy delicious food, including appetizers and a fully catered menu and bid on epic silent auction items provided by our supporters.
Rock out to the lively tunes of our favorite band, Leno, Cheney and Young, mingle with family, friends and hear about exciting CMT research updates from our internationally acclaimed CMT experts, Drs. Michael Shy and Steven Scherer.
CMT Experts – Drs. Scherer and Shy
Yohan and his Grandmother, Bev
The Love of an uncle for his nephew. Family is everything.
Our Fabulous Band
Walk 4 CMT!!
If you cannot make it this year – you can still participate by doing a VIRTUAL CYCLE or WALK or ROLL. It’s easy! Register: http://www.cycle4cmt.com. Here is some of the cool swag you’ll receive after you’ve reached your fundraising goal.
If you do a virtual event, send me pictures of your adventure! I need to brag about you! xoxo
Help us break the silence…get the word out about CMT!
“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.
“My high school years?” she shuddered. “After the doctors diagnosed me with polio at 13-years-old, they wanted to straighten my misshapen feet. So, for the next 5 summers in a row, my school vacations were spent either in the hospital or at home, immobilized, with heavy plaster casts weighing me down.” With raspy, uneven breath, Flora described those dreadful Mississippi summers as muggy, sticky and hot – really hot. “Neither the hospital nor my home had air conditioning in those days, so I was pretty uncomfortable – but I got through!” she added with her usual determination.
I first spoke with Flora in 2003, when I was getting to know the CMTA group leaders throughout the country. She told me that after her orthopedic surgeon “straightened her out”, she had left her polio diagnosis behind and moved forward with her life. She went to college, married her husband of 52 years – Billy Joe Jones in 1960 and had three children. After the birth of her youngest child, Cindy, she decided to stay at home to be a mother, wife, and homemaker.
Flora and her husband, Billy
Over the years, her legs weakened and her hands slowly lost function. In 1982, at the age of 45, she was finally diagnosed with Charcot-Marie-Tooth disease or CMT at an MDA clinic. Okay. There was a name for her progressive symptoms, but she wondered what she was supposed to do with the name. She couldn’t just “Google It” because home computers had not made their debut in society.
Luckily, by word of mouth, she heard about a CMT conference in Toronto, hosted by CMT International. No way was she going to miss this opportunity to learn and gather more information about CMT. Upon arrival, she was stunned to meet others with similarly-looking feet and hands – 200 other individuals with “the walk” including 30 people who admitted to breathing difficulties, like herself. One of her favorite parts of the conference was educating CMT clinicians about her own CMT.
In 1993, she was elated to discover a CMTA support group in her area. With her passion for spreading awareness and teaching others about CMT, it was not long before Flora stepped up as the leader of the Brandon, Mississippi CMTA support group. In fact, she involved the entire family in her mission and many weekends were devoted to mailing educational information, calling new members and organizing CMTA meetings.
Flora and Jeana Sweeney at the CMTA Support Group
Due to severely atrophied leg muscles, she lost her ability to walk at the age of 58, but that did not make one bit of difference. She continued to lead her group enthusiastically, informing the members about the CMTA, its research, and resources. Over the course of her lifetime, she touched innumerable lives, informed countless medical professionals and supported others who had a CMT diagnosis.
Toward the end of her life, she could barely use her hands, and her violent tremors made holding or sipping from a cup virtually impossible. Her paralyzed diaphragm made breathing harder and harder with each passing day and her weakened vocal cords affected her ability to talk. Yet, Flora did not let any of those worsening symptoms stop her from fulfilling her mission in a positive, cheerful and comforting way.
I just spoke to Flora a few months ago. She called to check in, to see how Yohan was doing after his foot surgeries. She told me he was always in her prayers. She admitted that her CMT was wreaking havoc on her body, but it would never, ever dampen her inner spirit. Even in her final months, she would not leave home without her brochures. She wanted to be ready to educate everyone and anyone who would listen to her about CMT. In a recent letter from Flora’s daughter, Cindy, she confided, “Even up to her last doctor’s appointment, she educated her doctors about CMT.” In February, Flora was given a new doctor, “a cute one!” she said with a wink. Super impressed that he really was interested in CMT, he even explained how he could help her live a productive life despite her limitations.
Flora and her daughter, Cindy
Following in Flora’s footsteps, I try to spread CMT awareness wherever I go, even if Yohan rolls his eyes out of his head like he’s screaming loudly. If we all speak about this progressive disease to our doctors, nurses, family, and friends think how many people would recognize the name, the symptoms, and the signs. “It’s CMT.” I say. “Charcot-Marie-Tooth disease.” And when the person looks puzzled, which they usually do, I remind them of the acronym – CMT. To make it stick, I joke that CMT does NOT stand for Country Music Television. And I might add, “ It’s the other CMT-the one that destroys nerves, causes muscles to weaken, and disables the young and old. It’s Charcot-Marie-Tooth disease and we desperately need a cure.” And a cure starts with awareness.
In memory of my friend Flora, who passed on April 1, 2018, at the age of 81 from CMT-related breathing complications, please help raise awareness of CMT. Think about it – even the most worthy causes won’t receive a dollar in donations if no one knows about them. Putting a face and a personal story on CMT may spur people into action, especially if they know their support will lead to a cure.
Flora, you are my inspiration to wake up every day and fight for a world without CMT. And now, I imagine you moving freely among the stars and dancing with angels. And I smile. I may not have told you during our phone conversations, but I admire you and love you deeply. Your memory will never be forgotten.
“His test came back positive for what? Sure, that explains a lot, but what’s it called again?” I asked stunned and a lot bewildered. “CHär/- Kät/ -Merēd -TOOTH, which sounded just wrong when he said it. I guess it sounds strange ANY way you say it, but he was completely butchering the French pronunciation of my son’s new disease (we are all French – can you blame me?) The take away was that our beloved 7-year old son Yohan had a progressive disease of the nerves, WITH NO TREATMENT OR CURE, which would make his muscles weak and useless over time. I felt dead inside – for a very long time.
Alpine Slide, Stowe, VT
My world shattered into millions of pieces that day, and I never thought we’d be able to pick up all the scattered bits to reformulate our dreams, our hopes, and our wishes for our son and our family. This reassessment took time, effort, and a lot of soul-searching, but we managed to accept the diagnosis and manage the Charcot-Marie-Tooth disease or CMT the best we could…. one day at a time.
As a mom, it was tough watching my child struggle with pain, braces, physical limitations, and obvious differences. Maternal instinct ordered me to protect, shelter, cajole, and, especially, do something—anything—to make the world a friendlier, more secure place for him.
For a while, school also became an inhospitable place, and bullies had their way of ruining a perfectly good day. Yohan’s self –esteem and self-confidence took major hits and his anxiety skyrocketed. The more I tried to lessen his burden by catering to his needs and lightening his load, the more resentful and withdrawn he became. Unknowingly, I was giving him the message he should not go out of his comfort zone because the world is a dangerous, scary place. And, I was setting him up to be reliant on me for simple tasks he was able to do all by himself.
We found an excellent therapist whose specialty was counseling kids with medical issues. Our goal was to empower him by making him accountable for his actions, giving him more and more responsibility, and providing him the space he needed to become autonomous and independent.
Sounds pretty good on paper, but implementation was another story.
On one of our many trips to Vermont, Yohan wanted to try snowboarding. I subtly, but unsuccessfully tried to talk him out of it because I did not want him to get hurt nor fail. He has CMT-related back issues (kyphoscoliosis), tight calves and heel chords.
Kyphosis + Scoliosis = Kyphoscoliosis
I assumed the worse – in my frightened mind, he had at least a 75%, maybe 80% chance of falling in the powdered snow, way over his head, and suffocating because he would not be able to stand up.
First Snowboarding Adventure
With the least amount of enthusiasm you can imagine, I rented the snowboarding equipment, grumbling under my breath the whole time, and when father and son left early one morning after Christmas, I stayed home and tried to keep very busy, blotting out what was happening on the slopes.
Later that day, after what seemed like an eternity, Yohan burst through the door, still in one piece and with a look of content satisfaction on his face. “I loved it!” he blurted out proudly. “I’m going to take this on seriously! I had such a great day!”
Like many first-time snowboarders, Yohan took a good many tumbles and falls in the snow, but he was confident in his abilities. He knew what he wanted, so he left home with determination and a can-do attitude, and came back fulfilled with a sense of accomplishment.
That event was followed by many more like it and served to reinforce what I already knew as a parent but still found difficult to wrap my head around. Even though Yohan has CMT, there are many things he can do with the right support, guidance, and freedom. Gilles, my husband was exemplary in providing many unique opportunities for him to explore his surroundings. An avid skier, mountain climber, biker, etc….he went out of his way to find activities that he and Yohan could do together, as a team. I am so grateful for my husband’s patience, effort and time with Yohan, as his childhood was full of fun adventures and learning experiences. I wasn’t as thrilled about some of these outings at the time, but I usually saw the photos after the fact and they speak for themselves-they have memories- good and bad- to last a lifetime! As parents, if we do not encourage our children to dream big, they might never know all the beauty, wonder, opportunity this world holds.
Kayaking with Gilles and friends
Yohan with Athos, Gilles with Chyna
Yohan is scuba certified, thanks to his dad.
Do you remember the movie about the life of Ray Charles, “Ray”? Despite his total and permanent blindness at the age of 7, Ray Charles’ mother treated him the same as any other child. He was made to do his chores, learn to get around without a cane, and fend for himself. “I won’t be here forever,” she repeatedly told him. Harsh though her treatment of the young Ray seemed, their story is filled with passion, love and many teachable moments. As hard as it may be, and still is, I have witnessed Yohan’s challenges, disappointments, and heartbreaks. But I no longer worry as I am confident he has the emotional fortitude to get through just about everything. If there is just one gift with which I wish to leave him, it is the knowledge that with an open and positive mindset, he can and will achieve his heart’s desire.
One day about 7-8 years ago, I get this random call from a young woman from Michigan. She wanted to volunteer with the CMTA. “Sure!”, I said enthusiastically. “We are always looking for volunteers-ALWAYS!” Now, compared to my loud, overly animated voice and my quick speaking conversational style, my new friend, Bethany, spoke slowly, methodically and in whispered tones. She actually takes a moment to think before she spoke – a new concept for me.
She wanted to volunteer for CMTA but she was about to have foot surgery, and she assured me that she’d get back to me during or after recovery. I had no expectations, but she did indeed get back. From this day forward, our friendship blossomed. I crept into her life like mold, and now, she’s never getting rid of me. We are stuck together like velcro. She moved to London last year, probably hoping the distance would give her some space-WRONG. We talk frequently, Facebook tons, and I’ll be seeing her next week in Miami.
Following her then boyfriend, Josh, to the Bay Area, California (a joke you’ll understand once you’ve read Bethany’s book), we got to know each other well. She really is not as quiet as you think when you first meet her. In fact, she’s quite chatty and holds her own in debates. From a shy, soft-spoken teen, to a master in digital communications, a successful fundraiser and a moving motivational speaker, Bethany has become a well known and loved figure in the world of CMT.
At 25, Bethany has published her first book, How Should a Body Be? which gives an intimate, honest and heartfelt portrayal of what it is like growing up with different abilities. She’s a wonderful writer and I am in awe of her strength and “determination” (I prefer the word stubbornness, but Bethany’s not thrilled with that word). Here are my thoughts on Bethany’s memoir:
Bethany Meloche’s thoughtful memoir—“How Should a Body Be?”— recounts the life story of a strong-willed young woman with a never-give-up, never-look-back stance to being alive in this world. In a culture that places so much emphasis on physical perfection, many are dissatisfied with their appearance and obsess over achieving unrealistic standards of beauty and fitness. Compound these everyday societal pressures with a progressive neuromuscular disease like Charcot-Marie-Tooth—which causes foot deformities, muscle weakness, tremor and breathing difficulties—and growing up with confidence and assurance becomes that much more arduous.
With wit and humor, Bethany relates the challenges of living in a world where people’s well-intentioned, but short-sighted commentary and feedback inadvertently amplify her feelings of self-doubt, uncertainty, and isolation.
Driven by a lust for knowledge and unquenchable curiosity, Bethany lives each day to the fullest, making her story both unique and inspirational. It would have been easy for Bethany to surrender, to lose hope, to fall into the depths of despair and depression, but by turning her anger outward she discovers strength, willpower, connection and success. “How Should a Body Be?” is a personal journey toward self-acceptance, healing and living life to its fullest, despite apparent limitations. Mature beyond her years, Bethany offers nuggets of wisdom to be shared, pondered and cherished. Honest, truthful and profoundly insightful, this book is for people with CMT, their families, their friends and anyone who struggles with self-image, confidence and the fear of being seen. This is the best book to date on growing up with physical differences, obvious or not.
I hear voices. Eavesdropping, as if I were an innocent bystander, I witness the back and forth volley of words, arguments, and reasoning. Often, I put a harsh stop to the banter, for fear of losing too many precious minutes to internal disputes and emotionally draining debates. Trying to reach my neutral, rational space often feels impossible, like I’m swimming against a strong current intent on overwhelming my frantic efforts. Yet, somehow, someway, I usually manage to quiet the noise, at least temporarily, and transition into a state of calm deliberation, frantic activity or self-imposed numbness.
Yohan had his first ever foot reconstruction surgery in June and 8 months later, he’s still not walking without using crutches or a knee scooter (see previous blog posts for the whole story). As soon as he starts to put full pressure on his foot, sores develop.
When that happens, staying off the foot until it heals is the remedy. To offset the pressure, he had his shoes modified, new orthotics made, which were adjusted again and again and again, only to have the sore reappear when weight-bearing. I know that all surgeries do not go as planned. You probably also know that surgeries are risky, in many, many ways. But I thought the surgeries that went awry happened to other people. But this time, Yohan is that other person.
The holidays came and went, and in January we found ourselves at a standstill, not knowing what direction to turn. The last pictures I sent to our surgeon showed how Yohan’s foot had healed, and it did not look right. For some reason, his heel looked misaligned, causing excessive pronation and weight distribution imbalance. Our surgeon, Dr. Pfeffer, was perplexed, but to his credit, he’s promised to make it right. Putting ego aside, Dr. Pfeffer is determined to make the next surgery the “last surgery” by asking for second and third opinions from well-respected colleagues. He wants to make 100% sure that no rock is left unturned before going back in to correct the lopsided foot and straighten Yohan’s toes. His humility, commitment, and compassion command our genuine respect.
Left Foot After Surgery
Nevertheless, I dread this second surgery. I just want Yohan to be able to walk with ease, even if it is only short distances. And in all honesty, I may have felt, if only for a microsecond, discouraged, angry and saddened by all the challenges Yohan’s already faced throughout his 23 years on this planet, due to the effects of CMT. He complains rarely and manages his day-to-day with laughter, humor, and hope. Yet it is difficult to witness his debilitating fatigue, chronic pain and now, successive surgeries. I wish it weren’t so, but it is so and that’s what is true.
In general, I tend to live in my head, not my heart. Why open up to intense emotion, when the risk is getting sucked up into a vacuum of never-ending despair and misery? When my thoughts become dark, the voices in my head try to cheer me up, scold me for being negative and/or neutralize the negative with positive thoughts. This process has become so automatic that I often no longer know what I feel inside. Many believe that raw emotions, in all their complexity, are an undeniable part of the human experience, serving as a profound source of inner guidance and direction.
Yeah, well my inner feeling mentor acts more like a sneaky stalker than a trusted ally, ready to pounce when I am least protected. I’d rather stay in the neutral zone and avoid the war-torn areas of my life. Yet I often wonder where those intense feelings go. Do they evaporate? Remain in the body unexpressed and ignored until the pressure builds so, they just explode? Do feelings have feelings? So many questions, so few answers.
The second surgery does not yet have a date, but it will be soon, in the near future. We’re all doing our best and trying to live in the moment. We’ll get through. Thanks for listening. And if you have a moment, let me know how you cope through difficult times. You never know, your advice and feedback may just be of help to others someone else.
Dedicated to all my friends who have a love/hate relationship with shoes.
“I love those shoes….OMG – they are sooooocute!” enthusiastically commented an unknown, young, attractive, athletically built woman. I looked around, certain that she was addressing someone behind me or outside my range of vision. Mouth hanging open, I stood stunned, realizing she was referring to my shoes, my size 12 purple and aqua blue Solomon running shoes. Managing to spit out a “Thanks!” her casual compliment rendered me speechless for all of about 5 minutes (which seemed like an eternity…..to me).
Above: The Complimented Shoes
The last time someone actually told me they liked my shoes was back in September of 1967. I was 5 and my mom had just bought me a pair of black, shiny patent leather shoes. The compliments I received! Overjoyed with my new shoes I ran, jumped, danced and then, never fail, I slipped on our hardwood floors, landing head first into the electric radiator, at the base of the wall. As blood gushed from the gash on my forehead, a cloth was applied to the wound where it stayed until we reached the ER. The stitches left a small scar above my left eyebrow, a foreboding symbol of future foot-related misery.
Above: Me, Age 5, Patent Leather Shoes
Nevertheless, I had not yet received the memo about imminent foot woes, so when my mom had to order new and very expensive shoes and winter boots from a shop in Montreal because my instep was so high, I thought that I was really something special. Although I hadn’t a clue as to what a high instep actually was, I didn’t care. I felt like a princess who needed the best of what money could buy, and from abroad, to boot (a 2-hour drive from my hometown of Burlington, VT). “I could get used to a life of royalty-Queen Elizabeth,” I imagined, my illusions of grandeur already a problem at such a young age. The thrill of ordering our butler around, “Andrew, Caviar, please! “or “I’ll wear the dazzling rubies this evening, Alfred! Snap, snap…I haven’t got all day!”
As I grew taller, my feet inevitably grew longer. By 8th grade, I was at least 5’7’ and my feet already demanded a size 10 shoe. Long-limbed and gawky, I looked like a baby flamingo and walked like a newborn giraffe learning to take its first steps. Between the giraffe and the flamingo, I must have looked a lot like a fliraffe.
Above: Baby Flamingo
Above: Baby Giraffe
Above: Fliraffe (a giraffe with baby flamingo feet)
If my parents had named me Grace, I would have been a laughing stock! It was bad enough with older brothers who had their own nicknames for me: clumsy, klutz, horse, big foot, clod, butterfingers, spazz, etc. I was always bumping into something and spent a lot of time on the ground, either cleaning up something I had spilled or nursing wounded knees.
If you have CMT, you may be able to relate to my story and have a few of your own. Does this sound familiar? I fall over air, get caught up in my own feet, trip up stairs, run into furniture and constantly drop things. Here are just a few concrete examples which come to mind: I dropped my cell in public toilets, twice, got my bike tire caught in the rails of a tram, and just simply fell over onto my side in the middle of a busy plaza, tripped on nothing and everything, sprained ankles, broken toes and sported many, many bruises. And this is just the tip of the iceberg, as the list is way too long and the catastrophes, too many to count.
Many of my friends in high school and college wore high heeled shoes for events. Not me. At 5’9’’ I was already taller than the majority of other students, especially the guys. Secondly, a size 10 high heeled shoe was impossible to find and third, I would have broken my neck. And have you ever found a sample size 6 or 7 shoe at the store, and when they brought out the size 10 or 11, it looked nothing at all like the size 7 you had already fallen in love with?
Above: The shoes I wanted (floor model,size 7)
Above: The shoes they brought out (size 12)
When I lived in France, the saleswoman wore a look of shock and disgust when I gave her my shoe size. As if being forced to wait on the Hunchback of Notre Dame, she nervously whimpered, “Madame, s’il vous plaît, look in zee secshun for zee man,” and she pointed in the direction of the men’s shoe department. How humiliating.
By adding padded and ultra cushy orthotics, my shoe size increased by 1 or 2 sizes!! On my body, an 11 or 12 shoe is not feminine. It just isn’t. I walk more like Herman Munster than a tall woman with long legs and big feet.
Above: My body and feet
So when my new best friend complimented me on my “cute” shoes, I decided to take the compliment and wear it with pride. And, honestly, I am just grateful to be able to walk. Some are not so lucky. So, I say screw femininity. The older I get, the less I care about what people think, especially if it is negative. Now give me positive commentary, and that my friends, is a different ball of wax.
The pizza had just arrived. Before I could take my first bite, the subject of my husband’s company cocktail party surfaces. “Thanks, but no thanks. I don’t want to go,” I say with steadfast determination. “I’ve already made sure that chairs will be available,” he counters, attempting to make the event seem easy, cozy, attractive even. Then, as he points at me with the tip of his piping hot slice of pepperoni pizza, he gets all serious and a little pouty. “Look, I run the company. It would mean a lot to me if you came. My staff and colleagues are looking forward to meeting you. ” On the inside, I roll my eyes, feeling a little irritated that some of the melted cheese from the pizza was still stuck to his face. If I’m not there, who will make sure he wipes off his chin, which was now dripping with grease and mozzarella?
Resigned, and feeling slightly manipulated, I agree to join in on the merriment.
Gilles’ mom once told me that sometimes you just have to do things you don’t want to do, like get a root canal, euthanize your pet lizard or unplug the toilet. And, going to this cocktail party was right up there in the category of things to avoid.
At cocktail parties, everyone usually stands around with a drink in hand, eating hors-d’oeuvres and chatting. I’m all good with the chatting and eating part. I also enjoy meeting new people. It’s the standing around that is the most difficult. When it hurts to stand for more than 5 minutes because of nerve pain in your feet, it is hard to feel relaxed at these events.
After no more than 5 minutes after our grand entrance, my anxiety rises and I desperately scan the room for a chair or something to lean on. Rocking back and forth, relieving pressure first on the right foot, then on the left, the right, the left, I wondering if the other guests are noticing my discomfort. So as to not look too silly, I even start bopping my head and swinging my hips to the beat of the music, as if I were lightly dancing and really into the melody. (This probably made me stand out that much more, but at the time, it seemed reasonable.)
10 minutes is my max tolerance before I excuse myself from whatever superficial conversation I am engaged in and plop down right into the arms of my new best friend- the chair. Thank God – the pressure is off of my aching soles. I wave to Gilles, just to show him where I’ve landed. Colleagues in tow, Gilles slowly makes his way toward me and over the noise, the music, the laughter, I overhear, “…… foot problems……can’t stand too long…..pain.” One of his cohorts, wearing a glittery cocktail dress and 10-inch heels chimes in, trying to relate to my situation, “Oh, my feet are killing me too! Ouch! I just have to slip these off….what women wouldn’t do to look their best! Staring down at my bulky size 10 (mens) hiking shoes, I could not take it anymore. “OMG, can we leave now?” I pleaded to deaf ears. “I just can’t relate to these people and they obviously can’t relate to me.” But, my protests were drowned by the noises of background laughter, glasses clinking and live, blasting music.
Loving the comfort of my chair, people politely stop by to say hi, but no one really wants to sit with me. Why? Because it is a COCKTAIL PARTY and people STAND at cocktail parties. As a seated attendee, engaging in casual or meaningful conversation with a standing guest just translates into craning of the neck in an upward position for an extended amount of time. The result? A trip to the chiropractor’s the following day. So, to bypass unnecessary appointments and self -afflicted neck pain, I set my gaze forward, looking straight in front of me. From this vantage point, the scenery is mostly just crotches and belt buckles. That’s exactly what I wrote – crotches and belt buckles!! Yep. Can you say, awkward? What’s worse, if the room is crowded, intimacy is quickly forgotten with up-close views of back pockets and butts. Feeling pretty helpless, I just end up praying that no one passes gas too close to my face. Escape would be difficult.
I usually get a few stragglers who spend some time talking about how mean Gilles is as a boss (just kidding), but mostly I pretend to be really busy on my phone, answering urgent messages, texting and making note of some vital, earth-shattering information.
I’m usually overjoyed when it is time to leave. Another holiday party over. Check! In all honesty, I think I would have rather unplugged the toilet.
There is no moral to this story, but here are a few thoughts:
If you want to see crotches and belt buckles up close and personal, attend a cocktail party. Make sure to sit for the entire duration of the party so you’ll be able to relate to my experiences.
Feign sickness and just don’t go to standing only events. Stay home, watch a movie and cuddle with your cat or dog. It’s much more relaxing and the therapeutic value is undeniable.
Ever had to use a wheelchair to get from here to there? Now, I’m not talking about temporarily using a hospital wheelchair to nurse a broken toe or to be wheeled out of the maternity ward after having a baby. I’m talking about relying on a wheelchair to get around for an indefinite amount of time because walking is too painful, extremely hard or simply not possible.
When I was pregnant with Yohan, I developed plantar fasciitis. It was 1993 and we were living in France at the time. My french PT chose to implement jackhammer “massage” therapy on the bare bottoms of both feet to loosen up the tightened fascia.
He turned on the pummeling device and went to work for what seemed forever on the right foot: GRRAKKA KKAKKAKKAKKAKKAKKAKK AKKAKKAKKAKK AKKAKKAKK AKKA KKAKKA AKK (OW, OW, OW), and then the left: GRRRAKKA KKAKKAKKAKKAKKAKKAKK AKKAKKAKKAKK AKKAKKAKK AKKA KKAKKA AKK (Double OW, OW, OW). It really hurt but I figured, “No Pain, No Gain” right?
WRONG!!!
Long story short, from that day forward, 24 years ago, nothing will ever be the same. My life had forever changed. My brain translated the pulsating vibrations as a threat, leaving the soles of my feet to ache, burn, freeze, stab, and just plain hurt. I no longer count the years, but the memory and the pain are forever etched in my feet and in my heart.
3 years later, Yohan, Gilles and I moved to California and my feet were still killing me. I tried everything, and I mean everything to alleviate the constant discomfort: acupuncture, medication, nerve blocks, psychotherapy, Tens, myofascial release, creams, gels, patches. Nothing worked, so after a lengthy and heated internal debate (the reality of using a wheelchair scared me), I purchased a custom-built wheelchair.
Here are just a few highlights from the first year or two:
“Oh dear,” laments an eighty-something-year-old in the grocery store. As I look up, she was peering down at me, pursing her lips and shaking her head slowly from side to side. “You are just too young to be in that chair.” Um. No duh, but I am, for now, and by the way, why am I even talking to you?
“Hey, this looks FUN!” yells a “friend” as he unexpectedly grabs the push handles in the back of the chair and starts to zoom me down the street, swiveling erratically to the right and to the left. He thought he was brightening my day, adding a little zest to my boring existence. Careening down the street, I was furious with my helplessness, vulnerability, and especially Monsieur Rémy. The guy who was pushing me around? We are no longer friends. He’s dead to me.
“Well, Heeellooooo Sunshine!” singsongs a salesperson at Macy’s. How are you doing today? she asks, enunciating every syllable with exaggerated grimaces which made me wonder if she thought I was deaf and had to read lips to communicate. Then she simply turned to my sister and asked if there was anything in particular “she” needed, referring to me. Oh, I get it, she assumed that I was mentally and physically disabled. You have got to be kidding me. Her strategy: avoid all eye contact with the sitter and go with the stander, the one “in control” and who looks the most normal. Normal must be in the eye of the beholder because on that particular day, my sister, Kathy, was dress as a blueberry…really. She was drumming up business for her summer business, Island Blueberries.
On another note, if you don’t get killed, some of the following situations could be translated as funny…..years after the fact.
This is the real Kathy.
Kathy, bless her heart, always volunteered to push me around downtown Burlington when I visited in the summer. Going into the mall, Kathy grunted several times in an effort to get the front wheels of the chair over the seemingly extra tall threshold (bottom of door frame). She pushed once (Hey, that was my back!…don’t use your knee!), she pushed twice and the third time….SCORE! The wheelchair unexpectedly jerked over the doorframe with such force that I fell forward, right out of the wheelchair and onto the cold, hard floor!
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That very afternoon, I Googled: manual wheelchairs + seatbelts+ overly enthusiastic sister.
On a different day, we encountered yet another obstacle. The wheelchair ramp to the store in question was short and steep. It looked something like this:
Going forward was out of the question, so Kathy, with all her might, pulled me into the store backward. Gravity was pulling me forward (I had not yet received that damn seatbelt). It felt like I was going to tumble out of that chair, and roll into the street. She got me to the top of the ramp and wouldn’t you know it…the bottom of the doorframe was again, extra high (What is it about buildings in VT?) I pleaded with her to just leave…it was not worth the trouble, but my sister does not back down when facing a challenge. And she rarely listens to me, so I held on tightly, trying to shift my weight backward. Then I heard 2 employees scream, “WE’VE GOT HER!” and before I could say, “this is really embarrassing“, one woman helped my sister pull, when the other suddenly appeared in front of me, and started pushing the arm rests to get the chair into the building. With three people pushing and/or pulling, we made it into the store, but I had had it, I was done shopping for the day.
Today, we laugh when remembering those mortifying incidences, but to those who experience similar or worse situations daily, it’s really frustrating. Here are just a couple of tips when interacting with a person with a disability:
Respect Personal Space-many use mobility aids, so don’t touch, use, lean on or move the person’s wheelchair, walker, cane, etc
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