Life with CMT: When he left, I yelled, “Break a Leg!” So he did. 🤦‍♀️

We’re back! Yohan yelled, sliding the door to the garage open. I noticed he was walking funny, not CMT funny, but like he had a stiff leg, a heavy foot, an injured limb? My perplexed look begged the question… What now?

He brushed off my concern, acting if I were being overly paranoid and concerned (like usual). “We had a great ride and of course, on the last mile, I was tired and I took a spill. I just sprained my ankle… and…well my elbow is banged up. Oh…. and sharp stick pierced the palm of my hand when I hit the ground, but overall, I’m fine. It’ll be better in a couple of days, he said, limping down the hallway.”

After a hot shower and short rest, I took a look at the damage. “My ankle is hardly swollen,” he insisted. I pointed out that the last time I had looked at his ankle, I could see a prominent ankle bone. Now it looked puffy and bloated, as if a small jelly fish had snuck in there and took up residence. So, the crutches came out of the closet, along with the sickening memories of past orthopedic surgeries, months and months of plaster casts, pain, boots, stinky feet, scars, blood and sores.

Our bald kitties ran across the hardwood floor to greet him, but one look at the clanking crutch made them hit the brakes and off they went sliding uncontrollably, face planting into the wall. Thank God the cats make us laugh!

Recognizing the all too familiar clunk, thud, clunk, thud of Yohan’s footsteps as he made his way across the room, I too wanted to hit my head against the wall, cursing CMT to eternity and back.

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Curious Cats

Yohan’s had so many trips, falls, ankle sprains and surgeries, he knew the drill. No, not RICE. We changed that acronym to RICED. Rest. Ice. Compression. Elevation. Drugs…can’t forget the Tylenol, Aleve, etc.

There wasn’t a lot of pain, but by Monday, Yohan intuitively knew he’d better get it checked out by his doctor. This was the foot on which he has had 2 reconstructive surgeries, with mediocre results.

When the films were placed on the viewing pane, there were a lot of aahs and oohs. His x-rays lit up the room like lights on Christmas tree. The techs were amazed at the hardware holding his foot together. But who was the jokester who took a pen and drew a fine line across the outer leg bone?

What’s in your toolbox?

Fractured Fibula

Diagnosis? A hairline fracture of the fibula requiring 2 months in a walking boot, 24/7, except to bathe. Ugh. Well, it could have been worse, but it still sucks.

Home we went, trying to wrap our heads around the news. The first order of business was to purchase an even-up foot riser to avoid throwing his opposing hip out of joint. He learned pretty quickly that the even-up foot risers are treacherous as they get caught on everything…..even air. The utmost caution is warranted. And if you don’t catch the edge of the foot riser on something, the cats will make sure you fall…..hard.

Walking on 2 CMT feet is tough. Put one of those in a boot and now balance on one very high-arched foot, whose toes curl in and up, and sprinkle in some ankle supination (outward turning ankle). Not for the faint of heart!

Supination

Typical CMT Foot

Next, he looked for additional boot liners. Seriously, they send you home with 1 boot with liner. The “hand wash only” liner smells like death after the foot is enclosed within its sweaty fabric for only 24 hours. Yohan ordered a second boot (the lux version of the same brand) so he could wash the liner every other day. Luckily, when a piece of the plastic from the first boot started digging into his heel, he had a cushy alternative.

The pain from the fracture was bearable, but he started to get a painful pressure sore on the bottom of his foot which thankfully abated when slipping his orthotic into the boot. The first several nights sleeping with the boot were tough, but gradually his body got used to the inconvenience.

This morning, he woke up with a smile!! He was celebrating his 2 weeks down, six weeks to go in a boot! He’s a glass half full kind of guy.

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Taking a step back, here are my reflections:

Yohan’s new electric mountain bike offered a rainbow of opportunity, where, for the first time in quite a long while, Yohan could just be one with his friends, his dad, and his people. He did some pretty amazing rides, going places and seeing things not seen before. And these days are not gone, just suspended for a short period of time. Not the end of the world, but a bummer all the same.

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What was and what will soon be.

I guess we all have ups and downs. Personally, I prefer the ups, but don’t we all? I believe the ups build experience and self-esteem and the downs build strength of character. And then there are all the in-betweens. Every emotional state –happiness, sadness, anxiety, fear, anger – is transient, so accepting the peaks and valleys with calm and acceptance makes each situation a little easier to handle.

Life’s highs and lows are an inevitable part of being alive, so I try to enjoy the highs, learn from the lows and experience everything else in between with an open heart. I know….easier said than done.

Yohan will get through this small setback. It will become a faint but unforgettable memory that will be part of his ongoing arsenal when dealing with upcoming challenges, setbacks and successes. He’ll get back on that bike…of this I’m sure. Why? Yohan is no stranger to adversity.

He’s not giving up or giving in. He’s found an activity he thoroughly enjoys, an activity which is exhilarating and fun, creating long-lasting memories of freedom and adventure. Also, he’s determined to cycle in the Cycle 4 CMT event with his Uncle Chris, his dad and maybe even me! The Cycle 4 CMT (in person or virtual) is so much more than a cycling event……It’s a celebration of strength, resilience and community.

Vincent and Yohan

Want to know more about the Cycle (and Walk!) 4 CMT? www.cycle4cmt.com

Join us in the spirit of uniting with like-minded people, to fund research to put a stop to CMT. There is no cure for CMT… yet. But there will be because we, our CMT warriors, friends, family, loved ones, are going to make it happen. Grateful, so grateful for our community. Xo

In Flora’s Memory – Just Say It! Again and Again and Again!

“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.

To find out more about CMTA research, please visit:  https://www.cmtausa.org/breakthroughs-for-cmt/

CMT Is Exhausting: 5 Insider Tips For Friends

Judging a Book by its Cover-Just Don’t

“Who is that girl?” she asked, squinting warily while jerking her chin forward like a rooster, not so discreetly pointing out the slim, well-dressed woman in question. “She doesn’t have CMT! Look at those heels!! Pfft! Forget about it. She’s a fraud!” It’s true, Jeana did not appear to have CMT on the outside, but her past revealed a different reality. Surgeries on both feet when she was only 16 helped her gait immensely, enabling her to walk brace free today.

However, if you look real close, her pinky toes look funky, betraying a harrowing brush

.

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Jeana’s pinky toe reacts to the surgeon’s scalpel.

with a surgeon’s scalpel. “No need to worry about the little toes on each foot.” the surgeon grinned. “I simply removed all the bones! All fixed!” So, instead of hammer toes, sticking up and out, she ended up with cute, floppy, puppy dog ear toes, which prove to be quite the challenge when wearing sandals.

Floppy toes, like floppy ears, have a mind of their own!

People’s CMT vary dramatically from one person to another, and telltale symptoms of foot drop, muscle wasting, fatigue, pain are not always super obvious. So, to my embarrassment, I often forget she has CMT.

But, I forgot you have CMT!!

How many times has CMT gotten me into trouble? When Jeana and I organize CMTA conferences together, we always do our best to make sure that everything is handicapped accessible; tongs are not used, tables are separated, leaving enough room for scooters and wheelchairs to pass, boxed lunches are chosen over buffets. Inevitably, no matter how much we prep the hotel staff, someone always seems to make a mistake, and we have to think fast on our feet to rectify the situation.

At the Patient and Family conference we held in Los Angeles a few years back, I remember the room being stuffy and hot. After 90 minutes of sweating and fanning ourselves with the paper programs, we took our first 15-minute break. “A big round of applause for Dr. So and So…what an informative presentation!” I exclaimed into the microphone.   “Now, let’s take a few minutes to stretch and quench your thirst with some nice, refreshing beverages. We’ll see you back here in 15 minutes.” I walked out into the hall, and what do I see? Big, heavy pitchers of water and lemonade. Merde!

Making life difficult for people with CMT.

We quickly asked family and friends without CMT or hand weakness to serve the drinks during the break.  After about 5 minutes, I look over at the drink station and our beverage volunteer happened to have wandered off and just disappeared into thin air. People were thirsty….parched even and getting more and more impatient while shifting their weight from left to right, left to right just to stop from teetering over (keeping your balance while standing is difficult with CMT).

I ran over and filled the empty spot for a couple of minutes before my little eye spied Jeana, who was talking, and talking and talking (no surprise there, right?), to a conference attendee.

I spied Jeana with my little eye.

“Jeana,” I blurted out frantically and a bit too harshly,  “if you can’t find someone to stay at the water station, just stand here and serve the beverages to these poor people with CMT who are struggling.” She looked at me in disbelief. Her face expressed what she was thinking. “Really? You are asking me to fill glasses, dumb ass? I too have CMT!” But being the mature and respectful person we’ve all grown to admire and love, she nodded her head and made her way to the water station and tried her best to serve our conference clientele. When I realized what I had asked of her, I regretted it….immediately.  Not because I made her suffer, but because she would never let me live this situation down….EVER!  As much as she did not say in the moment, she let her thoughts be known loud and clear after the conference. In fact, she won’t hesitate to bring this up when she needs a little extra material to tease me with. I hear her voice now, “Oh, really? I’ve got a better one. Elizabeth, remember that day when….  “

Sassy!

People with CMT are fighters with motivation, determination, strength, and willpower. Some are a bit sassy, too! One day, wearing my very concerned and sad face, crying-face_1f622I said something like, “Bethany, I don’t know how you do it every day.  Just putting on socks, braces, and shoes takes so long. It all seems so limiting.”   I was attempting, albeit poorly, to relate to her emotionally in some small way.  Thankfully, I have a Master degree in Counseling Psychology, which makes communication with people from all walks of life so much easier (Like Judge Judy below, I’m inflicting some heavy duty eye rolling on myself right now).

If you know Bethany, she usually thinks through her answers before responding. Not that day! That fine afternoon she was quick and sharp, replying without missing a beat, “Yeah, it’s not the best, but I feel fortunate that I’m walking. From wheelchair to walking is a pretty huge deal.” There was a short hesitation before she emphatically reminded me that she was able to walk much further and longer than I can…a LOT further and a LOT longer. “You are limited by your foot pain.  My braces have set me free!” she replied without flinching.

IN YOUR FACE! OUCH! Insert knife and rotate slowly 3 times. I mean, what do you say to that? ……..Exactly. I said nothing and followed her progress as she trained to walk a 5-day 40 Mile Trek of Peddars Way in Norfolk, England! She succeeded and came through with flying colors!

Bethany walking and training. I am in the picture for effect only. 🙂

Just Ask

I had spoken over the phone to Bethany quite a few times before she moved to the Bay Area in 2012. We had some great conversations, talked about volunteer opportunities and CMTA-related projects. When she moved here, we made a lunch date. I drove. On the way to the restaurant, I asked 100 million questions to which she sequentially answered, “Yes. No, I don’t know”  over and over again until I got really bad cotton mouth from talking too much. So, I stopped blabbering and lived painfully through seconds upon seconds of dreadful silence (The longest ten seconds I’d ever experienced –  it felt more like 10 hours- can you tell I’m an extrovert and hyperactive?) Bethany started to loosen up by the end of lunch, and I caught a few glimpses of the witty and sardonic person living and breathing underneath that cloak of quiet reservation.

Heading back to the car, we crossed the road and I saw that she was struggling a bit to walk, but I did not know if she needed or wanted help. What to do? So, I did absolutely nothing. I pretended she wasn’t struggling and that everything was A-Okay. Walking a step or 2 in front of her, I heard a faint whisper, “Elizabeth?”  I turned around to an outreached hand.  “Can you give me your arm to cross the road?” she asked politely (her mother did a hell of a job teaching this girl manners). My elbow jutted out in a microsecond and off we went, arm in arm across the road. Walking harmoniously side by side, she interjected, with a mischievous grin,” Jeesh. You of all people did not think of lending a helping hand? I’m so disappointed.” WHOA! The girl is a live wire….a comedian! This snarky comment was proof that her dad was also very involved in her upbringing. From that moment on, we became forever friends.

Laughter Really is the best Medicine!

The CMTA held its first-ever leader conference in Las Vegas many years ago. Leaders from all over the US attended, forming bonds of friendship and caring. The first evening, after dinner, we all made our way to our rooms, preparing for a very long day ahead. Breakfast was at 8am sharp the following day. At 7 am, Pat D. our Director of Community Services at the time called me with an urgent request, “ One of our leaders needs help buttoning and zipping his pants.”  Apparently, he lived with family and on his own, buttoning and zipping were practically impossible. “Why do I have to go?” I groaned.  “Can’t we get Dana to go help him…man-to-man? Now, on the verge of yelling, Pat reminded me that, “Dana can’t button or zip his own pants, how is he going to button someone else’s pants?” Good point.

Zippers and Buttons: the enemy

So, off I go to room 3016. When he opened the door, I could tell by his red cheeks that he was quite embarrassed. I felt a bit awkward, but what were our alternatives? I could have insisted that he attend the conference in his boxers, but I’m not usually that mean. So, I simply knelt down in front of him, (please do not visualize this scene!) zipped and buttoned him up and made a joke about the entire scenario. It was a true bonding experience.  The rest of the conference was a no-brainer compared to that experience.

To all my friends with CMT, thank you for trusting me with your struggles, accomplishments, thoughts, and anxieties. And thank you for your patience. I’ve learned so much from every single one of you and continue to learn more every day. And, when I mess up, let me know  – my closest friends don’t let me off the hook, why should you?

Good Friends Are Hard To Find (which can be a very good thing). From Left to right: Me, Bethany, Jeana