CMT: An Obstacle To Success?

 

Yohan in PT – Prediagnosis

“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 The more I tried to lessen his burden by catering to his needs and lightening his load, the more resentful and withdrawn he became.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

 

304500_10151356814842995_627606583_n

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.

 

Archery is still one of his favorite pastimes.

 

 

The Inside Scoop: The Real Story Behind Bethany’s Book, “How Should a Body Be”?

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.

 

Bottom line: Buy it. It’s that good. Buy it here:  http://amzn.to/2lBC9cz

Still not convinced? How can you say “no” to this cute face?

Image may contain: 1 person

 

 

“FEEL” is a 4-Letter Word

Yohan’s Foot Surgery #19

 

 

voices-funny

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.

sore

 

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.

foot

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.

shark

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.

images-head

 

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.

Crotches and Belt Buckles: Cocktail Party Blues

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?

pizza-page

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.

cocktail-parties

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.

8_inch_and_higher_high_heel_shoes

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.

remote-mine-belt-buckle-1pockets

plasma-belt

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:

  1. 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.
  2.  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.

girls-and-their-cats-breanna-and-vivienne

 

 

Wheelchair Horror Stories – #1

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.

percus-o-matic-350

“Zis weel feel veghrry good. Eet weel ‘elp wit zee pain.” Monsieur Rémy promised.

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.

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. 

blueberry-costume-for-adults-bc-808603a

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-edited

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 (Heythat 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!

wheelchair.

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:

03c53b87f366d605c1df0f2742867243

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:

  1. Respect Personal Space-many use mobility aids, so don’t touch, use, lean on or move the person’s wheelchair, walker, cane, etc
  2. Speak in a normal tone
  3. Talk directly to the person with a disability
  4. Ask before you help

More tips to come. Have any to share?