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, Carrots and Cones- Rare Diseases to Care About

February 28 was Rare Disease Day!!

rare disease day

 

Whoop-Dee-Doo!

Is that what you are thinking?  Hold on. Before you get all judgmental on me, let me explain why I believe Rare Disease Day to be a noteworthy event.

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Rare Diseases- The Backstory

Passed by Congress in 1983, the Orphan Disease Act defined a rare disease as a condition that affects less than 200,000 people in the US. This piece of legislation provided incentives, like tax cuts, to encourage pharmaceutical companies to develop medications for rare or orphan diseases.

Here are some Rare Disease Statistics:

-There are over 7000 different types of rare diseases.

-25-30 million people in the United States are living with rare diseases. That’s 1 in 10 Americans.

-It is estimated that 350 million people worldwide suffer from rare diseases.

-80% of rare diseases are genetic in origin.

95% of rare diseases have not one single FDA approved drug treatment!!

My son, Yohan, has CMT or Charcot-Marie-Tooth disease. Though it is known as an inherited disease, Yohan’s rare disease is the result of a spontaneous genetic mutation.  I guess these things gotta start somewhere. There is currently no way to slow down the progression of CMT.

 

Over the years, Yohan has literally tripped and stumbled, learning to manage this diagnosis and its symptoms. Progressive in nature, CMT gets worse over time. So just when you think you’ve reached status quo, WHAM, something else goes wrong. It’s the nature of the beast – the stuff that life is made of.

 

 

So, having CMT is bad enough, but then life gets even more complex when:

-You trip and break your kneecap.

 

 

 

 

 

-Your tremor is so apparent that a friend tells you that maybe cold turkey is not the way to go.

– Your fingers go rogue, making it impossible to grasp your cell phone before it falls into the port-a-potty. To make matters worse, you panic and attempt to retrieve it. Ewwwwwwww! But, it no longer works. Shit happens!

 

 

 

 

 

 

 

-You accidentally hit “send” on that that email no one was supposed to see. Monday morning is going to be a bitch.

 

 

 

 

 

 

 

You are suddenly diagnosed with a second rare and progressive disease.

Yes. Really.

No one ever made a rule stating that one rare disease per person is the maximum you can have. Some lucky people get none, and others get 2 or even more.

This past summer (2017) Yohan was diagnosed with a second rare disease that has nothing to do with CMT. It’s called Keratoconus (Carrot-A Cone-Us), which also has nothing to do with carrots and a lot to do with cones.

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With Keratoconus, the clear, dome-shaped tissue that covers the eye (cornea) thins and progressively bulges outward into a cone shape. It is also progressive in nature.

THANKFULLY, there is a treatment to slow down the progression. It’s called Corneal Collagen Cross-Linking (CXL) whereby Riboflavin or Vitamin B2 is used to strengthen the cornea. Unfortunately, this procedure is not FDA approved and still in the investigational trial phase. Non-FDA approved means that insurance will not cover the procedure, so I guess we’ll be paying out of pocket.

Rare diseases are expensive.

The shock of the diagnosis is fading, and we are all coming to terms with the news. Compared to foot surgery, this “should” be a breeze. But, I have trained myself never to use the word “should” because it sets unrealistic expectations, ends up undermining me and adds weight to my already present feelings of anxiety, stress, and lack of control in the world.

 

 

 

 

 

 

My first reaction to unpleasant or distressing news is to stick my head in the sand, even if I might suffocate. Instead of facing the facts head-on, I hide out for a while and do a lot of self-talking before I can come up for air and move forward. And I’m not even the one who needs the eye surgery!

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Where I Hang Out Most Days

How’s Yohan taking this? Yohan lives in the moment, in the now. And he is resilient. He doesn’t dwell on what might happen at some point in the future. He weighs the pros, the cons, seeks out good counsel and forges ahead. He must have got that quality from his dad as he sure did not learn it from me. On the contrary, I am learning from him….again. Funny how that happens.

Deep inside I know that whatever the future holds, we’ll all be able to handle the outcome. We always do.

But I first better get the sand out of my eyes or I might be undergoing a similar eye procedure!

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

 

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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.

 

 

9 Surefire Ways to Feel More Positive

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

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

 

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

    Appreciate the beauty all around you.

 

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

 

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

    Daily life in the Middle Ages

 

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

 

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

 

 

 

 

 

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

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

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

Jeana Sweeney, this image is for you. xoxo

 

 

 

 

 

 

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

CMTA: Turning Science Into Therapies

CMTA Board Chair, Gilles Bouchard, explains STAR.

On Saturday, March 18, the CMTA put on a Patient/ Family Conference in collaboration with the University of Miami. We had a wonderful turnout, with people from all over the world in attendance. Gilles Bouchard, the CMTA’s Board Chair, explained progress to date on CMTA-funded research. I wanted to share my notes with You!

 

 

In 2008, the CMTA’s Board of Directors launched STAR, or Strategy to Accelerate Research.  It was based on two important ideas:

Idea #1: Causes Are Known

We know the causes of many types of CMT. The big breakthrough was in 1991 when the gene PMP22 for CMT1A was discovered. Today 90 different genes have been identified as causing CMT and more and more types of CMT are being discovered each year. This is the foundation of the STAR strategy because if we know the cause of the disease, we can duplicate it in the laboratory. It is often said that “a problem that is well stated is half resolved,” and this is the case for CMT, unlike most other diseases where causes are either unknown or very complex.

Gilles

  

Idea #2: Manage Research According to Sound Business Principles.

STAR is based on 5 core business principles:

  1. a) Strategy: based on knowing the cause of the disease and what to focus on.
  2. b) Our team finds the best researchers in the world and asks them to implement the projects to support our strategy, unlike most foundations who fund the best projects which are presented to them.
  3. c) Accountability is not the most prevalent value in the world of research. We hold our researchers to their goals. We take your money very seriously. Our researchers are not fully paid until they fully deliver.
  4. d) Collaboration: researchers still tend to work in silos. They are experts in one domain and have one focus. To solve CMT, we bring people from different fields together so that they work collaboratively. We are now seeing more and more technologies and therapies emanating from many different fields of study.
  5. e) Partnerships: developing a new drug is not inexpensive. It costs between 400 million to 1 billion dollars to bring a new drug to market. The CMTA does not have this kind of money. We have to work with those who have the money to develop the drugs – big, strong pharmaceutical companies. In the end, they will carry the ball over the line for us.

 

Our Strategy. There are 5 keys elements to our strategy:
1) Assays. Assays are tests. We recreate CMT in Petri dishes. And then with high throughput screening or HTS, we test hundreds of thousands of drugs. This gives us a way to see if the medications tested have any effect on CMT. We are looking for hits. What are hits? Hits are drugs that have a positive effect on CMT.

assays

 

2) Animal Models.  Once we have promising hits, we then test them on laboratory animals. From millions of potential compounds, we can narrow it down to a few of the most promising compounds or drugs.

rats

3) Stem Cells: We take human skin samples and put them through a stem cell process to create Neurons (nerve cells) or Schwann cells (which make myelin). This way, we create assays that better represent human biology. We have good models for CMT1A and have been successful with CMT type 2.


4) Partners: For CMT1A, we’ve tested millions of compounds and with the help of a major pharmaceutical company; we have several promising compounds which need to be fine-tuned for humans. With the assays, animals, and tests, we’ve created a “toolbox” for anyone who has new therapies for CMT. They can come and work with us and test them, including new technologies that may be from other domains. We can get solutions from the entire medical field. For example, 4 different drug companies who work on many different diseases reached out to the CMTA in the past couple of months alone to discuss potential therapies.

5) Clinical Trials: We are working to get ready to conduct clinical trials and develop outcome measures – how do we measure whether a drug is effective for CMT or not? See further for more details

How do we work?
We created an advisory board with top-notch researchers. The Scientific Advisory Board has 14 world class scientists. The work of STAR is not only about science, but about turning science into therapies. Another way of saying turning science into therapies is translational research. So we created the Therapy Expert Board (TEB) – a group of experts that tell us how good the science is in terms of turning it into therapies for those with CMT.

More recently, we realized we had to get ready for clinical trials and a lot of partners were coming to us for advice on how to design clinical trials and outcome measures. So we created a 3rd board, the Clinical Expert Board (CEB), where we brought together a set of world-class experts, who are helping us help our partners think about how to design clinical trials.
We have come a long way since the inception of STAR in 2008. Over the last 2 years, the CMTA has financed 40 active projects and spent 3.5 million dollars on research. We are spending your dollars wisely and in a very focused manner. We are spending 10 times more on research than when we initially started. Success breeds success. Thanks to the support from all our donors, there is huge momentum and promise.

 

 

CMTA Research Update by Disease.

 

CMT1A
Over the past 7-8 years, we’ve done animal studies, performed HTS, got hits and worked with a company called Genzyme. Today, we’ve narrowed it down to 2 families of compounds which are being fine-tuned in the lab. Genzyme uses a traditional, small molecule pharmaceutical approach. The entire biotech industry is based on an approach where you create biological living proteins and go directly after your target.

In parallel, another company came to us with a very different approach using RNA interference. RNA interference uses little pieces of DNA to get into your nerves and affect the way the cells creates the protein which overexpresses PMP22.  We’ve seen promising results in rat testing. This technology is currently used in 2 approved drugs on the market.

 

CMT1X

CMT1X is the second most common type of CMT. Researchers have identified a relationship between CMT1X and inflammation. We’ve identified the source of this inflammation and we are going after therapies to target this source. The approach comes from cancer research! Another approach is gene therapy: In CMT, there is a problem with small pieces of DNA, so you can send the right DNA via a virus into the nerves, replacing the wrong DNA. We are also investigating gene therapy for CMT4.

 

CMT1B

We have good assays and mouse models. We’ve also had several hits and potential compounds. As in CMT1X,  inflammation may play a role in CMT1B, so CMT 1X research might help CMT1B.

CMT2A

We’ve patented a rat model and have seen promising results using stem cells.  We will also complete a small screening of FDA-approved drugs this year.

CMT2E

We have stem cell assays and good animal models. Testing will commence soon.

Clinical Trials-How You Can Help

 Every person with CMT has a big role to play. There are currently 20 Center of Excellence in the US and abroad. You can help by joining our patient registry. Clinicians need as much data on as many patients as possible to help drug companies conduct successful trials. We are also developing “outcome measures” to be able to see the effect of a drug as soon as possible so that we are able to keep the trials short and inexpensive. The traditional CMT test scores require too much time to show if a drug is working or not,  so we are looking at various “biomarkers” such as fat content in calf muscles or certain chemicals in the blood.

To participate in CMT research studies, please join the Patient Registry https://www.rarediseasesnetwork.org/cms/inc/registry

You do NOT need to know the exact type of CMT you have to join this registry. And everyone in the world who has CMT can join!

 

 

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

 

 

Cracking Under Pressure

Yohan’s Foot Surgery – #17

6 months later, he is still crutching around the house.  Every time Yohan attempts to walk on the surgically repaired foot, he develops open sores and blisters on the ball of his foot. We were hopeful that the custom-made orthotic would shift his weight to a better, more functional position. It does, but his fragile skin just plain cracks under pressure.

Infection is also an on-going concern. With little sensation on the bottom of his feet, the seepage from the sore was the only indication something was amiss. Now, to avert danger, he inspects his soles day and night with a telescoping mirror.

download

“You need another surgery on that same foot?”  I repeated incredulously as if I had not heard him right the first time. Sounding deflated, he explained, “Yeah… Dr. Pfeffer wants to take the pressure off the ball my foot, by surgically lifting the bone.  He also wants to straighten my toes, and I’m not too sure about that. I’m going to think about it. Anyway, all in all, it’s a 6-8 week recovery period.”

Remaining calm and collected on the outside, I steadily asked a few more questions, showed my support and told Yohan we would do whatever it takes to get him walking again. But inside, I’m all like, WTF? Another surgery? You gotta’ be kidding me. My friend, Bethany had her feet surgically repaired and now she is walking all over the place. In fact, she can walk further and longer than most people I know. And Jeana had surgeries…ONCE on each foot, and now her feet look great! She has funky pinky toes, but hey, she can dance, walk, and exercise.

As I thought about this new piece of information, I suddenly remembered all the community members on our Facebook group who had mentioned having multiple surgeries – 10, 15, 20 operations over the years. I had put these people in the category of anomalies. They must have had really bad surgeons or maybe the procedures were done a long time ago when surgeons did not know CMT and really hadn’t a clue as to what they were doing. Today, surely, with advanced technology and techniques, a second surgery on the same foot within a 6-month time frame is probably unheard of.

Many DO have multiple surgeries to correct CMT feet. It may not be news to you, but it just hit me that I must be completely delusional.

delusional-unicorn

As I count, I realize that it is more common to require several procedures on each foot, and then some over the years, than to have one surgery and be done with it. In fact, just last week I ran into a woman at the local fruit stand who commented on the Shark-O-Marie-Tooth bumper sticker on my car. “Oh, I see you have that too,” she muttered as she whisked by me.  “What do I have?” I asked a bit confused. While feeling the ripeness of a cantaloupe, she offered, “ That thing, you know- Charcot-Marie-Tooth. My husband has that too.” She moved on to the kiwi, popping bite-sized samples into her mouth. “He’s in his 70’s now and he’s had over 23 surgeries on his feet over the years.”

I almost choked on the chunk of apple that lodged itself in my throat. Funny how reality changes once you open yourself up to the world. Haven’t you ever heard a new word, idea or process, and then you hear about it again and again, wherever you look. This phenomenon actually has a name: The Baader-Meinhof Phenomenon.   Never heard of it? You will. Now that you are in the know, your unconscious will be scanning the environment looking for this word and you will be surprised at how often it will crop up from this day forth. This phenomenon even has its own Facebook page: https://www.facebook.com/TheBaaderMeinhofPhenomenon

Where was I? Ah, yes…back to Yohan.

The second surgery on the same foot is not scheduled yet, but come January 3rd, a call to Dr. Pfeffer’s surgery nurse will be made. Welcome 2017…….it can only get better, right?

PS: It’s not all doom and gloom. Yohan is finally using the knee scooter we’ve had since day 1. He wheels himself around the house with ease, running into people, objects, walls. He’s become quite adept at steering and getting around.  Wheeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeee!

Just an FYI: CMT SUCKS

Yohan’s Foot Surgery- #16

Thanks to all who have asked for an update on Yohan. I’ve been all caught up and preoccupied with the best-selling book, “101 Practical Tips for Dealing with CMT”.  If you have not ordered one, do so once you’ve finished reading this post. As a bonus, we are shipping all the books priority mail through December. So if you buy your copy by Dec 20, you will most likely get it in time for December 25. Here is the link:

Thanks to all who have asked for an update on Yohan. I’ve been all caught up and preoccupied with the best-selling book, “101 Practical Tips for Dealing with CMT”.  If you have not ordered one, do so once you’ve finished reading this post. As a bonus, we are shipping all the books priority mail through December. So if you buy your copy by Dec 20, you will most likely get it in time for December 25. Here is the link: http://www.cmtausa.org/101tips

updated-fpss

As for Yohan, he’s more or less become a permanent fixture in our dining room. Every once in a while I’ll stroll on by, dust him off, rearrange the furniture and water the plants. In front of his computer screen, he keeps himself busy with grad school applications, gaming, writing projects, gaming, reading, and did I mention gaming? He’d be perfect at that mannequin challenge which has gone viral over the past month or better yet, he might be able to break the Guinness World Record for competitive sitting (72 hours).

yo-dining-roomedit

Looking for Yohan? Look no Further!

The only reason I know he’s still living and breathing is that nasty cough he’s had for the past 30+ days. It almost feels like a game of psychological warfare where he’s trying to make me crack, and all truth be told, he’s succeeded. I’m worn down, my will is broken and I’m more than ready to divulge any and all information I might harbor. Let the interrogation begin!

The last time I checked in, Yohan had been cleared to walk and start PT. His progress has been molasses slow since then. Why? Pressure sores. After a week or two, these sores heal, and as soon as he’s given the green light to resume walking, they break open again. It’s so frustrating!! After surgery and casting, all his hard-earned calluses dropped away, leaving fresh, pink, baby skin. Problem is –  Yohan is 23 years old and he’s totally over the baby skin stage. Like, WAY over.

baby

He’s had a shoe insert made to relieve the force on the pressure spots. And we’ve been back and forth to San Francisco 3 times in the last month to have the orthotic modified, but we are not there yet. Just this morning, that stupid sore reopened, which means one thing: Stay off the foot and get back on crutches. UGGGGGHHHHH.

Yohan will be seeing Dr. Pfeffer next week for a consultation and we’ll go from there. If we can’t take the pressure off that one spot, he may need another procedure on that foot. No way are we delving into the second foot surgery until he is 95% ambulatory with the reconstructed one. We do not know how long that will take and grad school applications have been submitted for a fall 2017 admission.

Yohan does not like surprises. He yearns for certainty and security. We all do. How do you plan your life when you can’t really plan your life? Message from the universe: Shit happens and you just have to go with the flow. You make the best of what you’re given and deal. It’s definitely not optimal, but what choice do you have? CMT sucks.

life

Question: Dealt with foot sores? Share how you managed them!

Phobias: I’ll Tell You Mine, If You Tell Me Yours!

That hairy tarantula crawling around the ranch the other morning would have, by definition, freaked out anyone who suffers from has arachnophobia, a fear of spiders. While it was basking in the sun behind a tire’s worn treads, I so wanted to put it in my palm and pet it, but before I knew it, someone had scooped it up into a bucket and displaced it into a secluded grassy area, away from me and the lethal parking lot.

And come to think of it, one of the last times I impulsively picked up a feral, furry animal, it bit me. Thankfully, that scraggly rat did not have rabies, or I would have been whisked away to the nearest hospital receiving treatment for rabies which, at the time, included 21 injections, with very long needles, into my 10-year-old abdomen.

I am not afraid of 8-legged creatures, but after reading yesterday’s news, I just might reconsider my position. “Invisible Bugs In Kansas City Are Jumping Out of Trees to Bite People, read the headline of the Wall Street Journal.  Apparently, it is oak tree itch mite season in Kansas City, MO and these microscopic arachnids are pouring out of trees and landing on people, making their lives an itchy living hell. Arachnophobes-beware! Don’t hang out under oak trees in Missouri.

mites

Oak Tree Mite

The next news story, Creepy Clown Craze Sweeps the Globe, peaked my interest.  I do not suffer from a fear of clowns (coulrophobia)…yet, but if I keep reading the news, I may have to ask for a lifetime prescription for Valium.

clowns

While we are on the subject of phobias, here is how I addressed my new-found support group of phobic cohorts:  “Hey everyone. My name is Elizabeth and I’m afraid of inclines.” Inclines? Yes, inclines, also referred to as slopes, ramps, hills, gradients, or ascents. “Welcome, Elizabeth,” they all chanted in a monotone kind of way.

There is a word for my malady. It’s called bathmophobia. For over a decade, this phobia has severely limited my ability to function well in this world. I avoid walking on even the gentlest of slopes. I’ll walk backward up a hill, but no way will I attempt to walk forwards. Sounds weird, right? Well, if you’ve ever seen me walking backward up the jetway to the airport terminal, it looks even weirder.  People stare, make jokes, avoid eye contact and get irritated at my slowness.  Generally, I ignore people by pretending to be pulling a very heavy, wheeled carry-on, or feign looking for the rest of my family who has already escaped to the terminal so as not to be associated with me.

20 years of chronic foot pain can mess you up a bit. Over the years my brain has learned to avoid potentially noxious stimulus. Protecting the area of the body that hurts is a normal response, one which is deleterious if left to linger for an extended amount of time. Muscles, tendons, and fascia tighten, and rigidity sets in. My gait has changed. There is little heel to toe motion.  My calves are tight. I’ve been walking as if I had big blocks of ice on my already very large, size 11 feet-bang, bang, bang. You can hear me coming for miles away. And you wonder why you never see me in a dress!

ice

Up until now, I’ve been half-heartedly facing my bathmophobia. Like everything else in my life, if the consequences are not dire, the task or challenge will most likely remain at the bottom of my never-ending list. Here’s the thing – Gilles and I own horses who have to move to a new pasture soon, a pasture where I will be faced with a 6 percent grade incline. Oh my……

A little bit about Athos: He is more canine than equine. Initially, Athos belonged to Yohan who discontinued riding because of CMT-related fatigue and pain. Then, I started looking after him and he quickly became my primary reason to get up early in the morning to walk and exercise.  Athos, lacking the 2 inches necessary to earn the title of horse, is technically a pony (shhhhh, don’t tell him), but he thinks he’s a Clydesdale, or maybe a Great Dane, depending on the day.  I ride Athos too, but standing 5’9” tall, with daddy longlegs limbs, my ice blocks nearly touch the ground when I get on his back. I can almost break with my frozen heels…..no joke!

athos-spider-legs

 

 

He loves kids, and kids love him. He is particularly fond of my 9-year-old “niece”, Bella, and vice versa.

:bella-and-athos

Athos loves performing.  In fact, he’s clicker trained and knows a lot of tricks, from nodding his head, “Yes” to pushing a ball around the arena to picking up sticks and retrieving. Athos will do almost anything for a carrot!

Watch the video below where Bella and Athos are playing!

video-bella

https://www.youtube.com/watch?v=gRWbpvvf0Mg

 

So, I need to get moving in the upward direction. I have my mind set on conquering the 6% grade hill below (it’s steeper than it looks, folks).  It’s become my Everest, my K2, my Annapurna. If I can do this, I can do almost anything I put my mind to.  I’ll keep you posted.

slope

 

PS: And in the meantime, stay away from 8-legged creatures, clowns, and oak trees!