Charcot-Marie-Tooth

CMT Awareness Month Quiz! New Questions – 2019

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1) Charcot-Marie-Tooth Disease (CMT) is also known as:

a. Inflammatory Nerve and Muscle Syndrome

b. Hereditary Motor and Sensory Neuropathy

c. Funky Foot Disorder

d. Chronic Tendinopathy with fatigue

2) How many different inheritance patterns does CMT have?

a. 1

b. 2

c. 3

d. Too many to count.

3) Can CMT skip generations?

a.Yes

b. No

4) Can CMT appear in a child if the parents’ DNA is normal?

a.Yes

b. No

5). Is CMT a type of Muscular Dystrophy?

a. Yes

b. No

6). What type of pain might you experience with CMT?

a. Neuropathic or nerve pain

b. Muscle pain

c. Joint pain

d. All of the above.

7). CMT is a heterogeneous disease. What does heterogeneous mean?

a. CMT is an inherited disease.

b. CMT is usually related to having a very high IQ.

c. CMT is caused by many different gene mutations.

d. CMT affects both men and women equally.

8). Can a person have 2 types of CMT?

a. Yes

b. No

9). Is HNPP a type of CMT?

a. Yes

b. No

10).  CMT has no cure. But, non-medicinal treatments include:

a. Physical and Occupational therapies

b. AFOs or leg braces

c. Orthopedic surgery

d. All of the above

ANSWERS

#1 – b

Hereditary Motor and Sensory Neuropathy (HMSN) Hereditary means that the disease tends to run in families and causes problems with the sensory and motor nerves, the nerves that run from the arms and legs to the spinal cord and brain.

#2 – c : 3 

The 3 types of inheritance are Autosomal Dominant, Autosomal Recessive and X-Linked. Autosomal Dominant – the faulty gene is located on one of the numbered, or non-sex, chromosomes.  Humans typically have 46 chromosomes or 23 pairs of chromosomes.  The first 22 chromosomal pairs are called autosomes. Autosomal dominant conditions affect men and women equally, and both men and women have a 50% chance in each pregnancy of passing on the condition.

If a child inherits the mutation, that child will have CMT and will have a 50% chance of passing it on again.  If the child does not inherit the change, that child will not have CMT, will not have symptoms, and will not be able to pass on the change that is in the family in the future. 

Autosomal Recessive – 2 copies of the mutation are needed to cause the disease, meaning neither copy of the gene is working properly.  In almost all cases, the changes in the gene were inherited from the parents. Each parent has one copy of the gene with a change, but because that person has one copy of the gene without a change, that person does not have symptoms of CMT and is called a “carrier.” Only people with autosomal recessive forms of CMT in the family can be considered carriers. Both males and females are affected equally with autosomal recessive conditions, and there is an equal chance of passing it on to a child, no matter the sex. If two people are carriers of an autosomal recessive form of CMT, there is a 1 in 4, or 25%, chance of both passing down the copies of the genes that do not work to a child in each pregnancy. It is only the child that inherits two copies of the gene that have mutations that will have CMT. For a person who has a recessive type of CMT, that person will pass on one of the copies of the gene with the mutation to all of that person’s children. However, only if that person’s partner is also a carrier of a mutation in this gene will it be possible to have a child that is affected with the condition. If the partner is not a carrier, it is not possible to have children affected with a recessive form of CMT, but all children will be carriers.

X-Linked – the last pair of chromosomes is called sex chromosomes. the Y and the X.  For a person with an X-linked form of CMT, the inheritance is different depending on the sex of the person affected. X-linked forms of CMT (such as CMT1X) are caused by a mutation in a gene carried on the X chromosome. Recall that females have two X chromosome and males have an X and a Y chromosome. If a female has a mutation in an X chromosome gene, she will have a 50% chance of passing on that mutation to each of her children, no matter the sex of the child. However, if a male has a mutation in an X chromosome gene, the sex of the child does make a difference. As males pass on their X chromosome to their daughters and their Y chromosome to their sons, all of the daughters of a male with an X-linked mutation will inherit the condition, and none of the sons will. Source: https://www.rarediseasesnetwork.org/cms/inc/Charcot-Marie-Tooth/What-is-CMT

#3. No. 

CMT does not skip generations.

#4. Yes.

Sometimes the parents’ DNA is normal and the CMT variation happens when the child’s DNA is forming. This is called a new or spontaneous mutation.

#5. No No, CMT is not a type of muscular dystrophy.

CMT is primarily a disease of the peripheral nerves. CMT causes weakness and impaired sensory perception because the signal can’t get to and from the brain to muscle and skin, among other things. The muscles atrophy because they aren’t getting the proper signals, but the muscles themselves are not directly diseased, per se. Muscular dystrophy is a group of diseases of the muscle itself, which causes weakness of varying degrees (there are many forms of MD).

Sometimes the heart is involved because it is a muscle too. The lungs can also be affected because the breathing muscles are weak (similar to CMT, although in CMT it is because the phrenic nerves are affected, which in turn weakens the diaphragm, our main breathing muscle). So, in summary, CMT is a genetic neuropathy which is of course a neuromuscular disorder (The euro part of the word comes from nerve). When they were expanding  the MDA early one, they included 41 of the neuromuscular diseases, including Muscular Dystrophy, CMT, Spinal Muscular Atrophy, Myasthenia Gravis and so on. The thing to remember is that when nerves stop sending the correct signals, muscles atrophy and you wind up with similar problems to those experienced by someone with a “muscle disease” like MD.

# 6. D – All the above. 

#7. C

Over 100 different genes have been identified as causing CMT  (and counting).

#8. Yes. 

The statistic is that about 1.5 percent of people will have two types of CMT. Not all variants are disease-causing. In many cases, the results of the genetic tests are often very difficult to decipher and seeing a trained CMT expert who also performs a physical exam is key for an accurate diagnosis.Please see a licensed genetic counselor (www.nsgc.org) to better understand your genetic testing results.

#9. Yes

Yes, HNPP is a form of CMT. There are three different types of CMT associated with PMP22.  People with a duplication of PMP22 have CMT1A, people with a deletion of PMP22 have HNPP, and people with a missense mutation (single letter mutation changing an amino acid) in the PMP22 gene have CMT1E.  All are hereditary forms of peripheral neuropathy, and as CMT is the umbrella name for all forms of hereditary peripheral neuropathy, that would include HNPP. (Written by genetic counselor, Shawna Feely)

#10. D. 

CMT has no cure and treatments are supportive. Foot orthotics and braces (ankle-foot-orthotics, AFOs) are commonly prescribed  to help with foot deformity and foot drop. Surgery to correct foot alignment or to lengthen or transfer tendons is often performed. Physical and occupational therapies are instrumental in providing long lasting quality of life. There is no cure for CMT nor any drug or vitamin known at this time to make CMT better.

Questions? WWW.CMTAUSA.ORG

Pony Takes Stand to End CMT

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OUR STORY –

Yohan and I had so much fun trail riding when we were younger. Oh, the stories we could tell!! I’ll never forget the time I stepped in a bee’s nest and ran for my life with Yohan on my back! He held on to my mane and stayed on. Phew! Close call!

 

 

When my friend got a little older, he rode me less and less. I thought it was my fault. Did I eat too much? Was he embarrassed about my being  just a tad shorter than a real horse?  Was I too messy? One day, we had a heart-to-heart. He explaining that he had CMT. At first, I was excited – Country Music Television?  Yee-Haw! He’ls going to be famous – a  Country Music Star! Nashville anyone? I was dreaming of lights, camera, pampering! I might even be on TV!

 

My enthusiasm was short lived when he explained that CMT stands for Charcot-Marie-Tooth disease – a progressive nerve disease that affects his hands, feet, legs, spine and balance. He just could not ride me anymore, and we were both very sad. But, he did right by me, kept me in his family and today I have the best life ever at the Horse Park in Woodside.

 

Yohan sees me every time he’s home from school and I want to celebrate his kindness, understanding and compassion. His CMT makes his nerves deteriorate and his muscles weak. He looks so happy all the time, but I know that deep down, he’s worried about what the future will bring.

This year, I’m giving back by walking for Yohan. I’m not that into exercise, so this is going to be challenging. I may not be the youngest horse in town, or the fittest, but I sure am the most determined. Please sponsor my walk. I’m walking for Yohan and all my friends with CMT. I’m walking for all who can walk no longer. I’m ready to go the distance for you. Please sponsor me: https://cmta.akaraisin.com/pledge/Participant/Home.aspx?seid=19065&mid=9&pid=4927675&fbclid=IwAR2W9g_HUpB0SXYMkphUJdpnCESvOz9E0VDy8O25VnbGoUk8fEdIzB9VMiU

 

And here’s the best part – for every $10 raised, I get a carrot! So give generously!! xoxo

Your friend,

Athos

PS: I chose $2,600 for a goal – Yohan and I are both 26, so I figured $100 for every year lived would be a good idea. Help us reach our goal!

Athos and Yohan

CMT: So Much More Than Hands and Feet!

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Yohan gets Rylee’s mind off her knee pain.

 

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

 

I will not stand for this. Will you?

Helping Yo with his drops!

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

I will not stand for this. Will you?

Bethany and Elizabeth

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

I will not stand for this. Will you?

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

I will not stand for this. Will you?

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

 

Highlights

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

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

WE will not stand for this.

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

Chris Ouellette-The organizer of the Cycle 4 CMT

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

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

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

 

Here is how to start changing lives:

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

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

Never Give up or Give In. Just GIVE!

 

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

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“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/

Raising Kids With CMT-Where In The Heck Is the Manual?

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Last week, I purchased a top-of-the-line 4K LED Smart TV with enough options to bedazzle even the most technologically gifted.  Some of the features were intuitive enough that I proudly disregarded the instruction manual altogether.  To my dismay, I soon discovered that a good number of even the simplest functions were complicated, puzzling and downright impossible to comprehend even with the handy user’s manual.

smart TV                                                           New Toy – LED TV

 

Realizing my dependence on this pamphlet of how-tos, I reflected upon all the parenting guides I have purchased over the years, all dealing with every different aspect of the child imaginable: the terrible twos, sleeplessness, anxiety, positive discipline, the emotional lives of boys, tolerating teens, etc  The one book I have yet to come across is called the “THE MANUAL: Raising Kids With An Unexpected Diagnosis (Like CMT, a Progressive Nerve Disease).”

 

Parenting-troubleshooting

 

My instruction booklet was not included in my take-home packet from the hospital when Yohan was born 25 years ago, and I still fantasize about getting my hands on a copy of this yet non-existent source of knowledge.

Initially, I learned to care for my bundle of joy by asking questions, reading a selective few of those parenting books I mentioned above, taking advice from others (even if unsolicited) and relied heavily on my own maternal instincts. Through trial and error, my husband and I, as do most parents, strove to raise and nurture our child to be a resilient human being, who would hopefully be happy, successful, compassionate, friendly, confident, etc….. None of these attributes are ever guaranteed, but parents can and do influence their children to a very large degree by modeling behaviors and values, including empathic listening, acceptance of the whole child, unconditional love, acceptance and positive discipline.

Born with unique dispositions and temperaments, every child will integrate what he or she witnesses, observes and experiences to form a personality, which is in a state of constant flux, molding to and shaped by the surrounding environment. Raising children is truly a humbling adventure, where perfecting parental skills comes through valiant efforts on the part of the father and/or mother to do the best they can at any given moment.

And just to keep us on our toes, life has this nasty habit of throwing wrenches into our well thought-out and admirable plans and dreams, forcing us to change directions or reroute our individual and family’s journey throughout life over and over again.

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For instance, when parents are told that their apparently healthy and precious child has a progressive neurological disease called CMT, (or any other chronic illness or disability) the world as they knew it comes to an abrupt halt. For a while, time just seems to stop as the shock of reality works its way through to the senses. With no current treatment or cure, CMT is a group of diseases whose effects vary greatly from one person to the next, even among members of the same family. The fear of an uncertain future for our beloved child creates a chaos so disruptive, parents may inevitably experience and re-experience a whole gamut of different feelings anywhere from grief, rage, disbelief, depression, anxiety, sadness, hopelessness, to guilt and disappointment. This flurry of unyielding and varied sets of emotions is unequivocally normal and expected in the face of such devastating and unwelcome news.

 

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Stages of Grief

 

We immediately wonder what this diagnosis means for our child’s future – will she be able to play the piano, have children and fulfill her dreams as an actress? Will his legs remain strong, what about his basketball career, and just how much will my son be affected?  Will he/she become more or less disabled than me, than my mother, than other family members? As concerned parents, we all have a tendency to get ahead of ourselves by asking a million questions about the fate of our children by continuously wondering and inquiring about what the future has in store, trying to predict what cannot be foreseen.

To top it off, in the midst of this traumatic news, unsettled parents must return home to their children, bearing the intolerable news that will inevitably change the lives of every single member of that particular family. How hard it is to remain calm, in control and at ease after you’ve been hit with the CMT hammer! In these delicate situations, let’s not forget that children are savvy readers of parental anxiety, tension, and stress. They are inexorably influenced by our attitudes, opinions, and expressions as they learn a great deal about themselves by watching, listening and absorbing all the subtle messages left behind in conversations to be had both in public and in private. They definitely do not wish to see us devastated by their disease (even if we are) and need most of all to be comforted, supported and understood.

So, when a parent unintentionally acts out his or her intense sadness, despair and anxiety in front of their children, the child is sure to take it all in, every bit of it. If our children receive messages confirming that their own situation is hopeless and the future bleak, he or she will surely feel out of control and helpless in the face of upcoming and ongoing adversity.  However, if our kids are met with reassurance and optimism, they will be more likely to cope adequately, if not conquer the intermittent hurdles that are bound to appear throughout their lives. So, the quicker we as parents learn to “deal effectively” with the challenges presented by CMT, the quicker our children will build the necessary coping skills and resilience to live well despite this disease.

Let me qualify the expression “dealing effectively with CMT” as an extremely subjective experience, which takes on a different meaning for each and every one of us. Coming to terms with your child’s progressive disease and limitations takes a lot of time and energy, and as you already know, this does not happen overnight, if ever at all.

images

There are still moments when  I am angry at this disease and obstinately think of Yohan’s CMT to be the ultimate definition of unfair lots in life, followed by a pathetic, “Why me?”, “Why him?”, “Why us?” I usually manage to calm down by focusing on what he can do, by living in the present moment and by appreciating the gifts of today. I also fervently practice replacing my negative and catastrophic thoughts with a more positive and realistic perspective which encompasses a broader, less self-centered approach to challenging situations.

The brutal reality is that I cannot control his disease or make it go away.  However, we as parents are far from powerless and impotent. On the contrary, by sharing an unwavering hope for the future, role modeling coping mechanisms such as humor, optimism, faith while remaining connected to others for support, we are fostering resiliency and courage in our children, traits on which they will rely heavily today, tomorrow and the days to come.

Let me reiterate that as parents, we have the capacity to guide and support our children, despite the absence of that darn manual.  There are always silver linings to all black clouds, you may just have to look for them. Hurdles and stumbling blocks exist to a greater or lesser extent in everyone’s life and many times, these challenges will bring families closer together or even offer a deeper, more meaningful purpose to life itself. Whatever afflicts us or our loved ones and no matter how severely, life is truly what we make of it. By planting the seeds of hope, confidence, and self-assurance, we are offering our children the right to a bright and fruitful future.

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Whether it be CMT or some other uninvited disease, our children will have acquired the tools with which to triumph over whatever may befall them, because YOU, as parents, have given them the means. No one ever said that a parenting is an easy endeavor, but your hard work, time and patience is and will make a marked difference in the minds and lives of your children and the generations to come. Manual or no manual, your kids will be forever grateful, which I deem the best award a parent could ever receive.

 

 

 

Seeking Alternatives to Narcotics: Cannabis

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We are in the midst of an opioid overdose epidemic.

As the laws on prescribing narcotics become stricter, the millions of people who suffer from chronic and debilitating pain are left to fend for themselves. Denied access to pain-relieving medication, those with excruciating conditions are suffering, searching for alternative forms of relief.

It is in this light that I asked neuromuscular specialist, Dr. Greg Carter, chief medical officer of St. Luke’s Rehabilitation Institute and clinical professor at Washington State University’s College of Medicine about his research on cannabis as it relates to neuropathic pain in CMT.

 

By Greg Carter, MD

Humans have used cannabis (marijuana) as a safe and useful pain reliever for thousands of years. With appropriate patient screening and physician oversight, it can be used to treat chronic pain, particularly neuropathic pain, which causes people with CMT much grief and suffering.

Originally delta-9-tetrahydrocannabinol (THC) THC was felt to be the main active ingredient in cannabis. However, over the past several decades, other compounds unique to cannabis (“cannabinoids”) were isolated and characterized. Cannabis is now estimated to contain over 100 such compounds, many of which are not psychoactive but have potential medicinal benefits. This includes compounds like cannabidiol (CBD) and cannabinol (CBN).

We now know there is an internal cannabinoid system in our bodies that is intricately involved in the control of movement, pain, memory, mood, motor tone, and appetite, among others. Activation of this internal “endocannabinoid system” is what produces the runners high, among countless other physiological effects.

Overall evidence indicates that cannabinoids are safe and effective if used properly and may relieve pain without serious adverse effects. You do not need to be “high” to get pain relief. Strains that have higher CBD content and lower THC strains are the best. Patients should not smoke cannabis but rather use concentrated tinctures, putting several drops under the tongue. Vaporizers can also be used, which allows for inhalation of active hot mist, without the smoke. For dosing, patients should “start low and go slow.” They can take two or three inhalations, stop, and wait 10 minutes to see what the effects are. Ingestion takes about an hour to get effects so it’s harder to dose but lasts longer. Cannabis is absorbed through the skin and may be used in a liniment for localized pain. Patients should not drive or do anything that requires full cognitive and motor function while medicated with cannabis.

Patients with CMT need to be aware of the laws in their particular state or country. Even in states that allow for medicinal use, there may be laws that require that all standard means of treating pain be tried and failed before cannabis can be offered. Arguably, any decision to offer medicinal cannabis as a treatment option will depend on the severity of the underlying pain condition and the extent to which other approaches have been tried. Patients also need to be aware that the use of cannabis for any reason remains illegal under federal law in the United States.

 

 

Here are a few of his sources, including one of his research papers:

Br J Clin Pharmacol. 2011 Nov

Cannabinoids for treatment of chronic non-cancer pain; a systematic review of randomized trials.

Lynch ME1, Campbell F.

 

Abstract

Effective therapeutic options for patients living with chronic pain are limited. The pain relieving effect of cannabinoids remains unclear. A systematic review of randomized controlled trials (RCTs) examining cannabinoids in the treatment of chronic non-cancer pain was conducted according to the PRISMA statement update on the QUORUM guidelines for reporting systematic reviews that evaluate health care interventions. Cannabinoids studied included smoked cannabis, oromucosal extracts of cannabis based medicine, nabilone, dronabinol and a novel THC analogue. Chronic non-cancer pain conditions included neuropathic pain, fibromyalgia, rheumatoid arthritis, and mixed chronic pain. Overall the quality of trials was excellent. Fifteen of the eighteen trials that met the inclusion criteria demonstrated a significant analgesic effect of cannabinoid as compared with placebo and several reported significant improvements in sleep. There were no serious adverse effects. Adverse effects most commonly reported were generally well tolerated, mild to moderate in severity and led to withdrawal from the studies in only a few cases. Overall there is evidence that cannabinoids are safe and modestly effective in neuropathic pain with preliminary evidence of efficacy in fibromyalgia and rheumatoid arthritis. The context of the need for additional treatments for chronic pain is reviewed. Further large studies of longer duration examining specific cannabinoids in homogeneous populations are required.

______________________________________________________________________________

 

J Neuroimmune Pharmacol. 2015 Jun;10(2):293-301.

Cannabinoids for the Treatment of Chronic Non-Cancer Pain: An Updated Systematic Review of Randomized Controlled Trials.

Lynch ME1, Ware MA.

Author information

1

Departments of Anesthesiology, Pain Medicine and Perioperative Care, Psychiatry and Pharmacology Dalhousie University, Halifax, Nova Scotia, Canada, mary.lynch@dal.ca.

Abstract

An updated systematic review of randomized controlled trials examining cannabinoids in the treatment of chronic non-cancer pain was conducted according to PRISMA guidelines for systematic reviews reporting on health care outcomes. Eleven trials published since our last review met inclusion criteria. The quality of the trials was excellent. Seven of the trials demonstrated a significant analgesic effect. Several trials also demonstrated improvement in secondary outcomes (e.g., sleep, muscle stiffness and spasticity). Adverse effects most frequently reported such as fatigue and dizziness were mild to moderate in severity and generally well tolerated. This review adds further support that currently available cannabinoids are safe, modestly effective analgesics that provide a reasonable therapeutic option in the management of chronic non-cancer pain.

 

Pain Manag. 2015;5(1):13-21. doi: 10.2217/pmt.14.49.

Re-branding cannabis: the next generation of chronic pain medicine?

Carter GT1, Javaher SP, Nguyen MH, Garret S, Carlini BH.

St Luke’s Rehabilitation Institute, Spokane, WA 99202, USA.

Abstract

The field of pain medicine is at a crossroads given the epidemic of addiction and overdose deaths from prescription opioids. Cannabis and its active ingredients, cannabinoids, are a much safer therapeutic option. Despite being slowed by legal restrictions and stigma, research continues to show that when used appropriately, cannabis is safe and effective for many forms of chronic pain and other conditions, and has no overdose levels. Current literature indicates many chronic pain patients could be treated with cannabis alone or with lower doses of opioids. To make progress, cannabis needs to be re-branded as a legitimate medicine and rescheduled to a more pharmacologically justifiable class of compounds. This paper discusses the data supporting re-branding and rescheduling of cannabis.

 

 

CMT: An Obstacle To Success?

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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.

 

 

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

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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

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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.

My Name is Not Grace

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Dedicated to all my friends who have a love/hate relationship with shoes.

“I love those shoes….OMG – they are sooooo cute!” enthusiastically commented an unknown, young, attractive, athletically built woman.  I looked around, certain that she was addressing someone behind me or outside my range of vision.  Mouth hanging open, I stood stunned, realizing she was referring to my shoes, my size 12 purple and aqua blue Solomon running shoes. Managing to spit out a “Thanks!” her casual compliment rendered me speechless for all of about 5 minutes (which seemed like an eternity…..to me).

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Above: The Complimented Shoes 

 

The last time someone actually told me they liked my shoes was back in September of 1967. I was 5 and my mom had just bought me a pair of black, shiny patent leather shoes. The compliments I received! Overjoyed with my new shoes I ran, jumped, danced and then, never fail, I slipped on our hardwood floors, landing head first into the electric radiator, at the base of the wall. As blood gushed from the gash on my forehead, a cloth was applied to the wound where it stayed until we reached the ER. The stitches left a small scar above my left eyebrow, a foreboding symbol of future foot-related misery.

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Above: Me, Age 5, Patent Leather Shoes

Nevertheless, I had not yet received the memo about imminent foot woes, so when my mom had to order new and very expensive shoes and winter boots from a shop in Montreal because my instep was so high, I thought that I was really something special.  Although I hadn’t a clue as to what a high instep actually was, I didn’t care. I felt like a princess who needed the best of what money could buy, and from abroad, to boot (a 2-hour drive from my hometown of Burlington, VT).  “I could get used to a life of royalty-Queen Elizabeth,” I imagined, my illusions of grandeur already a problem at such a young age. The thrill of ordering our butler around, “Andrew, Caviar, please! “or “I’ll wear the dazzling rubies this evening, Alfred! Snap, snap…I haven’t got all day!”

As I grew taller, my feet inevitably grew longer. By 8th grade, I was at least 5’7’ and my feet already demanded a size 10 shoe. Long-limbed and gawky, I looked like a baby flamingo and walked like a newborn giraffe learning to take its first steps. Between the giraffe and the flamingo, I must have looked a lot like a fliraffe.

 

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Above: Baby Flamingo

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Above: Baby Giraffe

 

 

 

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Above: Fliraffe (a giraffe with baby flamingo feet)

 

If my parents had named me Grace, I would have been a laughing stock! It was bad enough with older brothers who had their own nicknames for me: clumsy, klutz, horse, big foot, clod, butterfingers, spazz, etc. I was always bumping into something and spent a  lot of time on the ground, either cleaning up something I had spilled or nursing wounded knees.

If you have CMT, you may be able to relate to my story and have a few of your own. Does this sound familiar? I fall over air, get caught up in my own feet, trip up stairs, run into furniture and constantly drop things. Here are just a few concrete examples which come to mind: I dropped my cell in public toilets, twice, got my bike tire caught in the rails of a tram, and just simply fell over onto my side in the middle of a busy plaza, tripped on nothing and everything, sprained ankles, broken toes and sported many, many bruises. And this is just the tip of the iceberg, as the list is way too long and the catastrophes, too many to count.

Many of my friends in high school and college wore high heeled shoes for events. Not me. At 5’9’’ I was already taller than the majority of other students, especially the guys. Secondly, a size 10 high heeled shoe was impossible to find and third, I would have broken my neck.  And have you ever found a sample size 6 or 7 shoe at the store, and when they brought out the size 10 or 11, it looked nothing at all like the size 7 you had already fallen in love with?

shoes-2

Above: The shoes I wanted (floor model,size 7)

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Above: The shoes they brought out (size 12)

When I lived in France, the saleswoman wore a look of shock and disgust when I gave her my shoe size. As if being forced to wait on the Hunchback of Notre Dame, she nervously whimpered, “Madame, s’il vous plaît, look in zee secshun for zee man,” and she pointed in the direction of the men’s shoe department. How humiliating.

By adding padded and ultra cushy orthotics, my shoe size increased by 1 or 2 sizes!! On my body, an 11 or 12 shoe is not feminine. It just isn’t. I walk more like Herman Munster than a tall woman with long legs and big feet.

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Above: My body and feet

So when my new best friend complimented me on my “cute” shoes, I decided to take the compliment and wear it with pride. And, honestly, I am just grateful to be able to walk. Some are not so lucky. So, I say screw femininity.  The older I get, the less I care about what people think, especially if it is negative. Now give me positive commentary, and that my friends, is a different ball of wax.

Crotches and Belt Buckles: Cocktail Party Blues

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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.

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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.

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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

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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.

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“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. 

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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.

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Kathy, bless her heart, always volunteered to push me around downtown Burlington when I visited in the summer. Going into the mall,  Kathy grunted several times in an effort to get the front wheels of the chair over the seemingly extra tall threshold (bottom of door frame). She pushed once (Hey, that was my back!…don’t use your knee!), she pushed twice and the third time….SCORE! The wheelchair unexpectedly jerked over the doorframe with such force that I fell forward, right out of the wheelchair and onto the cold, hard floor!

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:

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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?