My Quiet Monsters
Life with Charcot Marie Tooth Disorder
by Lenka Vodicka
Winter, 2005

            I know quiet monsters.  I meet them in small white-light rooms with faded forest prints beside posters of spinal columns.  I am introduced with remarkable calm.  The names could almost be called pretty:  Scoliosis, Arthritis, Charcot Marie Tooth.
            The doctors walk away and I am left holding the hands of these quiet monsters.  I will know them.  I will become their world.  As they become mine.
            I was born into their embrace.  I walked late, had high insteps, grew up under a cloud of constant weakness and clumsy falls.  I marveled at the simple ease of others pulling ahead on trails.  Why didn’t my feet land where my eyes told them?  I walked on my toes, couldn’t walk on my heels, and avoided active sports. 
            When I was twelve, a routine school screening for scoliosis, or curvature in the spine, alerted my parents that I should be checked.  The doctor held my feet, my Barbie-doll feet with high arches, and he shrugged.  The x-rays showed a snaking curve in my spine.  He sent me to specialists at UC Davis.  They took one look at my feet and called in more specialists for more opinions.
            I was referred to an electronic diagnosis machine in a cold cement room that sent currents down my nerves.  They told me I would feel mild shocks.  To tell them when the intensity was too much.
            “Okay,” I said.   Lightning lanced needles of electricity shot down my legs. Faster.  Stronger.  “That’s enough.”
            “One more moment, kiddo.  We’re almost through.”
            Just when I thought I stood on solid ground, the waves of pain engulfed me.  The monsters rose up out of the murky depths, clinging to my ankles. 
            The doctors told my parents that I had nerve disorder called Charcot Marie Tooth Disease.  In 1984, Charcot Marie Tooth was rare.  Specialists wanted to involve me in studies, take nerve biopsies, and chart my symptoms.  Charcot Marie Tooth was incurable.  And degenerative. 
            An entire new world opened before me, with choppy gray waters and cold abysmal depths.  I knew only one thing.  Here be monsters.

            Charcot Marie Tooth was first identified by three physicians in 1886: Jean-Martin-Charcot, Pierre Marie, and Howard Henry Tooth.   
            CMT is one of over forty diseases helped by the Muscular Dystrophy Association.  CMT is not like the other diseases, though. CMT affects the nerves rather than the muscles directly. 
            Nerves use myelin to speed communication.  With CMT, myelin breaks down, causing nerves to lose conductivity, eventually dying.  The muscles depending on those nerves, in turn, atrophy.  Tendons shrink, causing hammer toes, high arches, and curled fingers.  The degeneration focuses in the hands and feet.  Generally, life expectancy is not affected.  CMT is the most commonly inherited neurological disorder.  In the United States, CMT affects 1 in every 2500 people.
            Symptoms vary intensely among individuals.  Thus, it is impossible to predict the progression of the disease.  Some people live their entire lives without ever suspecting anything beyond clumsiness.  Some people live normal lives, than develop symptoms in adulthood.  Some people have multiple surgeries on their hands and feet before they are fifteen. 
            I considered my symptoms mild.   
            I read old stories where the fairies chose to live among human kind.  Their blood could not adapt to our mortal world.  Their deep pining for their homeland made daily activities difficult, until they could no longer live into the world they chose.  They had to return to the fairy land where they belonged.
            So my secret fairy blood, I thought, made this world challenging.  Small comfort as I endured hours of waiting rooms and numerous x-rays, as acting aspirations were cut short because I looked “awkward” on stage, as friends sprinted to distant river beaches while I plodded far behind, as I passed by new sandals, unable to wear the latest styles. 
            Few people knew about my monsters.  They saw a trim blonde girl with sea-blue eyes and notebooks thick with scribbled stories tucked under her arm.  Every once in awhile, someone asked if I had hurt my foot because I limped a bit.  But not often. 
            For the next seventeen years, I looked the other way.  I earned a teaching credential and taught second grade.  I figured I would write in the downtime, though my unending exhaustion meant few stories were completed.  I married.  I visited neurologists about every five years to hear again that I had decreased sensation in my legs, weak muscles, etc.  My children would have a fifty percent chance of inheriting the disorder.  Was that a reasonable risk?  After all, would I want to have never been born?  Everyone has challenges of one kind or another.  We imagined life with a child who may not be able to participate in physical activities.  Perfection is an ideal.  We could offer unconditional love, safety, and support.  The doctors saw no reason why I should not have children.
            When I was thirty years old, I held my baby daughter Anika.  Her bright eyes and clenched fists reached right into my deepest chasms.  From the very beginning, she was strong.  She sat up, crawled, and walked right along with her friends.
            I felt firm earth underfoot again.  The path wound before me, with thorns and sunshine, but still a predictable, understood journey.  I had everything under control.
            I dreamed that if I didn’t believe in the monsters, they wouldn’t believe in me.  They would walk up to me, look in my eyes, and if I looked back unseeing, if I refused to call them real, they could not touch me.  They were nothing more than ghosts in the mists.  I could see right through them.

            I was wrong.

            I don’t remember the first day of pain.  The pain gathered as a wave offshore, moving from occasional annoyance towards a constant undercurrent.  By May of 2005, I grimaced every day as I put my feet up at lunchtime.  Friends insisted that I go to a doctor.
            “It’s nothing,” I said.  “I had tendonitis a few years ago.  I stepped wrong.  It must have flared up again.  Once I rest, I’ll be fine, really.”
            The next week I called my doctor.  She referred me to a podiatrist, who could not see me until mid-June.  I stayed off my feet as much as I could between teaching twenty second graders and caring for my own two year old.  The hurting gathered.
            My feet felt as though I had dropped dictionaries on them ten minutes before.  Not the intense hop-around-on-one-foot pain, but an insistent reminder of dull-aching-pay-attention hurting.  Sitting down no longer helped.  One morning, I woke up with sore feet.  I waited for the appointment.

            Meanwhile, I created treasures.  I knitted baby blankets in maroon and indigo yarns soft as dandelion fluff.  I painted delicate faces on beads for fairy-dolls, crocheting wild hair with aqua eyelash yarn.  I sketched illustrations for books yet to be written.  I embroidered felt with beaded accents. 
            I sat on the couch, hands busy winding folding threading working discovering gathering beauty.  The world muffled and unwound, my mind wandering into quiet.  And the gentle surprise of projects nearing completion, as hours of work arrived as a blanket, fairy, doll, inspiration.
            I gave them away, almost all of them, the blankets, fairies, dolls, inspirations.  They seemed endless, a stream of continuing projects that led one into the other over and over again.  I had no idea. 
            What treasures they truly were.
            My creative evenings.
            In May, 2005, my left pinky finger went numb.  At first it annoyed me.  I waited for a day or two without change.  The sense of fingers being asleep settled in for a long stay.  I made appointments.  The local neurologist leafed through old files.  “So, Charcot Marie Tooth,” he said. "I don't see that very often."
           He did a modern electronic-measurement of my nerve conduction.  These measured bursts didn’t hurt nearly so much as the tests of my childhood.  I did have responses above the numb area of my wrist.  Physical therapy could help.  I called to make an appointment.  The therapist could not see me for weeks.  I shook my hand in efforts to regain some feeling in my fingers.  I waited.

            In the middle of June, I walked into the podiatrist’s office.
            “You know,” he said, “The longest reconstructive surgery that I assisted was on a Charcot Marie Tooth patient.  Six hours in my residency.”
            He held my feet in his hands, pressed at tender places. He reminded me that I was not imagining my physical challenges. He said my description didn't sound like tendonitis.  
            He pulled out a rubber hammer to check for reflexes.
            "You won't find any," I said, laughing as I do when I'm nervous.
            "We'll see," he answered, tapping my knee.
            Sure enough, nothing. No Achilles reflex either. He even had me clasp my hands together in an effort to get some reaction from my tendons.
            "You're right," he conceded.
            He took x-rays.  There on the light-board in black and white, I could see how the bones of my foot just didn’t line up right. My bones were all angles, high arches and curled toes.  He pointed at gray shading where the big toe bone met the bones of the arch. "You shouldn’t have a joint there at all,” he said.  “The arch has caused those bones stress.  That looks like arthritis," he said.
            Arthritis? Isn't that for old people?  I wouldn’t allow the reality to settle into my thoughts.  This wouldn’t be so bad, I thought.  A few adjustments would be all. 
            I'd have to research. It made sense, that the pressure would make the joint swell. I really didn’t have any tools to help.
            He taped up my foot so we could see how that felt. In a couple of weeks he’d measure me for orthotics. Hopefully the orthotics would take some stress off and make my feet feel better.
            I went for a walk with Anika and suffered the whole next day. I had to stay off of my feet.
            My greatest fears loomed massive in the quiet.  What if the dreaded degeneration caught up with me at last?
            But I would not travel those thorny paths. Because my whole life was a continuum. Things must be approached day by day. Some days felt better than others.
            For the first time, I thought of the word disabled.  The idea stuck between my thoughts like a tall spiked tree laced with poisons.  I would not climb to that place.
             The doctor said that I had a classic Charcot Marie Tooth foot. I even had the characteristic “bullet hole” in my heel where two bones were so out of place that a channel for the tendon could be seen in the x-ray.
            "You can put me in your learning materials," I joked.  "As an example. I'm unusual."
            "No," he said, "You're unique."

            I started my research.  My case was osteoarthritis caused by trauma to the joint, caused by ongoing stress of bones not lined up properly due to my high arches.  I found scary words like incurable, pain, and irreversible.  I found that I am way too young to be having such strong symptoms. 
            I also read about a plateau, that pain can decrease and increase.  Which I found over the spring, as some days were worse than others.  I had to work towards the good days. 
            I stood on the crest of a gathering wave.  I spent years looking out at the wide sea, out to a horizon where the future could not touch me.
            Now I saw the falling.  The crash just ahead, unavoidable and foaming with chaotic currents.
            Only I had to reach deep, deep to the stronger currents of family and love, the center self that did not depend on outside circumstances for validation. 
            I missed the long walks, the hikes through damp woods, the sense of wandering already.  But I wasn’t giving up.  I had physical therapy the next week.  The podiatrist was going to cast my feet so they could make custom orthotic supports.  The plastic inserts for my shoes would cradle the arch and, hopefully, redistribute weight so that the arch would get relief.  The orthotics could help.  I waited.

            In late June, I met with a physical therapist specializing in neurology.  She measured my abilities to flex my feet, to push back against her hands.  She pulled out the rubber hammer.
            “You won’t find any reflexes,” I said with a smile.
            “We’ll see.” 
            Sure enough, my leg stayed relaxed and stationary as she tapped for a response.
            She had me stand to see how I balanced on my ballerina feet.  “You haven’t seen doctors for seventeen years?” she asked.
            I shook my head.  “Not beyond a check up.”
            She smiled.  “You have managed amazingly well.  You should feel good about that.”
            No one had shared that perspective before.  I was so used to being shown weakness, to being directed towards problems.  To have recognition for the incredible strength it took to get through the day brought tears to my eyes. 
            “Think about swimming to maintain muscle tone,” she said.  “We have strategies to relieve the pain.  We’ll tape up your feet today to see if that helps.”
            Another therapist came in to tape up my arches, adding strength to the aching joints.  She grinned as she struggled to make the tape lie flat along my arch.  “You win the award,” she said with a smile.  “These are the highest arches that I have ever taped.”
            I sat at home with my feet up.  My two year old, Anika, toddled up and touched the winding white tape.  “Mommy, owie?” she asked in her lilting little girl music.
            “A little,” I said.
            She kissed my arches.  “Better?”
            Better.

            The next day, I picked up paperwork qualifying me for a lifetime disabled parking permit due to “foot deformities caused by Charcot Marie Tooth.” 
            Was that the official moment, then?  Was I disabled?  Was I “rising above my circumstances” or “overcoming daily obstacles” or the hundreds of other catch phrases that attached to the disabled experience? 
            I would use the placard in challenging places like malls and fairs.  I moved into a place of permanence.  My new reality crowded against me.  My husband, Giovanni, and I playfully looked for “blue spots” to use.  Each time we pulled into the advantaged parking space, a twinge of sadness tugged at me.  I would rather walk. 
           
            By mid-July, my feet had healed considerably.  I walked through street fairs without fearing repercussions the next day.  I learned that the injuries ran deep and only time could repair the damage.  Sleep was important.  I would sleep nine hours and still nap in the afternoons.  I began to dread August and the return to teaching.  But I would manage.  I would find a way to balance the needs of the monsters and the needs of my life.  I could do it all.
            I now had permission to relax.  I could read books all afternoon.  I could put my feet up and enjoy movies in the evening.  I could allow laundry to pile up in favor of rest.  These things could not be considered troubling.  They were liberating.  The press to achieve had to be realigned towards a quest towards health.  That was not a bad thing.  Not bad at all.

            In late July, I walked into a certified hand therapist’s office.  My finger had remained persistently numb for the past two months.  She held my hand between her own, had me close my eyes while she poked my skin with what looked like stiff fishing wire. 
            “Tell me when you feel something.”
            I knew she must be touching me.  I felt nothing.
            She made a custom brace by immersing fiberglass in heated water, molding it to my wrist. 
            She tested my grip strength.  My left and right hand could squeeze 34 and 36 pounds.  “The chart ends at 38 pounds for someone your age,” she said.
            “So that’s good,” I said.  “I’m close.”
            “No, you don’t understand.  The bottom of the chart is 38 pounds.  You’re off the chart.  That’s not good.”
            I heard similar news before.  She turned my hand over, tested my finger strength.  “Well,” she said, “You have a compressed ulner nerve.  You must rest your hands.  Limit activity to thirty minutes a day.  That’s everything.  Knitting, sewing, anything needing a grip.”
            I lay on the couch at home with idle hands no longer measuring time in created pursuits.  Thoughts crowded against the edges of the evening, unable to wind themselves around my fingers. 
            CMT led to weakness through my wrists.  Joints did not have adequate support.  They could easily slip.  Pinch nerves.  In both hands.
            I must be cautious.  Avoid writing by hand.  Avoid prolonged gripping, holding, and pinching.  Rest.
            The liberation no longer sounded appealing.  I sat on the couch in my imposed rest and struggled against the sense of panicked doom rising up out of the stillness.  I had known, in high school, how one day I may need a wheelchair, how I could have physical limitations.  But I never thought about that in real terms.  I knew my feet were troubled, but I trusted my hands.  The therapist showed how muscles were atrophying around my thumb.  My joints could all hyperextend, meaning that what I thought was flexibility was actually weakness, where muscles did not have the rigidity necessary to protect the ligaments and nerves.  I had to pay attention.
            My husband and I started investigating a move to a more reasonable community, out of the exclusive foothill town where I grew up and planned to raise my daughter.  We could increase our pay and lower our bills.  I wanted to stay in our community, but the limitations of our jobs and high housing costs had me grudgingly admit that we may need a drastic change.  Perhaps next summer we would move . . .

            By early August, I knew a small peace.  I finished the last of my physical therapy appointments for my feet.  I walked around the county fair all day without trouble.  I had feeling in my fingers again.  I joined a health club so I could swim regularly.  Renewed optimism replaced the dread.
            Then school started.
            Within two days, the pain returned.  The numbness flared up.  After working to get the class ready, I collapsed into underwater naps, feeling like a stone falling into deep water, rising up disoriented, unsure of the day of the week.  It wasn’t going to last forever, I told myself.  The first few weeks would be tough.  But once the art classes, reading groups, and routines were established, I would get back to a manageable place.  I just had to tough it out for a little while.
            Right?

            Seven year old children needed so much.  “Someone stole my pencil!” was actually a dropped pencil under the desk that could not be discovered until I walked over to investigate.  Lining up needed a teacher nearby to keep hands from messing with the person ahead or behind.  Forms needed filling out and Johnny lost his homework so could he get another copy?  From the machine down the hall that confuses everyone who hasn’t learned how to use it so I better just make the copy myself?
            I typed up a letter, as my turbulent summer brought tears to my eyes when I tried talking about my health.  I shared with staff.  Music was now in a building five minutes walk away.  I arranged for help walking students so I could drive. 
            At Back to School Night, I passed out a shorter, less emotional summary of what families could expect.  Seeing me sitting down did not mean I was being lazy or negligent.  After most parents had left, a mother approached me.
            “I’m so surprised that you have Charcot Marie Tooth,” she said.  “I do too.”
            A rush of emotion swelled inside.  After 33 years, I met the first person to share my condition.  Her mother and uncle were also affected.  She had found out about CMT shortly after a cancer diagnosis, so she had a perspective that CMT was not a heavy sentence.  She beat the cancer.  She had two children who she wasn’t going to test for CMT.  If they started showing signs, that would be answer enough.  She had a wonderful doctor who knew about genetic testing and encouraged an active, engaged lifestyle that kept muscles strong and spirits positive.  Hearing that was fresh air in my gloomy outlook.  I wanted to visit this doctor.
            Unfortunately, my insurance would not work with that doctor’s network.  I should have kept seeing my local doctor who responded to my questions with questions of his own. I started entangling myself in the complicated networks of insurance policies.  After generous help from my primary care doctor and numerous phone calls to the insurance company, I finally got permission to have one second opinion appointment.  In my many calls to insurance representatives, I had to spell out Charcot Marie Tooth.  They asked what it was.  And then they wondered why I sought a specialist. 
            I called for my appointment.  I couldn’t be seen until late December.  I waited.

            September became October.  I visited the podiatrist for the casting.  He wet strips of plaster in warm water and smoothed them over my feet.  He fussed along the arch, pulling the damp strips off and trying a different angle.  “I’ll probably get a call from the lab,” he said with a grin.  “They won’t believe these are real.”
            I smiled.  What could I say?

            The custom orthotics helped a bit, but not enough.  The pain came roaring back.  I pushed through.  Day after day.  I visited my podiatrist.  “There’s not much more I can do,” he said, shrugging.  “We could chip away at the bone spurs forming over the joint, but they’ll just come back.  I’ll refer you to a surgeon.  He may be able to help.”
            “But I thought we didn’t want extensive surgery.”
            “You’re too young.  You’re healthy in all other respects.  You shouldn’t have to cope with this much pain.  You might as well get some information.  We’ll refer you.  Let us know if he doesn’t contact you within two weeks.”
            He didn’t call.  They faxed the information again.  He still didn’t call.  So I called today at the end of December to find that he no longer returned calls.  I needed to call him.  So I wait for them to return from lunch.  To make another appointment.

            In mid-October, I visited my hand therapist for the last of the insurance-approved visits.  She noticed my improved sensation.  “You need to think long and hard about your future,” she said.  “You need to stop focusing on surviving day to day.  Instead, make a list of what you would like to do in ten years.  Do you want to ride bikes with your daughter?  Because you need to make careful choices.  Your energy is valuable.  Most people with your challenges last about ten years with active jobs like yours. How long have you been teaching?”
            “Nine years.”

            I researched further schooling so that I could move out of elementary schools; teach at a college where lost pencils were not an emotional crisis.  I started an online MFA program in Creative Writing.  My husband gave Anika her baths while I analyzed Creative Writing teaching pedagogies online.
            By report cards in November, I thought, I’d have some relief.  I wouldn’t be spending weekends avoiding family outings so I could get some rest for the week ahead.  Yet, report cards arrived and my feet were constant aching soreness. 
            I broke down with colleagues at work.  Of course I would take a leave of absence if my family could afford such a thing.  But we couldn’t.  I was trapped.  I wasn’t managing.  I skated on thinning ice.  Emotions ran high.  I cried often. 
            Again, I met with my family doctor.  She explained that chronic pain can physically lower serotonin levels.  This brings stormy moods and increased perceptions of pain, leading to a cycle of worsening pain and depression.
            She gave me anti-depressant medication along with stronger pain medication.
            I met with a counselor.  She planned to help me adjust to changing shifting sands underfoot. 
            I tried to find similar stories.  I visited CMT sites online, found a wide community of people looking for answers.  Yet, only three books are available about CMT, all geared towards physicians and filled with technical language.  I asked in Barnes & Noble where they have books about disability.  The sales clerk gave me a puzzled look.  “We have a section for diseases,” he said.  No books relating to disability. 
            Part of me would like to be the Hollywood hero conquering all obstacles through indomitable will and absolute spirit, who inspires others by demonstrating that being held back doesn’t mean being held down, showing that sheer force of will can overwhelm all worldly setbacks.  Part of me would like to push to those heights, talk about the ‘gifts’ of my challenges.
            But a greater part of me listens.  Wants to honors the differences that set me apart.  Wants to hold the changes sacred, wants to slip into the gentle space with these quiet monsters and leave worldly expectations behind.  Part of me sees society as flawed, pushing me into an able/disabled choice that does nothing to accommodate the path set before me. 
            As I sit here with the monsters settled into my skin, I think perhaps they are not monsters at all.  Perhaps it is in forcing them to conform to a narrow world, a world of fast-paced, self-sacrificing, money-driven success.  I now question everything; take nothing for granted.  I research the intricacies of disability benefits.
            Perhaps the challenge is not in forcing the monsters to adapt to the outside world, but in helping the outside world understand the monsters.  Perhaps the demands to make them conform to my expectations are misguided.  Instead of struggling to stay away from the gray oceans labeled Here Be Monsters, I could swim right into the depths.  Perhaps I should ask what they want.  Perhaps I should listen.  Dance with them instead of suffocating them.  Perhaps then they will not be monsters.  They will simply be a part of the whole of who I am.

Update:  March, 2006:  Disability application is in the works.  I am waiting for approval which can take up to six months.  The financial implications no longer matter against my health.  I rely more and more on medication to get through the day.  Insurance will not approve one anti-fatigue medication that costs 100s of dollars a month yet helps immensely. 
            We are not moving from Nevada County.  I am unable to work full time anywhere, anymore.  We are looking for a house closer to town.  Our house is currently on the market in Cascade Shores.  I can no longer manage walking down our driveway in the sporadic snow.
            The orthopedic surgeon says the only option for my arthritis is reconstructive surgery in both feet, involving removing a wedge of bone and securing the bones with screws.  Each foot takes 6 months of recovery and must be done separately.
            Genetic testing came back with proof of Charcot Marie Tooth type 1A.  I have a duplication of the PMP 22 protein gene, meaning two genes where one belongs.  I actually have an extra helping of genetic material.  This is the same diagnosis as my friend.
            My school, The Nevada City School of the Arts, has selected me to receive the Teacher who Makes a Difference Award.

Update: May, 2007: Disabilty application was approved. I worked part time this past year teaching a kindergarten homestudy program that was a great success. Hooray. I completed coursework for my MFA in Creative Writing, sending off the thesis project: Dirt, Lord of All Things Dark and Mysterious.
I was selected as Teacher of the Year for Twin Ridges School District.
I now longer take daily pain medication and my arthritis is being managed through careful activity and rest.
The Cascade Shores house sold and we now live in a beautiful single story home close to town.
What a difference a year makes :)

Update: August, 2008: I continue to work part time. Dirt is finished and being considered by agents. Anika starts Kindergarten this year. We are expecting a new baby any day now. Life takes curious and wonderful new turns!

This narrative is ongoing . . . at Lenka's Living with CMT Journal

Further resources on Charcot Marie Tooth can be found at:
The Charcot Marie Tooth Association
CMTnet
The Muscular Dystrophy Association