February Newsletter 2002

[Guest guest]

PERSPECTIVES

Vol 2., Issue 11

February 16, 2002

In This Issue:

1) Happy Trails with Nora, Pagan the Horse, CMT and Us! - by B.

2) Golf, the Casino and the " Age " of the Aquarian - by E.

3) Teaching Challenges: CMT, My Friend and Enemy - by Rev. Devin Ann E.

4) Weight a Minute! Redirection and My Journey Now! - By D.

5) Wait! Take My Advice, I Did And Lost 40 lbs! - by Paolo V.

1) Happy Trails with Nora, Pagan the Horse, CMT and Us - B.

I had never even heard of Charcot-Marie-Tooth when my daughter, Nora was

diagnosed two and a half years ago at age 12. My husband, Nora and I

had gone out to Oregon to visit my family. We were on a hike to get to

an overlook over the Pacific Ocean and that is when I knew something was

really not right with Nora. The trail was a bit rocky in parts and

mostly uphill. She had a terrible time and had to hang on my husband's

arm to just walk on this trail.

So when we got home I called her pediatrician and told her about how

Nora seemed to have difficulties in lifting her legs, like in going

upstairs or on this uphill hike. I remember her saying something about

evaluating Nora for neurological problems and I thought to myself, " What

on earth is she talking about? Nora doesn't have any neurological

problems! She has some problem with her legs! "

The pediatrician did some basic tests in her office. I remember one was

having Nora walk on her heels, which Nora was completely unable to do.

The doctor referred us to a doctor specializing in pediatric

rehabilitative medicine at the University of Michigan Hospital. This

physician, after her evaluation, told us that Nora probably had

Charcot-Marie-Tooth disorder and sent Nora to have an EMG, which

apparently confirmed her initial diagnosis. She recommended that Nora

get AFOs, which shocked all three of us. However, after Nora wore them

once, she has basically worn them most of the time since as they make it

so much easier for her to walk.

One of Nora's initial reactions was relief. She said, " Well, I am not

just clumsy after all. " I felt very sad at first: I wished it was me

that had it and not my baby! Nora has adjusted to it very well. She

tells her very closest friends, but struggles with when to tell people

she is just getting to know. She doesn't want to be a part of anything

related to others with CMT, like the list or communicating with

other teens with CMT. We had an opportunity to meet with another

teen-aged girl and her mother and Nora refused to come with us. I

understand this: it is hard enough to feel like you belong as a

teen-ager!

The CMT diagnosis explained some things, like the fact that Nora had

never been able to learn to ice skate. She did learn finally to ride a

bike, but never really felt comfortable doing it. She has always been a

fairly cautious kid with physical things. There is one sport that she

absolutely loves and that is horseback riding. She took lessons at age

8 and just fell in love with the whole deal: horses, riding, even stall

cleaning.

When she was 10, her then trainer gave us the colt borne by the

trainer's mare. So Pagan has been a part of our lives. My husband now

manages our co-op barn with 12 boarders and he and Nora are there at

least 4 days a week. (We don't own the farm, just lease one of the barns

there.) We even have a loaner horse (I call him our " emergency back-up

horse " ) that Nora is riding while Pagan gets more training.

Riding has been a wonderful thing for Nora. She is a bit more anxious

about it than some other riders, but she is a good rider and she keeps

forging ahead. Her CMT has affected her riding only a couple of ways.

She obviously cannot mount from the ground. We have a tall mounting

block with steps with a railing that my husband built. (The dude ranch

we went to out West had a similar one they called " the dude launcher " .)

We had a physical therapist who was also a rider work with her a bit to

help her straighten out her hips. She had a tendency to keep one side a

bit more forward with the result that the horse went more to that side.

Nora wanted very much to be on the high school's equestrian team and was

this last fall. It was a challenge as Pagan wasn't ready and the horse

we leased to use developed hoof problems. But Nora was able to ride

that horse in 2 of the 3 meets. Despite her nervousness and

inexperience in showing, she did the classes the coach had signed her up

for and even decided to do one of the gaming events. I was so proud of

her!

Nora also volunteered last summer at our local therapeutic riding

program. She helped get the horses tacked up before the classes. They

were thrilled to have a volunteer who actually knew how to do that.

Of course, we worry about what the future will hold, but none of us

knows what the future holds for us. I remember shortly after we got

Nora's diagnosis, that the daughter of one of my husband's co-workers

was diagnosed with MS, so I thought, it could be worse.

2) Golf, the Casino, and the " Age " of the Aquarian- by E.

I am a brother in our CMT family of thousands! With so many new members

in our fold I thought you might like to know a little about the guy that

pops in on our now and then. Needless to say my CMT is inherited,

CMT1A, but never had a label put on it until I was 45yrs old. I have

been fortunate, my CMT has not disabled me as much as others seem to

share. I hope and pray that the day will come when we all will have to

look in the archives to find out what CMT was.

Growing up I was constantly involved in sports. About the age of twelve

I can remember my mother at times would say " You looked like your

walking on eggs. " I also remember going to the family doctor and hearing

him saying " this boy has excellent feet, he will never have flat feet,

look at that beautiful high instep. " Bless his heart, if he could only

see them now.

I continued with sports, but I begin to have problems with the ankles

turning over, falling, my knee caps popping to one side, you know the

drill. At age seventeen the high arches and hammer toes were in full

bloom and I was having a problem finding shoes that fit. I finally went

to an orthopedic doctor who wasted no time in operating on both feet. I

not sure exactly what he did, but it took seven hours to do it. No

fusion or athrodesis, probably tendon transfers. He never identified

what the cause of the deformities were. Poor man, I don't think he knew.

During and after high school I had a job with a grocery chain, on my

feet constantly. Still not being able to find a good shoe. (where were

you people when I needed you?) :-) I can remember one place that made

the insoles out of steel and the owner of the store would get mad when I

put on the new shoe and said it hurt. " Well, it takes time to break them

in " he would say. I don't know if he meant my foot or the shoe. The shoe

always won. Working and playing (go dancing almost every night) on the

feet continuously took its toll.

One day I come out of work and seeing the bus coming I reached in my

pocket to find change. I couldn't feel a thing. I doubled up what I

thought was my fist and fortunately change was in the fist when I took

it out of my pocket. I went to step up on the bus and I could not feel

the step under me, I had to look down to see if I had made contact. To

make a long story short, my nerves had given up and were shutting down

from the abuse the feet were taking.

Well, I went to night school (that's why I can't read during the day)

and earned an associates degree in accounting, hopefully to find work

off my feet. Good paying jobs were not that plentiful back then so I

took work that did involve being on my feet, and wearing normal shoes.

Hurt, wow, ever see a grown man cry?

Finally after a few years I went to work at a school for the mentally

retarded. Having lunch with the neurologist one day, he suggested I take

the NVC test and that's the first time I could put a label on my

disease.

I married and had a family of four (2 girls, 2 boys). One my girls has

inherited the CMT. She has three children and her first born has

inherited CMT. She had the blood test, that's the way I found out I had

the CMT1A. I have 11 grandchildren ranging from 4 - 17 and only the

afore mentioned young lady has it so far. Please God none of the others

will!

My CMT has been the slow progressing type. Starting with the feet and

legs, hammer toes, high instep wasting of the calf muscles in the

earlier part of my life, then progressing to the hands in the later

part of my life to a point where in my next pair of shoes I will

probably be looking at Velcro instead of shoe laces.

Back in 1992 I had my feet operated on again, this time they did an

ankle fusion on the right foot. The foot not only turned over but was

turning to the left, I was literally walking on my ankle. I now wear P.

W. Minor in depth shoes with a molded innersole. The right one has what

I call a rocker sole with an added half inch to compensate for the

fusion. They have they been a blessing.

I walk about three miles a few times a week with absolutely no pain or

discomfort. I was active in a program at the YMCA which included weight

lifting, swimming, and some aerobics (rowing machine) It really did

help. We expend so much more energy in a given task then most people do.

I'll be shall be seventy-one this February, an Aquarius. Did you know

most of the genius's are Aquarians? The thing they don't tell you, you

guessed it, most of the nut cakes are Aquarians too. I want tell you

what side I am on, but I'll let you guess.

I have been retired for ten years. I'm keep busy baby sitting, (the baby

is twenty-six, now you know what side of that line I'm on), playing at

the computer, Foxwood Casino treks now and then, out to lunch a couple

times a week, golf, playing penny ante and so much more! I don't really

know how I found the time to work!

As you know the majority of people in this world don't have CMT, but

I'll bet that majority don't have as many loving friends that I have

found on . God bless you all and keep up the good fight.

3) Teaching Challenges: CMT as My Friend and My Enemy - By Rev. Devin

Ann E.

For 27 years now, CMT has been my constant companion. Together, we have

gone to camp and national meetings. We made it through 4 years of

seminary, carefully traversing marble staircases and bumpy brick

sidewalks. I have sat many hours, alone, just me and my CMT, recovering

from surgeries and countless sprained ankles. We have walked many miles

together, just me and my CMT. Sometimes I have stumbled and sometimes I

have fallen, but I am always able to get up again, because otherwise, I

would be stuck there, sprawled on the ground, watching life pass me by

and letting my CMT win.

I was diagnosed with CMT when I was three years old. I do not remember

what it is like to be " neurologically normal. "

In my childhood, my CMT was never denied but it was not spotlighted

either. My mother, who was diagnosed with CMT in her mid-20's, taught

me that my physical abilities made me special and that I could

accomplish anything I set my mind to.

In short, CMT was not an excuse for not trying. CMT has never stopped

me from doing anything I wanted, although it has forced me to approach

ordinary tasks differently and more creatively.

As an ordained minister, who works with children and youth, I have the

opportunity to teach young people that we all have challenges in our

lives and that we need to accept and adapt to them. I feel that CMT has

opened doors for me that make it easier to relate to children and

teenagers. When teens see my wobbly stance and awkward gait, I believe

they see a part of themselves: the part that is always scared and

uncertain. They see how their lives feel out of control and

unbalanced. But then, as they get to know me, they experience the real

me, the inside being who confident and strong and funny. Suddenly, they

realize that awkwardness and instability can be conquered. I hope they

see in me that it is not the way you get around in life that matters,

but the way you face and experience life does count. I hope that my

struggles and challenges, my successes and victories, and see that they

too can achieve anything.

CMT is not something I have. It is simply a part of me. Like my smile

and my tears. Like my faith and my love. Like my determined spirit and

my compassionate soul. I am not a victim of CMT, nor am I a " patient

patient " of CMT.

CMT is a part of me, whether I like it or not. It is not something to

overcome or beat. But neither is it something I can completely accept.

Like my temper or my impatience, I seek to acknowledge it without giving

more power than it already has. CMT is a part of me and I am a part of

it. I am a face of CMT. I am young, bright and driven, yet my legs do

not work the way I wish they would. I cannot run or wear high-heeled

shoes or climb stairs well, but that's OK. These are merely actions.

They cannot and do not affect my being.

CMT will not stop me. It may make me change my path or slow my pace but

it is not a barrier. CMT is not something I have. It is simply a part

of me . . . and I am a part of it.

4) Weight a Minute! Redirection and My Journey Now! - By D.

My struggle with weight began at the age of four, when I underwent a

tonsillectomy. Until that time, I had been skinny as a toothpick, and

not very healthy. During the following year I put on about 40 pounds, a

lot for a five year old, and it's been a battle ever since.

Since childhood, I have had a passion for pottery. It has been my life

long dream to be a potter, and I spent three years in Japan in my early

twenties studying pottery, including two years as an apprentice in the

workshop of Tatsuzo Shimaoka, who was designated as a Living National

Treasure by the Japanese Government in 1997.

Pottery was not my only passion; as a teenager, I also took up growing

orchids, playing bagpipes, and studying ballet. As long as I continued

to dance, I was able to keep my weight in check, but once I settled

into the work-a-day life, the fat seemed to creep back on.

Unable to make a living from the pottery (still my dream), I received an

MBA from Tulane University in Finance in 1988 (if you recall, the stock

market crashed in the fall of 1987, so job prospects were dismal). By

the time I graduated, I weighed over 270 lbs.! My first year out of

school, I made loosing weight a priority, so I put myself on a low-fat,

high fiber diet, and began exercising regularly. At one point, I was

running three days a week and even competed in a few 10K races.

In 1992, I moved to New York City, and took a job with a firm with

offices on the 98th floor of the North Tower of the World Trade Center.

I was eating lunch at my desk when, around noon on February 26, 1993, a

truck load of explosives was detonated in the parking garage almost a

quarter mile below. This event changed my life forever. I became very

depressed, and gained almost 60 pounds in the first three months

following the bombing.

About five or six years ago, I awoke one morning to find my toes were

numb. My doctor checked me for diabetes, and said I was fine, and not to

worry. Gradually, the numbness spread back to my ankles, and I began to

experience similar symptoms in my hands. Then I began to have trouble

doing basic dance steps, and could no longer run up stairs. I finally

insisted on seeing a neurologist, who diagnosed me CMT II and Carpal

Tunnel Syndrome in both hands.

Of course, this is not something a would-be potter wants to hear - I

likened it to a neutron bomb, that kills people, but leaves buildings

standing: this wouldn't kill me, but there was a good chance it would

kill my dreams. As the CMT gradually eroded my physical activity, I

once again found solace in food. I finally tipped the scales at 315

lbs.

A year ago I decided enough was enough, and joined Weight Watchers. The

Weight Watchers program is very good, as it encourages very healthy

eating habits - lots of vegetables, moderate amounts of fruits and

grains, with a garnish of meat and a wee bit of fat. During my first

eight months with Weight Watchers, I lost 70 lbs., and was doing great!

Then, 9/11, and the second attack on the Trade Center.

One former colleague of mine lost 68 co-workers, although he made it

out. Another friend who survived worked at the Port Authority, which

lost hundreds. Once again, sleepless, anxious nights, bad

dreams,exhaustion. I found myself unable to stick with the Weight

Watchers program, and started packing on the pounds.

Then, on Christmas Eve morning, on my way to work, I was involved in a

serious car accident, in which I received numerous injuries, the most

serious being a shattered right knee. I believe that my CMT directly

contributed to this accident, due both to exhaustion and poor

proprioception in my feet (I had brought this up only a month earlier

with my doctor - he just rolled his eyes...). I was in the hospital for

three weeks, and have been confined to either bed, a walker, or a

wheelchair since. Although I have not regained all the weight I lost

last year, I have regained about half of it.

The good news is that I am using this down time to try to redirect my

efforts in life. At the moment, I am seriously thinking of going back to

school and getting a Master of Fine Arts in Ceramics and then teaching.

When I'm feeling down, I wonder who would possibly hire a ceramics

professor who is losing the use of his hands; at other times, I feel I

have a lot to offer, based on my experiences living and working in

Japan. I am no longer thinking of having to stop potting, but how to

marshal resources to be able to continue. This may require completely

rethinking my approach to my art as my CMT progresses, but that journey

itself may make a better teacher of me.

In the meantime, pass me another piece of chocolate cake! Er, celery

sticks, I mean!

6) Wait! Take My Advice, I did and Lost 40 lbs! - by Paolo V.

I've always recommended people with CMT stay slim, because muscles and

joints in the lower limbs cannot hold an increased body weight,

deformities are more severe and falls can be more damaging.

For example, knee hyper extension, which is a common feature of CMT, is

worsened by the increased weight, with knee pain and instability; so,

many patients need to keep their knees slightly bent and have cramps in

their thighs.

Moreover, CMT gait is heavier and therefore more shocking for joints

than normal gait. In case of overweight, the joints in the lower limbs

suffer even more and degenerative ostheoarthritis happens earlier and is

more severe.

People with CMT are often overweight, because they do not walk

enough.Many think they are not overweight, because their weight is not

much greater than the theoric one. But since CMT causes muscle atrophy

(wasting) and the muscles are heavy, their theoretic weight should be

diminished by the weight of the atrophied muscles!

Now my personal experience. Until twelve years ago I was 20 kg (= 40

pounds) heavier than I should be, due to good Italian food and sedentary

life. So I often fell down, despite I used AFOs. The result of these

many traumatisms was chronic knee swelling, pain, increased

hyperextension and, more important, quadriceps weakening (this muscle

always weakens when the knee joint suffers because of the inactivity

after a sprain,

and when you start to walk again, it does not hold the knee and you can

fall again).

When I fell down 3 times in the same day - once in a supermarket where I

broke several bottles - I realized I was going to be chair bound soon. I

reacted positively. I started a diet and did not stop - not even once -

until I lost all my excessive 40 pounds. Of course the damage I had

caused to my knees could not be fixed, but I became more agile and, even

in case of fall, I did not hurt so much as I did before. I needed a

strong will to lose all those pounds without medications and even more

to remain slim in time. But it was worth it, because I can still walk!

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