Sisters in CMT

So alike, so far apart: sisters in CMT.

As soon as Iveta came through the door, I knew we were kindred souls. I noticed the unsteady walk right away.

Iveta standing

The crutch on her left side told me there was likely a balance problem so common with people who have CMT. She smiled and her huge eyes seemed to be asking are you like me, are we the same, how will you react to me, will we be able to talk?

Immediately I knew there was so much in common between us. One look at her hands and I knew she had to learn to cope the same way I do.
A little about my guest from afar: Iveta (pronounced Eveta,) Leipa, 45, is from Liepaja in Latvia. Leipaja is on the west coast of the country very near the Baltic Sea.

When Iveta was born, her father, who had problems with his hands and feet, noticed that there was something wrong. Her feet weren’t right. There were similarities between his feet and hers. Her father’s maternal cousins also have CMT, both male and female, but no one knew what it was.

At 10 months, Iveta began walking and did a lot of tripping and falling. She simply kept going. Her parents sent her to a regular school and for part of her elementary school experience she was teased terribly due to the way she walked and her apparent clumsiness. “They made fun of

Agra and Iveta

everything I did. Even my eyes were too big,” she said through her friend and interpreter, Agra Vagners, a special education teacher from Toronto.

At 33, Iveta went to university as a mature student, studying the Latvian language. She graduated after seven and a half years with her master’s degree and a specialty in editing.
She told me she has always worked and she has take on jobs in offices doing accounting, dispatch, for the local phone company, in statistics and on a computer.

I had also known that she was a poet from our previous talks on e-mail and I asked her how that came about. She told me that all of her relatives had said that ever since she first opened her mouth to speak, she spoke in symbols and rhymes. She’s been putting words to paper since she could write and has kept her work from age 14. She’s been published in the local press and across Latvia since 1993 and does “read- alouds” and poetry for children’s workshops.

Does her poetry talk about her life with CMT? Not directly, she said, but there is a lot in it about feelings and emotions. Several of her poems were translated into English for a muscular dystrophy event and the words have become lyrics for songs.

I had to keep remembering that Iveta does not speak or understand much English but thanks to our mutual CMT bond and Agra’s excellent understanding and ability to translate both English and Latvian, we nattered on for almost three hours.

The first thing on the agenda was the obvious: our hands and feet.

Our hands: Iveta's left, Linda's right.

We both could see that our hands were alike. Our fingers are curled and clawed, unable to open fully and partial paralyzed and our feet either fully or partially paralyzed. We have both had operations on our ankles and both have had toes fused so they wouldn’t curl under. Walking on curled toes is extremely painful.

Comparing CMT feet

When I took off my boot, I showed Iveta the skate hooks on it that allows me to do and undo them very quickly and passed the boot to her to show her how light it is. Custom-made boots don’t have to be heavy, clunky things. When the right boot maker works for you, he can use fiberglass, cork and thin leather to build what you need.

We discussed how we managed. Iveta can still move her thumbs which makes daily functioning a bit easier for her. My thumbs are paralyzed and Iveta said that was likely something in the future for her. She has had tendon releases to uncurl her fingers so she can grasp the steering wheel of her car and drive. My fingers open with a shake or help from the other hand. My left hand fingers are more open than my right that has folded because I use it for my quad grip on my steering wheel. My right hand is great for driving but not particularly good for much else. I use hand EZ Grip hand controls to drive. Love ’em.

Iveta mentioned that she has had the button on her automatic transmission shift disengaged so she didn’t have to push it in with her thumb every time she put the car in drive or park. I didn‘t know that could be done. She also said she still drives with her feet as she feels confident that she can get her foot from the gas to the brake fast enough to avoid an accident. She has an extra wide brake pedal so she can’t miss it.

I also gave my two visitors a tour through our home. I designed the house back in 1988 when I could still stand and walk a little but, now that I can no longer walk, it has turned out to be fine for driving a scooter in as well.
In every room there were questions. What kind of cutlery do I use? I showed her my lightweight coin silver antique spoons and the little tool normally used to spread frosting on a cake that I use for taking eggs out of the frying pan. A regular lifter is far too heavy for me.

What was that on the wall over the sink? It was my wall mounted Swing-Away can opened. Iveta has a counter top can opener that her father has adapted so it doesn’t move around as she uses it.

In my bedroom, we also discussed the light, plastic urinal I use at night so I don’t have to go into the bathroom and wake up my husband. As it fills, the urinal gets heavier, so I’ve adapted it with a long strong cord that goes through the urinal handle and around my neck. With the cord holding it, I can use both hands to hold the urinal and not worry about dropping it.
In the bathroom, we talked about the loop of binding tape I have sewn into my slacks that goes over my head to help me pull up my slacks when I get up off the toilet. If my slacks fall down around my ankles, I can’t lean over to pull them up. The tape keeps the slacks around knee height and I stick a finger through a belt loop and can pull them up.

When Iveta asked me through Agra how I wiped my bottom, I thought Agra would sink to the floor. You don’t often ask a comparative stranger how they take care of intimate chores but when you have CMT and very weak hands, wiping your behind can be a real problem. I understood her question and how important it is to both of us.

No one wants to lose their independence and losing the ability to perform basic hygienic tasks can be devastating. I showed her how I am able to hold the toilet paper with my left forefinger and thumb running the paper along my thumb and using part of the heel of my hand to do what needs to be done. I also thought to myself as I did, how much I hope I never lose my ability to do that.

We discussed hand-held toilet paper holders and the Japanese made TOTO toilet that washes and dries you, but neither is as a good as your own hand. Being able to feel what you are doing and where you are doing it makes a big difference.

We laughed over the fact that Iveta has jump rings one everything: her purse, her wallet, everything. I showed her the ring on my pants zipper and I knew that everything I own with a zipper also has a jump ring on it. If we can’t grasp something between our forefinger and thumb, we can slip a finger through a jump ring and pull. I also use an old-fashioned buttonhook to do up buttons but ever year the number of garments I’ve bought with buttons on them has decreased to almost nothing.

When Iveta was diagnosed at age 20, it was the first time the rest of her family had heard of the disease, even though many of them had it. This happens with many families. CMT is a common, inherited progressively debilitating neuromuscular disorder. It is estimated that one in 2500 people are born with it. That’s just as prevalent as multiple sclerosis. Pretty well everyone has heard of MS, almost no one has heard of CMT.

Three hours went quickly, we took more pictures, and then it was time to part. I was getting very tired. Talking does me in because my diaphragm is partly paralyzed. Excitement and emotion mixed with constant talk only adds to the fatigue. I hate that this happens because I’d like to go like the Energizer bunny but at 68, I have to take into consideration my limitations or I’ll get weaker and weaker and someday I won’t be able to talk. I know whereof I speak. For two and a half years in the early 90s, I could barely get a word out. My left vocal cord wouldn’t work and my voice was a double, scratchy mess. I had an Ishiki laryngoplasty done where a small silicone wedge is put against the paralyzed cord to push it against the working one. You think blowing air through vocal cords that don’t touch isn’t tiring? I hope you never have to find out.

Visiting with Iveta made me look at my own CMT. I don’t often do that. I just get on with it. I don’t worry about what people think about the fact that I can’t walk and use a scooter or that my hands aren’t normal. I’ve come to grips with my CMT. It’s part of me. You want me? You get my CMT.

I’ll think of Iveta climbing up to her fourth floor one-room apartment in Latvia and rejoice for her when she gives a poetry reading in front of her admirers. I wish her love and laughter and all the happiness in the world.

Whether or not we can understand each other’s language doesn’t matter.

A goodbye hug

Iveta and I will always be friend in CMT. We’ll e-mail each other from time to time but most of all we’ll remember the hugs that came so easily – one sister with CMT to another.

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Daily thoughts

Some things flit through my mind and others stick…sometimes for days..sometimes forever.

Last night I watched a movie called A Short Stay in Switzerland starring Julie Walters as a physician suffering, and I mean suffering, from a disease that was eventually going to see her unable to speak, swallow, feed herself, walk and, in general, function.  Her husband had died of the same thing and she knew full well what was ahead.  She decided to leave England and travel to Switzerland where she could end her life without being labeled a criminal. Her three children were told and supported her although it wasn’t easy being told by your mother that she wanted to die. They went with her to Switzerland and it was heartbreaking to see what they went through. She did the deed and it was over.

The emotional relief I felt when she was finally at rest, when her jaw dropped and you knew it was over, was just incredible for me.  I’ve long thought about this and often wondered if I’d have the intestinal fortitude to kill myself if I got to the point where my CMT life simply wasn’t worth living. And worst of all, if I had a life full of constant pain that couldn’t be relieved without being in a drugged haze. The pain I have had for the last 20 years is bad enough.  I can’t imagine it worse but then I couldn’t imagine back then living with what I have already lived with for so long. The human spirit is an amazing thing.

I think the most difficult part of the movie was when she was saying goodbye to Flora, her beautiful cat.  That just did me in.  Animals don’t understand that you are going away for good. They just love. People understand and they love. Perhaps it was because my 15-year-old deaf/blind poodle was lying on a foot stool under the television that I felt so emotional about this scene. I know his days are numbered.

It takes tremendous courage to kill yourself, it takes tremendous courage to live on with the stresses of progressive deterioration. Either way, I think it should be our choice unfetted by laws or politics. We need to know we have the option of ending it when it simply becomes too much for an intelligent, brave, optimistic soul to go on.

If you get a chance to see the movie, don’t pass it up.

Speaking of stress, I had an e-mail yesterday from someone with CMT whose family is under a great deal of stress due to a conflict with a neighbor. He was asking if stress can make your CMT worse and have there been any research articles printed on it.  I’ve been out of the CMT research loop for about eight years. But, he can search Medline for journal articles and any good university library can get them for him.

When I was in the loop and publishing the CMT Newsletter the question of stress came up often.  We who have CMT know that stress can really do a number on our body.  It only makes sense that if we have a compromised nervous system anything that unduly stresses that system will see a decrease in our ability to function.

When I asked the physicians who worked with me about stress they could only say that because they couldn’t, or didn’t have the opportunity to, measure the person’s level of function before the stressor, they couldn’t say definitively that the stress had made them worse.  I think they were being asked because someone was proposing a lawsuit. We know if we’re being affected by the stressors around us. Trying to prove it is something else.

I remember a young woman with CMT who went blind after an automobile accident and from what everyone could figure out, it was from the stress. A seemingly simple fall resulting from catching your toe on a crack in a cement sidewalk can see you truly stressed for days. It changes your entire body composure, your homeostasis. That’s why we can easily fall again… everything is out of whack from the first fall.

Fighting with a neighbor is one of those situations that makes you feel helpless. It concerns your home: the place you are supposed to be able to retreat to for rest and healing. Unfortunately, the person you are fighting with is there, in the house next door, day after day. The problem needs to be resolved. The continual stress can be devastating.

I painted outside for three hours today. During those three hours I didn’t feel the heat, had very little pain and only looked at my watch when I heard the school bus pull up next door to left my neighbour’s daughter out at her driveway.  It was some kind of heaven: water splashing over the rocks in the pond, the boat-tailed grackles gnawing and crackling in the cedars, the seeds from my Shademaster locust dropping like heavy flies on my drawing table. The bright colored pigments dropping off my brush give me more than food. Watching the pigment disperse over the water-laden paper is like watching feathers grow. The entire process lights up my brain and makes me feel alive.  I have to paint more.

I promise I’ll make these shorter.   It’s easy to go long; tough to be succinct. I have to get tougher on myself for your sake.  Thanks for being there.