Going, going, not gone, yet

I can’t even give my body away! Don’t get me wrong, I’ve never tried to sell it, but after my sister’s sudden death, my husband Ron, and I, decided we’d better get our affairs in order. My sister was our power of attorney for everything and the executor of our estate. Now that she is gone, everything needs to be changed.

Thinking that I’d like to be useful up to and including the end, I thought that surely some organization, medical school or teaching hospital would delight in a really well worn CMT2A2 body to study. Boy, was I wrong.

My search began with a call to the mortuary people who will be picking up our bodies when the time comes.

Nope, they don’t even know who to ask but there is a transport company in Toronto that takes bodies everywhere. Call them.

I make the call. Yes, certain places take bodies but the body has to meet certain criteria: no recent surgeries, no communicable disease, etc, etc.

I don’t expect to be dying after surgery but who knows how the end will come. As far as a communicable disease goes, they are far and few between here in North America but you never know what will be going around the old folks home if I check out in my 90s with only a few of my marbles left like my mother did.

McMaster University, that trains many of our young area doctors, said they would take me but upon further questioning couldn’t guarantee that I wouldn’t just be used for regular anatomy studies. My old body is so far from regular that those med students would most certainly be led astray. And, what a waste of a perfectly good CMT 2A2 body!

I feel like my body is akin to a used car with added accessories. Along with one terrific 1942 model they get fused toes, feet and ankles and they can play with the metal screws and staples that hold them together.

Also included are very slim calves with no added padding to get in the way of often stressed out, splintered shin bones.

An extra heavy thigh with added padding and a luxury-style derriere beautifully spread from years of expert sitting is a real feature.

A liver likely damaged from tons of painkillers and heaven only knows what the thousands of acid inhibitors I’ve taken over the years have done to my stomach is thrown in for examination.

My intestines are thickened from decades of constipation, sort of like winter tires with a few bulges here and there.

My heart, the main spark plug, is weak and working hard to keep my ever-cold extremities at a constant just below room temperature.

There is a fault in my electrical system because something is burning across my derriere all the time and down the back of both of my legs.

My hands feature curled fingers that only work part of time but there are great strong fingernails for prying things open and scooping up small things. My thumbs haven’t moved in maybe 30 years…a great study in atrophied muscles. My wrists feature wrist drop and they can have my wrist braces if it makes any sense to show those kids how a simple piece of plastic works even better than surgery.

One thing I do have that is really good is great upholstery. My skin is terrific – hardly any wrinkles at all. Yet.

Above the neck there is only one vocal cord working but it does the trick (is that the car horn?) and my eyes have both had double cataract surgery done on them plus lovely new lens implants. I see well. I guess they’d be similar to windshields.

My headlights are terrific round DDs.

Someone, somewhere once said I had killer eyebrows. Nobody really cares about eyebrows when they are taking your body apart.

What my brain will be like when I finally leave this mortal coil, nobody knows. The doctor looking after my mother said hers looked like lace when she died. I may go the same way if something else doesn’t get to me first.

I guess you have figured out that I’m not too broken up about dying. I don’t want to die in pain or neglect in some rest home but you take what you can get.

I did hope that a good CMT 2A2 body would be of use to someone for research purposes but I guess not in Canada, anyway. Shipping me to the United States would likely be a logistical nightmare, so that’s out as well. I can just imagine the questioning at the border crossing. The customs officer leans over and asks the driver of the hearse: “And what do you have to declare today?” Answer: “One terrific CMT2A2 body for research.” And then the fun begins.

Realistically, there likely are legitimate ways a body can be shipped into another country but I don’t think I can put my husband through the stress. If I could arrange everything beforehand, it might work.

My husband and I bought a double cemetery plot when we were married 34 years ago. It cost us $35. Yep, that’s right, $35. It’s in a lovely old cemetery out in the country and there is a big herd of Jersey or Guernsey cows, I’m never sure which is which, right across the road. It’s our kind of place.

It’ll be a lot easier for Ron to just have me picked up and shipped to the crematorium rather than having to fill out a stack of papers to have my body transported to a medical school somewhere, but hey, you can’t blame a girl for trying.

I love this photo by Paul Alexander of me on my scooter. It makes me feel good.

I love this photo by Paul Alexander of me on my scooter. It makes me feel good.

I’ve always wanted to have, She finally got warm, on my tombstone. I don’t know if Ron will do it but I might just get that done before I go. If I can’t give my old body away, it will at least rest with the cows that I love so much. Maybe Ron will mix my beloved dog’s ashes with mine. I have always said I’d like to go where dogs go. Who knows, I may find out where they go and my journey will continue.

Too bad about the medical schools missing out on a great 2A2 body, though… their loss.

I hope I’m not going anywhere anytime soon but I’ve learned that there is a very thin line between life and death and you never know when the end will come. I’ve always believed that if you can’t be useful, at least being prepared.

Until next time – stay warm.

Vanity, thy name is woman!

In 1988 my husband and I lived in a century-old home in a little old village by Lake Ontario called Port Dalhousie. Our home was really three houses that had been joined together over the years. It had two basements and a crawlspace and two furnaces. It was impossible to air condition. And, it just so happened, that summer was one of the hottest we had experienced in Southern Ontario to date.
I remember the sweat dropping off my nose onto the keyboard of my huge computer as I tried to type CMT Newsletter copy. It was then that my husband and I decided to sell the house and and build a new one that we could air-condition and that was fully accessible for me. I could stand and I could walk at the time but fell often in the old house as the floors rolled in all directions.
It took us about three weeks to sell the house using a home-made sign on the front lawn and an ad in the Toronto Globe and Mail newspaper. A neighbour saw the ad in the paper, came over, took a look through, brought some friends through, and bought the house. It all happened so fast, I wasn’t prepared to sit down and design a new house but I had to. She wanted to move in in six months. I had three months to design the house, find an architectural draftsman to draw up the plans and, most importantly, find a lot to build the house on. Then, after all that was done, I had to find a builder who could make it happen in three months. Not sure how but I found one.
Having been interested in architecture all my life and having a mother who dealt in antiques and knew houses, I got to work and pulled together a design with all the knowledge I had in me regarding accessibility.
The house was ready for us to move into the spring of 1989 and worked very well for us even during the time that I took to using a small electric scooter in the house. I had made all the doorways 36 inches wide and most were pocket doors. All the halls were five feet wide. There were 14 skylights to make it bright. Everything was totally flat. I thought I’d thought of everything, however, the bathroom medicine cabinet was about 8 inches above the counter backsplash and when I could no longer stand, I could only see the top three inches of my head in the mirror. Image
In order to put on make-up I bought a round magnifying mirror with a pull-out mechanism and Ron attached it to the wall. This worked fine for close-up work but I could never see the full effect nor could I see how my clothes looked on me from the waist up. Believe it or not, I put up with this for about 12 years. I even had a little piece of mirror framed and placed in the space between the medicine cabinet and the sink. It really didn’t work very well but it was better than nothing.
Last fall, I decided that I would do something about the bathroom situation and I had it all planned for the month of February of this year but you know what happens when you make plans. Last fall my sister, Kathie, and I visited a place that sells granite and a particularly huge slab of beautiful grey, yellow, black and white stone that would cover both vanities in the master bathroom and the vanity in the guest bathroom struck my fancy. A friend of hers is a handyman and certified electrician. He said he would do all the work involved including regrout the shower, paint the entire room and put up all the small incidental chrome pieces such as towel rings and hooks plus install the lighting fixtures I’d chosen. And, he had time to do it then, February was booked.
If you read this blog as regularly as I write it, you’l know that Kathie suddenly died in February. You know what they say about the best laid plans. Fate knew best.
The granite installers measured for and had the counters ready so quickly it was like magic. The plumber came in the morning, unhooked the plumbing, the granite guys came and put in the sinks and counters and the plumber came back in the evening to hook it all up again. Everything went like clockwork.

Now for the mirror. No more dinky mirrors. No more medicine cabinet that I couldn’t reach. I ordered the biggest piece of plate glass mirror that would fit the room. I think it is four by six feet. It is beveled on three sides and goes right down to the top of the back of the counter. I can see my head and the rest of me. Image
Observation: When you are 72, that may not be such a good thing. Gravity has a way of gaining ground. I had no idea I’d sagged so much. Now I see it all. It’s right there in plain view after every shower. Not pretty, but at least I can see it!
I did find something neat though. Moen makes a hand-held shower head with a vinyl grip that you can slip your hand through so you don’t drop it. My hands are so weak I can barely hold the soap much less grip a shower head. I love having that extra grip around the back of my hand although I still manage to drop it from time to time.
The bathroom looks great. The walls are painted a colour called “First Light” to match the light yellow in the granite. I even did a big painting to go with all the chrome and the colours in the stone. Using silver leaf to give it some pizzaz was a new venture for me and I had fun putting it together. It hangs over the new toilet with its new riser.

Image
I learned from this not to put up with something that doesn’t work for you. If you can, change it. I should have done the bathroom over years ago or, at the very least, installed a mirror I could use.
My next project is the kitchen. Ron says I’m not to tell him what I’m doing, just do it. He can’t stand the thought of spending money on a “perfectly good” 25-year old kitchen. I can.

I begin the new year on my back

What a way to begin the new year: chills, fever, a terrible cough and stuck in bed for nine days not being able to breathe well.
I won’t bore you with the gory details but there are several things I’ve learned that might help those of you with CMT who experience a bad cold or flu. (I had my flu shot back in October and I think what I’m recovering from is bronchitis.)
1) If you think you are coming down with something that will require antibiotics (something not viral), start taking acidophilus right away. Antibiotics kill all the bacteria, good and bad, in your gut and intestines and you don’t want diarrhea as well as whatever it is you have. A probiotic will keep your intestines healthy. I take one every morning sick or well. They are cheap and help keep your intestines functioning well.
2) If your diaphragm is affected by your CMT, coughing for days may weaken it to the extent that you don’t have enough push to blow your nose. If this occurs, talk to your doctor. Your sinuses can drain down into your lungs and without clearing your sinuses, you can really be miserable. I use a spray called Rhinocort (Budesonide) that helps reduce sinus swelling and a continually dripping nose. The only problem with it is that my hands aren’t strong enough to twist the dispenser. I have to ask hubby, Ron, to do it for me every morning. And, too much of it can see a nosebleed. Once my nose stops dripping continually, I designate one lip balm stick to my nose and use it to keep the inner tissues soft and lubricated. There are also products on the market that will open your nasal passages so you can breathe, whether you can blow your nose or not.

orchidwhitejan.2014
My respirologist had me take a sniff test. They do a live x-ray on your diaphragm and can see if it moves when you sniff. This is fine for one or two blows but when you are coughing maybe 50 times a day and night your diaphragm gets worn out. (I used five boxes of tissue with 65 in each.) It weakens. A sniff test doesn’t take this into consideration. I’ve even had my cough reflex conk out. I just couldn’t cough anymore even though I knew there was stuff that needed to come up. In this case suctioning can be done if the cough reflex doesn’t return.
3) This one is for me a lifesaver. Not only does it do away with the fear of having to go to the hospital where the wait is usually a grueling eight hours or more but it means I can control what happens to me when it occurs. Often with bronchitis, my lungs get so congested that I can hardly breathe. I’m fighting for every breath and can’t sleep unless I’m sitting up which is very painful as my neuropathic pain is in my behind and legs and sitting up on wrinkled sheets caused by the automatic bed is torture.
I use a portable home DeVilbiss Pulmo-Aide compact compressor nebulizer that turns the drug, Salbutamol, into a vapor and I inhale it. The vapor lets me breathe more easily and helps loosen the phlegm in my bronchial tubes. At first I might need it four times in 24 hours but the times grow further apart as my breathing becomes easier and the antibiotic (I use Biaxin slow release) begins to work.
Having a standing prescription for antibiotics, that I might or might not use once a year, and that nebulizer with the Salbutamol vials makes all the difference in the world for me. I know when I’m getting sick. I’ve had 70 plus years to get to know my body. I know the signs of a bronchial infection. Having a bit of control over my own body means a lot. For this I thank my respirologist.
4) Being female, my use of antibiotics is always followed by a vaginal yeast infection that itches like crazy (forgive me guys, but it happens). The antibiotic kills everything that keeps a good balance in that area of the female anatomy. The drugstore can supply you with an anti-fungal cream that, when used for three nights, should do the trick.
5) The last thing I do is wipe everything down with alcohol, especially my TV and electric bed remotes and my computer keyboards and mice. My drug paraphernalia is washed or wiped clean and put away for (heaven forbid) next time.
It was nine days in bed. I missed New Year’s Eve and our 33rd wedding anniversary. Then spent three days on the couch and now I’m slowly back at the computer. This is the most typing I’ve done in, maybe, three weeks and I’m heading for the couch.
My dear man, Ron, looked after all of my meals, changed my bed, did the housekeeping as he usually does, helped me in and out of the shower and was there for my every need. Thankfully, for him, I slept a lot. Without him, I’d have been in hospital. We were only rooms apart most of the time but I know I was lonely for him and he kept asking me when I thought I could get up so he wouldn’t be eating alone. After 33 years, it’s not easy being alone, even if it is temporary. And, the longer we’re together, the more we value each other.

jan2014orchid
I hope I haven’t grossed you out too much but I wish someone had told me about the standing prescription, and especially the nebulizer, years ago when I thought I was going to die, or just drown in bed, trying to get my next breathe.
The good news is that I’ve found the Smithsonian and Oasis HD channels and along with PBS (I’m hooked on Downton Abbey) and our Ontario TVO, I haven’t watched a commercial in three weeks…and my beloved orchids are all beginning to come out.
Stay well,
Linda

Wrist drop and simple bracing

Well, we are officially into fall… one of the most beautiful seasons of the year in southern Ontario. Most of the leaves have yet to turn colour but Ron brought a small branch of brilliant maple leaves home from his Sunday walk and they are as beautiful as any flower could ever be. He rarely buys me flowers but when he’s out on his walks he usually brings home something that he thinks I might like. Sunday was a branch of gorgeous leaves and a sprig of deep purple asters.

Red and orange maple leaves

Red and orange maple leaves


Fall also makes me think that in less than three months I’ll have to brace myself for the cold of winter.

However, it’s not the cold I want to talk about today but bracing.

Like many people with CMT, my feet and legs were affected first. I haven’t been able to walk in more than 20 years. Very slowly, my hands and wrists have been getting worse. My thumbs became paralyzed maybe 30 years ago. Then I was unable to straighten my fingers and shortly after that the area across my knuckles began to weaken.

For many years I had typed with a small pencil woven in and out of my fingers to straighten the longest one so I could hit the keys with it. When I lost the strength in the muscles across the knuckles in my palm, the pencil just dragged on the keyboard. I no longer had the strength to hold it up and that’s when I went to turning my hands sideways, thumbs towards the heavens, and typing with the knuckles of my little fingers. That way of typing is very hard on your shoulders so I eventually bought Dragon Dictate.

My old brace

My old brace


In the last six months I have again experienced wrist drop. I say again because many years ago I experienced this daunting progression of CMT and had a wrist brace made by the fellows at the orthotics clinic at the McMaster Health Sciences in Hamilton. My doctor had prescribed a brace that covered my entire palm and straightened my thumb so that I would have a pinch. Once measured for that brace both the orthotist and I realized that it was too much brace for my small hand. We then came up with a simple wrist enclosure with straps and a small tongue that went under the heel of my hand to hold it up. I have used that brace for 30 plus years off and on and found that I really didn’t even need the straps.
Complete wrist drop

Complete wrist drop


When you type with the knuckles of your little fingers you have to turn your hand sideways to do it. Your wrist can’t drop if it is turned sideways. However, you can’t eat easily with your wrist turned sideways, or at least, I can’t. I can start a meal – and I use a plastic fork because it’s so light – and halfway through, my wrist will no longer allow me to lift the fork to my mouth. There is simply no strength in my wrist at all.
My new brace - reminds me of a Calla lily

My new brace – reminds me of a Calla lily


I realized not too long ago that that tongue was starting to crack off the brace and it was time for a new one. A visit to my local orthotist at Niagara Prosthetics and Orthotics saw her use a Rodin 3D scanner system to calibrate the new brace. No more plaster moulds.
My wrist held up by the new brace

My wrist held up by the new brace


The first time I tried my new brace on it didn’t fit well but two subsequent visits and I had a light Duraflex brace with no straps that I could slip on by myself. The tongue was more substantial than that of my old brace and it held my wrist up beautifully. She also put holes in the underside for ventilation as plastic can get mighty hot. And, I still have some of the wrist socks they use when they put an arm in a plaster cast that I use under the brace if it is really hot out and I need to use it.
Holes in the underside of the new brace and a good sturdy tongue

Holes in the underside of the new brace and a good sturdy tongue


I can now feed myself, wash dishes, hold soap – even though my fingers don’t grasp it very well my fingernails do – and paint again, amongst other things that I couldn’t do when I experience complete wrist drop.

It’s a strange thing about wrist drop, at least in my case, anyway. Some days my wrist is strong and I don’t need the brace at all and other days three or four spoonfuls into my morning yoghurt and my wrist is completely limp and I’m searching for my brace.

Holding a fork wearing my new brace

Holding a fork wearing my new brace


I also make sure I have it in my purse when I go out to restaurants. My husband likes his food and for him to have to stop eating to feed me would really take the fun out of going out for a meal. I know he would do it, but I’d rather be able to feed myself, and my wrist brace makes it possible.

By the way, this new brace cost slightly over $1,000. The Assistive Devices Program (ADP), run by the government of the Ontario paid 75%, and my insurance the rest. Sometimes I wonder why I pay $200 plus a month for private health insurance then I run into something like this and it becomes abundantly clear.

If you have had any experience with wrist bracing, will you please share it with us?

Until next time,

Cheers!
Linda

Is it carpal tunnel syndrome or not?

Most people think that CMT causes the most problems when it affects your lower extremities but losing the use of your hands can add insult to injury. Losing your independence is no fun but you can get around with assistive devices. Losing the ability to dress yourself, put on makeup or shave, to wash your own hair, lift a fork or do your job without pain really puts CMT up front and centre.

Hi Linda,

Thank you for writing your blog, it is excellent!

My name is Erica and I was diagnosed with CMT when I was an infant. Since then, I have been aware of my condition but really have not done much research about it or have done anything to accommodate it.

I have always had difficulty with certain activities. Now, I am 25 years old and have recently started working in an office, I am feeling the painful effects of CMT. My right hand has become particularly bad and it is suspected I may have carpal tunnel syndrome. I have noticed my right hand is significantly weaker ex: I am unable to open bottles of water and it feels most comfortable when I have my hand in a fist.

I would like to find out much more about my disease and would like to learn preventative measures or therapies I should be doing.

Would you perhaps have any information for me? Or perhaps you could suggest a doctor who specializes in CMT I could see?

I find that not many people are familiar with CMT and don’t know where to begin addressing my condition. I live in Peterborough, ON.

Any information or guidance would be greatly appreciated.

My answer:

My initial reaction to your question was too asked Stuart Patterson, a hand surgeon I’ve known for years who now practices in Florida. Stuart said that there was no way he could comment on your case without seeing you, of course, but he did give me the names and addresses of several good hand surgeons not too far from you which I will put at the end of this post.

You mention in your letter that you have not done much research about CMT and carpal tunnel syndrome and that’s the first thing I’m going to suggest as the CMTA at CMTA.org has some good information about carpal tunnel and CMT.

You should also be aware of the fact that CMT can mimic carpal tunnel syndrome. You don’t want to go through an operation and have it not help you because it wasn’t what the surgeons thought it was.

Although I hate to recommend one, an electromyogram or EMG will most likely tell a neurologist whether it is carpal tunnel or CMT. And, yes, I would see a neurologist before I go to surgery, just to be sure. The neurologist will be the one who’ll likely order the EMG.

There are also medical journal articles written on CMT and carpal tunnel syndrome and just by typing CMT and carpal tunnel syndrome into my browser, I came up with several very good items.

I also have several articles about CMT hands and wrists on Linda Crabtree.com that might be helpful.

And I will suggest that you join CMTUS at https://www.facebook.com/groups/cmtus/
The discussions on that group are very helpful and you are most likely going to encounter people with CMT who also have what you are experiencing right now.

So, do your homework and you’ll find out all kinds of things on your basic CMT and about hands and wrists.

Bracing might also be an option and/or physiotherapy but make sure whomever looks after you understands that overusing the hand or wrist can make your CMT worse as in squeezing a rubber ball for exercise, etc. If you overwork the nerves that serve the muscles in your hands, the nerves will no longer fire the muscles and the muscles will atrophy with use. That’s why my hands no longer work. I’ve been typing since 1970. I now use Dragon Dictate as my main communication aid and it saves my hands.

I’ll also suggest that you talk to your doctor about having a blood test done to find out what genetic type of CMT you have, if you don’t know it, so you’ll know what to expect in the future. Being prepared and proactive will help you manage your CMT for the next 50 years or so.

You have a long and wonderful life ahead of you. I wish I was 25 again but I also wish I knew then what I know now about CMT. You have the benefit of some 30 years of writing and research so go to it and good luck.

Here are the names of some surgeons in your area courtesy of Dr. Patterson:
Richards, Dr. Robin
Sunnybrook Health Sciences Centre
2075 Bayview Ave., D5-75
Toronto, ON
M4N 3M5
Fax: 416 480-4925
Email: robin.richards@sunnybrook.ca
Specialty Interest:
Upper Extremity
Kim Mason, Executive Assistant
Tel: 416 480-5051
Email: kim.mason@sunnybrook.ca
Professor, Department of Surgery, University of Toronto
Medical Director, WSIB Shoulder and Elbow Clinic, Orthopaedic and Arthritic Institute
Deputy Editor for the Upper Extremity, Journal of Bone and Joint Surgery (Am)
Secretary and Past President, Ontario Orthopaedic Association
Secretary-Treasurer, American Shoulder and Elbow Surgeons
and
Dr David Pichora
University Position:
Professor of Surgery and Mechanical Engineering
Current Appointments:
Attending Staff, Kingston General Hospital
Attending Staff, Hotel Dieu Hospital
Areas of Clinical Interest:
Hand and Upper Extremity, Reconstructive Microsurgery, Orthopaedic Trauma, Elbow
Areas of Research Interest:
• Computer assisted surgery
• Wrist and shoulder biomechanics
• Kinematic studies of the rheumatoid wrist
• 3D motion and imaging studies of the upper extremity
• Clinical outcome studies in orthopaedic trauma
Contact Information:
Department of Surgery
Kingston General Hospital, Nickle 3
Kingston, ON K7L 2V7
Tel: 613.549.6666 x. 3395
Fax: 613.548.2526
(Secretary available in the a.m. only)
You’ll need a referral to either of these two surgeons but I would see a neurologist in your area first and take journal articles or something that says CMT can mimic carpal tunnel with you.
All the best,
Linda
p.s. – comments from readers are always appreciated.
I’m going to do an item on CMT and wrist drop plus bracing next so stay tuned.

The fear of falling

The question this time is about sudden falls and how upsetting and unsettling they can be as you never know when and where you’ll end up on the ground.

Hi Linda:

My name is Jamey Jo Steele and I am 24 years old and live in Washington State in the USA. I was diagnosed with CMT when I was 8 years old. Near as we can tell my CMT is a result of a spontaneous mutation as nobody else in my family is affected.

I had surgery on both my right and left feet when I was 8 to relax my tendons in my arches. I then had surgery at the age of 16 on my right foot which included a heel slide, hammer toe relaxation and fusion of my big toe. Recently at the age of 24, last December I had a subtalar fusion on my right foot.

I have been wearing AFOs for about 3 years now and have had nothing but trouble with them. They cause a bad callus on the outside of my foot which on both feet has got infected with staph infection and caused a lot of problems. In the past 3 years I have had 3 different AFOs made for my left foot and 4 for the right.

Recently, while working (I am a school teacher) I was simply walking around my classroom lecturing and my left knee gave out causing me to fall. I went to the hospital, nothing broken, etc., given a knee brace and told to come back in a week if things weren’t better. That same afternoon I went to stand and the same knee gave out again. Two days later while getting into a car, with a knee brace on, not putting any weight on my leg, the knee gave out again!

I saw an orthopedic surgeon yesterday and he has ordered an MRI to see what’s going on in there. He fit me with a higher quality brace. My knee is completely black and blue from bruising. I have never ever had any knee pain or problems until now.

Is this something you have experienced or know of other CMT’ers experiencing? If so, what is done to help this NOT happen. I am literally scared to death to ever walk again with out a knee brace and crutches for fear for my knee giving up on me again!!

Just looking for some advice, insight, words of wisdom from someone who seems like she knows a lot and had lived with CMT for a long time.
Thanks-
Jamey Jo

Hi Jamey Jo: Having lived with CMT a long time I can relate to your problem. CMT can affect your knees and having them give out without any warning seems to be a common occurrence for many of us. One minute we’re up and the next second we’re down. It’s not only frightening, extremely unsettling but very painful and you can’t help but wonder what’s down the road for you.
I would often fall crossing the road where the road crests because it is a bit of a hump. I recall falling in the middle of Saint Catherine Street in Montréal. If it hadn’t been for a Good Samaritan scooping me up and carrying me to the curb, I would have been human fodder for all of the taxis and sundry vehicles revving their motors to move as soon as the light turned green. I also used to knee it up curbs because my knees simply weren’t strong enough to hold me.
Angela Graham’s Facebook page CMTUS just had a discussion on what to do when you trip over a speed bump and land on your face or hands or boobs or whatever. The consensus there was that it was time for a small portable electric scooter and I fully agree. I really don’t know how you school board would react to you teaching seated on an electric scooter. If they are at all humane there should be no problem. It’s not what you are sitting on that counts but what’s in your brain.
See what the doctor comes up with after the MRI is done and try a stronger brace but if it’s like the knee problems most of us have a mobility device is the answer.
Pride makes a little light scooter called a GoGo. I bought one in the spring two years ago for about $1400 and I believe it would likely be cheaper in the United States. I also ended up buying myself an extra battery pack so that I would always have one charged and ready to go. I get up at night and have to move my scooter to use my urinal beside my bed and the picture you have to have a battery pack at night so there really isn’t any time to let it fully charge. Having two means that I can always have a charged battery pack ready to go.
A couple of things you should be aware of before you buy: the charging unit is not easy to plug-in to the battery if you have weak hands. It’s under a flap under the seat. I find the easiest way to do it is to have the charger somewhere where I can get at it very easily and then move the battery pack to it.
The battery packs are heavy so figure that into the equation.
The seat swivels but you have to have the strength to pull up on the release under the seat to let it swivel. My hands are very weak and I can barely do it but I can do it. If your hands are normal, or almost normal, you will have no trouble.
The coloured lights on the tiller that tell you if you need to charge your battery are so bright that they can impede your night vision. I have put a little black circle over them that I can lift up to see how my charge is.
I find the seat a little hard so I have an Action gel pad on it that seems to do the trick.

Linda on her GoGo. The basket is loaded and the seat swivels to be in front of the keyboard and to use Dragon Dictate

Linda on her GoGo. The basket is loaded and the seat swivels to be in front of the keyboard and to use Dragon Dictate


All in all, I like the Go-Go very much for indoor use but I would not use it if I were going to do anything on a trail or anywhere where there are a lot of bumps. It is great for a shopping plaza and would be great for school as long as there was an accessible entrance.
You can also attach a GoGo basket on the front and carry books, papers, etc. in it. I carry a water bottle, a small box of Kleenex, a Tele-Stik reacher, my wrist brace and my Kobo and in four glasses cases, the kind with the clip on the side so I an keep them from slipping down into the basket, I carry: pens, pencils, scissors, an emery board, Lipsyl, a letter opener, you name it, ready at hand.
You can also buy a device to lift a GoGo or any scooter into a trunk or the back of a van and out again at the touch of a button.
Why not contact a dealer of assistive devices near you and ask to borrow one for a weekend and see how it feels. I think the relief will be palpable.
I will also mention that there are foot ankle knee orthoses aka KAFOs. Some people with CMT use them and you could talk to your doctor and a good orthotist about them but I know that riding in style on a nice little scooter is a heck of a lot nicer than strapping on a great big KAFO every morning. But that’s just my take on it.
Will exercise help? I very much doubt it. In fact, exercise could simply exacerbate the problem.
I can hear the fear of falling in your written word and I remember it well. It’s horrible and you feel as if you want to be hanging onto something all the time, using crutches to hold you up or be carried. That reminds me of Footprints in the Sand and knowing that when you are at the worst time in your life and the most frightened, you are being carried. I’m not a religious person but I’ve learned to have an open mind and perhaps the suggestion of a Go-Go is the answer for you. God knows answers come from just about anywhere.
Good luck and let us know how you do.
If anyone out there has had the same experiences please comment on this blog post.
Cheers!
Linda

Pain and peace

Four weeks ago I was in utter despair. My pain drug, clonezapam, was no longer working and my pain level was up to an 8/10. I didn’t know where to turn. My doctor at the pain clinic had told me to go to my general practitioner to renew my prescription but I couldn’t figure out what I was going to do because it was no longer working and I had been handed over to my GP who referred me to the pain cliic in the first place. I was going in circles and in real pain.
My husband and I made an appointment to see my GP and the first thing he did was tell me that I shouldn’t be on clonazepam but I should be on Lyrica. I had tried Lyrica many years ago and had gone into a shock state where I was cold and clammy and felt just terrible. I didn’t think it was for me but I was willing to try anything.

pink hyacinths

pink hyacinths

The first night I took the Lyrica, I slept like the dead. Ten hours after I had taken the pill I still could barely move but I actually felt as if my body was relaxed. Muscles in my shoulders, my back and rib cage didn’t hurt. Lyrica was maybe going to work wonders for me! But, as most things work out, during that day the neuropathic burning pain came back and I couldn’t shake the drowsiness of the Lyrica. It seemed as if the Lyrica lasted 12 hours, and relaxed me for that length of time, then took another 12 hours to wear off so I could actually think straight and didn’t help the burning pain.
I knew my GP didn’t want to prescribe clonazepam and had told me that he would not prescribe marijuana when I asked him. So the next step was to make an appointment to go to back to the pain clinic in Hamilton and talk to my original team. I was pleasantly surprised when they said they could take me in two days time.
Ron and I made the two-hour trip to Hamilton (Ron likes to take the back roads) and Dr A.J. told me that I should be taking the clonazepam along with the Lyrica. “Two completely different drugs,” he said. Lyrica will help with my fibromyalgia pain and the clonazepam will dull my brain enough to cope with the burning neuropathic pain. I had been previously afraid to take the two of them together because I didn’t know how they would interact with each other since they both made me drowsy. I really don’t want to unknowingly overdose on prescription drugs.

My yellow roses

My yellow roses

The first night, I took two clonazepam and one Lyrica. I couldn’t get out of bed until 2 o’clock the next afternoon. I was completely out of it, but the pain was around 3/10. The next night, I took one clonazepam and one Lyrica, and realised that I had to take the drugs early. The clonazepam acts quite quickly but the Lyrica takes about an hour and a half to start working for me. So I’ve sort of changed my life around to accommodate my drugs. I go to bed around 9 o’clock; sometimes with a good book on my Kobo and sometimes watching taped TV shows, and I take my drugs early so that I’m conscious and can think by 9 or 10 o’clock in the morning. The Lyrica has pleasantly surprised me by continuing to take away that horrible back pain I used to have when I sat at my computer and it also does away with the cramping in the muscles around my ribs, pain in my shoulders and a lot of other incidental aches and pains that I have always contributed to just plain overwork but was probably fibromyalgia that I ignored from a diagnosis about 25 years ago. It’s so easy to simply ignore something when you think nothing can be done for it. I had no idea that Lyrica could do that. Feeling that I’m really in bed and not suspended on top of the mattress is a real joy. And, a huge bonus with taking Lyrica and clonazepam together is that I rarely have to get up in the middle of the night to go to the bathroom, in fact I don’t think I’ve done it more than once in the last 3 weeks, and that really helps contribute to my quality of sleep.
I hate to be talking about pain and drugs all the time but after all this blog is about CMT and me.
On a lighter note, spring has sprung hear in Niagara and it’s absolutely gorgeous out there with temperatures in the high 60s to mid 70s.

Eva Schmitz of Palatine Roses selecting rose bushes for me in the spring rain

Eva Schmitz of Palatine Roses selecting rose bushes for me in the spring rain

My sister, Kathie, and I took a trip in the rain out to a place called Palatine Roses in Niagara-on-the-Lake two weeks ago and I’ve never seen so many roses, or rather rose bushes, in one place. I bought 15 – 6 miniatures and 9 tea roses – all in various shades of pink, yellow and white. I love the ones with the cream and green on them. For my 71st birthday which was 16 April, my sister and my husband paid for my new roses. Thanks to both. You are terrific.

Harold uses a huge bulldozer to pick our 700 plus pound piece of granite out of the pile

Harold uses a huge bulldozer to pick our 700 plus pound piece of granite out of the pile

We’re having some landscaping done and hubby and I went to Montgomery Landscaping in Niagara Falls which is also a rock yard. I’ve never seen so many different types and sizes of rock in my life. The man who runs the yard, Harold, used a huge bulldozer to pick out a piece of granite I liked, weighed more than 700 pounds, that we are going to have drilled and made into a bubbling rock so the birds can take a bath and drink out of the top and small animals can drink at the base as it falls down into a hollowed out piece of stone at the bottom.
Our back garden is my place of peace, my safe haven, and I intend to be out there most of the summer. I have my Kobo reader and I have an old adjustable single bed out there. I’ll have a nice new slippery cover made for it so that I can transfer sideways onto it from my scooter, cover up with the mohair blanket, if needed be, and read while I’m surrounded by birds, chipmunks, squirrels, roses and rhododendrons.

Dogwood

Dogwood

And, were buying another dogwood tree this week. I think they are my favourite flowering tree and they grow so well here in Niagara because we are part of the Carolinian Forest zone of Canada and the United States. A lot of things will grow here that won’t grow elsewhere in Canada or the United States including beautiful dogwoods in a variety of colours.
I’ll write more as the landscaping takes off mid-May. I find it fascinating to watch the ground being moulded into something that accommodates my scooter and me plus makes my world a more beautiful place.
I’m no longer desperate from the pain but it sure wasn’t fun not knowing how things would turn out.
One thing you can never do is let go of hope. When all things look bleak and dark, hope is the one thing that can keep you going and that’s what I hung onto while I was desperately searching for ways to dull the pain that I know is always lurking under the surface of the drugs. I’m not deceiving myself that I have a long term answer but for now, I’ll take what I can get, and keep on chugging along.
Till next time, take care,
Linda

Getting through until it warms up

Our chipmunk visitor

Our chipmunk visitor

This little fellow lives in our backyard and has for about three years. We’re having some landscaping done and I’m hoping he doesn’t relocate somewhere else. He’s so much fun to watch scurrying around.
Mar. – Went on Palatine Roses website and picked out 10 roses – yellow and white, some tea, some small and one fairy light pink tiny beauty. Ordered them for pick up April 19. I’m going to try a few in the backyard where I can see them while I’m resting out there. Will there be enough sun? That’s the question.
Mar. 20 – Sister Kathie and I went to Canada Blooms in Toronto. Walked/rolled for about 5 hours. Picked up some good ideas for the yard. Bought a yellow Plumeria cutting for $10 and it is rooting. Bought Heavenly Blue Morning Glory seeds and Passion Flower vine seeds ($3. ea.). I’m thinking that when the landscaper replaces the lattice around the air conditioner, I can plant the Passion Flower seeds and a blue clematis that won’t grow on the north facing garage wall.
Mar. 29 – I haven’t driven in months and had to try to see if I could transfer from the swivel seat to the driver’s side without too much trouble. I did it but don’t think I can do it alone. I can’t pull the transfer board out from under me once I get to the driver’s side. I’ll work on it.
Ron and I went to Sunshine Express and I drove. It’s a huge garden centre, and I bought a flat of 48 blue and white pansies. They are little but very healthy. Once home they all went into the edges of the barrels in front of the existing rose
bushes and into a large pot.They should look spectacular in about 3 weeks.
This is what’s keeping me going these days…working slowly towards my garden and the new landscaping as the weather very slowly turns from really cold to manageable then warm. Yesterday it was 56F. It’s getting there but it seems to take so long and I hurt so much.
Someone on the CMTUS chatline Angela Graham runs was talking about fibromyalgia. I was diagnosed with it many, many years ago and just sort of ignored the diagnosis. CMT was enough, I thought. But now, really thinking about how much pain I am in all over my body, I’m realizing that I likely do have fibromyalgia and should be looking after myself better. The clonazapam no longer works. Nothing works.
But, I notice when I’m gardening I have very little pain. When I’m painting, I have very little pain. When I’m doing an update on AccessibleNiagara.com and people aren’t getting back to me and the computer won’t do what I’m asking of it, everything hurts from the hump on my neck (I’m told I now have scoliosis from leaning over a computer for 40 years) to my shoulders, spine, ribs cage, behind, legs and feet. There isn’t much that isn’t either aching or burning.
I like this blog to be upbeat but someone said to me, ‘Yes, but you need to tell it like it really is’. Well, it’s really hard getting older. I’ll be 71 April 16. Trying to work when you ate old and have CMT as bad as do, and stress, makes the pain a lot worse.
I intend to do everything I can this year to keep the pain at bay and am actively looking for someone to take over my huge AccessibleNiagara.com website. It has been my baby for 13 years. Time to let it loose.

Clara's coins

image-556″ /> Clara’s coins

This is my latest collage. I started it during the Christmas break and finished it last week. It’s called Clara’s Coins because it features many pennies. Pennies are being phased out in Canada so it is also an homage to the penny. Clara is the lovely lady in the photo. She was my grandmother and she is about 20 in this photo, around 1899 – 1900.
And, I forgot – our St. Catharines Downtown Association puts banners on the light posts in our downtown area featuring community leaders, etc. I have been honoured and am hanging with winning athletes, fundraisers, artists, writers, philanthropists and other community notables. Over a serigraph, that sort of resembles me, is the word “Activist”. Funny how you just do what you do and all of a sudden you’re an activist but I guess the accumulation of shout outs over the years about disability issues have added up.

Banner featuring a sort of likeness

Banner featuring a sort of likeness

Sometimes I think I’ll not have anything to do when I give up the stressful bits but little projects and interests have a way of flooding into the holes you leave when you toss out stuff you can no longer handle.
My garden is my priority right now and finishing my website update plus delivering the remaining 6,000 Accessible Niagara brochures we have left from the 40,000 we had printed three years ago. I’ll not do another brochure. Smarter ways of getting information to tourists with disabilities are out there. I just have to find them.
Happy easter everyone and may you live pain free or a least keep trying.

Swivel seats, bed heads and hawks

During the fall I found that I didn’t have the confidence I needed to stand up, turn around, and sit down to transfer, thus making the trip from my scooter through the two front seats of our van to either the passenger side seat or the driver’s seat, a really harrying experience. I would lift myself up from the scooter, grab ahold of the back of the front seats and manoeuvre my body sideways to go through the opening. It was when I was turned sideways, and had nothing to really hold onto, that I felt as if I was just simply going to crumble to the floor of the van. It got to the point where I was almost panicking. We knew we had to do something.

I was familiar with swivel seats because I had one on the very first van we owned back in the early 80s and, although I didn’t use it very much then, I knew how they worked.

We called Kevin Rabe, our local expert on all things disability transportation at Northend Mobility in Welland, ON, and he recommended a B and D Independence Transfer Seat Base. This base is a 5 way marvel that goes forward and back, up and down and swivels. It uses the nice leather seat that we have in the van so nothing looks out of place. I opted to have it on the passenger side because Ron does most of the driving now. But, I don’t want to lose my edge so I’m going to figure out how to transfer using a transfer board from the passenger seat to the driver’s seat when the weather gets a little warmer.

We had the swivel seat installed a couple of weeks ago and I’ve used it three times and every time it gets easier. It cost us $2,600. I’m sure that by the time spring rolls around I’ll be able to transfer very easily and quickly without all the panic. I may even be able to figure out a way to transfer over to the driver’s seat without a transfer board but that’s to come.

Swivel seat turned toward the driver's seat so I can transfer sideways onto my scooter

Swivel seat turned toward the driver’s seat so I can transfer sideways onto my scooter


Swivel seat in position to transfer. I'm already on my scooter and backing down the ramp.

Swivel seat in position to transfer. I’m already on my scooter and backing down the ramp.


Speaking of transferring, I’ve had to teach myself to transfer from my scooter into bed without standing up. I do it by simply driving up to the side of the bed, lifting up the edge the armrest of the scooter, pulling my leg up on the bed and then just rolling sideways. It works fine. The only problem with that little manoeuvring is that when I have to get up in the middle of the night to use the urinal, I have to pull myself back onto the seat, and I use a very slippery towel that I have between the bed and scooter seat to make it easier, and then slide onto the seat. And then I engage the scooter’s power, drive forward, drive back to give myself enough room to spread my legs to get the urinal under me, and use it.Then I put the urinal on the bedside table, drive forward and drive back up against the bed and then roll back into bed. The whole thing takes about 12 minutes, and if I’m lucky, by the time I’m finished, I’m not wide-awake. Fortunately, I’m usually able to go back to sleep after this little manoeuvre because I don’t keep dreaming that I’m looking for a bathroom somewhere.

Speaking of bed, there are three different ways of raising the head of the bed for those with CMT breathing difficulties in the latest issue of the Hammacher Schlemmer catalogue. It’s the spring preview 2013 issue. One of them is a polyurethane foam wedge, one is a memory foam inclined mattress topper and the last one is a blowup and you can regulate the lift according to the amount of air you put in it. All of them are cheaper than buying an automatic bed. Hammacher Schlemmer always amazes me with the interesting things they have in their catalogue and a lot of them are extremely useful for people with disabilities.
hawk
And, speaking of sleep (I know this is one segue too many), I was lying in bed this morning and noticed a large bird in the hemlock tree near our little pond. It’s snowing like crazy today but the bird was obviously holding down and pulling apart a small rodent or bird. When Ron came into the bedroom I asked him to get the binoculars out and for about 15 minutes we watched a beautiful hawk have breakfast…and took some pictures, of course.
hawk2
As usual, my orchids are keeping me company and doing their best to drive away the winter blues in spite of the cold and huge amount of snow piling up out there.

Beautiful purple phal orchid

Beautiful purple phal orchid


Until next time, take care of yourself.
Linda

Hand-saving ideas (part two)

Personal care and maintaining your independence can be a huge issue when your hands weaken because of CMT. Here are a few of my ideas and, please, feel free to send in yours.

For personal care buy the lightest hairdryer (usually a travel model) you can find, and lightweight washcloths and towels. A hairdryer will dry your pubic area, under breasts and arms more thoroughly than a towel. This was suggested to me by a doctor years ago when I developed a heat rash. I’ve been plugging in my hairdryer every night after my shower, ever since. No more rashes.

If you continually drop your hairbrush look for a child’s brush or go to a salon supply store and search for a small lightweight brush. They usually have a large selection of hair and makeup brushes.

Search out a lightweight showerhead if you use a hand-held shower and get yourself a lightweight long handled back brush. There are spa products online that allow you to use two hands to scrub your back and look like a long rope with loops at each end or a loofah sponge with loops. Check out Merben.com under bath and beauty and then sisal. I just bought one through Amazon and it was delivered in a week.

Have a one handled thermostatic valve installed a your shower so you don’t have to fiddle with temperature control. I have a Moen and love it.

If you continually drop large bars of soap, consider cutting them in half. Also try using a soap mitt.

If you install grab bars in a shower or tub area consider whether you will be able to actually grip them. There are various sizes and shapes of grab bars and you might want to consider sitting instead of standing in the shower if you’re to rely on your hands to continually steady yourself.

Consider a sonic toothbrush. You don’t really have to brush, just place it on the tooth and let it do its work. I have a Philips Sonicare. It’s a bit heavy and I need to turn it off by keeping it in my mouth and leaning it on the counter but it’s worth the effort.

On to getting dressed: Velcro can be your best friend. Especially good on waistbands where finicky little buttons can be impossible to do up. I have used it on pant and skirt waistbands, down the front of shirts after sewing the buttons on top of the buttonholes so it looks like it’s done up, and I know it is often used on shoes. Velcro can also ruin good material so be very careful when using it as it can pull anything it touches.

If your hands are strong enough to work an elastic waistband, it does away with buttons and zippers.

Button hooks can be purchased new or in antiques shops. They were used for boot and glove buttons in the 17th and 18th century and still work on any kind of buttons today.

This is an old bone handled utton gook likely used for gloves or children's shoes but the concept remains the same

This is an old bone handled button hook likely used for gloves or children’s shoes but the concept remains the same

An O-ring available at any hardware store or Walmart, usually in the key department, if threaded through the pull of a zipper, will allow you to slip your finger through the ring and work the zipper. You can usually tuck the ring in if you’re using it on a fly. Often just a loop of ribbon or strong thread through the top of the zipper will work. If you go to craft shows you can also find handmade zipper pulls for jackets and sweaters that are quite imaginative and fun.

Try buying tops the just slip over your head rather than button up. Lands’ End and L.L. Bean have a hug selection of T-shirts, turtle necks, cowl necks and polos for men and women in cotton knit that don’t need ironing if you smooth them right out of the dryer.

If you must wear a clean dress shirt every day think about working a professional laundry into your budget. Ask them to leave the top buttons undone so that you can just slip the shirt over your head and use a button hook for the cuffs and the 2 top buttons. A necktie can always be left tied and simply slipped over your head and then pulled back into place again when needed. Lands’ End and L.L. Bean also have no-iron dress shirts.

Socks and weak hands can be a real challenge. I simply cannot put on my own socks without a great deal of trouble. I think part of the problem is because my ankles are fused in a standing position and I had no grip. If anyone has any suggestions for putting on socks, please comment.

Consider using an extra long shoehorn – you can get them with a 2 or 3 foot extension.

If shoelaces are a problem look into elastic shoelaces that can be kept tied and you simply slip your foot into the shoe.

If you wear boots with laces consider having skate hooks put on your boots so that you don’t have to thread the laces through the holes using forefinger and thumb or buy boots with skate hooks already on them.

If you continually drop things, which I do, consider buying a device called a Telestik. (Telestik.com) It looks like two old-fashioned radio antennas in a thin black tube. Once extended, one of the antennas has a very sticky pad on it that will pick up everything from water bottles to makeup brushes. The other one has a strong magnet on it and will pick up anything metal. For a while, I had problems with the replaceable sticky pads failing, but once I got a batch of good ones, I am able to use it 5 or 6 times a day and not have to ask Ron to pick up after me.

If you have a problem with your hands being too weak to pull your slacks and underwear up from the floor after you’ve used the toilet, consider attaching a ribbon to either side of your slacks on the inside waistband. Put it round your neck as you get up and it will bring your slacks, with your underwear in them, up to a point where you can grab them. I used this on all of my slacks until I could no longer stand. Just tuck the ribbon in your underwear at the front so it is hidden before you do up your zipper.

If you use a urinal at night beside the bed but are afraid you’ll drop it as it fills and gets heavier, tie a string around the handle and make it long enough so it will loop around your neck. I use an extra long shoelace from one of my winter knee-high boots. The string around your neck holds the urinal leaving you with two hands free to grip and guide. Even if you do drop it, it’ll just dangle there until you can get a grip again.

All of the things I’ve mentioned or practical items that can help someone with weak hands but here are a couple of things that everybody with CMT can do to protect their hands and keep them as strong as possible:

Think about what you’re doing with your hands on a daily basis. Is there anything repetitive that you are doing that you don’t need to do? If your hands are getting weaker and you are texting, typing, playing games on your computer, knitting, crocheting, painting, sculpting, etc. you could be wearing out the nerves that need to fire the muscles. Remember, CMT is a problem with our nerves, not our muscles. But, our muscles require a signal from our nerves to go into action.
When our CMT affected nerves can no longer send that signal because they have been overused through the years, or simply used through daily living, the muscles atrophy. We use our hands every day for all manner of tasks for a lifetime. No wonder they give out. But not everyone has this problem. Like I said at the beginning, even people with CMT in the same family can vary in degrees of hand atrophy and some never experience it.

Do not exercise your hands purposely. They get enough wear and tear on a daily basis.

Make sure anyone advising you about your hands knows CMT well. Hand surgeon and specialist, Dr. Stuart Patterson in Lakeland, Florida (Google him), knows CMT. Sometimes surgery can help; sometime it makes the problem worse. An Occupational Therapist (OT) and/or a Physical Therapist (PT) may not know CMT and their recommendations could do you harm. Know CMT yourself. Don’t always rely on the so-called professionals. CMT is still an unknown to many. If what is recommended doesn’t sound right to you, speak up. It’s your body and your life and you have to live with the damage no matter how unintentional.

Do not use exercise equipment that relies on your hands to work other parts of your body. For instance, a rowing machine may be great for various muscle groups but you are also using your hands to grip the handles for the entire time you’re using it.

If you want to lift weights, use wrist weights.

And to end this may I suggest that you think about the future and how your hands will be five, 10 or 15 years down the road. What you do now will make things easier for you then. If you start planning and buying now for the independence you want in the future, you’ll thank yourself for thinking ahead.

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