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

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

Nathan’s story and request

I am Nathan Miller, 41, who was diagnosed with Charcot- Marie Tooth Syndrome (CMT).  I have a Bachelor of Science in Social Work. 

I only share this profile and details for informational purposes and not for pity. 

Looking back, I often wished that I had more information as to the limitations and the abilities of one who has CMT.  I think that it would be fortunate at the time of one’s diagnosis with CMT to arm himself/herself with an holistic base of knowledge regarding this disability and abilities at the point of the person’s life, as well as over the whole lifespan.  It would be important to know what tools at different life stages one might need to arm himself/herself, so as not to be publicly embarrassed.  For example, there have been a few times where I wondered whether I was going to be able to button my pants, zip my pants, or get these items undone for restroom purposes, as well as dressing and undressing.  I am unaware of the tools out there to help me, as well as where to look for these items.

I am a social worker by trade, but I am currently between jobs and looking for full-time work. 

It’s important to note that even though the United States has made some significant progress with the area of workers with disabilities, more public awareness is needed, as I have had to work in environments where I had to climb stairs on a daily basis, even though climbing stairs is a chore now. 

Also in the area of disability law, it’s unfortunate that even survival of the fittest becomes the rule when supervisors are faced as to whom they will let go.  It’s a lot easier to let someone go if they can always file for disability, than a worker who isn’t disabled.

I was born in January of 1971. My father was in the U. S. Military.  Because CMT was seldom heard of in the 70’s, I always tried to be competitive in physical education and activity against my peers.  Trips and falls, foot drop, and exhaustion for no explanation were commonplace for me. 

At a young age, I often noticed that I had difficulty holding a steady hand when it was most required, little fine motor coordination, and difficulty handwriting, carrying fragile things or things that could be spilled. 

I often thought I was just like everyone else.  However, I noticed at a young age, as young as second grade, that I didn’t run as fast as others; I often got ankle sprains, eventually got clawing of the toes (hammer toes– which became more pronounced by the age of 13 to 14).  I tried as hard as I could at physical activities but often thought that I had to accept that I was just different. I didn’t have an explanation for what was wrong with me. 

In 1982, at the age of 11, my brother was diagnosed with Hodgkin’s disease, cancer of the lymphatic system.  It was shortly afterwards, not knowing he had CMT, he was prescribed the drug, Vincristine, which had an adverse affect on him. He went from being a high school football player, to falling more, then to crutches, to a wheelchair, to an electric wheelchair, to bedridden, and then nearly died.  He was on a ventilator and respirator. He eventually made it through the ordeal, was cured of the cancer, and walks again with the assistance of ankle-foot-orthoses.  It was at this time, that all of my family members were tested to see whether we had the CMT that my brother had.  We found out it was passed through my father, down to five out of six of us children.  My mother didn’t have it. 

Looking back on my life, had I known all of the problems that I experienced with CMT, I probably would have wanted to request a doctor’s order to have me taken out of physical education right away.  I was frequently exhausted the remainder of the day due to overexerting myself to do well in physical education.  By the time I was a freshman in high school; it was suggested that I visit a physical medicine and rehabilitation specialist.  The nearest doctors at the time were at U. C. Davis Medical Center in Sacramento, CA.  By this time, I had suffered foot deformities and hammertoes. The doctors at U. C. D., fitted me for short leg ankle foot orthoses, and referred me to a podiatrist.  I was also given a medical note to immediately withdraw from physical education at high school.  This was quite a relief for me due to extreme difficulty of the demands, exhaustion, and breathing problems (I’ve had lifelong sleep apnea).  In a month, I received the orthoses and within six months had seen the podiatrist and had surgery on both of my feet —  they operated one at a time, let the one heal, and then operated on the other.  After about a year of surgeries, castings, and healing of the feet, the podiatrist then fitted me for foot orthotics.  To prevent tripping, clumsiness, and the weight of short leg braces, I was thankful to go down to just the foot orthotics.  To this day, because of the weight of the ankle foot orthotics, and the fatigue of having CMT, I only use foot orthotics and not the ankle foots orthotics (AFOs). 

I have difficulty walking in snow and ice with the AFOs.  If others haven’t been around when I’ve fallen, I often fear how I’d be able to get myself back up.

Work Life:  This area has been unpredictable due to exhausted fingers and mobility.  Climbing stairs and clumsiness continues to be an issue for me; however, this past year, I have noticed a significant decrease in my hand mobility, strength, and adequate strength for activities of daily living: dressing, buttoning, and zipping of clothing and walking. My gripping is getting worse, as holding a toothbrush, a pen, writing and typing are all becoming significant chores. Sometimes, I don’t know whether to exercise my fingers and hands, or save the strength in my hands for activities later in the day. 

I often wish I knew what to arm myself with for prevention– dressing, buttoning, snapping, and knowing which type of activities to proceed more moderately or assertively with due to the lack of predictability of the CMT. 

I write you, Linda, to know where I can get help and products that would help arm me to be more independent as my CMT progresses.  I know that it can vary among individuals with CMT; however, we all share similar features and issues. 

I know that we as a team of individuals with CMT, we shouldn’t fret about it or predict how this disease will take its toll, but hold our heads high in stride as we face our days of challenges we embark; but, you and others that have the maturity of having already been through my life stage might know how we, of the younger generations, might plan ahead, and not be surprised when things take their toll. 

Knowledge and preparation, as well as knowing what products and resources are out there can be beneficial. I’d also like to know if teams of engineers are developing future needed products that can make living with CMT easier. I appreciate your carrying on this site and the education materials for future generations that might deal with CMT. 

Sincerely, Nathan Miller

Linda here – My next post will be a partial list to help Nathan and I’ll post another on Thursday to finish my ideas. Please feel free to post to this blog if you have  recommendations along with solutions.