David Wright
His Journey of Determination with the Rare Disease, CMT
I first became aware that there was such a thing as Charcot-Marie-Tooth disease (CMT)
when my father was (finally) diagnosed with it. CMT is a group of inherited neurological
disorders that affect the peripheral nerves – the nerves that transmit signals from the
brain to the muscles of your arms, legs, and limbs. CMT causes progressive damage
to these nerves, leading to muscle weakness, loss of dexterity and coordination, and
foot and leg deformities. The disease progresses at different rates for each patient. It
can become very difficult to walk, pick up small objects, get dressed, and engage in
almost any form of robust physical activity.
People with CMT suffer a lifetime of physical pain, emotional distress, and often
humiliation. Along with the challenges of navigating daily life and the inconveniences
brought on by a lack of balance and hands that don’t work properly, given the progressive
nature of CMT, there is also the constant uncertainty of what will happen to their body
next.
We lived in a small town in deep South Texas. My father had suffered both polio and
rheumatic fever as a youth, so a correct diagnosis would be difficult. The first doctor
said my father had a form of muscular dystrophy. He was sent to the University of Texas
Medical School in Galveston, where the (mis)diagnosis was confirmed. The treatment
was to keep him comfortable and pain-free, which was done with copious quantities of
pills. After two years of living with a drug addict, my mother, a chemist, sent him to the
Mayo Clinic, where first, he dried out. Then he was thoroughly evaluated by Dr. Donald
Mulder, a neurologist, identified the cause of my father’s symptoms as CMT. At this
point, I was a sophomore in college.
Little did my younger brother and I know that CMT is often hereditary, so we viewed
CMT as unique to him. We both finished college. I was commissioned and went into the
Army; my brother married and went into the insurance business. When I was 28, Dr.
Mulder reached out to us and asked that we be tested for CMT. This entailed an
electroencephalogram and an electromyogram. I was tested at Ft Bragg and a few days
later, the neurologist called me to come to the hospital. He had consulted with Dr.
Mulder and my responses confirmed that I had CMT. He advised me to resign my
commission to preclude being medically discharged. My brother was tested, and it was
confirmed that he also had CMT Type 1A which is the mildest form of CMT.
My brother had a daughter prior to knowing he had CMT and chose to have no more
children to prevent the risk of passing it on. I chose not to have children and adopted
two.
Over the years, I watched my father deteriorate. He first walked with a cane, then with
crutches and leg braces, and finally was wheelchair bound. He died at 81 of a heart
attack. Based on my father’s experience, I decided to make my muscles work as hard
as they could for as long as they could, to keep my weight down so there was less bulk
to carry, to eat a healthy, low-fat diet, and stopped drinking for nine years.
The first symptom that I experienced was drop foot when I was about 42. At the time, I
had a solid regimen of lifting, biking, running, and swimming. I even started doing
triathlons. My weekly training goal was 80 running miles, 180 biking miles, 6 swimming
miles, and lift three times. It was a way of life.
With the drop foot, I would trip and fall while running. I always had a bloody knee and a
hole in a pair of $60 tights. So, I looked into braces and got a splint for my right leg. It
was a miracle. I actually ran three marathons with it. Later, I added one for the left side
and kept on going. I no longer do triathlons, but I have continued a serious exercise
program. I have a home gym, a treadmill, a water rower, and a Peloton. Most every day,
I work out for one to two hours with a combination of aerobic exercise and weights. I
keep my weight at 158 – 160, I eat a healthy low-fat diet, I limit alcohol intake to a few a
week. I wear carbon fiber braces on both legs. I also maintain a peptide regimen, which
started as a treatment to accelerate recovery from a hip replacement, and, I believe, but
can’t prove, that it has slowed the inevitable advancement of the disease. Even so,
CMT advances. It comes in increments, i.e., you are level for a period then you enter a
decline, then level off for long periods. Right now, I’m level and have been for about two
years. I’m now 79.
How do I feel about CMT? I wish I didn’t have it. I wish I could still button my shirts and
not wear leg braces. But, given a choice, I would take it over many other things. My
wife, Linda, passed away 5 and a half years ago from esophageal cancer. The CMT that
I have is a minor inconvenience compared to that. By contrast, my brother chose a
radically different way of living with CMT. In a word, he is old. I’m not.
Linda and I selected Charleston as a place to live after we quit working. She began
looking at places to live and picked Daniel Island and I’On. Once we got here and
looked, I’On was the only choice. We bought our home in November of 2015 and love it
here. It’s convenient to everything - twelve minutes to downtown. It’s filled with beautiful
homes. It's peaceful. It’s mostly filled with wonderful people. The two lakes are great, as
is the trail. It’s by far the best place to live in this area.
Neighbors (as I’On neighbors do so well) introduced me to Cleary and George Simpson,
who help run the CMT Research Foundation and also live in I’On. They have a singular
mission: to find a cure for all types of CMT. They are in touch with the top scientists and
researchers around the world working on a CMT cure. It is great to have them as a
resource, as my condition tries to outrun my determination to keep it at bay.