Mind over matter
by Ian Thomas, Wales, U.K.
My name is Ian and I have CMT type 1A. I am a very happily married, 41-year-old father of three children, two of whom also have CMT. I am also a proud young grandfather of a 15-month-old grandson.

First, a brief history of my CMT. Like many people with CMT, I had symptoms from an early age, four years old in my case, and was thought to have Duchenne muscular dystrophy. Thankfully, this wasn't so and, at 19, I was correctly diagnosed with CMT.

Over the pat 22 years, I have had the following surgeries on both my left and right : triple arthrodesis; wedge tarsectomies; Dwyer osteotomies; Keller procedures; P.I.P. arthrodesis; excision of 3rd and 4th metatarsel head; tenotomies ; Steinler procedure (left); amputation of 2nd and 4th toes (right); excision of cuboid (right); and various other small procedures.

The combination of having so much surgery only compounded the progressive nature of CMT. The result was a breakdown of my ankle joints, so much so that widespread osteoarthritis was causing so much pain. I had worn out the ankle mortice holding the bones of the leg to the feet meaning the tibia was sliding and giving way across both ankles.

Having researched options myself and their long-term prognosis, I decided to discuss the future with my family, friends and my specialist, consultant orthopaedic surgeon, Mr. Malcolm Downes. Keeping mobile and, if possible, pain free was my aim, and if that meant amputation, then I was physically and psychologically ready for it. The alternatives were ankle fusions or ankle replacements. Neither were the real answer, at least that is what I believed and my surgeon agreed.

Fortunately, in the U.K., we have a National Health Service that is free to all citizens, although waiting lists are long, and I waited for 18 months for my amputations, long enough to have changed my mind, but I knew this was the right option for me.

August 1998, my first below knee amputation took place. I have to say it was quite a painful experience, more than I had anticipated, but within seven days I was released from hospital and was walking again five weeks later at the local limb centre. Walking with an artificial limb is like walking on stilts, good balance is needed, and providing you have no tender areas on the stump, then it should be totally pain free as is in my case. Phantom pains lasted some three weeks. I also had cramps in the stump but only in the first three weeks.

November 1998, three months to the day after my first surgery, I had the second amputation. This time I had no pain, not even phantom pain or cramps.

Walking with two limbs began in earnest the beginning of December 1998, and the first week of January 1999 I was back at work, unaided, and indeed I drove myself to work. The only adaptations to my car is that I have larger foot pedals. I have worked as a volunteer advisor for seven years in a local Citizens Advice Bureau. My aim, within the next year or so, is to gain full-time salaried employment.

My walking gait is now so good that an aunt said to me recently, "Ian, I have not seen you walk so naturally since you were a young child." Indeed, whenever I visit the local limb centre, I often get asked by other amputees, "Are you calling to collect someone," not realizing that I walked in as a bilateral, below knee amputee patient and will walk out as such.

May 1999 I collected my new limbs. They are state of the art with a spring mechanism within, so I have a greater bounce when I walk. I now feel like running everywhere. When making up these new limbs, the prosthetist asked what height I was prior to amputation and whether I would like to be shorter or taller. I told him I had stood six feet tall but would like to be even taller...a big mistake! For two days I was walking around with my head in the clouds. Why? Because I was raised to six foot three, and believe me, those extra few inches make a whopper of a difference; my centre of gravity was way out and all my trousers were half mast. So, reluctantly, I had to agree with the prosthetist and he lowered to six foot one, which is quite nice.

My birth feet grew only to a U.K. size 3 shoe. Now I wear a size 10, and believe me, what a difference and probably why I now have such good balance.

I am under no illusion that amputation is a salvation for everyone but I do say if you are like me and have undergone so much traumatic surgery and can see no end to it all then carry out your own research, talk to medics, better still talk to amputees at your local limb centre. Of course, amputation will throw up its own problems, but think positive...mind over matter.

The limb centre I attend is attached to a major trauma hospital, and I have been asked by the sister in charge if I would give some of my time to speak to trauma patients who find the experience of losing limbs literally ‘the end of their world.' They just don't know it yet but just a few weeks down the road they will be walking again.

My dear wife, Leona, children, Ceri-Ann, Alastair and Leona, have been a tower of strength. Morever, not many 15-month-old children have the capacity to assist their grandfather in taking off his legs! Notwithstanding, my parents and extended family and friends have been fully supportive, and I am eternally grateful for that. Indeed, I cannot thank everyone at Morriston Hospital, Swansea, South Wales, enough.