| Amputations
Dear Readers: Linda here - Every now and then something comes along that may mean a great deal to some. The following articles all concern amputation and the reasons why people with CMT sometimes have an amputation performed. They aren't easy articles to read but they testify to man's intestinal fortitude to get on with life and I hope they will help others who have reached the limits of present-day medical knowledge to decide whether amputation is the right thing for them.
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. 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.
Doctor-patient with CMT opts for foot-lift instead
of face-lift Middle age crisis in the aging CMT male Homo sapiens may invite all sorts of death-defying acts of malcontent. In my case I was focused to correct a clubfoot that was only getting worse and not holding up to the heretofore lifesaving AFO. I was constantly nursing callouses on the outer edge of my right foot. Sometimes they festered into abscesses that put my foot out of commission for weeks on end. So, when the time was right (my pediatric practice enlisted two new pediatricians), I began to shop for an orthopedic surgeon; one who would agree to doing an ankle arthrodesis (ankle fusion) and at the same time reshape a caved-in ankle into one that is frozen upright and enabling me to walk on the bottom of my foot, not the outer edge. There were three in this area for me to choose. Once done, the date was set and I never looked back. Undaunted, my wife and I vacationed to the northeast for the fall foliage - breathtaking vistas twice out. We even walked a five mile circle in Old Boston. Returning home, I was ready for surgery on 10-21-97, uneventful although Dr. Keith Donatto was concerned he did not wedge the ankle fusion as much as he would have liked, but in my mind's eye, another procedure was out of the question. I would just wedge the shoe to make up the difference. Recovery was a slow tedious process, but getting about on crutches was the only alternative to being a couch potato'. I can vouch for CMT patients who complain with balance problems. Crutches hardly improved my balance, except when I found myself touching ground with my surgerized foot, probably sooner than my doctor allowed. At six weeks, X-rays showed bone filling and limited weight bearing was allowed. However, I knew best, so it was limitless weight bearing henceforth. In the next four weeks I was parading through Wal Mart for Christmas presents, riding the 4-wheeler at my farm and driving the car, all with my right foot in a walking cast. At 10 weeks I bought a right shoe with me to the doctor's office expecting to rid myself of any more casts. When the X-rays were read by Dr. Donatto to me, a deterioration of last month's healing, and even microfractures, my hopes for a renewed life of activity and less dependence on my very supportive wife were doomed. There loomed the possibility of non-union, a complication of fractures, and fusion surgery...a complication I never considered. I, being a doctor, could not entertain a complication as a possibility. I, being a doctor, knew what progress could be made and when to make it. I, being a doctor, even though admitting to pain and swelling that was not relenting, thought of nothing going wrong. So we face the music of all my actions and their consequences. Back into a cast, back to crutches, no weight bearing to the right foot was my sentence for the next four weeks. As my eyes flooded with tears, I realized that I was no longer in control, that I had to rightfully submit to being the patient, following doctor's orders. Rationalization was a lark of the past. My only solace, fortunately, was at my side, my wife who had been there for me in all these weeks, and with her continued support, we would get through this setback. For four weeks I no longer used stairs for fear of accidentally bearing weight on my foot. I took vitamins, used an electric bone stimulator and confined myself to the bed or the couch. By the end of January, and with the next four week visit looming, I began to feel the anxiety of whether the X-ray would reveal bone generation. But, it was not to be to our dismay, nor would it be in the next visit a month later. Now four months post-operative and on total disability, I began to despair at the thought of losing my foot and forced early retirement. When I returned the next month and the X-rays were again unfavourable, the recommendation was surgery... repeat the fusing, repair the wedge to the angle he was hoping to improve, to remove screws and put in an L-plate and seven screws...essentially starting from scratch. My morale was so low I didn't hesitate to say, "Go for it," on Good Friday, April 10, 1998. I consider myself an optimist, allowing the transcendence of pain and
inconvenience of major surgery to the other side of the mountain'
with each day, week, month reaching to recovery. There were more books
to read...anthologies by John Jakes, Ken Follette, Grisham, etc., were
great saviours from boredom over the next four months. Alas! It was not to be. On Tuesday, August 14, 1998, and four months post-operative, I saw Dr. Donatto for the final visit. He was being transferred to San Francisco and a higher step on the ladder of academia. His first remark as he studied the films of my right foot was, "and the end of a saga." They showed continued bone growth up to 80% this time and probably no further X-rays were needed. "What about the swelling that never goes down until I recline in the evening," I proclaimed. "And what about the pain when I try to do more?" I knew that an AFO was going to be necessary but not until the edema (swelling) was controlled. I had many questions, none of which could be answered by the good doctor, unless he was also a shaman. Indeed, I wondered if my next visit to Dr. Donatto's replacement might be better served by those medical magicians. The swelling and pain continued, and each night as I lay with the foot elevated, I pondered the outcome but could not yet come to grips'. I had returned to my clinical practice and found that if I spent more than two hours afoot the pain and swelling was intolerable. But I held to the prescription to return for follow-up in three months, which finally took place in November. Another quandary...I would have to place my future with an orthopedic surgeon I didn't like. He was first recommended by retiring orthopedist, Dr. Luke Bordelon, a highly sought after specialist in foot problems from CMT, who had operated on both feet twice previously, and was may saviour in the 80s. This foot specialist, a pediatric orthopedist, also operated on adults with CMT. The visit was not received well, as he suggested surgery on both feet and allowing one year to recover. His aloofness or lack of compassion didn't add to his credibility and his name had been removed from a list of candidates. So the appointment was made and the consternation began, right up to
the moment when I reported to his office only to be told that he was in
emergency surgery and would not be able to see me today. My fuse was lit,
as I could have been called to prevent the unnecessary trip to his office.
I asked if I could at least be X-rayed, that he could read the results
later. No, he couldn't do that. He would have to see me first. Understandable,
if I was a new' patient, but my case was a follow-up that, on appearance,
wasn't even discussed with Dr. Donatto. I left, defeated and deflated.
It didn't take long for him to listen to my history, read the X-rays, and examine my foot. "Jim, your foot is not healing. You still have nonunion, and it's a matter of time as to when infection will begin, osteomyelitis," proclaimed Dr. George. "What's more, I suggest you undergo a Syme's amputation (stump is like a heel) so you can get on with your life." He came down hard but true, and his emphasis on "getting on with my life" made it palatable. This is the least invasive amputation of the lower limb - at the lower level. The heel pad is salvaged as a flap, to cover and cushion the end of the stump, and allowed to heal in four to eight weeks before a prosthesis could be employed. Before Mary and I could blink our eyes and allow the pangs of devastation, a plan of attack was set in motion. George called in his youngest associate, Dr. John Burvant, who would be the surgeon and George would come out of retirement and assist, to my satisfaction. We scheduled the operation for December 10, the eve of my 63rd birthday, thus allowing two of my associates their predetermined vacation time. The next three weeks crawled by as the pain of a very loose, swollen foot became intolerable, only because I knew its days were numbered. But I plodded along and the surgery and healing process were uneventful. I have been discharged once more by my surgeons. I am awaiting a new'
foot, as the prosthetist made an impression of the stump this past week.
I have no phantom pain but sensations that the foot is still there...wiggle
my toes, feel an itch.
Linda here - I asked Dr. Robert Sampson, one of our long-time CMT International advisors and an orthopedic surgeon who has CMT, to write about union, nonunion and amputation to help us understand exactly what Dr. Anderson had done and how it could impact on our lives if needed. This is what he wrote: UNION is what is supposed to happen when a fracture occurs. It is the final knitting of the bone. This same term defines the satisfactory healing of a man-made fracture such as a fusion or a reshaping of the bone (osteotomy). In both these cases a surgeon has cut the bone and when it heals in a satisfactory period of time it is united. If the time it takes to heal is too long then it is called a delayed union. If the bone heals on time, but is crooked, then the healing is called a malunion. So, in general, when a fusion becomes a delayed union three to six months have gone by without a satisfactory healing. After six months it is usually termed a nonunion. Depending on the area of fusion (ankle 20%) or hindfoot (10%) or surgical technique (arthroscopic 99%) or (open 20%) different rates of nonunion may occur. A nonunion is treated in a variety of ways. Notice that once a nonunion has occurred there is always less than 100% chance of getting a good union. Factors creating a nonunion are: luck or no luck, general nutrition, ability to rest the fused joint, surgical technique especially getting a nice flat surface area and compressing it with screws or other hardware. Tobacco is a no-no as its use increases the rate of nonunion by 500%! Prior infection decreases successful union. Neuropathies such as diabetes or CMT also increase the risk of nonunion. My experience has been that diabetic neuropathy is far more likely to cause nonunion than is the neuropathy of CMT. Signs of a nonunion are continued pain, swelling, redness and a feeling
of heat at the surgical site. Confirmation is by regular and sometimes
by special X-ray studies. Amputations are done when all else fails and repeated surgeries would be fruitless or perhaps wasteful of a person's time and financial resources. Typically, a good fusion of the foot or leg takes four months to heal and another two months to be useful. Amputations of the foot or leg usually heal in about six weeks and, in the younger person, the patient can even be fitted for the first leg prosthesis in the operating room. Amputations can take place just about any place on the leg, but certain
ones are more common and predictible. Above the knee amputation is disabling. It takes a lot more energy to
walk with this type of prosthesis and the knee joints tend to be heavy,
mechanical and artificial. Linda here - I've known of Brian Christie for many years through letters from his parents, Mabel and the late Charlie Christie, but I've never really heard Brian's entire story. I found it fascinating. I think you'll agree Brian is one stubborn, gutsy guy! He spoke to me by telephone from his home. He's got his life back! How did his foot problems begin? By this time, maybe 15 years ago, I went to apply for Canada and provincial pension. I had been diagnosed as having CMT Type 1 with what my doctor calls a "Wild Card"... my CMT does things it isn't supposed to do. My doctor, Joe Dooley, has researched this disease for many years and is still at it as far as I know. I have extremely limited sensation in my hands, lower arms, lower legs and feet. I'd say, I've lost 90% of the sensation in those areas and 80% of the strength. I got into an argument with a table saw three years ago and cut off four fingers. It took six hours for surgeons to sew them back on. I only had one shot of morphine and that was immediately after I did it. I had general anesthetic while they managed to sew three of them back on but I couldn't feel much of anything when I first cut them off. Fortunately, I have about 90% movement in those fingers although my hand is very weak. From the beginning, when I first developed open foot ulcers after the
plantar wart surgery, I was running back and forth from the hospital three
times a day to have the ulcers packed and my dressings changed. Needless
to say, there was no time for anything else with the hospital and work.
Then the doctors showed me how to change my own dressings but insurance
wouldn't pay for the bandages. The cost was about $350 a month. I finally
bought them from a medical supply outfit and did them myself. Twice they tried skin grafts and they just fell off. I've tried home remedies, just everything. Anything anyone told me, I tried. I had nothing to lose. In September of 1997, I woke up one morning and was walking from the bedroom to the living room and my son asked me, "What's with the blood?" I took off my sock and bandage to see what was happening and apparently I had an infection so bad that it had literally exploded the end of my foot. The skin was all off. I went to the hospital and the surgeon looked at it and admitted me to try a course of antibiotics to see if he could control the infection. Five days later he said he couldn't, that amputation was my only option. At that time he didn't know how much he would have to take off. I still had these big ulcers on the sole of my foot. He amputated right behind the ulcer on the ball of my left foot. It was called a forefoot amputation. He left the amputation open, put a couple of stitches in each end and the rest was packed with Kaltostat, a seaweed dressing. You could actually see that stuff drawing the infection out of the rest of my foot. Within three months my foot was totally healed over, the first time in 20 years. I can actually sleep without a sock on! The right foot was worse. Between Christmas and New Years 1997, the open ulcers got infected. I went to the doctor on Jan. 2, was admitted to hospital the same day. The surgeon took one look at my foot and told me it was gas gangrene and he didn't know what he could do with it. They couldn't amputate because of the gangrene but they did operate on that foot to try to clear the gangrenous tissue. It took three months for it to heal although it still had the open ulcers. All they did was remove the gangrene. Three months later, March 1998, they did the same procedure on the right foot that they'd done on the left. I was walking on it three months later. I was even walking around a campsite, up and down hills. After 20 years, they finally described the organism they couldn't identify in the swabs they had taken of my ulcers for so many years. Apparently, it takes four or five organisms combined to create gas gangrene. The one element that was missing was in this right foot. When they had my actual forefoot in their lab, after the amputation, they found the element they needed to confirm the diagnosis of gas gangrene. To look at me today, and if I had on a pair of boots, you'd think I had nothing wrong with me but one finger missing. In reality, I have both feet partially amputated, and I can't feel anything from the knees and elbows down although I can move those areas. The major things that are wrong with me just don't show. How does Brian feel about the amputations? I was offered prosthetics to replace the parts of my feet that are missing but they wanted $1,400 apiece for them. So, I buy a pair of high-top sneakers, stuff the end of the sneakers with gauze bandage, put 'em on, tie 'em up and walk. I use a cane for balance sometimes. I have a wheelchair and a walker and a ramp on my house that I fought for years to get and a bathroom specially made for me all done through grants I had to fight for but I'm doing okay, I'm stubborn. I've never used my CMT as an excuse. It is not an excuse. To me and my family it is an inconvenience and that's all it is. We're doing fine. My wife, Linda, is a personal care workers (PCA) and has worked in nursing homes all her life. She takes all this in stride, but without her, I wouldn't be where I am now. I don't think she realized the marriage vows included what she's been through. I love her so much! My family doctor, Dr. Yvonne King, has kept me alive. She is incredible.
The two surgeons, Dr. Ralph Burnett and Dr. George Sanson, and Dr. Walter
Shlech, head of the infectious disease centre for the Maritime Provinces,
and Dr. Joe Dooley, the CMT researcher, are all to be highly commended.
If it wasn't for them I wouldn't be here. Amputation - One Smart Move! The recovery years were not exactly a piece of cake. I had a husband who moved out, and two young daughters to care for. All of my extended family lived 1,000 miles away. But you know the old saying...you do what you gotta do. And I did. Twenty-seven years later, and I am still coping well. Some things are downright hard to do and others are as easy for me as they are for the normal' population. I have noticed as I approach 60 that I am quite a bit stiffer. I still have pain, but sometimes I can actually block it out, and if I can't, I don't let it stop me from doing what I want to do. I had to quit working full-time four years ago because of osteoporosis. I had fractured my pelvis and the extra pain was just too much. So now I do volunteer work at the school one day a week. I take a quilting class and sew in my free time. My greatest enjoyment, though, is the three grandchildren. We love having them here at the house overnight. I drive five miles about three times a week to take my two-year-old grandson for a walk down the street. He seems to know that I can't chase him so he never lets go of my hand. So much of the information in the newsletter is pertinent to my situation, but then there is always the feeling that "I am better off than you are."
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