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Personal stories

Coping with CMT swollen feet and ankles
by Darlene Weston, OR, U.S.A.

My husband, Dean, is the fifth generation of people in his family with CMT. Each generation has shown more severe symptoms than previous generations.

Over the years, the health problem that has most complicated Dean's health and CMT treatment has been uncontrollable high blood pressure. Other health issues have been numerous bouts of pneumonia, kidney complications, and in recent years, severe swelling and ulceration of the lower legs and feet. Depression was our biggest enemy and the hardest to fight over the years.

In 1989, Dean skinned his lower leg. It became infected... then ulcerated. The skinned area opened to the bone and the wound abscessed. He was able to stay semi-active at this time so no significant swelling was involved. A NuPatch (Duoderm) was prescribed and worked well. It's only been since 1994 that Dean's legs began to swell periodically.

In 1996, our primary care physician, Dr. Conrad, sent him to a lung specialist who ordered a sleep study. The doctor's report said, "Periodic EMG activity in sleep was found markedly at 116 per hour." The doctor described it as having very severe periodic limb movements separate from the problems of severe sleep apnea. He put Dean on a Bipap machine for sleep at night which helped tremendously.

This past year, 1997, has been our worst year fighting swelling, edema, and leg ulcerations. Dean was instructed to try to keep his legs elevated but his pain was far worse with his legs up (even with increased Percodan usage) so it wasn't effective in his case. In March, he got an infected toe... antibiotics were prescribed. In May, when he developed a rash on his lower legs, stronger antibiotics were used and his water pills increased. In July, the toe began oozing yellow fluid so the doctor removed part of the toenail. We were using a variety of ointments for the terrible itching. The doctor tried all kinds of combinations of medications to bring the swelling down... nothing worked. In late July, the toe begin to bleed and the leg ulcerated. We had been using warm epsom salt soaks to no avail. Diagnosis: atrophied skin changes (or stasis dermatitis) caused by the swelling and edema breaking down the cell tissues. We had the toe under control but not the leg yet.

In August, chronic fatigue from the infection set in and Dean slept hours on end. We changed the topical salve from Nizorel to Neosporin and began wrapping his legs. His shoulder arthritis flared unmercifully. He begin having severe hot flashes. This mixed with the chilling spells was unnerving to watch. The left leg developed the same itchy blisters that we were fighting on the right leg as well as a huge water sack.

The next thing we knew both legs were seepage plants. I couldn't put his compression hose on fast enough to keep the water from getting the sheets wet. His socks and shoes during the day were wet despite the bandages. By the end of August, both legs were red, inflammed, and the rash was up to his kneecaps.

Again the doctor increased his water pills, and changed other medications hoping to find the culprit. Nothing worked. More massive doses of antibiotics were prescribed, and UnoBoots were applied in the doctor's office. Culture tests revealed some staph infection and our ever-patient doctor smiled and said at least it wasn't of the Eboli strain! Even Dean's eyes were red and itchy by this point. We were referred to a skin specialist, Dr. Miller, who confirmed the diagnosis and prescribed Triamcinolone (.1%) cream.

In September, the doctor changed the UnoBoots and thought we might be improving the legs (we were) but Dean was getting sicker. He begin having bad nightmares, bloated stomach, back kidney pain, and severe diarrhea. Our doctor was seeing Dean every week and found bad bacteria in a stool culture. During this time, Dean began to be very jaundiced, the hot flashes were not improving, he was very pale, and felt cold to the touch (unusual for him). The end of September found the doctor taking off the second set of Uno Boots and the legs finally improving.

By the middle of October, we were out of the woods and into the throes of compression hose problems. Dean's feet are quite deformed (multiple surgeries in his youth), and when the swelling is down he has the typical CMT stork legs. I have health problems of my own and have very little strength in my hands, compounded by a breathing problem, so the amount of exertion required to put the hose on and off is a very real problem! Dean cannot put them on himself so it's a real joint effort.

We are getting ready to go pick up the second attempt at custom-made Jobst socks. If these do not work, we won't try again. The first pair didn't work and were agony to put on and impossible to get off! We've been using regular Jobst knee- high support hose every other day (versus daily wraps) until just recently. For Dean, I think we need the toes out kind because he is fighting another large (over half inch diameter) water blister between two toes and we're watching it like a hawk! The leg wraps are easier but they allow some water retention in ridges so they're not the perfect solution by any means. For now, we watch the swelling and hope to keep it under control.

The best explanation we've received concerning the leg swelling was from Dr. Miller. Her explanation was that muscles have many responsibilities including proper blood circulation (which is necessary to keep swelling down) and the CMT atrophied muscles get a failing grade on this part of their job! On top of this, Dean has mild heart failure (weakened heart muscles) so it also complicates the circulation problems.

Linda here - Most people with mild or moderate CMT who are active do not have problems with swelling but it certainly can be part of our CMT symptoms.

Readers write about their foot problems
Andy AldrichFrom Andy Aldrich, U.S.A.: Due to CMT related foot and ankle problems, I had a triple arthrodesis performed on my left foot in 1967 and on my right in 1968. I was in junior high school at the time and these surgeries allowed me to lead a fairly normal life. I jogged for several years and also was able to go deer hunting for almost 30 years.

As the years went by my ankles started to turn over like they did before the surgeries and the pain levels in my feet became worse and worse. Occasional trips to see foot doctors resulted in statements like, "There's nothing that can be done to help you, you will just have to learn to live with this." I stupidly listened to these doctors and did learn to tolerate increasing pain levels and walking difficulty.

By 1997 my ankles were turned over to the point that I could barely walk and I could no longer control the pain. I needed help so I saw my PCP(primary care physician). She sent me for physical therapy and to see a local orthopedic surgeon. The therapy helped a lot but was not the answer. The surgeon wanted to fuse both ankles. He also had the courage to admit he knew next to nothing about CMT and sent me to Massachusetts General Hospital, in Boston, for another opinion. The surgeon at Massachusetts General knew a lot about CMT and also wanted to fuse both ankles.

At this point, I was still not satisfied with the doctors' opinions and asked for a third opinion. To my surprise, the HMO sent me to the Lahey Clinic. Again, the surgeon recommended fusing both ankles but wanted me to see another surgeon who had more experience with severe foot and ankle problems.

The fourth surgeon was completely different from the others. He spent over an hour examining my feet and X-rays and was much more thorough than the other three. He said the right ankle would have to be fused but held out hope that he could save the left. He also said that if I had come to see him four or five years sooner he could have saved both of my ankles. I can't explain it but as soon as I saw him I knew he was the best man for the job. I told him to schedule the surgery.

I was given the option of doing both feet and ankles at the same time or doing them one at a time. I opted for one at a time, as doing both would mean six weeks in a wheelchair and a stay in a rehab hospital.

The first surgery was on my right ankle in October 1997 at the Lahey Clinic. My ankle was fused and the Achilles tendon stretched. After six weeks on crutches the forefoot was operated on. An osteotomy straightened my foot and my hammertoes were straightened. The left foot was done in September 1998, again at the Lahey Clinic. The doctor wouldn't know if the left ankle could be saved until after the surgery was started. He was able to save the ankle, only 10 degrees of motion left but much better than no motion. This was also a two-part surgery with the forefoot and toes to be done after six weeks on crutches.

I am now fully recovered from all four operations. I had six weeks of physical therapy and gait training, as I had to learn to walk with straight ankles.

So far, the surgeries have been a huge success. What a difference straight ankles and feet make! Almost all of the pain is gone, although I do have some nerve pain in the left foot. My energy levels have improved now that I'm not constantly battling the pain and I find that I have a lot more ambition than I had before the surgeries. My balance is also much improved.
It was a tough two years but being able to walk without pain makes it all worthwhile.

The moral to this story is don't listen to doctors who don't know about CMT. Get help as soon as you start having problems and get as many opinions as you can. The more informed you are the easier it is to make the correct decision on how to proceed.

For anyone in the New England area who needs an opinion on whether to have surgery or not my doctor's name is Mark Slovenkai and he is an orthopedic surgeon at the Lahey Clinic in Burlington, Massachusetts.

Lengthening the Achilles Tendon
Julia Sherman, U.S.A.
Apologetically, my physical therapist said, "In order to lengthen the Achilles tendon, you need to stand on a slant board for 20 minutes continuously...understand, I mean you must maintain that stretch for at least 20 minutes in order to have any effect. I know that will be hard to do."
Solution: Every morning I got breakfast all lined up, turned on the TV, opened a lower kitchen cupboard door, and put my slant board in place on the bottom shelf of the open cupboard. This provided a convenient 3 to 4 inch slant to the floor. Then, I stood on the slant board for 20 minutes while I ate my breakfast...a perfect fit for my routine, and it worked.
For a slant board, you can use a ready-made shelf board purchased from a hardware store.