RESPONSES to Elective TT Amputation secondary to painful CMT
Duane Nelson
Description
Collection
Title:
RESPONSES to Elective TT Amputation secondary to painful CMT
Creator:
Duane Nelson
Date:
2/2/2016
Text:
Thank you for the incredible responses regarding my query about experiences with elective TT amputations secondary to painful CMT disease. Some of you requested to see the responses....here they are!
Responses edited for brevity and listed in random order:
1) limb salvage surgeries including fused ankles for CMT disease and we have seen many of these patients have long term successful results from elective BKA surgeries once the surgeries are longer feasible with positive outcomes. The healthcare team that we have put together including a Psychiatrist, PT's, Social Workers, and ourselves meet monthly to discuss these patients. Our experience with these patients living with CMT disease is very positive and more often than not, allows them to once again become independent, often times return to work, and more importantly decrease or cease to use pain killers any longer. In my opinion, the paradigm shift in the surgical field to accept amputation as a more functional choice than continued surgical procedures with risk of infection has made a world of difference for the patient to see a light at the end of the tunnel in reduction of pain, ability to ambulate and be independent, return to hobbies or work, reduce stress on family members, and the financial burden of paying for surgical procedures long term and the therapy and/or recovery time
2). I worked with a gentleman who had CMT quite a few years ago who underwent a BKA. I believe it was elective only after battling a wound on his foot that wouldn't heal. He did great as an amputee and his friends and family all said he walked much better as an amputee.
3). Had a very interesting and positive experience with a patient a few years back when I was in private practice. She was a little younger, different Dx, but similar circumstances. She opted for a unilateral elective amputation after many years of trying to convince a surgeon that it was a more viable option than her present circumstances of constant breakdowns, hospital stays and pain. She had undiagnosed Spina Bifida Occulta with feet that would be very similar to Charcot-Marie-Tooth disease. She will tell you the choice was life transforming for the positive
4). I have had a few people with the same experience. One is a woman in her 50s, that is a unilateral BKA. She had several surgeries and orthotic devices to try and use her foot, but she was in severe pain. She is much more mobile after receiving her prosthesis. My other patient is a male in his 40s, who is now a bilateral BKA from his elective surgeries. He is able to go dog sledding and hiking in Alaska. He definitely had his quality of life improve. It should be noted that he does not have any other major health complications.
5).Yes Yes Yes ! this is a great candidate for an elective surgery. I had a 43 yo with Charcot and did an elective Trans Tib, It was such a good outcome he elected to do the other side making him a bilateral Trans Tib.
6). My old office administrator had sever CMT and opted to become a bilateral BK amputee. Having worked in the industry she knew the benefits as well as draw backs of being an amputee. She did very well and never regretted her decision. She was much more mobile as a BK amputee with much less pain.
7). I have no experience with amputations. However, I have a lot of experience with CMT feet (I am assuming a classic Cavo-varus CMT foot/ankle) and I find that often the pain they experience is the result of bracing that is too simple and too painful, then rejected leading to further deformity, instability and pain. I would describe my approach as aggressive accommodation and correction of the foot thus reducing the force needed to correct the ankle from the side.
8). I have worked with several patients who have made this choice and they do not regret it or suffer from any unique, debilitating problems associated with CMT that have reduced their mobility with a prosthesis. I should note that one requires a DFA AFO on the opposite side and often feels this is more limiting than her amputated side. It remains to be seen how they will do as the CMT progresses however and I would expect their mobility to deteriorate more rapidly than similar age-matched peers.
I should also note that each of these individuals had undergone multiple and, at best, semi-successful attempts at fusions. One had problems with chronic and recurring infections and the others, debilitating pain. I would never suggest this as a first option if they have not seen a highly skilled foot and ankle surgeon and I know one of the patients was turned down by several Orthopedists who viewed this as extreme and unnecessary
9). Has she already been to see an orthopedic surgeon? TT amputation seems extreme. I have several CMT patients who have had very good results with osteotomies, fusions, and tendon transfers...some who have been able to ditch the AFOs.
10). I have treated several, including a bilat we are working on now. They all have done very well.
11). Hi. That is interesting indeed since all the Charcot I have seen has a presentation of numbness, or no feeling at all. If this patient has been battling this problem and the paint certainly she deserves pain relief. Though I have not had a patient having gone for surgery your patient needs to educate herself thoroughly
12). No way DONT DO IT !!!! You failed to mention, was she fit with PTB support AFO'S? Make her some she will love you.
13). Wow that is a great question. I would consult with a neuro surgeon experienced in de-myelinating diseases. If you cut the nerves, will it exacerbate the process farther up the chain?
14). I have had this situation a few times in my 20 plus year career. The amputation has yet to alleviate the pain to any measurable degree and because of the continued pain it was no help with mobility either. I would advise otherwise.
15). Yes, regularly. Pain however is subjective, and an amputation does not mean that the person will be pain free afterward. Most surgeons that I work with believe that a spinal block preoperatively will reduce the incidence of post amp-pain
16). I have encountered exact situation two times with one individual having pain eliminated and the other not, both patients as amputees performed better in there ADLs post amputation. It has been my experience that pain isn't always eliminated by amputation regardless of the underlying condition.
17). This is a hard call for pain relief. The longer she has dealt with the pain the lower are chances she will get full or significant relief. Our experience has been..you just don't know how sever the phantom pains will be. Mobility can be significantly better. Depends on how impaired she is currently
18). We have several CMT patients that finally had to have a BKA and they increased their activity level. Sadly, with CMT they may also become bilateral which adds another level of complexity for them.
19). I have a CMT patient who is a TT. He had a very unstable ankle (not Charcot foot) and is a unilateral TT. He wears at times an AFO on the other side. He walks well but balance is off. He biggest problem is step width and sometimes puts his foot (either) too close to midline and has to recover. My patient has been able to work, geocache and travel over the last 10 years I've known him.
20). I see a fair amount of CMTers. It would be unusual to need an amputation for relief of pain that derives from CMT itself. Pain that derives from the mechanical secondary effects though is common. DBS type bracing is pretty extraordinary at 1) relieving that kind of pain, 2) restoring the balance that CMT erodes, and 3) controlling the anatomic alignment changes that accompany the progressive muscle power loss. I have patients who's next option was reconstructive, multiple fusion surgery secondary to severe, progressive pain who have been years without the surgery or the pain. I have not had any offered amputation related to CMT, but some with similar mechanically driven pain from post traumatic arthritis have, and have also had relief without surgery. I think the functional capacity as measured by pain, walking speed, ambulatory range (the distance a person can walk without having to stop), ambulatory symmetry, and device management issues is better with this bracing than with prosthetics for CMT case.
21). I have experienced several ( at least 5) elective amputation cases similar to yours. None with Charcot-Marie-Tooth. The cases that I experienced had unremitting pain from afflictions such as poorly healed Crush Injury; Land mine explosion; Gunshot wound; Chronic bone infection; Joint fusion; Severe Arthritic conditions and etc. As I recall the amputation outcome in almost every case was quite negative! On your and your patient's behalf please stay on the conservative side. Your patient has many years ahead of her.It is stating the obvious that Amputation is ablative and irreversible. Many of the patients I knew - who elected to have amputation - were not able to overcome the psycho- social aspect. Most did not get rid of their pain. Often the surgeons could find no physical cause. Some thought the nerve pain pathways had been so thoroughly established that pain continued as though the causative factor was still present. Others believed it was a form of phantom pain. While some psychiatrists believed there was a psychiatric generation of pain.Medicine advances every day.
thanks,
Duane Nelson CP(c)
Saskatchewan Abilities Council
Responses edited for brevity and listed in random order:
1) limb salvage surgeries including fused ankles for CMT disease and we have seen many of these patients have long term successful results from elective BKA surgeries once the surgeries are longer feasible with positive outcomes. The healthcare team that we have put together including a Psychiatrist, PT's, Social Workers, and ourselves meet monthly to discuss these patients. Our experience with these patients living with CMT disease is very positive and more often than not, allows them to once again become independent, often times return to work, and more importantly decrease or cease to use pain killers any longer. In my opinion, the paradigm shift in the surgical field to accept amputation as a more functional choice than continued surgical procedures with risk of infection has made a world of difference for the patient to see a light at the end of the tunnel in reduction of pain, ability to ambulate and be independent, return to hobbies or work, reduce stress on family members, and the financial burden of paying for surgical procedures long term and the therapy and/or recovery time
2). I worked with a gentleman who had CMT quite a few years ago who underwent a BKA. I believe it was elective only after battling a wound on his foot that wouldn't heal. He did great as an amputee and his friends and family all said he walked much better as an amputee.
3). Had a very interesting and positive experience with a patient a few years back when I was in private practice. She was a little younger, different Dx, but similar circumstances. She opted for a unilateral elective amputation after many years of trying to convince a surgeon that it was a more viable option than her present circumstances of constant breakdowns, hospital stays and pain. She had undiagnosed Spina Bifida Occulta with feet that would be very similar to Charcot-Marie-Tooth disease. She will tell you the choice was life transforming for the positive
4). I have had a few people with the same experience. One is a woman in her 50s, that is a unilateral BKA. She had several surgeries and orthotic devices to try and use her foot, but she was in severe pain. She is much more mobile after receiving her prosthesis. My other patient is a male in his 40s, who is now a bilateral BKA from his elective surgeries. He is able to go dog sledding and hiking in Alaska. He definitely had his quality of life improve. It should be noted that he does not have any other major health complications.
5).Yes Yes Yes ! this is a great candidate for an elective surgery. I had a 43 yo with Charcot and did an elective Trans Tib, It was such a good outcome he elected to do the other side making him a bilateral Trans Tib.
6). My old office administrator had sever CMT and opted to become a bilateral BK amputee. Having worked in the industry she knew the benefits as well as draw backs of being an amputee. She did very well and never regretted her decision. She was much more mobile as a BK amputee with much less pain.
7). I have no experience with amputations. However, I have a lot of experience with CMT feet (I am assuming a classic Cavo-varus CMT foot/ankle) and I find that often the pain they experience is the result of bracing that is too simple and too painful, then rejected leading to further deformity, instability and pain. I would describe my approach as aggressive accommodation and correction of the foot thus reducing the force needed to correct the ankle from the side.
8). I have worked with several patients who have made this choice and they do not regret it or suffer from any unique, debilitating problems associated with CMT that have reduced their mobility with a prosthesis. I should note that one requires a DFA AFO on the opposite side and often feels this is more limiting than her amputated side. It remains to be seen how they will do as the CMT progresses however and I would expect their mobility to deteriorate more rapidly than similar age-matched peers.
I should also note that each of these individuals had undergone multiple and, at best, semi-successful attempts at fusions. One had problems with chronic and recurring infections and the others, debilitating pain. I would never suggest this as a first option if they have not seen a highly skilled foot and ankle surgeon and I know one of the patients was turned down by several Orthopedists who viewed this as extreme and unnecessary
9). Has she already been to see an orthopedic surgeon? TT amputation seems extreme. I have several CMT patients who have had very good results with osteotomies, fusions, and tendon transfers...some who have been able to ditch the AFOs.
10). I have treated several, including a bilat we are working on now. They all have done very well.
11). Hi. That is interesting indeed since all the Charcot I have seen has a presentation of numbness, or no feeling at all. If this patient has been battling this problem and the paint certainly she deserves pain relief. Though I have not had a patient having gone for surgery your patient needs to educate herself thoroughly
12). No way DONT DO IT !!!! You failed to mention, was she fit with PTB support AFO'S? Make her some she will love you.
13). Wow that is a great question. I would consult with a neuro surgeon experienced in de-myelinating diseases. If you cut the nerves, will it exacerbate the process farther up the chain?
14). I have had this situation a few times in my 20 plus year career. The amputation has yet to alleviate the pain to any measurable degree and because of the continued pain it was no help with mobility either. I would advise otherwise.
15). Yes, regularly. Pain however is subjective, and an amputation does not mean that the person will be pain free afterward. Most surgeons that I work with believe that a spinal block preoperatively will reduce the incidence of post amp-pain
16). I have encountered exact situation two times with one individual having pain eliminated and the other not, both patients as amputees performed better in there ADLs post amputation. It has been my experience that pain isn't always eliminated by amputation regardless of the underlying condition.
17). This is a hard call for pain relief. The longer she has dealt with the pain the lower are chances she will get full or significant relief. Our experience has been..you just don't know how sever the phantom pains will be. Mobility can be significantly better. Depends on how impaired she is currently
18). We have several CMT patients that finally had to have a BKA and they increased their activity level. Sadly, with CMT they may also become bilateral which adds another level of complexity for them.
19). I have a CMT patient who is a TT. He had a very unstable ankle (not Charcot foot) and is a unilateral TT. He wears at times an AFO on the other side. He walks well but balance is off. He biggest problem is step width and sometimes puts his foot (either) too close to midline and has to recover. My patient has been able to work, geocache and travel over the last 10 years I've known him.
20). I see a fair amount of CMTers. It would be unusual to need an amputation for relief of pain that derives from CMT itself. Pain that derives from the mechanical secondary effects though is common. DBS type bracing is pretty extraordinary at 1) relieving that kind of pain, 2) restoring the balance that CMT erodes, and 3) controlling the anatomic alignment changes that accompany the progressive muscle power loss. I have patients who's next option was reconstructive, multiple fusion surgery secondary to severe, progressive pain who have been years without the surgery or the pain. I have not had any offered amputation related to CMT, but some with similar mechanically driven pain from post traumatic arthritis have, and have also had relief without surgery. I think the functional capacity as measured by pain, walking speed, ambulatory range (the distance a person can walk without having to stop), ambulatory symmetry, and device management issues is better with this bracing than with prosthetics for CMT case.
21). I have experienced several ( at least 5) elective amputation cases similar to yours. None with Charcot-Marie-Tooth. The cases that I experienced had unremitting pain from afflictions such as poorly healed Crush Injury; Land mine explosion; Gunshot wound; Chronic bone infection; Joint fusion; Severe Arthritic conditions and etc. As I recall the amputation outcome in almost every case was quite negative! On your and your patient's behalf please stay on the conservative side. Your patient has many years ahead of her.It is stating the obvious that Amputation is ablative and irreversible. Many of the patients I knew - who elected to have amputation - were not able to overcome the psycho- social aspect. Most did not get rid of their pain. Often the surgeons could find no physical cause. Some thought the nerve pain pathways had been so thoroughly established that pain continued as though the causative factor was still present. Others believed it was a form of phantom pain. While some psychiatrists believed there was a psychiatric generation of pain.Medicine advances every day.
thanks,
Duane Nelson CP(c)
Saskatchewan Abilities Council
Citation
Duane Nelson, “RESPONSES to Elective TT Amputation secondary to painful CMT,” Digital Resource Foundation for Orthotics and Prosthetics, accessed November 5, 2024, https://library.drfop.org/items/show/242010.
