• I have CMT and my doctor wants me to have shoulder replacement surgery. Will my CMT have an adverse effect on healing?

    If the person has good shoulder strength going in, the proposed surgery should be perfectly fine.

  • A 29-year-old CMT patient had surgery on the ball of his foot. The incision failed to heal and he is receiving intravenous antibiotics several times a day. Do CMT patients have healing problems?

    CMT’s circulation problems and decreased sensory loss could slightly affect healing.

  • I am 43 years old and have pain in my knee. The doctor said my CMT is pulling my kneecap to the side, causing the pain. What can be done for this?

    Generally, unless the CMT starts in childhood, the quadriceps, the muscle that pulls the kneecap to the side is not affected. However, there may be deformity in the knee caused by arthritis or other disease of the knee joint, unrelated to CMT, that could cause pain. If that happens, the quadriceps might pull the kneecap to the side and the treatment for that is to take care of the underlying problem.

  • I’ve seen reference to the potential need for foot/ankle surgery in some cases of CMT. Is the surgical procedure specialized? Are there specific indicators or contraindicators for this surgery?

    Surgery should only be done if there is a need and a benefit and the patient and the surgeon have a frank discussion about both sides of the equation. Before surgery, the patient must give “informed consent” and all the patient’s questions need to be answered so that the patient knows exactly what is being done, the expected results, and the problems, complications, difficulties and benefits. Alternative measures need to be discussed. This is universal practice in the United States.

    Operating on CMT patients is not considered routine surgery for any orthopedic surgeon. Some fellowship-trained (foot/ankle) orthopedic surgeons have had special training in foot and ankle surgery and may even limit their practices to foot and ankle surgery. Because of the variability of surgeons’ experience and the ongoing muscle weakness of CMT patients, each case has to be assessed individually and surgery planned carefully.

  • While I was wearing my AFOs, my knee suddenly gave out and I sprained the top part of my right foot. Is this associated with CMT?

    >Experiencing the knee giving out in a backward direction and hyper-extending the foot and ankle is not an uncommon occurrence in people with Charcot-Marie-Tooth Disease. Due to poor proprioception (the ability to feel position) and atrophy of leg muscles, it is difficult for all CMT individuals to maintain good balance. The calf muscle (behind the lower leg) crosses and helps stabilize both the knee and ankle. Because of weakness in this muscle due to CMT, the knee can hyperextend and cause instability and balance loss. This can cause the individual to fall backwards, thus hyperextending the foot and ankle.

    Strengthening exercises usually offer little value due to the muscles’ inability to function from CMT. A knee brace can help, but they are bulky and cumbersome to wear especially if one is already wearing AFO bracing. There is no specific treatment for this problem other than being aware that it is present and taking precautions to avoid being injured in the fall. Continued use of a crutch for extra stability would also be recommended.

  • Is there a relationship between recurrent patellar dislocation and CMT in teen girls?

    Assuming there are no congenital anomalies or growth development problems, the dislocation of the patella (which is generally lateral) to the outside is usually not related to CMT disease, but does happen quite frequently in girls, because of the positioning of the patella (kneecap) in its “groove” or “track” as it extends the knee.

    Girls who are obese may have kneecaps that “track” towards the outside and with a bit of trauma or with a “trick” movement, can dislocate them. Once that happens, the dislocation may occur more frequently. A pediatric orthopedic surgeon should easily recognize this problem and if a “brace” does not work, a surgical repair may be needed.

  • What surgeries are typically done to fix the foot deformities common in CMT?

    There are many surgical procedures available for people with CMT, but one that is quite effective is reconstruction of the foot and ankle. Options include the straightening of hammered toes, the repositioning of an in-turned heel and the lowering of the arch. The end result is a foot that is stable and is able to stand flat on the ground. Tendon transfers on the lateral side of the foot and ankle can also provide increased strength and a more normal gait. AFOs, or ankle-foot orthoses, help with stability and balance, and should always be considered before surgery.

  • Are people with CMT prone to reactions from anesthesia when having surgery?

    Most people with CMT will have no problem with anesthesia. Only those with respiratory involvement (very rare) or vocal cord paralysis (a very rare variant) may have problems with anesthesia above that seen in the general population.

  • I’m having surgery and will be given Versed. I read somewhere about being careful with Versed. I have CMT 1A and am wondering if that medication will be okay.

    I know of no special concerns with Versed and CMT unless it is used for an extended period of time, unlike the brief exposure for anesthesia before surgery.

  • I am 78-years old and I need a knee replacement. Will this be successful with my CMT?

    While I cannot tell you if your surgery will be successful or not, I can tell you that many people with CMT get joint damage because their ankles/knees/hips have to do more work to stabilize them than people without CMT. This is due to muscle weakness, loss of proprioception (knowing where your body is in space), and frequent abnormal foot structure. When the arthritis becomes severe in the knees, for example, it needs to be treated, which sometimes requires knee replacement. There is no contra-indication to surgery because of CMT. Exercise is part of building up the knee again. It should probably be low impact but I suspect that is what the surgeons would plan.

  • My right ankle is very unstable, causing me to stagger and pronate. I’ve tried various types of AFOs, but they are all very uncomfortable (though they work well on my other foot), and I still stagger. My ankle is stiff. If I had an ankle fusion, would a bone stimulator (on the outside of the cast for two months) be safe?

    It sounds like your unstable ankle really affects your day to day life, including your hips and spine.  The lack of motion in the ankle is usually from end-stage arthritis.  Ankle replacement surgery may be an option for you; get several opinions from fellowship-trained (foot/ankle) orthopedic surgeons, as well as board-certified reconstructive rear foot/ankle podiatric surgeons. Ankle fusions are great when healed and aligned correctly, but can put undue stress on your knee.  The current trend is more towards ankle replacement, as opposed to fusion.

    Bone stimulators should be safe and helpful for bone healing with CMT. TENS units are also commonly used after surgery to control pain.  Both are electric stimulators that should be preset so as to not give excess electrical current, which could burn someone with lack of sensation from a neuropathy like CMT.

    Rehabilitation will be paramount after your ankle surgery; therapy should be started right away.  There is no doubt other muscles and joints in your legs, hips and back have developed “compensation patterns” that will negatively affect your recovery and learning to walk without your “stagger.”  Hopefully you can find a physical therapist trained in kinesiology and body mechanics before your surgery–and make sure the therapist agrees to help keep your “swagger,” but get rid of your “stagger.”

    You might also consider working with a pedorthist, podiatrist, or an orthotist to have shoe modifications done–like a rocker bar or lateral sole flare–to give you more stability and more efficient gait patterns.  Please let us know your progress. We are all in this together!

  • Is surgery more effective in certain types of CMT?

    The most common CMT symptoms are: foot drop, high arches, claw toes, lower leg weakness, numbness and pain. As the disease progresses, similar symptoms may also appear in the hands and arms. The effectiveness of surgery is linked more to the competency of the surgeon and the severity of the deformities than to the type of CMT a person has.

  • Does CMT1A pose any risks for cataract surgery?

    There should be no risk for cataract surgery based on CMT. As far as we know, there is no evidence that the optic nerve is affected in CMT1A. The optic nerve is a central nervous system nerve and is structurally different than peripheral nerves. There are other forms of CMT that have optic nerve involvement, but not 1A. CMT1A should not have a direct effect on vision. In general, vision problems are probably due to something other than CMT.

  • Which bone should be used for an ankle fusion?

    TThe best bone to use is your own bone, either from the fibula or proximal tibia.

  • Why are ankle replacement surgeries not recommended for CMT?

    A total ankle replacement is usually not done in a patient who has any decreased feeling because the failure rate is too high.