• How do you avoid getting pressure spots from braces?

    Braces need to fit perfectly to avoid pressure spots. Custom-made braces that are properly designed, fabricated and fitted for the patient are the best type of brace. Well-fitted braces should be comfortable and used with appropriate shoes, such as laced or Velcro-fastened sneakers. Braces need to be adjusted periodically to account for weight gain or loss and need to be replaced periodically

    If the disease progresses to produce severe heel varus (inverted feet) or curled toes (hammertoes), surgical procedures may need to be considered. You need to be evaluated periodically by a neurologist or an orthopedic specialist for advice regarding the adjustment or replacement of the braces and/or the need for surgical procedures.

  • Is there any type of brace for people whose hands are affected by CMT? I’m experiencing weakness and tremor.

    Hand involvement in Charcot-Marie-Tooth disease (CMT) usually occurs late and produces weakness and wasting of the hand and finger muscles, causing difficulty in extending the fingers. The thumb gets weak and rotates externally, making movements such as pinching, opening jars, buttoning and unbuttoning very difficult. A good occupational therapist can not only teach you some hand and finger exercises, but he or she can get you some tools such as button hookers, tools to hold eating utensils and other tools that will help you with some of the daily activities. If indicated, the therapist, along with your physician, can also recommend some type of splint or braces. Tremor is a frequent occurrence in CMT and is aggravated by the nicotine in cigarettes and caffeine in coffee and tea. Some medications improve tremor, including beta-blockers and anti-convulsants. A neurologist should be consulted before taking any of them.

  • Should people with CMT wear support stockings or socks when flying? Are there any reasons not to wear these stockings when flying?

    Unless the person has Hereditary Neuropathy with liability to Pressure Palsies (HNPP), which is typically caused by a deletion of PMP22, wearing compression stockings should not be a problem. The nerves are not more sensitive to compression for most kinds of CMT.

  • I wear Ottobock Knee-Ankle-Foot Orthoses (KAFOs) on both legs. Would it be okay to ride a regular bicycle to strengthen my legs and help me lose weight?

    First, I salute you on staying active. The bicycle will help you lose weight and tone your leg muscles.

    With your history of knee braces and AFOs, it would be a wise idea to work with a reputable bike shop to obtain a proper “fit” on your bike. Varying seat heights, as well as changing out stem length on the handlebars can make a big difference in the way your body handles riding your bike.

    There are many new pedal variations and new styles of cycling shoes that could benefit you. Most high-end bike shops have at least one trained technician who can work with you to help you ride efficiently and prevent many of the aches and pains that comes from a misaligned frame (your frame on the bike frame).

    Your physical therapist and/or orthotist might need to be involved if you can’t decide whether to wear braces while riding. Above all, you want safety—to be able to get off the bike in a hurry. You also want to know how to best benefit your leg muscles and keep the joints aligned correctly while exercising. Have fun outside and don’t forget to wear a super comfortable helmet.

  • What type of foot orthotics should I get?

    The type of orthotic will depend upon your “foot type.”

    With a more rigid foot that is also high arched and rolling to the outside, many times-a soft over-the-counter (OTC) device works fine. This is especially true if AFOs are worn only occasionally. A true custom “posted” foot orthotic made to a mold or a laser scan of the feet is appropriate in the rarer instance of the flat (pronated) and mobile type CMT foot.

    The foot orthotic often becomes ineffective as the CMT progresses and the foot deformity advances and worsens. This is when shoe modifications like rocker bars or wedges, and one of the newer spring- type carbon fiber AFOs begin to help more than just an in-shoe orthotic device.

    Consultation with an orthotist/prosthetist might be helpful to gain further understanding of options. Usually the answer lies in a correct combination of shoe types and a specific type of orthotic (orthosis).

  • Is the WalkAide system recommended for those with CMT?

    The WalkAide and similar products that operate using a neuromuscular electrical muscle stimulation unit (NEMS) are contraindicated for those with CMT. These systems use electrical stimulation to activate the muscles and create a contraction. This works best for patients who have central motor neuron processing problems and intact peripheral nervous systems. For those with CMT, the electrical signal will not travel through the nervous system appropriately and as a result a patient can get an unsafe amount of electricity to elicit a muscle response. With prolonged use, this could lead to electrical burns and potential nerve damage. The WalkAide may be able to elicit a muscle response in patients with minor CMT involvement, but it is not recommended in these cases because it can damage healthy nerve cells. This has not been directly reported with CMT as far as I am aware, however it has been reported in spinal cord injury (SCI) patients with lower motor neuron lesions, versus SCI patients that have upper motor neuron lesions. This device is best suited for patients with multiple sclerosis, whose movement is affected by the upper motor neuron involvement.

  • How does one go about finding a good orthotist who knows CMT?

    Please check in with your regional Branch leaders for suggestions: https://www.cmtausa.org/resource-directory/wpbdp_category/cmta-branches

  • How can I keep my toes from curling up when wearing shoes with a carbon fiber AFO

    It can be challenging to keep your toes straight with the muscle imbalances brought on by CMT. These toe deformities are called hammer toes, or sometimes “claw” toes. Two products have proved successful in our patient population:

    1. A crest pad (sometimes called a buttress pad) may help. These gel pads have a loop around two to three toes, giving them a spongy silicone bumper on which to ride, and to help keep the toes straight. They also reduce pressure on the tips of the hammer toes when walking. Here is a link to a typical crest pad: http://www.drjillsfootpads.com.
    2. Many CMTers have found relief with a product called Correct Toes, silicone squares that give each digit its own little “garage” in which to rest and keep the toe straight and protected. These require a little more room in your shoe around the toe box, however. They work very well if you wear sandals or a stretchy closed-toe shoe. Here is a link: http://naturalfootgear.com/products/correct-toes.
  • I need to wear my braces while I’m in the pool. Is there a way to cushion the bottom of water shoes?

    Dr. Greg Stilwell replies: “Yes there is! I recommend a Dr. Scholl’s flat gel insole. Big box retailers (Target, Walmart, etc.) typically have a good selection of these insoles. These are inexpensive; you could even buy two pair at low cost. Also, the insoles can be easily trimmed to fit your water shoes. Look for the ones that have very little absorbent cloth on the top, since they dry quickly. When you’re done swimming, be sure to pull the insoles out of your water shoes and hang dry the shoes and insoles separately to eliminate any moisture. Excess moisture can easily grow fungus – not a friend to those of us with CMT!”