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·0 If your muscle is able to contract with weight applied,you should see a physiotherapist to find out the maximum weight the muscle is able to tolerate.There was a study done of exercise in Neuromuscular Diseases, by Dr.W. Fowler Jr of the Rehabilitation and Research Training Centre in Neuromuscular Diseases at UC Davis. The muscle can then be exercised three days a week, starting with three sets of four repetitions; this can be increased over a twelve week period to eight repetitions per set. A modest increase in strength may result.
·0 Strengthening exercises which use heavy weights should always be avoided: increased weakness and muscle cramps may occur.
·0 It is very important to avoid immobilization, if possible, because it causes disuse muscle atrophy as well as loss of the motor patterns you have learned, which compensate for the weakness in some muscle groups: it is advisable to be physically active for 2 or 3 hours every day.
·0 Exercises designed to improve coordination and balance can help, especially after a period of bed rest. (e.g. after illness or a fracture requiring rest in bed).
·0 It is impossible to reduce the tiring of your muscles: the
only therapy is... rest.
·0 Leading the most normal life possible is the best active therapy
2. Management of deformities and contractures Bone and joint deformities are caused by muscle contractures. Deformities are caused by an imbalance of strength in the muscles that act across joints. In CMT, the dorsiflexor muscles [tibialis anterior, peroneus longus and brevis],(which pull your foot up) are affected more than the plantarflexor muscles [tibialis posterior and gastrocnemius],(which push your foot down).
As CMT progresses, dorsiflexion of the foot becomes impossible and you get foot drop. Sometimes the heel will not reach the floor, [pes equinnus] while you are standing. (Your achilles tendon has a contracture). Usually, this is associated with rotation of your foot, [pes equinnus-cavus-varum-supinatum] causing a reduction in size of the area of your foot which contacts the floor. This causes pain along the outside border of your foot.
The same mechanism may occur in the wrists causing
a claw hand deformity.
Contractures
can help be prevented by:
·0 Stretching calf muscles:
the best stretching is obtained by standing and walking, with flat shoes
and foot orthoses.
·0 Wear night braces: to keep your feet in the proper position while you are asleep.
·0 Avoid the weight of blankets on your feet: a light duvet or a bar for the blankets are recommended, especially during adolescence.
When stretching is no longer effective and your heel will not reach the ground when you are standing, surgical Achilles lengthening (or other surgical procedures) are necessary. If a contracture forms in one foot only, standing position may be altered and scoliosis may develop.
Contractures may occur in your hands, leading to claw hand deformity: a daily self-made stretching exercise of your fingers and wrists is useful to prevent them becoming fixed in one position.
Muscle contractures may cause deformities that can only be corrected
with bone surgery, especially if they occur while the bones are still growing.
Avoiding becoming overweight or frank obesity, is very important, as weak lower limb muscles are unable to support an increased body weight while standing or walking.
* The shoe should be higher and stronger than normal, as your ankle needs support, in order to prevent sprains: I recommend a 15-20 cm. (6"TO 8") high sport boot as its synthetic larger sole works like a shock-absorber.
* When there is foot-drop and tripping is a problem, an ankle-foot-orthoses (AFO) is necessary: I recommend a plastic, low, AFO, open at the heel, with a complete sole, and the sides joined by foot orthoses.
* For people who don't want to wear AFO's, a 15-20 cm. (6" TO 8") high sport boot, will do the same thing, if the back of the shoe is strengthened with strong leather or polypropylene. A latex pad inserted into the back of the shoe will allow the ankle some flexion while walking, and help prevent pain in your Achilles tendons.
* If the quadriceps muscle (above your knee) becomes weak, i.e.
when you are unable to straighten your leg while seated, a knee-ankle-foot
orthoses (KAFO) is necessary, but two canes are necessary to
walk, so, an assessment of strength in your upper limbs should precede
this prescription.
Falls are prevented by proper shoes with orthoses and, overall, by paying attention:
·0 watch the floor: even the lowest step can cause a trip or sprain
·0 avoid uneven ground: unless a cane is available to improve balance
·0 beware of carpets and broadloom.
·0 avoid dark places: balance worsens where you can't see
·0 use a railing while going up and down stairs
·0 avoid haste
In rare advanced cases, walking outside the house can become unsafe and tiring. When falls are frequent, it is advisable to use a wheelchair outside of the house and a walker inside .
An Electric wheelchair or scooter will protect your upper limbs from
overwork, saving you energy which may allow you to continue to walk inside
at home or at work, and give you more independence.
Soft woolen gloves with a calf-skin palm can protect your hands, and prevent your hand slipping on railings etc. However, sometimes they restrict flexibility in your fingers.
Most people with CMT, have difficulty with activities which require
hand control and sensation during daily living (ADL) : special tools or
simple changes in the tools you use, can be very helpful. An Occupational
Therapist can give advice for your specific needs. On the web page http://www.maddak.com
you can find a catalogue of special tools for ADL.
·0 Deep sighs should be done regularly, to air those parts of the lungs that are not ventilated during quiet breathing.
·0 Assisted cough exercises are useful if you have the flu or respiratory problem in which you produce phlegm.
·0 Assisted ventilation is
rarely necessary, but may be in severe cases of CMT with respiratory failure
due to involvement of the phrenic nerve.
·0 Low back pain:
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"Management and Rehabilitation In CMT"� 1997-1998 by Paolo Vinci M.D.
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Dorothy B. Gosling (Retired R.N.)
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