Exercise, Management and Rehabilitation in CMT

[Guest guest]

By Paolo Vinci 2005

http://www.geocities.com/dgosling_rn/rehab.html

1. Improvement of muscle strength, resistance and balance

·0 If the muscle to be strengthened is only able to contract with no resistance

of any kind, a strengthening exercise program is either ineffective or may cause

increased weakness.

·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 . 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

3.Promotion of mobility:

Walking is the best physiotherapy for contractures; so, even in late stages of

severe cases who use a wheelchair , daily exercises with a walker are

recommended.

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.

Orthoses for walking

* Custom-made foot orthoses are always recommended, even in mild cases, because

they reduce impact on the bones of the foot, (plantar muscles on the soles of

your feet in CMT do not protect the bones); the best ones are in polyurethane.

The orthotics also help prevent callouses by keeping the foot in the correct

position, and give more stability 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.

Prevention of falls

It is very important to avoid falls, because fractures in people with CMT are

common, require more time to heal than in normal individuals, and the inactivity

may cause worsening of your CMT.

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.

4.Improvement of hand function

Sensation and strength in everyone's hands decreases with exposure to cold

temperatures. In CMT, cold may cause complete loss of strength and sensation, in

both hands and feet, resulting in increased disability, even in moderately low

temperatures.

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.

5. Management of respiratory failure

·0 Deep breathing exercises, take a deep breath in (pushing your stomach out),

hold 5 seconds, then blow out all the way. Hold 10 seconds and then repeat.

·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.

6.Solutions to psychological problems

Individuals with CMT often have psychological problems related to:

acceptance of the disease and their limitations.

progression of their disability

accepting orthotics,canes or other mobility aids

relating with the opposite sex and decisions about having children.

embarassment about their appearance

loneliness

difficulty finding a suitable job, and financial problems.

As these may cause depression, with a decrease in mobility and/or a rise in

aberrant behavior (eg.:alcoholism), psychological support is always recommended.

7. Solutions to other problems

·0 Difficulty swallowing: eating small bits of food instead of big ones with

your neck bent forward, can be more helpful than taking medication.

·0 Low back pain:

physiotherapy

corset or back brace : never use while walking

passive pelvic tilt exercises

·0 Knee pains can be prevented by foot orthoses and shock-absorbing shoes;

physiotherapy may be beneficial.