by Paolo Vinci M.D.
by Paolo Vinci M.D.
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Improvement
Of Hand Function
Management Of Respiratory
Failure
| 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 fallsIt 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 functionSensation 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 problemsIndividuals with CMT often have psychological problems related to:
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:
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