Physiotherapy in FSH Muscular Dystrophy

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Physiotherapy in FSH Muscular Dystrophy

• Originally presented by Marina Morrow at a FSH
  Information Day

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FSH Muscular Dystrophy

• It is the third most common muscular dystrophy
  with approximately 1/3rd of people diagnosed in
  later life
• The earlier the diagnosis, the more severe the
  clinical presentation
• Affects both males and females and is present in
  all racial groups
• Muscle involvement varies from person to person
  (both within families and between families)

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Clinical features of FSH

• Progressive weakening of skeletal muscles
• Weakness round the face, neck, shoulders and
  upper arms
• Legs can also be affected leading to ankle
  tightness

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Early Symptoms

• Unable to open and close eyes fully
• Unable to fully smile, whistle, suck from a straw
• Weakness of the shoulders unable to stabilise
  the shoulder girdle
• Tendency for the weakness to be asymmetrical

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Muscle weakness

• Assessment of muscle strength, endurance and
  fitness by a specialist physiotherapist
• Learn to work within your capabilities
• Avoid true muscle strengthening which aims to
  overload the muscle

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Muscle Strengthening

• Avoid muscle endurance techniques as the basis of
  these tends to focus on repetition
• Vary exercise techniques e.g. Walking/swimming/cyc
  ling/gym to avoid adaptation to the stimulus
• Use resistance rather than weights if you are
  unsure
• Warm up and cool down phase with stretches of the
  appropriate muscle group

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Exercise

• Exercise will improve muscle endurance and
  muscle strength (prevent disuse atrophy) and help
  to improve mood. In the first six weeks of an
  exercise programme, the muscles will become more
  effective and efficient
• BUT
• Avoid muscle fatigue Learn to work within your
  capabilities and pace yourself

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Foot and ankle instability

• Can affect balance as feet tend to slap down
• Unable to respond to differing surfaces very
  quickly i.e. Slopes, hills, kerbs, gravel, grass,
  snow and sand
• Unable to respond to inclement weather, busy
  areas as fast changes in direction can affect
  balance

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Ankle tightness / Contracture

• Regular stretching and warm muscles
• Hold the stretch for a minimum of 20s

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Possible solutions

• Shoes with a wide, deep toe box and large base of
  support are generally found to be beneficial

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Back problems possible solutions

• Stretching and exercise
• Postural management
• Orthoses good clinical assessment is essential
• Surgical interventation

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Fatigue

• Muscles can get tired more quickly and recovery
  takes longer
• Avoid strengthening work
• Look for ways to conserve energy
• Take frequent breaks
• Pace yourself think about your daily schedule
• Ergonomic seating an positioning. Frequent
  changes of position
• Beds / chairs / work surfaces

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Balance and falls

• Look where you are going and assess the situation
• Ensure paths/doorways etc. are well lit
• Appropriate orthoses and footwear essential
• Avoid soft tissue injury
• Develop a fall strategy
• Progression of muscle weakness coupled with the
  aging process

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Postural Management

• ... The use of any technique to minimise
  postural abnormality and enhance function
• Farley at al (International Journal of Therapy
  and Rehabilitation October 2003)
• Techniques to improve posture include surgery,
  orthoses, static seating, adaptive seating in
  wheelchairs, physiotherapy and postural
  equipment such as standing frames, sleep systems,
  etc.

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Beds

• Height adjustable
• Powered back rest

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Wheelchairs

• Shop mobility
• Long distances
• Powered v manual
• Tilt and recline

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Pain Relief

• Postural management
• Massage
• Acupuncture / heat therapy (if sensation is
  unaffected) / TNS machine
• Medication
• Referral to pain team