Duchenne Muscular Dystrophy: Rehabilitation Management

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Duchenne Muscular DystrophyRehabilitation
Management
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Introduction

• Different types of rehabilitation needed through
  life
• Delivered mainly by physiotherapists and
  occupational therapists, but others may be
  involved
• Rehabiliation specialists
• Orthotists
• Providers of wheelchairs/other seating
• (Potentially) orthopaedic surgeons
• Key management of muscle extensibility and joint
  contractures
• Stretching aims to preserve function and maintain
  comfort
• Programme should be monitored by PT, but must
  become part of the familys daily routine

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Contractures

• Factors contributing towards tendency towards
  contractures
• Muscles becoming less elastic due to limited
  use/positioning
• Muscles out of balance around the joint
• Maintaining good range of movement and symmetry
  is important
• Maintains best possible function
• Prevents development of fixed deformities
• Prevents pressure problems with the skin

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Management of muscle extensibility and joint
contractures

• Physiotherapist key contact for contracture
  management
• Ideally input from local PT supported by a
  specialist PT every 4 months
• Stretching should be performed at least 4-6 times
  a week as part of familys daily routine
• Effective stretching may require a range of
  techniques including stretching, splinting, and
  standing devices

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Stretches

• Regular ankle, knee and hip stretching is
  important
• Later, regular stretching at the arms becomes
  necessary especially fingers, wrist, elbow and
  shoulder
• Additional areas requiring stretching may be
  identified on individual examination
• Standing programes (in a standing frame, or power
  chair with stander) are recommended after walking
  becomes impossible
• Resting hand splints are appropriate for
  individuals with tight long-finger flexors

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Splints

• Night splints (ankle-foot orthoses/AFOs) can help
  control ankle contractures
• Should be custom-made, not off the shelf
• After loss of ambulation, daytime splints may be
  preferred
• Daytime splints not recommended for ambulant boys
• Long-leg splints (knee-ankle-foot-orthoses) may
  be useful at stage when walking is becoming very
  difficult or impossible
• Can help control joint tightness, prolong
  ambulation, and delay the onset of scoliosis

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Wheelchairs, seating and assistive equipment

• Early ambulatory phase
• Scooter, stroller, or wheelchair may be used for
  long distances to conserve strength
• Posture is important customisation of chair
  normally necessary
• With increased difficulty walking, provision of
  powered wheelchair is recommended
• This should be adapted/customised for comfort,
  posture and symmetry

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Wheelchairs, seating and assistive equipment (2)

• Arm strength becomes an issue over time
• PTs/OTs can recommend assistive devices to
  maintain independence (e.g. alternative
  computer/environmental control access)
• Proactive consideration of equipment allows
  timely provision
• Additional adaptations in late ambulatory and
  non-ambulatory stages may be needed to help with
  getting upstairs, transferring, eating/drinking,
  turning in bed, and bathing

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Recommendations for exercise

• Limited research on type, frequency, and
  intensity of exercise that is optimum for DMD
• High-resistance strength training and eccentric
  exercise are inappropriate across the lifespan
• Concerns about contraction-induced muscle-fibre
  injury
• To avoid disuse atrophy and other secondary
  complications of inactivity, all ambulatory and
  early non-ambulatory boys should participate in
  regular submaximal (gentle) functional
  strengthening/activity, including a combination
  of swimming-pool exercises and recreation-based
  exercises in the community

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Recommendations for exercise (2)

• Swimming may benefit aerobic conditioning and
  respiratory exercise highly recommended from
  early ambulatory to early non-ambulatory phases
  (can be continued as long as medically safe)
• Additional benefits may be provided by
  low-resistance strength training and optimisation
  of upper body function
• Significant muscle pain or myoclobinuria in 24h
  period after a specific activity is a sign of
  overexertion and contraction-induced injury. If
  this occurs, the activity should be modified

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Surgery Introduction

• No unequivocal situations where contracture
  surgery is invariably indicated
• May be appropriate in some scenarios if
  lower-limb contractures are present despite
  range-of-motion exercises and splinting
• Approach must be strictly individualised
• Ankles (and to a lesser extent, knees) are most
  amenable to surgical correction/subsequent
  bracing
• Hip responds poorly to surgery for fixed flexion
  contractures cannot be effectively braced.
  Surgical release/lengthening of iliopsoas and
  other hip flexors may further weaken them, and
  make the patient unable to walk even with
  contracture correction.
• In ambulant patients, hip deformity often
  self-correcting if knees/ankles straightened
• Various surgical options exist none can be
  recommended above any other.

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Surgery Early Ambulatory Phase

• Procedures for early contractures include
• Heel-cord (tendo-Achilles) lengthening for
  equinus contractures
• Hamstring tendon lengthening for knee-flexion
  contractures
• Anterior hip-muscle releases for hip-flexion
  contractures
• Some clinics recommend that procedures are done
  before contractures develop this approach is not
  widely practiced today

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Surgery Middle Ambulatory Phase (1)

• Interventions aim to prolong ambulation
  contracted joint can limit walking even if
  overall limb musculature has sufficient strength
• Some evidence suggests walking can be prolonged
  1-3 years by surgery
• Difficulty of objective assessment consensus
  difficult to achieve
• Prolonged ambulation due to steroid use has
  further increased uncertainty of value of
  corrective surgery
• Certain recommendations can be made irrespective
  of steroid status
• Muscle strength/range of motion around individual
  joints should be considered before deciding upon
  surgery

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Surgery Middle Ambulatory Phase (2)

• Approaches to lower-extremity surgery
• Bilateral multi-level (hip-knee-ankle/knee ankle)
  procedures
• Bilateral single-level (ankle) procedures
• Rarely, unilateral single-level (ankle)
  procedures for asymmetric involvement
• The surgeries involve tendon-lengthing, tendon
  transfer, tenotomy (cutting the tendon) along
  with release of fibrotic joint contractures
  (ankle) or removal of tight fibrous bands
  (iliotibial band at lateral thigh from hip to
  knee)

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Surgery Middle Ambulatory Phase (3)

• Single-level surgery (e.g. correction of ankle
  equinus deformity gt20) not indicated if there
  are knee flexion contractures of 10 or greater
  and quadriceps strength of grade 3/5 or less
• Equinus foot deformity (toe-walking) and varus
  foot deformities (severe inversion) can be
  corrected by heel-cord lengthening and tibialis
  posterior tendon transfer through the
  interosseous membrane onto the dorsolateral
  aspect of the foot to change plantar
  flexion-inversion activity of the tibialis
  posterior to dorsiflexion-eversion.
• Hamstring lengthening behind knee generally
  needed if knee-flexion contracture of more than
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• After tendon lengthening and tendon transfer,
  post-operative bracing may be needed, which
  should be discussed pre-operatively.
• Following tenotomy, bracing is always needed.

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Surgery Middle Ambulatory Phase (4)

• When surgery performed to maintain walking,
  patient must be mobilised using a walker or
  crutches on the first or second postoperative day
  to prevent further disuse atrophy of
  lower-extremity muscles.
• Post-surgery walking must continue throughout
  limb immobilisation and post-cast rehabilitation.
  
• An experienced team with close coordination
  between the orthopaedic surgeon, physical
  therapist, and orthotist is required.

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Surgery Late ambulatory early non-ambulatory
phases

• Late ambulatory
• Generally ineffective
• Obscures benefits of more timely interventions
• Early non-ambulatory
• Some clinics perform extensive lower-extremity
  surgery/bracing to regain ambulation within 3-6
  months of loss of walking ability
• This is generally ineffective not currently
  considered appropriate

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Surgery Late non-ambulatory phase

• Severe equinus foot deformities (gt30) can be
  corrected with heel-cord lengthening or tenotomy
• Varus deformities (if present) can be corrected
  with tibialis posterior tendon transfer,
  lengthening, or tenotomy.
• This is done for specific symptomatic problems
• Generally to alleviate pain/pressure
• Allow the patient to wear shoes
• Correctly place the feet on wheelchair footrests.
  
• This approach is not recommended as routine

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Pain Management

• Very little currently known about pain in DMD
• Patients should be asked whether pain is a
  problem, so it can be addressed/treated
• Appropriate intervention relies on determining
  cause of pain
• Pain often results from posture problems and
  difficulty getting comfortable. Interventions can
  include
• Provision of appropriate/individualised orthoses
• Standard drug treatment approaches (muscle
  relaxants, anti-inflammatory medications)
• Consider interactions with other medications
  (e.g. steroids, NSAIDS) and side-effects,
  especially those which might affect cardiac and
  respiratory function
• Rarely, orthopaedic intervention may be indicated
  for pain that cannot be managed in any other way,
  but which might respond to surgery
• Back pain, especially in steroid-treated
  patients, should prompt careful checking for
  vertebral fractures which respond well to
  bisphosphonate treatment.

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References Resources

• The Diagnosis and Management of Duchenne Muscular
  Dystrophy, Bushby K et al, Lancet Neurology 2010
  9 (1) 77-93 Lancet Neurology 2010 9 (2) 177-189
• Particularly references, p186-188
• The Diagnosis and Management of Duchenne Muscular
  Dystrophy A Guide for Families
• TREAT-NMD website www.treat-nmd.eu
• CARE-NMD website www.care-nmd.eu