Duchenne Muscular Dystrophy: Rehabilitation Management - PowerPoint PPT Presentation

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Duchenne Muscular Dystrophy: Rehabilitation Management

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Duchenne Muscular Dystrophy: Rehabilitation Management Introduction Different types of rehabilitation needed through life Delivered mainly by physiotherapists and ... – PowerPoint PPT presentation

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Title: Duchenne Muscular Dystrophy: Rehabilitation Management


1
Duchenne Muscular DystrophyRehabilitation
Management
2
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

3
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

4
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

5
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

6
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

7
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

8
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

9
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

10
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

11
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.

12
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

13
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

14
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)

15
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
    15
  • After tendon lengthening and tendon transfer,
    post-operative bracing may be needed, which
    should be discussed pre-operatively.
  • Following tenotomy, bracing is always needed.

16
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.

17
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

18
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

19
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.

20
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
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