Duchenne Muscular Dystrophy:

Rehabilitation Management
 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 family’s daily routine
 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
 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 family’s daily routine
• Effective stretching may require a range of
techniques including stretching, splinting, and
standing devices
 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
 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
 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
 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
 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
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
 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.
 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
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
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)
Surgery: Middle Ambulatory Phase (3)
• Single-level surgery (e.g. correction of ankle equinus deformity
>20°) 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.
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.
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
 Surgery: Late non-ambulatory phase
• Severe equinus foot deformities (>30°) 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
 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.
 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