1. Duchenne Muscular Dystrophy: Rehabilitation 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 family’s 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 family’s 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 >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.

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
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 (>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

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 (1) & Lancet Neurology (2)
Particularly references, p
The Diagnosis and Management of Duchenne Muscular Dystrophy: A Guide for Families
TREAT-NMD website:
CARE-NMD website: