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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 lowresistance 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 kneeflexion 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 lowerextremity 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 nonambulatory 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