2 Introduction Different types of rehabilitation needed through life Delivered mainly by physiotherapists and occupational therapists, but others may be involvedRehabiliation specialistsOrthotistsProviders of wheelchairs/other seating(Potentially) orthopaedic surgeonsKey: management of muscle extensibility and joint contracturesStretching aims to preserve function and maintain comfortProgramme should be monitored by PT, but must become part of the family’s daily routine
3 ContracturesFactors contributing towards tendency towards contractures:Muscles becoming less elastic due to limited use/positioningMuscles out of balance around the jointMaintaining good range of movement and symmetry is importantMaintains best possible functionPrevents development of fixed deformitiesPrevents pressure problems with the skin
4 Management of muscle extensibility and joint contractures Physiotherapist: key contact for contracture managementIdeally input from local PT supported by a specialist PT every 4 monthsStretching should be performed at least 4-6 times a week as part of family’s daily routineEffective 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 shoulderAdditional areas requiring stretching may be identified on individual examinationStanding programes (in a standing frame, or power chair with stander) are recommended after walking becomes impossibleResting hand splints are appropriate for individuals with tight long-finger flexors
6 SplintsNight splints (ankle-foot orthoses/AFOs) can help control ankle contracturesShould be custom-made, not “off the shelf”After loss of ambulation, daytime splints may be preferredDaytime splints not recommended for ambulant boysLong-leg splints (knee-ankle-foot-orthoses) may be useful at stage when walking is becoming very difficult or impossibleCan help control joint tightness, prolong ambulation, and delay the onset of scoliosis
7 Wheelchairs, seating and assistive equipment Early ambulatory phaseScooter, stroller, or wheelchair may be used for long distances to conserve strengthPosture is important: customisation of chair normally necessaryWith increased difficulty walking, provision of powered wheelchair is recommendedThis should be adapted/customised for comfort, posture and symmetry
8 Wheelchairs, seating and assistive equipment (2) Arm strength becomes an issue over timePTs/OTs can recommend assistive devices to maintain independence (e.g. alternative computer/environmental control access)Proactive consideration of equipment allows timely provisionAdditional 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 DMDHigh-resistance strength training and eccentric exercise are inappropriate across the lifespanConcerns about contraction-induced muscle-fibre injuryTo 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 functionSignificant 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 indicatedMay be appropriate in some scenarios if lower-limb contractures are present despite range-of-motion exercises and splintingApproach must be strictly individualisedAnkles (and to a lesser extent, knees) are most amenable to surgical correction/subsequent bracingHip 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 straightenedVarious surgical options exist: none can be recommended above any other.
12 Surgery: Early Ambulatory Phase Procedures for early contractures includeHeel-cord (tendo-Achilles) lengthening for equinus contracturesHamstring tendon lengthening for knee-flexion contracturesAnterior hip-muscle releases for hip-flexion contracturesSome 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 strengthSome evidence suggests walking can be prolonged 1-3 years by surgeryDifficulty of objective assessment: consensus difficult to achieveProlonged ambulation due to steroid use has further increased uncertainty of value of corrective surgeryCertain recommendations can be made irrespective of steroid statusMuscle strength/range of motion around individual joints should be considered before deciding upon surgery
14 Surgery: Middle Ambulatory Phase (2) Approaches to lower-extremity surgeryBilateral multi-level (hip-knee-ankle/knee ankle) proceduresBilateral single-level (ankle) proceduresRarely, unilateral single-level (ankle) procedures for asymmetric involvementThe 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 lessEquinus 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 ineffectiveObscures benefits of more timely interventionsEarly non-ambulatorySome clinics perform extensive lower-extremity surgery/bracing to regain ambulation within 3-6 months of loss of walking abilityThis 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 tenotomyVarus deformities (if present) can be corrected with tibialis posterior tendon transfer, lengthening, or tenotomy.This is done for specific symptomatic problemsGenerally to alleviate pain/pressureAllow the patient to wear shoesCorrectly 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/treatedAppropriate intervention relies on determining cause of painPain often results from posture problems and difficulty getting comfortable. Interventions can includeProvision of appropriate/individualised orthosesStandard 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 functionRarely, orthopaedic intervention may be indicated for pain that cannot be managed in any other way, but which might respond to surgeryBack 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, pThe Diagnosis and Management of Duchenne Muscular Dystrophy: A Guide for FamiliesTREAT-NMD website:CARE-NMD website: