Duchenne’s Muscular Dystrophy: A Multidisciplinary Approach. By: Brittany Annis, Student PT Ithaca College 2010. Objectives. Introduce background information on Duchenne’s Muscular Dystrophy Identify associated pathologies
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By: Brittany Annis, Student PT
Ithaca College 2010
Contractured Tensor Fascia Latae:
Tightened IT Band
Resultant hip and knee pain/deformities
Ankle plantarflexion and supination
Tight gastrocnemius mm/Achilles tendon
Associated weakness of tibialis anterior and peroneal mms
Difficulty wearing shoes
Concern about foot’s appearanceAssociated Pathologies: Joint Contractures
Can alter the natural progression of the disease and improve function and quality of life.
Comprehensive set of recommendations to manage the wide spectrum of complications
Gastroenterology/ NutritionThe Multidisciplinary Team
Stretching/Positioning function and quality of life.
Management of contractures
Objective measures and testing to monitor progressionPhysical Therapy Interventions
Strength testing function and quality of life.- MMT to monitor disease progression and predict functional losses, assess responses to treatment and monitor muscle imbalances
Test LEs every 6 months when ambulatory; test UE and LE every 6 months when non-ambulatory
ROM: goniometry to identify hypomobility, jt contractures that may contribute to functional deterioration or to musculoskeletal/integumentary complications, or to note need of splinting/orthotics.
In ambulatory phase, measure hips, knees, ankles (ITB, H/S, heelcords). In non-ambulatory phase, measure UE as well as LE (elbows, wrists, finger flexors)Objective Measures
Timed Testing function and quality of life.: Standardized tests are responsive to change, easy to administer, relevant to function (when ambulatory only)
Timed 10 meter walk, timed Gower’s manuever, time to climb 4 stairs, 6 min walk test
ADLs: Assessment of impairment in home, school, community settings
Frequency of falls, self-care skillsObjective Measures, Con’t
Combination of AROM, AAROM, PROM, prolonged elongation (splinting, positioning)
Minimum of 4-6 days per week, at home/school and in the clinic
Heelcords: Gastrocnemius/ Achilles Tendon lengthening into dorsiflexion
Tensor Fascia Latae/Iliotibial Band: stretch hip into adduction, internal rotation, and extension.
Other muscles throughout the hips, knees and ankles
In non-ambulatory phase, also focus on upper extremities: finger flexors, wrist flexors, elbow and shoulder jointsStretching
Wheelchair: Need appropriate postural positioning to prevent scoliosis and back pain/aches
Powered for more involved patients.
Manual Lightweight for less involved patients so can self propel and increase independence, as well as provide arm exercise
Standing Frames: A few hours per day, even with minimal weight bearing, to prevent and reduce the severity of contractures, decubitis ulcers, and scoliosis.
Also improves bone mineral density, circulation, and GI and respiratory functions.Assistive Devices Con’t
Type I muscle fibers are slow twitch for slower contractions, and fatigue-resistant, so good for continuous contractions (postural muscles)
Type II muscle fibers are fast twitch for quick contractions, and are easily fatigued (“sprinter” muscles). They are also less resistant to mechanical stresses (more easily injured)
It is possible to switch Type II muscle fibers to Type I with exercise training, so with an increased number of Type I fibers, the muscles are more durable and more resistant to degeneration.
But the form of exercise needed to switch Type II fibers to Type I is very specificType I vs Type II Fibers
In a mouse study, a greater percentage of Type I muscle fibers were observed after low intensity, long-term exercise
Sedentary mice with DMD had a higher percentage of Type I fibers than sedentary mice without DMD
It has been observed that Type II muscle fibers are subject to greater degeneration, and the majority of research using animal models and some human studies suggest that increasing activity may actually slow the degeneration in dystrophic muscle.Current Evidence Regarding Exercise
Low resistive and aerobic exercise are justified to: fibers were observed after low intensity, long-term exercise
Prevent deconditioning, decreased fitness, disuse atrophy and joint contractures
Counteract secondary complications of inactivity such as obesity, Diabetes, osteoporosis and cardiovascular diseases
Long term, low-intensity, preferably no load (or low-load) weight-bearing activity to reduce mechanical stress on the muscle
Increased Type I fibers, which are less vulnerable to degeneration
Will not promote hypertrophyRecommended Exercise for DMD
Use caution to ensure that cardiovascular or muscular complications do not develop
High-resistance and eccentric exercise is NOT recommended, although it has not actually been proven harmful for a DMD patient
Aquatic Therapy! Excellent for improving/ maintaining mobility, strength, flexibility, and aerobic conditioning and cardiopulmonary fitnessRecommended Exercise for DMD, Con’t
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