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Spinal Muscular Atrophy

Spinal Muscular Atrophy. Occupational and Physical Therapy Jo Anne Maczulski, MA, OTR/L Kristin Krosschell, PT, MA, PCS. What is SMA?. Loss of function of spinal motor neurons causing progressive weakness of muscles Muscles include extremities, respiratory muscles Sensation is normal

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Spinal Muscular Atrophy

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  1. Spinal Muscular Atrophy Occupational and Physical Therapy Jo Anne Maczulski, MA, OTR/L Kristin Krosschell, PT, MA, PCS

  2. What is SMA? • Loss of function of spinal motor neurons causing progressive weakness of muscles • Muscles include extremities, respiratory muscles • Sensation is normal • Brain function is normal

  3. What is the lower motor neuron?

  4. Therapeutic Intervention • Physical therapy • Occupational therapy • Bracing/ Orthotics

  5. PT • Gross motor development • Maintain flexibility, ROM and strength of the LE’s and minimize contractures • Upright weightbearing • Mobility/ Ambulation

  6. OT • UE/hand function • Maintain flexibility, ROM and strength of the UE’s/hands and minimize contractures • Perceptual motor development and experiences • Social emotional growth and development

  7. Child’s occupation • Importance of play • Importance of therapeutic activities

  8. Coordinate with other disciplines “Best care” Important to coordinate all care with others managing child’s care(therapists, doctors, teachers, caretakers)

  9. Therapy Intervention • Respiratory • Maintain flexibility and avoid contractures • Facilitate sensorimotor development and exploration • Innovations for play and positioning • Strengthening/ Exercise/ Fitness • Energy conservation • Mobility

  10. Therapy Intervention • Maximize a child’s function • A player and a learner • Promote and facilitate independence in functional activities • Sensorimotor experiences

  11. Developmental therapy • Maximize function • Optimize play • Facilitate mobility

  12. Developmental Handling • To promote movement • Facilitate skill development • Exploration of environment • Movement control • Movement experience and comfort with movement

  13. OT/PT- Developmental handling

  14. Therapy (OT, PT)

  15. Promote and facilitate independence in functional activities • Expectations for independence • Self care activities • Interaction within the community • School • Adaptive equipment • Positioning

  16. Occupational Therapy

  17. Maximize flexibility, ROM, strength of UE and hands and avoid contractures • Maintain strength and ROM of trunk, UE’s and hands as able • Assess functional abilities as related to strength • Develop compensatory strategies that maximize the child’s physical abilities • Watch for fatigue and overuse, endurance • Splinting for UE’s

  18. Facilitate sensori-motor development and exploration • Play • Body awareness, vestibular and tactile input and experiences • Developmental postures and activities • Adaptive positioning and innovations for play • Toys • Types and controls • Environmental accessability • Environmental controls/ computers

  19. Sensorimotor experiences • Movement experiences • Body awareness • Motor planning • Motor control

  20. Sensorimotor development and exploration

  21. Social-emotional growth and development • Behavior • Motivation

  22. UE/ Hand function • Fine motor development • Bilateral coordination • Shoulder girdle integrity • Splinting • Positioning

  23. Hands and splints

  24. Slings and springs

  25. Play and positioning

  26. Play and exploration

  27. Perceptual motor development and experiences • Importance • Perceptual motor development

  28. Adaptive equipment • Facilitate independence • Play • School • Resources

  29. Play and adaptations

  30. Adaptive toys

  31. Adaptive switches/ toys

  32. Switches for environmental control

  33. Assistive technology • Evaluation • Resources

  34. Physical Therapy

  35. Respiratory • Respiratory play • Postural drainage • Breathing exercises • Positioning for improved respiration • TLSO, Benek’s vest, Abdominal binder

  36. Respiratory

  37. Maintain mobility and avoid contractures • Prevent deformity • Range of motion exercises • Stretching to preserve or increase flexibility • Positioning • Splinting • Infancy throughout lifespan • Be Pro-active

  38. Passive stretching? Florence, Glanzman, Krosschell, Maczulski, Main, Morrison 2006, Wang's Journal of SMA Care

  39. Gross Motor Development • Stability • Mobility • Skill acquisition • Compensatory strategies

  40. Strengthening/ Exercise/ Fitness • Maintain strength as able • Assess functional abilities as related to strength • Train to use to maximal advantage • Watch for fatigue and overuse • Strengthening does not change progression • Too much can cause overfatigue

  41. Strengthening/ Exercise/ Fitness

  42. Maintain flexibility

  43. Strengthening, exercise and fitness

  44. PT

  45. PT

  46. Energy conservation • Do not overwork areas of increased weakness- may lead to overuse syndromes, pain and increased weakness • Scheduling to optimize energy and strength

  47. Upright weightbearing • Physiologic advantages • Prevention of contractures • Decrease osteoporosis • Prevention of UTI’s • Increase cardiopulmonary tolerance • Development of head and trunk control • Strengthening

  48. Upright weightbearing • Quality of life enhanced • Increased self esteem • Participation in group activities with greater independence • Positioning for upper extremity use

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