Occupational and Physical Therapy

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Spinal Muscular Atrophy

• Occupational and Physical Therapy
• Jo Anne Maczulski, MA, OTR/L
• Kristin Krosschell, PT, MA, PCS

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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

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What is the lower motor neuron?
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Therapeutic Intervention

• Physical therapy
• Occupational therapy
• Bracing/ Orthotics

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PT

• Gross motor development
• Maintain flexibility, ROM and strength of the
  LEs and minimize contractures
  
• Upright weightbearing
• Mobility/ Ambulation

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OT

• UE/hand function
• Maintain flexibility, ROM and strength of the
  UEs/hands and minimize contractures
  
• Perceptual motor development and experiences
• Social emotional growth and development

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Childs occupation

• Importance of play
• Importance of therapeutic activities

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Coordinate with other disciplines

• Best care
• Important to coordinate all care with others
  managing childs care(therapists, doctors,
  teachers, caretakers)
  

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Therapy Intervention

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

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Therapy Intervention

• Maximize a childs function
• A player and a learner
• Promote and facilitate independence in functional
  activities
  
• Sensorimotor experiences

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Developmental therapy

• Maximize function
• Optimize play
• Facilitate mobility

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Developmental Handling

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

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OT/PT- Developmental handling
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Therapy (OT, PT)
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Promote and facilitate independence in functional
activities

• Expectations for independence
• Self care activities
• Interaction within the community
• School
• Adaptive equipment
• Positioning

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• Occupational Therapy

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Maximize flexibility, ROM, strength of UE and
hands and avoid contractures

• Maintain strength and ROM of trunk, UEs and
  hands as able
  
• Assess functional abilities as related to
  strength
  
• Develop compensatory strategies that maximize the
  childs physical abilities
  
• Watch for fatigue and overuse, endurance
• Splinting for UEs

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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

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Sensorimotor experiences

• Movement experiences
• Body awareness
• Motor planning
• Motor control

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Sensorimotor development and exploration
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Social-emotional growth and development

• Behavior
• Motivation

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UE/ Hand function

• Fine motor development
• Bilateral coordination
• Shoulder girdle integrity
• Splinting
• Positioning

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Hands and splints
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Slings and springs
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Play and positioning
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Play and exploration
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Perceptual motor development and experiences

• Importance
• Perceptual motor development

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Adaptive equipment

• Facilitate independence
• Play
• School
• Resources

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Play and adaptations
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Adaptive toys
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Adaptive switches/ toys
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Switches for environmental control
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Assistive technology

• Evaluation
• Resources

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• Physical Therapy

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Respiratory

• Respiratory play
• Postural drainage
• Breathing exercises
• Positioning for improved respiration
• TLSO, Beneks vest, Abdominal binder

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Respiratory
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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

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Passive stretching?
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Gross Motor Development

• Stability
• Mobility
• Skill acquisition
• Compensatory strategies

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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

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Strengthening/ Exercise/ Fitness
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Maintain flexibility
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Strengthening, exercise and fitness
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PT
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PT
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Energy conservation

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

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Upright weightbearing

• Physiologic advantages
• Prevention of contractures
• Decrease osteoporosis
• Prevention of UTIs
• Increase cardiopulmonary tolerance
• Development of head and trunk control
• Strengthening

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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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Upright weightbearing

• Positioning/ Alignment
• A-frame/ Parapodium/ Supine/Prone standers
• A start towards ambulation
• Adaptations for control and alignment in
  standing
  
• Critical time periods
• 12-24 months
• Post surgery

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Monitor equipment needs

• Braces and splints
• Assistive devices
• Walkers and crutches
• Standers/ Upright Positioners
• Wheeled mobility
• Strollers
• Wheelchairs/ Scooters
• Stand and Gos

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Ambulation

• Pros and cons
• When and how
• Training and practice
• Effects of strength
• Changes with TLSO

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Bracing for standing and ambulation

• AFOs
• KAFOs
• Ischial weightbearing KAFOs or LLBs
• HKAFOs
• RGO
• Parapodium
• Adaptations to aid in ease of donning and doffing

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Braces for standing and ambulation
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Bracing and assistive devices for
standing/ambulation
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Learning to walk with KAFOs
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Gait training
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Standers
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Mobile standers
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Standers
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Adaptive equipment at home
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Bathing and toileting
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Car seats
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Lifts and ramps
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Wheeled mobility

• Positioning
• When to correct alignment and when to accommodate
  alignment
  
• Adaptive seating
• Carriers for medical equipment
• Safe mobility and transportation

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Wheelchairs

• Manual or power
• Options
• Elevating legrests, swing away legrests
• Adjustable height arms
• Seating and support
• Headrest
• Tilt in space and/ or recline systems
• Power controls- placement and types
• Seat belt or harness
• Tray- to assist in ADL and for UE support

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Wheelchairs and strollers
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Recreational intervention

• WC sports
• Hippotherapy
• Adaptive swimming
• Modified bowling
• Summer camp programs
• FUN- of utmost importance

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Windsurfing
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Hippotherapy
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Skiing
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Canoeing at camp
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Companion canines
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Thank you!!