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Key Management: SMA

/ Resources by Condition / Intro to SMA / Key Management: SMA

Key Management of Spinal Muscular Atrophy

The main goals of physiotherapy in individuals with SMA are to maintain and improve motor function, maintain joint mobility, and prevent complications such as joint contractures and scoliosis. It is also important particularly in those with type 1 or type 2 SMA to manage any pulmonary symptoms.

  • Patients and their carers may require teaching of airway clearance techniques, lung volume recruitment bags and /or provision of cough assist machines. Respiratory care may include non-invasive ventilation secretion management and suction.
  • In non-ambulant individuals, early power chair provision is important, and they may be able to use a powered wheelchair from as young as 18 months. Encourage mobility on the floor using floor scooters for play in young children.
  • Standing frames may be provided from normal standing age. Gaiters and AFOs may also make standing possible. Light weight ischial weight bearing knee ankle foot orthosis or KAFOs may be considered for standing or assisted mobility, but they do need sufficient neck and  trunk strength.
  • In ambulant individuals, balance difficulties and falls do occur and can make travelling long distances more difficult. A lightweight self-propelling wheelchair may be useful to manage fatigue and energy. Some young people may prefer a mobility scooter. Power assist wheelchairs are also available.
  • Simple orthotics may aid foot position and ambulation such as heel cups or, supra malleolar foot ortho (SMAFOs)
  • Those with poor neck control may need neck support, particularly when travelling in a car. Headmaster collars can be useful.
  • Fatigue management is important to put in place for all levels of SMA.
  • In weak individuals who cannot maintain an upright trunk position without arm support, you may consider supplying spinal bracing to aid function.
  • Evidence suggests that exercise can help improve strength in SMA and practising tasks can also improve function particularly in this new environment of treated individuals. For children, exercise is play and sports and activity should be promoted.
  • For the weakest type of child, exercise can be performed in side-lying by taking the weight of the limb and working in a gravity free environment.
  • For all individuals, hydrotherapy and water can be a useful medium for improving stamina, improving respiratory performance and improving muscle function as well as stretching joints and muscles.
  • Specific muscle groups can be targeted for strengthening using theraband or light weights, however it is important that exercises are generally symmetrical and strengthening exercises should be combined with aerobic exercise.
  • An adapt trike or bike may be useful and horse riding has been shown to be beneficial.
  • Contractures are common in SMA but often present with a mixture of hyper mobility and tightness. It is certainly not necessary to stretch hyper mobile joints but important to review all joints on a regular basis especially in children and growing teenagers. The most commonly contracted joints in SMA are ankles, hips and knees and ITBS can get tight if they spend too much time in a frog-sitting position / externally rotated at the hips.  Supported seating can help with this issue.
  • It is important to manage posture and prevent spinal asymmetry. This is particularly true in children as they grow and if they’re spending a lot of time in a wheelchair. Ensuring that all positions used throughout the day promote a promote good spinal posture is key – 24-hour postural management.

Resources

This is a useful guide to home exercise in SMA and covers all ranges of ability. Tina Duong is an experienced Physical Therapist from Stanford, California, USA Exercise at home:

Step-in SMA offer several useful training modules including multidisciplinary care guidelines for spinal muscular atrophy and physical therapy and rehabilitation care guidelines for spinal muscular atrophy.

Leaflet: SMN managing fatigue

Considerations for Managing Rehabilitation in Adult Patients with SMA

MDA "At home physical therapy exercises for SMA"

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  • Who We Are
    • Our Team & Governance
    • Partnerships & Collaborations
  • Resources by Condition
    • Becker Muscular Dystrophy (BMD)
      • Intro to BMD
    • Congenital Muscular Dystrophy (CMD)
      • Intro to CMD
      • Key Management: CMD
      • Key Assessment Guide: CMD
    • Congenital Myasthenia Syndrome (CMS)
      • Intro to CMS
      • Key Management: CMS
      • Key Assessment Guide: CMS
    • Congenital Myopathies (CM)
      • Intro to CM
      • Key Management: CM
      • Key Assessment Guide: CM
    • Duchenne Muscular Dystrophy (DMD)
      • Intro to DMD
      • Key Management: DMD
      • Key Assessment Guide: DMD
    • General Neuromuscular Diseases (NMD)
      • Intro to NMD
      • Key Management: NMD
      • Key Assessment Guide: NMD
    • Glycogen Storage (GSD)
      • Intro to GSD
      • Key Management: GSD
      • Key Assessment Guide: GSD
    • Inherited Neuropathies
      • Intro to Inherited Neuropathies
      • Key Management: Inherited Neuropathies
      • Key Assessment Guide: Inherited Neuropathies
    • Limb Girdle Muscular Dystrophy (LGMD)
      • Intro to LGMD
      • Key Management: LGMD
      • Key Assessment Guide: LGMD
    • Myotonic Dystrophy (DM1)
      • Intro to DM1
      • Key Management of DM1
      • Key Assessment Guide: DM1
    • Spinal Muscular Atrophy (SMA)
      • Intro to SMA
      • Key Management: SMA
      • Key Assessment Guide: SMA
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  • Managing NMD
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