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  1. Musculoskeletal Support and Interventions
  2. Nutritional Support and Interventions
  3. Common Nutritional Issues
  4. Respiratory Support and Interventions

Musculoskeletal support and interventions

Musculoskeletal issues in spinal muscular atrophy (SMA),
such as scoliosis, may require bracing or surgery

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Spinal curvature is a common concern, with scoliosis affecting 60-90% of children with SMA types 1 and 2 and initial presentation in early childhood. This may be addressed by surgical correction or positional support (e.g. bracing). The decision to perform surgical correction of complications such as scoliosis is based on the child’s spine curvature, pulmonary function, and bone maturity.1,2

POTENTIAL BENEFITS

CONSIDERATIONS

SURGERY

POTENTIAL BENEFITS

  • May help preserve balance when sitting and realign the distorted thorax2
  • May increase overall comfort, quality of life, and independence1

CONSIDERATIONS

  • May impact ability to perform lumbar puncture
  • Surgical treatment of spinal deformity should be delayed until after the age of 4 years2
  • Individuals with spinal muscular atrophy may be at higher risk of surgical complications than the general population3

BRACING

POTENTIAL BENEFITS

  • May improve sitting balance, endurance, and overall physical appearance3
  • More conservative approach than surgery that allows for further growth3

CONSIDERATIONS

  • Unable to prevent or delay development of scoliosis2,3
  • May cause some discomfort1
  • Lung function may be adversely affected by rigid bracing in children ≤8 years of age
  • Expiratory lung volume may be lower with bracing4
  • Thoracic bracing is recommended for children with a major curve Cobb angle >15-20 degrees2
Muscular Atrophy

The clinical spectrum of SMA is highly variable and requires comprehensive medical care involving multiple disciplines.2