Spinal curvature is a common concern, as it develops in more than 50% of individuals with SMA, most commonly in those who are nonambulatory. 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 spine curvature, pulmonary function, and bone maturity.1,2

POTENTIAL BENEFITS

CONSIDERATIONS

SURGERY

POTENTIAL BENEFITS

  • May improve sitting posture, balance, endurance, and cosmesis2
  • May slow respiratory decline2,3
  • May increase overall comfort, quality of life, and independence2

CONSIDERATIONS

  • May impact ability to perform lumbar puncture
  • Optimal timing of procedure is controversial3
  • Individuals with SMA may be at higher risk of surgical complications than the general population3

BRACING

POTENTIAL BENEFITS

  • May improve sitting balance, endurance, and overall physical appearance1
  • More conservative approach than surgery that allows for further growth1

CONSIDERATIONS

  • Likely does not prevent or delay development of scoliosis3
  • May cause some discomfort1
  • Lung function may be adversely affected by rigid bracing in children ≤8 years of age4
  • Expiratory lung volume may be lower with bracing4

Limb contractures are common in neuromuscular diseases such as SMA5

Contractures may occur in patients who have lost, or never achieved, the ability to ambulate. They contribute to reduced functional range of motion, loss of function for activities of daily living, and increased pain. One study found reductions in range of motion by >20° among 22% to 50% of patients with later-onset (consistent with Type II) SMA. Hip, knee, and wrist contractures were most common. Orthopaedic treatment often consists of range-of-motion exercises in order to prevent fixed joint contractures.6

Mobility-assistive technology

Power-assistive mobility devices may be considered for individuals with severe or progressive disease who suffer from fatigue and lack of endurance, allowing them to conserve energy and focus more on activities of daily living. Children with SMA who are as young as 20 months of age have demonstrated safe, independent use of powered wheelchairs.7,8

POTENTIAL BENEFITS

CONSIDERATIONS

POWERED WHEELCHAIR

POTENTIAL BENEFITS

  • Powered mobility may allow children to independently explore and interact with their environment, which may encourage development of communication, cognitive and social skills7,8
  • Seat-to-floor option may allow children to participate in floor activities7

CONSIDERATIONS

  • Young children may need constant supervision8
  • Seating, hand controls etc. may need to be configured to match patient’s physical needs8

STANDING WHEELCHAIR9

POTENTIAL BENEFITS

  • May relieve pressure on pelvic bones while sitting
  • May improve lung capacity
  • May reduce occurrence of gastrointestinal complications
  • May increase bladder emptying
  • May prevent reductions in bone mineral density (BMD)
  • May improve ability to perform activities of daily living

CONSIDERATIONS

  • Potential to overstretch contractures
  • Skeletal deformities may impede patient comfort
  • Lack of standing tolerance may limit use
  • Potential for fracture in severe BMD loss
  • Potential for causing postural hypotension

CONTROLLERS

POTENTIAL BENEFITS

  • Sensitivity can be programmed
  • Force-sensing joysticks
  • Sip-and-puff may be used by patients with breath control7

CONSIDERATIONS

  • Controllers may need to be periodically re-designed to compensate for progressive weakness10

References

1. Spinal Muscular Atrophy Clinical Research Center. Physical/occupational therapy. Available at:  http://columbiasma.org/pt-ot.html. Accessed November 2017. 2. Wang CH, et al. J Child Neurol. 2007;22:1027–49. 3. Mullender M, et al. Scoliosis. 2008;3:14. 4. Tangsrud SE, et al. Arch Dis Child. 2001;84:521–24. 5. Skalsky AJ, McDonald CM. Phys Med Rehabil Clin N Am. 2012;23:675–87. 6. Tsirikos AI, Baker ADL. Curr Orthoped. 2006;20:430–45. 7. Lin W, et al. Phys Med Rehabil Clin N Am. 2012;23:885–94. 8. Jones MA, et al. Phys Ther. 2003;83:253–62. 9. Arva J, et al. Assist Technol. 2009;21:161–8. 10. Hilliard K. Special Effect’s controllers enable every gamer to enjoy video games. Available at: http://www.gameinformer.com/b/features/archive/2014/06/25/creating-controllers-for-all-disabilities-special-effect-enables-every-gamer-to-enjoy-video-games.aspx. Game Informer website. Accessed November 2017.