Spinal muscular atrophy (SMA): general treatments

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Spinal muscular atrophy (SMA): general treatments. Cough assist devices, Airway clearance: for impaired cough. Fully referenced neurology checklist.

Respiratory treatment

  • Cough assist devices
  • Airway clearance: for impaired cough
  • Non-invasive ventilation (NIV): with bilevel positive airway pressure (BiPAP)
  • Routine vaccinations to prevent chest infections

Nutritional management

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Measures to improve swallowing

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Measures to improve intestinal function

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Orthopaedic care

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Prevention of contractures

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Other treatment considerations

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References

  1. Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol 2007; 22:1027-1049.
  2. Burnett BG, Crawford TO, Sumner CJ. Emerging treatment options for spinal muscular atrophy. Curr Treat Options Neurol 2009; 11:90-101.
  3. Lorson CL, Rindt H, Shababi M. Spinal muscular atrophy: mechanisms and therapeutic strategies. Hum Mol Genet 2010; 19:R111-R118.
  4. Giovannetti AM, Pasanisi MB, Černiauskaitė M, Bussolino C, Leonardi M, Morandi L. Perceived efficacy of salbutamol by persons with spinal muscular atrophy: a mixed methods study. Muscle Nerve 2016; 54:843-849.
  5. Wijngaarde CA, Huisman A, Wadman RI. Abnormal coagulation parameters are a common non-neuromuscular feature in patients with spinal muscular atrophy. JNNP 2019 (Epub ahead of print).

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