What are the International Standard of Care Committee treatment guidelines for respiratory complications in patients with congenital muscular dystrophy?

Updated: Aug 17, 2020
  • Author: Twee T Do, MD; Chief Editor: Jeffrey D Thomson, MD  more...
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Answer

The International Standard of Care Committee consensus recommendations include the following [76] :

  • Regularly scheduled physician visits and patient or family awareness of potential signs and symptoms are elements of a proactive approach to recognize early pulmonary problems prior to the onset of chronic respiratory compromise
  • Pneumococcal and influenza vaccines are suggested for any patient with CMD
  • Palivizumab, a humanized monoclonal antibody against respiratory syncytial virus, should be given to children under 2 years of age as prophylaxis
  • Spinal bracing is required to promote activities of daily living, ensure functional sitting posture, and delay the progression of scoliosis; this allows adequate thoracic growth until optimal timing for spinal surgery
  • Spirometry both in and out of the brace is recommended to evaluate the impact on respiratory function
  • Adjustment is needed between the degree of correction and the compression pressure on the thorax to avoid compromise of respiratory capacity

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