What are the AAN/AANEM 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 AAN/AANEM guidelines recommend the following [77] :

  • Inform families of patients with CMD that respiratory insufficiency and associated problems may be inconspicuous at the outset (level B)
  • Monitor pulmonary function tests such as spirometry and oxygen saturation in the awake and sleep states of patients, with monitoring levels individualized on the basis of the child's clinical status (level B)
  • Refer to pulmonary or aerodigestive care teams, when available, that are experienced in managing the interface between oropharyngeal function, gastric reflux and dysmotility, and nutrition and respiratory systems, and can provide guidance concerning trajectory, assessment modalities, complications, and potential interventions (level B)

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