American College of Chest Physicians Consensus Statement on the Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy Undergoing Anesthesia or Sedation

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

“…155 Preoperative training in and postoperative use of non-invasive ventilation is strongly recommended for patients with a baseline forced vital capacity of below 50% predicted and necessary with a forced vital capacity of below 30% predicted. 155 Incentive spirometry is not indicated owing to potential lack of efficacy in patients with respiratorymuscle weakness and the availability of preferred alternatives, such as mechanical insufflation-exsufflation.…”

“…155 Preoperative training in and postoperative use of non-invasive ventilation is strongly recommended for patients with a baseline forced vital capacity of below 50% predicted and necessary with a forced vital capacity of below 30% predicted. 155 Incentive spirometry is not indicated owing to potential lack of efficacy in patients with respiratorymuscle weakness and the availability of preferred alternatives, such as mechanical insufflation-exsufflation. After careful consideration of the risks and benefits, patients with significant respiratory-muscle weakness might be eligible for surgery, albeit with increased risk, if these patients are highly skilled preoperatively in the use of non-invasive ventilation and assisted cough.…”

Airway Clearance in Duchenne Muscular Dystrophy

“…For example, a forced vital capacity of Ͻ40% to 50% of predicted or Ͻ1 L are associated with nocturnal hypoventilation and the need for noninvasive ventilation, independent of clinical symptoms. 1,[19][20][21] The use of such support heralds increased risk for diminished airway clearance and the need to augment the patient's weakened cough.…”

“…Hypoxia, however, suggests the presence of poor airway clearance and/or hypoventilation with secondary ventilation-perfusion mismatch. 6,18,20 Thus, a patient who is clinically well with new-onset daytime hypoxia might require assisted ventilation because of hypoventilation, whereas a patient with new-onset hypoxia who is acutely ill with a viral process more likely has a diminished ability to clear airway secretions and would benefit by cough-assisting maneuvers. 7 Similarly, measurement of CO 2 levels via capnography can aid in the assessment of overall respiratory muscle function.…”

“…8,12,16,20 CPFs are performed by using a standard peak flow meter with the attachment of a full face mask at the mouthpiece. 18 The patient then coughs into the mask.…”