Difficult decannulation: impact of an individualized strategy on the survival of patients with neuromuscular diseases.

<bold>Background:</bold> Patients with neuromuscular diseases (NMDs) faced with respiratory failure, infectious processes and severe bulbar impairment are frequently intubated and tracheostomized in Intensive Care Units worldwide. For this patient profile, there is no defined proposal regarding weaning from mechanical ventilation and decannulation outside the Reference Centres. <bold>Objective:</bold> Evaluate the 10-year survival of patients with NMD who tolerate decannulation. <bold>Methodology:</bold> Retrospective study that included patients from 11/2013 to 10/2023 managed in an NMD Reference Center in Belo Horizonte/Brazil. The 33 decannulated patients followed an individualized NMD decannulation protocol, which was carried out based on non-invasive ventilation trials with the tracheostomy tube still in place, capped and the cuff deflated. Inspiratory pressure was titrated to optimize SaO2 ≥ 94% without supplemental oxygen. Patients were divided into three groups according to diagnosis: Amyotrophic lateral sclerosis (ALS), Duchenne Muscular Dystrophy (DMD), and other uncommon neuromuscular diseases (other NMDs). Survival estimates for each group (ALS, DMD and Other-NDMs) were expressed by Kaplan-Meier curves and compared using Log-Rank. <bold>Results:</bold> The median survival assessed by diagnosis for patients who succeed in the decannulation process was approximately 52 months for ALS patients, 64 months for DMD patients and 38 months for Other NMDs. <bold>Conclusion:</bold> All efforts must be undertaken in an individualized and safe protocol to decannulate patients with NMDs. A tracheostomy should be considered for the patient, their family members and the health professionals team who assist them in a shared decision.