Indicators of Need for Mechanical Ventilation in Duchenne Muscular Dystrophy and Spinal Muscular Atrophy

Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients

“…R espiratory insufficiency appears in the course of Duchenne muscular dystrophy (DMD), first during sleep [1] and then at a later stage during the day [2]. With progressive respiratory failure, ventilation needs to be assisted.…”

Pediatric home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline executive summary

“…Several studies of adolescents and young adults with DMD and other Neuromuscular Disorders have demonstrated that low vital capacities of < 35-40% are very sensitive for continuous nocturnal hypoventilation and the need of nocturnal ventilation, however patients may be quite symptomatic and may have daytime hypercapnia. [38][39][40] However in at least one other study, patients with DMD already had daytime hypercapnia with an FVC in the 40-50% predicted range. 41 In more recent studies in mixed groups of adults with neuromuscular myopathies, an FVC < 60% was approximately 90% sensitive and specific for the onset of sleep disordered hypopneas (REM related hypopneas) whereas an FVC < 40% was 94% sensitive (79% specific) for continuous nocturnal hypoventilation.…”

“…6 Finally, management of associated comorbidities of the underlying condition that contribute to impaired airway clearance need to be addressed, including obesity, aspiration of oral secretions and/or food, as well as gastroesophageal reflux. 6 For individuals with artificial airways, 39,40 suctioning via catheter is a method to remove secretions from the large airways. The mechanical in-exsufflator can be used to clear secretions from the peripheral airways.…”

“…Additional adjunctive treatments to enhance airway clearance in this population include increasing patient mobility and repositioning, 39,41 and the use of heated humidity. 39,40,42 Tracheostomized patients also require adequate fluid intake to avoid dehydration, which can result in inspissated secretions. Uncuffed tracheostomy tubes are preferred over cuffed tubes if ventilation can be provided adequately.…”

Elaboração e análise de confiabilidade de escala de avaliação funcional do subir e descer escada para portadores de distrofia muscular de Duchenne (DMD)

“…Essa escala mostrou grande correlação com medidas de força muscular periférica, intensidade das contraturas musculares, anos de uso de cadeira de rodas e capacidade vital forçada (Hapke, 1972). Além disso, também tem sido sugerido um papel auxiliar para essa escala, na tomada de decisão do melhor momento para introdução de ventilação mecânica nesses pacientes (Lyager et al,1995).…”