Mechanical Ventilation (MV) in patients with Duchenne Muscular Dystrophy (DMD) in our area

Introduction: DMD affects 1/3500 males. The most frequent cause of death is ventilatory failure.90% of patients who do not use MV die before 20 years. Aim: To describe the characteristics of patients with DMD treated in our hospital in the last 20 years Methods: Retrospective study. We collected:age, cardiopathy, date of death, use of MV and starting age, elective or after an exacerbation, ventilatory mode, use of Cough Assist, gastrostomy and scoliosis surgery. Functional tests and polygraphy previous starting respiratory theraphy were analyzed. Results: 19 DMD males.57,8% has MV and is controlled by Pulmonologists.52,3% require Cough Assist and 10,5% gastrostomy. Cardiac affectation in 57,8% and 36,8% has scoliosis surgery. During this time 5 patients died at the age of 26 +/- 6,52 years. MV was initiated in 11 patients at 19y(15-25) and mean time with MV was 6 y(1-9).90.9% non-invasive/1 Invasive.45.4% MV was initiated acutely during an admission. Characteristics of MV are shown in Table1. A combined Pediatric-Pneumology assestment has been started since 2017. Conclusions: DMD is a rare disease in our area.57,8% of patients require MV and Cough assist and are controlled by pulmonologists Mainly MV starts in the adolescence and in 45,4% of cases after an acute process. Recently, a process coordination between Pediatrics and Pulmonologists has begun to improve the care of these patients.