Duchenne Muscular Dystroph

Progressive muscular weakness characterizes Duchenne muscular Dystrophy (DMD). In the second decade of life, lung function decreases to such an extent that spontaneous ventilation needs to be assisted. Nocturnal nasal intermittent positive pressure ventilation (NIPPV) is effective in providing 24 h normocapnia during several years but needs to be extended with disease progression when diurnal dyspnoea is present. Non-invasive interfaces may be used for continuous assisted ventilation. Some DMD patients however may require tracheostomy when bulbar function is severely altered. Other patients may undergo tracheostomy in the emergency room after acute respiratory insufficiency. Long-term intermittent positive pressure ventilation via tracheostomy is reported with good acceptance in 2/3 of the DMD patients. In spite of advantages (i.e. safety and possibility of endotracheal suction), tracheostomy also puts patients in danger of side effects such as excessive mucus production, tracheal stenosis, tracheobronchomalacia, bleeding, chronic bronchitis and bronchiectasis. Intensive Management of Chronic Bronchorrhea in a Tracheostomized Duchenne Patient with Bronchiectasis: Michel Toussaint

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Last date updated on September, 2024