Intensive Management of Chronic Bronchorrhea in a Tracheostomized Duchenne Patient with Bronchiectasis

Duchenne muscular dystrophy (DMD) is a severe X-linked hereditary neuromuscular disease characterized by progressive muscle degeneration and functional tetraplegia. Advances related to the use of mechanical ventilation and techniques of airway clearance have improved the lifespan of these patients until 30-35 years. In a 27 years old home-ventilated tracheostomized DMD patient affected by bronchiectasis and atelectasis with excessive bronchorrhea (>300ml mucus production/24h), endotracheal suctioning act became so frequent that it severely affected his mental health and that of his family. An intensive programme of airway clearance including ventilation via Intrapulmonary Percussive Ventilation (IPV) and suctioning via mechanical insufflation-exsufflation (MI-E) associated with strong manual chest compression improved mean nocturnal SpO2 from 91 to 95% and reduced mucus production and suctioning rate by 50%. The perceived physical and emotional health (SF-36) increased in the patient and in his family.