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

Abstract

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. In the patient, the general mental health score increased from 16 to 48%, the vitality from 10 to 40% and the general health perception from 10 to 35%. In the patient’s father, the social functioning improved from 38 to 63%, the general mental health from 36 to 60% and the vitality from 35 to 60%. In this report, intensive respiratory therapy with IPV in combination with MI-E and chest compression was safe and spectacularly improved the quality of ventilation, the mucus production and the perceived mental health in an adult Duchenne patient with bronchorrhea.