Cough and airway clearance in Duchenne muscular dystrophy

Abstract

People with Duchenne muscular dystrophy (DMD), develop a respiratory muscle weakness that results in weakened cough, airway clearance impairment and over time respiratory failure and death. Assessment of cough effectiveness through vital capacity, peak cough flow and maximal inspiratory and expiratory pressures has been used to identify the optimal timing of cough augmentation techniques initiation. The choice of therapies depends on physician knowledge, and patient/care giver abilities. The purpose of this review is to clarify mechanisms of action, benefits and disadvantages of available techniques, such as manual cough-assisting manoeuvres, glossopharyngeal breathing, air stacking by resuscitator bag or by volume-cycle ventilator, and mechanical insufflator-exsufflator. Mechanisms of mucus mobilization, like intrapulmonary percussive ventilation, may have a therapeutic role in the case of persistent atelectasis. It is also crucial to recognize the initial phase of an acute respiratory exacerbation, increase the use of these techniques which may reduce morbidity and mortality.

Keywords: Air stacking; Cough; Duchenne muscular dystrophy; Mechanical insufflator–exsufflator; Neuromuscular disease; Peak cough flow.