Neurol. praxi. 2020;21(2):114-119 | DOI: 10.36290/neu.2020.058

Management of respiratory muscleweakness in advanced stages of Duchenne muscular dystrophy

doc. MUDr. David Kemlink, Ph.D.

Neurologická klinika a Centrum klinických neurověd, 1. LF UK a VFN v Praze

Duchenne muscular dystrophy is a progressive neuromuscular disorder inevitably leading to respiratory muscle weakness with subsequent impaired airway clearance and hypercapnic respiratory failure. These complications were the main cause of death in this patient population. The main intervention is early and regular assessments of respiratory muscle strength and adequate compensation of the impartment. Thanks to modern technical devices it is possible to prolong the survival of the patients with preserved quality of life relative to the state before respiratory failure. The main methods are airway clearance techniques including regular use of mechanical insuflator - exsuflator (cough assistance) and non-invasive ventilation. The latter in more advanced stages of the disease requires providing the patient with multiple interfaces including the mouth-piece (or straw), and advanced life - support ventilators providing also volume controlled modes.

Keywords: Duchenne muscle dystrophy, noninvasive ventilation, airway clearance techniques, mouth-piece ventilation.

Published: June 2, 2020  Show citation