Respiratory care in Duchenne muscular dystrophy.

Respiratory complications are amajor cause of morbidity and mortality in Duchenne muscular dystrophy (DMD). Progressive weakness of inspiratory and expiratory muscles leads to ineffective cough, mucus retention, sleep-disordered breathing, nocturnal hypoventilation, and ultimately chronic respiratory failure. Before the advent of mechanical ventilation, respiratory failure was the leading cause of early death. Advances in multidisciplinary care, including noninvasive ventilation (NIV), airway clearance techniques, corticosteroid therapy, scoliosis management, and cardioprotective treatments, have markedly improved survival and quality of life. Respiratory decline typically follows loss of ambulation, reflecting the progressive loss of muscle strength. Hypoventilation first appears during REM sleep and evolves into daytime hypercapnia. Regular monitoring-annually before and biannually after loss of ambulation-is essential. Key respiratory monitoring parameters include decreased vital capacity, maximal inspiratory and expiratory pressures, and peak cough flow. Sleep studies are critical for early detection of nocturnal hypoventilation and sleep-disordered breathing. NIV remains the cornerstone of therapy, improving gas exchange, sleep quality, and life expectancy. As weakness increases, patients may become ventilator dependent; invasive ventilation is reserved for NIV failure. Adjunctive strategies such as lung volume recruitment and mechanical insufflation-exsufflation enhance cough efficacy and secretion clearance. Long-term corticosteroid therapy delays respiratory decline, while scoliosis correction and infection prevention further optimize outcomes. Nutritional management also supports respiratory health. Overall, early detection, individualized ventilatory management, and comprehensive multidisciplinary care are critical to improving survival and quality of life in patients with DMD.

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