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Duchenne Muscular Dystrophy: The Effect of Glucocorticoids on Ventilator Use and Ambulation

Bach JR, Martinez D, Saulat B: Duchenne muscular dystrophy: The effect of glucocorticoids on ventilator use and ambulation. Objective:To describe the effect of glucocorticoid treatment on age at wheelchair dependence and at dependence on part-time (<23 hrs/day) and continuous noninvasive mechanical ventilation. Design:In this retrospective study, patients with Duchenne muscular dystrophy who received glucocorticoid therapy were compared with those who did not for ages at wheelchair dependence and when beginning part-time (nocturnal) and continuous noninvasive intermittent positive pressure ventilation (NIV). Respiratory symptoms, end-tidal carbon dioxide, oximetry, and vital capacity were noted every 4-12 mos, and NIV was initiated for symptomatic nocturnal hypoventilation. The daily NIV use increased until some required it continuously to survive. Results:The 117 untreated patients became wheelchair-dependent at 9.7 ± 1.3 yrs of age. Four then died from cardiac failure, and five were older than 19 yrs without using NIV. The remaining 108 began nocturnal NIV at 19.2 ± 3.7 yrs of age. Ninety of the 108 became continuously NIV-dependent at 21.9 ± 4.5 yrs of age, and the 17 treated with glucocorticoid therapy became wheelchair-dependent significantly later at 10.8 ± 1.3 yrs of age (P = 0.02). Three died from cardiac failure, and three were older than 19 yrs without using NIV. The remaining 11 began nocturnal NIV at 22.9 ± 5.3 yrs of age (P = 0.05). Eight of the 11 became continuously NIV-dependent at age 28.9 ± 7.3 yrs (P = 0.005). Conclusions:Intermittent glucocorticoid therapy delays wheelchair dependence and may delay ventilator dependence for patients with Duchenne muscular dystrophy.