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.

Related posts:

1. The effect of caregiving on women in families with Duchenne/Becker muscular dystrophy.
2. Preclinical Studies for Gene Therapy of Duchenne Muscular Dystrophy.
3. Change in Natural History of Duchenne Muscular Dystrophy With Long-term Corticosteroid Treatment: Implications for Management.
4. Impact of tracheostomy on swallowing performance in Duchenne muscular dystrophy.
5. [Limb-girdle muscular dystrophy type 2M with adult-onset loss of ambulation. A case report].

Tags: Muscular Dystrophy

This entry was posted on Sunday, July 25th, 2010 at 7:07 am and is filed under Muscular Dystrophy. You can follow any responses to this entry through the RSS 2.0 feed. You can skip to the end and leave a response. Pinging is currently not allowed.