Lung volume recruitment in patients with Duchenne Muscular Dystrophy in the early non-ambulatory stage

Abstract

Ventilatory and cough insufficiency are the main causes of morbidity and mortality in patients with Duchenne muscular dystrophy (DMD). Lung volume recruitment (LVR) might decrease the decline in vital capacity (VC).

Objective: To determine the changes produced by respiratory training performed at home with LVR techniques and manually assisted cough on VC, maximum insufflation capacity (MIC), and peak cough flow (PCF) in adolescents with DMD in the early non-ambulatory stage.

Patients and Method: Quasi-experimental study with repeated measurements every three months. 16 adolescents with DMD close to the CV plateau and cough insufficiency were evaluated and trained daily in LVR and assisted cough, with follow-up for 12 months, assessing respiratory function, quality of life, and caregiver overload.

Results: Eleven users (69%), aged 12.7 ± 2.8 years, completed the follow-up. Supine position VC and PCF + compression increased significantly (p = 0.047; p = 0.012, respectively). Seated VC and MIC tended to improve by 11.2% and 21.6%, respectively. Quality of life and caregiver overload did not present significant changes.

Conclusion: In adolescents with DMD in the early non-ambulatory stage, significant improvements were observed in VC and cough capacity. This provides an intervention of easy implementation, capable of preventing the expected respiratory functional decline.