Hemant Sawnani1,2, Oscar H Mayer3, Avani C Modi2,4, John E Pascoe1,2, Keith McConnell1, Joseph M McDonough3, Anne M Rutkowski5, Md Monir Hossain2,6, Rhonda Szczesniak2,6, Dawit G Tadesse6, Christine L Schuler2,7, Raouf Amin1,2. 1. Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. 2. Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio. 3. Division of Pulmonary Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. 4. Division of Behavioral Medicine and Clinical Psychology, Center for Treatment Adherence and Self-Management, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. 5. Cure CMD Foundation and Kaiser SCPMG, Los Angeles, California. 6. Division of Epidemiology and Biostatistics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. 7. Division of Hospital Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Abstract
OBJECTIVE: Respiratory compromise in congenital muscular dystrophy (CMD) occurs, in part, from chest wall contractures. Passive stretch with hyperinsufflation therapy could reduce related costo-vertebral joint contractures. We sought to examine the impact of hyperinsufflation use on lung function and quality of life in children with CMD. STUDY DESIGN: We conducted a randomized controlled trial on hyperinsufflation therapy in children with CMD at two centers. An individualized hyperinsufflation regimen of 15 minutes twice daily using a cough assist device over a 12 months period was prescribed. We measured lung function, quality of life, and adherence. To demonstrate reproducibility, pulmonary function was measured twice on the same day. A mixed-effects regression model adjusting for confounders was used to assess the effects of hyperinsufflation. RESULTS: We enrolled 34 participants in the study; 31 completed the trial (n = 17 treatment group and n = 14 controls). Participants in the treatment group demonstrated a relative gain in lung volume measured at 4 and 8 months, but not at 12 months. The control group required increases in the maximum insufflation pressures to achieve maximum lung volumes while the treatment group did not. Adherence was best early in the study, peaking at the first visit and decreasing at subsequent visits. Caregiver-reported quality of life was higher in the treatment group. CONCLUSION: Hyperinsufflation therapy is effective in increasing and sustaining lung volume over time. Adherence, however, was inconsistent and difficult to maintain. Further research should determine if improved adherence leads to sustained benefits of hyperinsufflation.
OBJECTIVE: Respiratory compromise in congenital muscular dystrophy (CMD) occurs, in part, from chest wall contractures. Passive stretch with hyperinsufflation therapy could reduce related costo-vertebral joint contractures. We sought to examine the impact of hyperinsufflation use on lung function and quality of life in children with CMD. STUDY DESIGN: We conducted a randomized controlled trial on hyperinsufflation therapy in children with CMD at two centers. An individualized hyperinsufflation regimen of 15 minutes twice daily using a cough assist device over a 12 months period was prescribed. We measured lung function, quality of life, and adherence. To demonstrate reproducibility, pulmonary function was measured twice on the same day. A mixed-effects regression model adjusting for confounders was used to assess the effects of hyperinsufflation. RESULTS: We enrolled 34 participants in the study; 31 completed the trial (n = 17 treatment group and n = 14 controls). Participants in the treatment group demonstrated a relative gain in lung volume measured at 4 and 8 months, but not at 12 months. The control group required increases in the maximum insufflation pressures to achieve maximum lung volumes while the treatment group did not. Adherence was best early in the study, peaking at the first visit and decreasing at subsequent visits. Caregiver-reported quality of life was higher in the treatment group. CONCLUSION: Hyperinsufflation therapy is effective in increasing and sustaining lung volume over time. Adherence, however, was inconsistent and difficult to maintain. Further research should determine if improved adherence leads to sustained benefits of hyperinsufflation.
Authors: A Nadeau; M Kinali; M Main; C Jimenez-Mallebrera; A Aloysius; E Clement; B North; A Y Manzur; S A Robb; E Mercuri; F Muntoni Journal: Neurology Date: 2009-07-07 Impact factor: 9.910