Sherri L Katz1,2,3, Nicholas Barrowman2,4, Andrea Monsour3, Santana Su2, Lynda Hoey4, Douglas McKim2,5,6. 1. 1 Division of Respiratory Medicine, Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada. 2. 2 Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada. 3. 3 Children's Hospital of Eastern Ontario Research Institute, Ottawa, Ontario, Canada. 4. 4 Clinical Research Unit, Children's Hospital of Eastern Ontario Research Institute, Ottawa, Ontario, Canada. 5. 5 Division of Respiratory Medicine, The Ottawa Hospital Rehabilitation Centre, Ottawa, Ontario, Canada; and. 6. 6 The Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.
Abstract
RATIONALE: Lung volume recruitment therapy slows rate of decline of lung function in neuromuscular disease, possibly due to enhanced airway clearance, reduced atelectasis, or prevention of chest wall contractures. OBJECTIVES: To determine if lung volume recruitment maintains maximal insufflation capacity (MIC), despite decline in VC. METHODS: This was a retrospective cohort study (1991-2008) of individuals with Duchenne muscular dystrophy at pediatric and adult tertiary centers. Lung volume recruitment was prescribed twice daily, according to protocol. Changes over time in MIC, VC percentage predicted, the difference between MIC and VC, maximum inspiratory and expiratory pressures, and assisted and unassisted peak cough flow (PCF) were assessed using linear mixed effects models. MEASUREMENTS AND MAIN RESULTS: Sixteen individuals, 8.6 to 33.0 years old at initiation of lung volume recruitment, with median VC percentage predicted of 13.5 (interquartile range, 8.0-20.3), were followed over a median of 6.1 years (range, 1.7-16.1 yr). MIC-VC differences were stable (change, 0.02 L/yr; P = 0.06). Post-lung volume recruitment, compared with pretreatment, rate of decline in VC decreased from 4.5% predicted/yr to 0.5% predicted/yr (P < 0.001). Maximal inspiratory and expiratory pressures were unchanged (P = 0.08, 0.59 respectively). Assisted-spontaneous PCF difference was maintained (slope, -1.59 L/min/yr, P = 0.35). CONCLUSIONS: With lung volume recruitment therapy, MIC-VC differences were stable over time, indicating that respiratory system compliance remains stable, despite a loss in VC, in individuals with Duchenne muscular dystrophy. Decline in VC was significantly attenuated, and assisted PCF was maintained in a clinically effective range.
RATIONALE: Lung volume recruitment therapy slows rate of decline of lung function in neuromuscular disease, possibly due to enhanced airway clearance, reduced atelectasis, or prevention of chest wall contractures. OBJECTIVES: To determine if lung volume recruitment maintains maximal insufflation capacity (MIC), despite decline in VC. METHODS: This was a retrospective cohort study (1991-2008) of individuals with Duchenne muscular dystrophy at pediatric and adult tertiary centers. Lung volume recruitment was prescribed twice daily, according to protocol. Changes over time in MIC, VC percentage predicted, the difference between MIC and VC, maximum inspiratory and expiratory pressures, and assisted and unassisted peak cough flow (PCF) were assessed using linear mixed effects models. MEASUREMENTS AND MAIN RESULTS: Sixteen individuals, 8.6 to 33.0 years old at initiation of lung volume recruitment, with median VC percentage predicted of 13.5 (interquartile range, 8.0-20.3), were followed over a median of 6.1 years (range, 1.7-16.1 yr). MIC-VC differences were stable (change, 0.02 L/yr; P = 0.06). Post-lung volume recruitment, compared with pretreatment, rate of decline in VC decreased from 4.5% predicted/yr to 0.5% predicted/yr (P < 0.001). Maximal inspiratory and expiratory pressures were unchanged (P = 0.08, 0.59 respectively). Assisted-spontaneous PCF difference was maintained (slope, -1.59 L/min/yr, P = 0.35). CONCLUSIONS: With lung volume recruitment therapy, MIC-VC differences were stable over time, indicating that respiratory system compliance remains stable, despite a loss in VC, in individuals with Duchenne muscular dystrophy. Decline in VC was significantly attenuated, and assisted PCF was maintained in a clinically effective range.
Entities:
Keywords:
VC; cough; neuromuscular diseases; pulmonary function test; respiratory therapy
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