efficacy of noninvasive positive pressure ventilation for improving the respiratory function, use of accessory respiratory muscles, quality of sleep and nutrition of cystic fibrosis patients

Background: respiratory failure secondary to recurrent respiratory infections is the most common cause of death in cystic fibrosis (cf). objectives: to assess the efficacy of noninvasive positive pressure ventilation (nippv) on respiratory function, use of accessory respiratory muscles, ease of physiotherapy for respiratory clearance, quality of sleep and nutritional status in cf patients with respiratory failure. methods: all cf patients admitted to the pediatric department of masih daneshvari hospital, tehran from march 2015 to january 2016 were studied. the inclusion criteria were clinical evidence of respiratory distress and signs of respiratory acidosis. the exclusion criteria were nausea and vomiting, not tolerating nippv, need for intubation, pneumothorax, presence of giant bullous emphysema or decreased level of consciousness. baseline spirometry and venous blood gas (vbg) was obtained before and after nippv. a simple questionnaire was filled out for the nutritional status, sleep quality, ease of physiotherapy for airway clearance and the volume of secretions after nippv compared to baseline. paired samples t-test and wilcoxon signed rank test in spss version 16 were used for the before and after comparison of numerical and ordinal variables, respectively. results: out of a total of 53 cf hospitalized patients 17 met the inclusion criteria. there were 10 (58.8%) males and 7 (41.1%) females with a mean age of 11 ± 4.57 (10 - 25) years, mean weight of 31.85 ± 10.11 (13 - 48) kg and mean bmi of 14.16 ± 2.71 (5.9 - 18). the mean saturation of peripheral oxygen (spo2 ), respiratory rate (rr) and mean partial pressure of co2 (pco2 ) of patients before the intervention were 75 ± 13.25% (52 - 90%), 32 ± 11.17 (22 - 55) mmhg and 55.20 ± 17.62 (30.3 - 108), mmhg respectively. a significant difference was noted after the intervention in spo2 , venous pco2 and rr compared to baseline (p < 0.05). the change in sleep quality (p = 0.001), nutritional status (p = 0.001) and ease of physiotherapy for respiratory clearance (p = 0.008) after nippv was statistically significant while the change in use of accessory respiratory muscles (p = 0.785) and the volume of secretions (p = 0.1) was not significant. conclusions: nippv in cf patients with respiratory failure can improve the respiratory function, sleep quality and nutritional status of patients. also, nippv enhances airway clearance during respiratory physiotherapy.