Pulmonary function, functional capacity and quality of life in adults with cystic fibrosis.

BACKGROUND: As there are few studies on the impact of respiratory and functional status on the quality of life domains in adults with cystic fibrosis, this study aimed to evaluate the association between respiratory function, functional capacity and quality of life in these subjects. METHODS: This is a cross-sectional study, where adults with clinical and laboratorial diagnoses of CF fibrosis underwent pulmonary function tests, the six-minute walk distance test (6MWT) and responded to the Cystic Fibrosis Questionnaire-Revised (CFQ-R). Descriptive statistics was used to summarize the findings. The associations were tested by means of Pearson's or Spearman tests, and the significance level was set at 5%. RESULTS: The 21 patients who completed the study presented with reduced quality of life in all CFQ-R domains, obstructive pulmonary disease and reduced 6MWT distance. The following associations were found between pulmonary function and CFQ-R domains: forced vital capacity - FVC (%) and treatment burden and digestive symptoms (r=-0.433, p<0.05; r=-0.443, p<0.05, respectively), forced expiratory volume in one second - FVC ratio - FEV1/FVC (%) and physical functioning, social and respiratory symptoms (r=0.5, p<0.05; r=0.58, p<0.01; r=0.45, p<0.05, respectively), residual volume (%) and physical functioning (r=0.49, p<0.05), airways' resistance - Raw and physical functioning and emotional functioning (r=-0.44, p<0.05; r=-0,46, p<0.05, respectively), carbon monoxide diffusing capacity (%pred) and physical functioning (r=-0,51; p<0.05). CONCLUSION: Adults with CF have reduced quality of life, which in part is associated with the severity of their lung function.

A long-term pulmonary rehabilitation program progressively improves exercise tolerance, quality of life and cardiovascular risk factors in patients with COPD.

BACKGROUND: Support and treatment options have been widely discussed in recent decades with the aim of improving morbidity, mortality and quality of life of chronic respiratory disease (COPD) patients. Although it is believed that longer pulmonary rehabilitation programs can provide better results, most of the evidence comes from short-term programs. AIM: To determine the effects of an outpatient pulmonary rehabilitation program on exercise tolerance, dyspnoea, hemodynamic variables and quality of life. DESIGN: Case series study. SETTING: Rehabilitation Centre. POPULATION AND METHODS: A convenience sample of COPD patients was enrolled in this study. The intervention consisted of a 96-wk exercise training program, including aerobic training, upper-limb exercises and inspiratory muscle training. Pulmonary function tests, blood biochemistry, six-minute walking distance test and health-related quality of life were recorded at baseline and after completion of the 6th, 12th, 18th, 24th months. RESULTS: Forty one consecutive COPD patients were recruited and thirty six completed the study. There was a significant improvement in hemodynamics, demonstrated by the gradual reduction in heart rate, blood pressure and MvO2 (double product) starting from the 12th month. Lipid profile showed a reduction of low density lipids and an increase of the high density lipids levels starting from the 6th month. Exercise tolerance, dyspnoea, respiratory muscle strength and quality of life also improved starting from the 6th month. CONCLUSION: A 24-month pulmonary rehabilitation program leads to a progressive improvement in quality of life, dyspnoea and exercise tolerance, and reduces cardiovascular risk factors in patients with chronic obstructive pulmonary disease. IMPACT: Our study suggests that long-term pulmonary rehabilitation programs can result in further improvements in the aforementioned cardiorespiratory variables.