Quality of life in patients awaiting lung transplant: cystic fibrosis versus other end-stage lung diseases.
ABSTRACT The symptoms associated with chronic lung disease can impair quality of life and psychosocial functioning. The purpose of the present study was to provide a thorough baseline assessment of quality of life in patients with end-stage lung disease and being evaluated for transplant; and to assess potential differences in quality of life between patients with cystic fibrosis (CF) and those with other types of end-stage lung disease (e.g., chronic obstructive pulmonary disease (COPD), interstitial pulmonary fibrosis (IPF)). We evaluated 58 patients with CF and 52 patients with other types of end-stage lung disease who were recruited for this study during an assessment of their candidacy for lung transplant. Subjects completed a battery of questionnaires that assessed demographic factors (including work and educational status), the presence of psychological distress (anxiety and depression), availability of social support, coping styles, and physical functioning. Despite significant impairment in physical functioning in the areas of recreation, household activities, sleep, and ambulation, other indices of life quality suggested good adaptation in the majority of patients. Also, quality of life differed for patients with CF and for those with other types of end-stage lung disease. Patients with CF were more likely to be working, had lower levels of anxiety and higher levels of social support, and used more functional coping strategies than did patients with other end-stage lung disease. These results highlight the fact that patients with different types of lung disease may require different psychosocial services as they await transplant. These findings also raise the question of whether there is a difference in quality of life after transplant between patients with CF and those with other types of lung disease.
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ABSTRACT: Disrupted sleep and nocturnal hypoxia are common in cystic fibrosis (CF). However, the predictors of nocturnal hypoxia in CF are still controversial. In order to identify the risk factors for nocturnal desaturation and sleep disturbances, we carried out a clinical and polysomnographic investigation of CF patients. We studied 30 clinically stable CF cases with clinical lung disease (mean age = 12.8; mean FEV1 = 65.2), 10 CF cases without significant lung disease (mean age = 13.3; mean FEV1 = 99.8), and 20 controls (mean age = 15.5). Patients were evaluated by spirometry, 6-min walk test, the Shwachman–Kulczycki (S–K) score, and full overnight polysomnography. Cases with clinical lung disease had lower body mass index, forced vital capacity, and S–K scores. During sleep, five CF cases with clinical lung disease (15%) had SaO2 <90% during more than 30% of total sleep time and 11 cases (36.6%) had a nadir SaO2 below 85%. FEV1 values for CF cases with clinical lung disease were related to nadir SaO2 (P < 0.03) and to mean SaO2 (P = 0.02). A receiver operating characteristic (ROC) analysis determined FEV1 at 64% to be predictive of nocturnal desaturation as defined by minimum SaO2 <85% (sensitivity = 92.3%; specificity = 77.3%) or SaO2 <90% for 30% of sleep time (sensitivity = 81.8%; specificity = 85.2%). Frequency of impaired sleep was not different in CF cases with (N = 2) and without significant lung disease (N = 5, P = 0.53). Sleep architecture was not significantly different between the two groups. Sleep apnea was present in three CF cases with clinical lung disease and in one case without significant lung disease. In summary, desaturation during sleep can be predicted by FEV1 <64% with good sensitivity and specificity. There are no significant differences in sleep architecture between clinically stable CF cases with and without significant lung disease. Pediatr Pulmonol. 2009; 44:1143–1150. ©2009 Wiley-Liss, Inc.Pediatric Pulmonology 10/2009; 44(11):1143 - 1150. · 2.53 Impact Factor