High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests.

OBJECTIVE: To assess pulmonary abnormalities detected by high-resolution computed tomography (HRCT) in young children with cystic fibrosis (CF) and mild to moderate lung disease. STUDY DESIGN: High-resolution computed tomography was performed in 60 children, 6 to 10 years old, with mild to moderate lung disease (forced expiratory volume in 1 minute [FEV(1)], 52-137; mean, 102; SD, 15% predicted). HRCTs were scored by using a system that evaluates each lobe for severity and extent of CF lung disease. Findings of CF lung disease were tabulated in all subjects and in a subgroup with normal pulmonary function tests. HRCT scores were correlated with FEV(1), forced vital capacity (FVC), and forced expiratory flow between 25% and 75% of expired vital capacity (FEF(25-75)) in 57 patients. RESULTS: Bronchiectasis was present in 35% of subjects, mucous plugging in 15%, and air trapping in 63%. No abnormality was detected in 25%. In 37 subjects with FEV(1), FVC, and FEF(25-75) >85% predicted, bronchiectasis was present in 30%. In 17% of these subjects, bronchiectasis was seen in > or =4 lobes. Correlations between HRCT scores and FEV(1) were significant and showed fair to moderate correlation (r=0.36-0.46). CONCLUSIONS: High-resolution computed tomography demonstrated a broad range of pulmonary abnormalities in young patients with CF with mild to moderate lung disease. In this study, abnormalities, including bronchiectasis, were common in young children with CF and normal pulmonary function tests.

The impact of treatment of pulmonary exacerbations on the health-related quality of life of patients with cystic fibrosis: does hospitalization make a difference?

OBJECTIVES: To investigate the impact of pulmonary exacerbations on the health-related quality of life (HRQOL) of patients with cystic fibrosis (CF) and to elucidate factors that are associated with changes in HRQOL post-exacerbation. STUDY DESIGN: Participants completed questionnaires at a pre-exacerbation visit, at the beginning of the exacerbation, and at the post-exacerbation visit. To measure HRQOL, we used the Child Health Questionnaire-Parent Form (PF-50) and the Medical Outcomes Study Short Form-36 (SF-36). We examined demographic, clinical, and treatment-related predictor variables for association with change in Physical Summary (PhS) and Psychosocial Summary (PsS) HRQOL scores after an exacerbation. RESULTS: We collected data from 48 subjects. Patients experiencing exacerbations who were treated as outpatients had a mean (SD) age of 19.5 (12.7) years and a mean pre-exacerbation forced expiratory volume in 1 second (FEV(1)) of 70% (36%) of predicted; 53% were female. Patients treated as inpatients had a mean age of 14.6 (5.5) years and a mean FEV(1) of 56% (24%); 56% were female. In univariate analyses, exacerbations were associated with a statistically significant decrease in FEV(1) (-10.0%) and PhS scores (-4.5 points). PsS scores did not significantly change with exacerbations (-0.7 points). In multivariable analyses examining change between pre-exacerbation and post-exacerbation visits, no variables were significantly associated with change in PhS scores. However, not being hospitalized for the exacerbation was associated with deterioration in PsS scores, and being hospitalized was associated with improvement in PsS scores (beta coefficient: 9.4; P <.01) by the post-exacerbation visit. Results were similar from the exacerbation to the post-exacerbation visit. CONCLUSION: In patients with CF, on average, pulmonary exacerbations affect physical HRQOL more than psychosocial HRQOL. Being hospitalized for exacerbations is associated with improvement in psychosocial HRQOL after exacerbations, whereas not being hospitalized is associated with deterioration.

Health values of adolescents with cystic fibrosis.

OBJECTIVES: To assess health values (utilities) in adolescents with cystic fibrosis (CF) and to evaluate how health status and clinical factors affect their health values. METHODS: Adolescents 12 to 18 years of age completed the Child Health Questionnaire (CHQ), Health Utilities Index Mark 2 (HUI2), and 3 health value measures: the visual analog scale (VAS), time tradeoff (TTO), and standard gamble (SG). Severity of illness was measured by percent of predicted forced expiratory volume in 1 second (FEV(1)) and frequency of pulmonary exacerbations. RESULTS: The mean age (+/- SD) of the 65 adolescents was 15.1 (+/- 2.1) years; 53.8% were male; their mean FEV(1) was 72.8% (+/- 27.0%) predicted. The mean TTO utility was 0.96 (+/- 0.07) and the mean SG utility was 0.92 (+/- 0.15). In multivariable analysis, the General Health Perceptions domain from the CHQ was the only health status scale significantly associated with the VAS, TTO, and SG. No clinical or demographic measures were significantly related to both TTO and SG scores. CONCLUSIONS: Direct utility assessment in adolescents with CF is feasible. Their TTO and SG utilities are generally high, indicating that they are willing to trade very little of their life expectancy or take more than a small risk of death to obtain perfect health. Their self-rated health perceptions are related to their health values, but, as in adult populations, only moderately so, indicating that health values are highly individualistic. Therefore, health values should be ascertained directly from adolescents.