QUALITY OF LIFE IN ADULTS AND ADOLESCENTS WITH SEVERE CYSTIC FIBROSIS

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 囊性纤维化是最常见的遗传性致死性疾病之一；在过去的30年里，中位生存年龄从1969年的14岁提高到1999年的29.1岁(95%可信区间为27.5岁至31.6岁)。随着存活率的提高，这种疾病现在必须被认为是一种慢性和进行性肺部疾病。不幸的是，关于不同方法测量CF患者与健康相关的生活质量的实用性和相对优势的研究非常有限。这项应用提出了一项针对严重CF患者的前瞻性队列研究。将评估四种不同的健康相关生活质量(HRQL)工具，包括最近开发的CF特定生活质量工具，每隔6个月进行一次，总共为期三年。这项建议的总体目标是促进测量患有严重囊性纤维化的青少年和成人患者的HRQL的最新水平。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Cystic Fibrosis (CF) is one of the most common inherited fatal diseases; over the past thirty years, the median age of survival has improved from 14 years in 1969 to 29.1 years (95% CI's 27.5 to 31.6) in 1999. With this improved survival, this disease must now be considered a chronic and progressive lung disease. Unfortunately, there is very limited research on the utility and relative strengths of different methods to measure health-related quality of life in patients with CF. This application proposes a prospective cohort study of patients with severe CF. Four different health-related quality of life (HRQL) instruments will be assessed, including a recently developed CF specific quality of life instrument at 6 month intervals for a total of three years. The overall object of this proposal is to advance the state of the art in the measurement of HRQL in adolescent and adult patients with severe cystic fibrosis.