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的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目及 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者的研究机构。 囊性纤维化（CF）是最常见的遗传性致死性疾病之一;在过去的三十年中，中位生存年龄从1969年的14岁提高到1999年的29.1岁（95%CI为27.5至31.6）。 随着生存率的提高，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，有非常有限的研究，不同的方法来衡量CF患者的健康相关的生活质量的效用和相对优势。 本申请提出了一项重度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个月评估一次，共评估3年。 本提案的总体目标是推进严重囊性纤维化青少年和成人患者HRQL测量的最新技术水平。

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.