QUALITY OF LIFE IN 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。子项目和 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所在的机构。 囊性纤维化（CF）是最常见的遗传性致命疾病之一。 在过去的30年里，美国的中位生存年龄从1969年的14岁提高到1999年的29.1岁。随着存活率的提高，这种疾病已经从一种婴儿死亡率非常高的疾病转变为一种现在必须被视为慢性和进行性肺病的疾病。 在过去的30年里，提高这些患者的生存率一直是主要目标，但现在临床研究人员必须扩大他们对这种疾病结局的评估，以包括以患者为导向的结局，如健康相关的生活质量。 本研究是一项针对重度CF患者的前瞻性队列研究，总体目的是推进青少年和成人重度囊性纤维化患者健康相关生活质量（HRQL）测量的最新技术水平，以促进改善患者生活质量的治疗方法的开发和评估，并帮助预测肺移植评估的最佳时间。这是一项在入组时年龄≥ 14岁的CF患者中进行的HRQL前瞻性、纵向、描述性研究。 每例受试者将在入组后6个月、12个月、18个月、24个月、30个月和36个月的基线时完成4份HRQL问卷、抑郁评分问卷、移植问卷，进行肺功能测定和定时6分钟步行。

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 in 1999 in the United States. With this improved survival, this disease has been transformed from a disease with very high infant mortality to a disease that must now be considered a chronic and progressive lung disease. Improving the survival in these patients has been the primary goal over the last 30 years, but now clinical researchers must broaden their assessment of outcome in this disease to include patient-oriented outcomes such as health-related quality of life. This study is a prospective cohort study of patients with severe CF with the overall object to advance the state of the art in the measurement of health-related quality of life (HRQL) in adolescent and adult patients with severe cystic fibrosis in order to facilitate development and assessment of treatments that improve the quality of patients' lives and help predict the best time for evaluation for a lung transplant. This is a prospective, longitudinal, descriptive, study of HRQL in patients with CF who are 14 years of age and older at enrollment. Each subject will complete 4 HRQL questionnaires, a depression score questionnaire, a transplant questionnaire, perform Spirometry, and a timed six-minute walk at baseline 6 months, 12 months, 18 months, 24 months, 30 months and 36 months after enrollment.