Cystic Fibrosis as a Model of Health Care Transition for Chronically Ill Youth

描述(申请人提供)：尽管囊性纤维症(CF)是高加索人群中最常见的限制生命的遗传病，但存活率已经增加，因此更多患有囊性纤维症的儿童和青少年可以很好地活到成年。这一增加的存活率为青少年和患有CF的年轻人的护理带来了新的机遇和挑战。一项重大挑战是，在慢性疾病可能恶化和慢性病自我管理挑战增加的时期，如何最好地为青少年和家庭从儿科向以成人为中心的卫生保健过渡做好准备。萨维基医生是哈佛医学院儿科学助理教授和波士顿儿童医院CF中心的儿科肺科专家。他感兴趣的主要领域是健康结果和质量提高，特别是关注患有CF的青少年和年轻人的医疗过渡脆弱时期。萨维基博士的直接职业目标是发展患有复杂慢性疾病的儿童的临床结果研究方面的专业知识。他完成了儿科健康服务研究方面的奖学金，但需要通过高级研究方法和分析技术的正式课程进行进一步培训。他将与一个由国家专家领导的导师团队合作，该团队在CF健康结果研究、定性研究和医疗保健过渡领域。他的长期职业目标是成为一名独立研究员，拥有针对患有CF的青少年的健康结果和医疗保健提供系统方面的专业知识，长期目标是调查青春期的干预措施如何能够在这些患者成年后导致自我管理和健康结果的持续改善。他职业发展计划中的工作和研究与NHLBI的使命相辅相成。Sawicki博士将进行一项关于患有CF的青少年和年轻人之间的医疗过渡的研究。这项建议的目的是：1)了解患有慢性纤维性心脏病的青少年及其父母所认为的过渡到成人护理的障碍和促进者。2)研究一组CF青少年在过渡准备状态方面的纵向变化，以及3)检查疾病认知、治疗信念、社会支持、疾病严重性和健康相关生活质量对一组CF青少年健康护理过渡准备状态的发展和进展的影响。他将在这个项目中使用定性和纵向调查方法。这项研究将极大地有助于了解青少年和患有CF的青壮年的医疗过渡障碍和促进者。这项研究的结果可能对社区卫生保健的提供具有重要的意义。此外，这项工作也可以作为研究其他青壮年慢性疾病人群的医疗过渡的模式。

DESCRIPTION (provided by applicant): Although cystic fibrosis (CF) is the most common life-limiting genetic disease in the Caucasian population, survival has increased such that many more children and adolescents with CF are living well into adulthood. This increased survival has led to new opportunities and challenges in the care of adolescents and young adults with CF. One major challenge is how best to prepare adolescents and families for the transition from pediatric to adult-centered health care during a time in which CF disease may worsen and challenges of chronic disease self-management increase. Dr. Sawicki is an Assistant Professor of Pediatrics at Harvard Medical School and a pediatric pulmonologist at the CF Center at Children's Hospital Boston. His principal areas of interest are health outcomes and quality improvement, particularly focused on the vulnerable period of health care transition for adolescents and young adults with CF. Dr. Sawicki's immediate career goal is to develop expertise in clinical outcomes research of children with complex chronic medical conditions. He completed a fellowship in pediatric health services research, but requires further training through formal coursework in advanced research methods and analytic techniques. He will work with a mentorship team led by national experts in the fields of CF health outcomes research, qualitative research, and health care transition. His long-term career goal is to become an independent researcher with expertise in health outcomes and healthcare delivery systems for adolescents with CF, with the long-term objective of investigating how interventions during adolescence can lead to sustained improvements in self-management and health outcomes when these patients reach adulthood. The work and research in his career development proposal complement the mission for NHLBI. Dr. Sawicki will perform a study of health care transition among adolescents and young adults with CF. The aims of this proposal are: 1) To understand the barriers and facilitators of transition to adult care as perceived by adolescents with CF and their parents. 2) To examine longitudinal changes in transition readiness among a cohort of adolescents with CF, and 3) To examine the impact of illness perception, treatment beliefs, social support, disease severity, and health-related quality of life on the development and progression of health care transition readiness in a cohort of adolescents with CF. He will use both qualitative and longitudinal survey methodology in this project. This study will substantially contribute to the understanding of barriers and facilitators for health care transition among adolescents and young adults with CF. The results of this research may have significant implications for the delivery of healthcare in CF. In addition, this work may serve as a model for the study of health care transition for other populations of young adults with chronic medical conditions of childhood onset.