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

描述（由申请人提供）：尽管囊性纤维化（CF）是高加索人群中最常见的限制生命的遗传性疾病，但生存率有所提高，因此更多患有CF的儿童和青少年能够很好地活到成年。这种生存率的提高给患有CF的青少年和年轻成人的护理带来了新的机遇和挑战。一个主要的挑战是，在CF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医疗保健转变做好准备。Sawicki博士是哈佛医学院儿科助理教授和波士顿儿童医院CF中心的儿科肺病专家。他的主要兴趣领域是健康结果和质量改进，特别关注患有CF的青少年和年轻人的医疗保健过渡的脆弱时期。Sawicki博士的直接职业目标是在患有复杂慢性疾病的儿童的临床结果研究方面发展专业知识。他完成了儿科健康服务研究的奖学金，但需要通过高级研究方法和分析技术的正式课程进一步培训。他将与CF健康结果研究、定性研究和卫生保健转型领域的国家专家领导的指导小组合作。他的长期职业目标是成为一名独立的研究人员，在青少年CF的健康结果和医疗保健服务系统方面具有专业知识，长期目标是研究青少年时期的干预措施如何在这些患者成年后导致自我管理和健康结果的持续改善。他的职业发展建议中的工作和研究补充了NHLBI的使命。Sawicki博士将对患有CF的青少年和年轻人的医疗保健过渡进行研究。该提案的目的是：1)了解患有CF的青少年及其父母认为的向成人护理过渡的障碍和促进因素。2)研究CF青少年群体的转变准备的纵向变化，以及3)研究疾病认知、治疗信念、社会支持、疾病严重程度和健康相关生活质量对CF青少年群体医疗转变准备的发展和进展的影响。他将在本项目中使用定性和纵向调查方法。本研究将极大地有助于理解青少年和青年CF患者的医疗保健过渡的障碍和促进因素。本研究的结果可能对CF患者的医疗保健提供具有重要意义。此外，本工作可以作为研究其他儿童期慢性病青年人群的医疗保健过渡的模型。

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.