Staab D, Schwarz C
Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland.
Internist (Berl). 2018 Nov;59(11):1138-1145. doi: 10.1007/s00108-018-0498-y.
Cystic fibrosis (CF) is an inherited incurable multi-organ disease. Improvement in treatment approaches over the last 20 years have led to an increased life expectancy where the number of adult patients has doubled and will continue to increase exponentially. Due to the use of new substances which modulate the basic defect, a substantial improvement in the prognosis can be assumed but the existing healthcare structures in Germany do not meet these rising needs. With more than 50% of patients being adults, there are only very few internal medicine centers available. Only approximately one third of the patients are treated in adult health centers. Adolescence in particular is a very vulnerable phase of the disease, the risk of comorbidities is increased and adherence to the very laborious treatment recommendations is as a rule low. While in many other countries transition programs have been evaluated and implemented for more than 20 years, in Germany there have only been rudimentary approaches to transition. Meanwhile investigations are available on the perceptions of adolescents with respect to coping with the disease and their treatment needs, including the perception of the time when the transition process should begin. Successful transition seems to be performed best in combined pediatric and adult centers, with the back-up of an experienced multidisciplinary team of healthcare providers.
囊性纤维化(CF)是一种遗传性、无法治愈的多器官疾病。过去20年治疗方法的改进使预期寿命延长,成年患者数量翻倍且将继续呈指数增长。由于使用了可调节基本缺陷的新物质,可以预期预后会有实质性改善,但德国现有的医疗结构无法满足这些不断增长的需求。超过50%的患者为成年人,而可用的内科中心却非常少。只有约三分之一的患者在成人健康中心接受治疗。青春期尤其是该疾病非常脆弱的阶段,合并症风险增加,而且通常对非常繁琐的治疗建议的依从性较低。在许多其他国家,过渡项目已经评估和实施了20多年,而在德国,过渡措施还很不完善。与此同时,已有关于青少年应对疾病及其治疗需求的认知的调查,包括对过渡过程应何时开始的认知。成功的过渡似乎在儿科和成人联合中心进行得最好,并有经验丰富的多学科医疗团队提供支持。