Gerritsen J, van der Laag H, Sinnema G, Knol K
University Hospital, Groningen, The Netherlands.
Lung. 1990;168 Suppl:474-80. doi: 10.1007/BF02718167.
Cystic fibrosis is the most common serious genetic disorder in people of European descent. Treatment of these patients is ongoing throughout life and until now has been aimed at the consequences and is still not curative. Over the past 10-20 years, there has been a dramatic improvement of mortality rates for cystic fibrosis, due in large part to advances in medical care. The average age of survival for young people with cystic fibrosis is pushing well into the 20s with one third living into their 30s. Consequently, education plays a major role in management of patients with cystic fibrosis, and starts directly after being sure of the diagnosis. Growing up, these patients experience a lot of problems, and these are especially marked in the adolescent. A special problem, for many cystic fibrosis patients is becoming an adult. Continuity in care for these patients from the pediatric to the adult department is not always guaranteed. It is concluded that patients with cystic fibrosis should be treated in specialized centers, and such treatment cannot be carried out sufficiently by one person, but has to be embedded in a team of caregivers.
囊性纤维化是欧洲血统人群中最常见的严重遗传性疾病。这些患者需要终生接受治疗，迄今为止，治疗一直针对疾病后果，且仍无法治愈。在过去10至20年里，囊性纤维化患者的死亡率有了显著改善，这在很大程度上归功于医疗护理的进步。患有囊性纤维化的年轻人的平均存活年龄已顺利进入20多岁，三分之一的人能活到30多岁。因此，教育在囊性纤维化患者的管理中起着重要作用，确诊后应立即开始。在成长过程中，这些患者会遇到很多问题，在青少年时期尤为明显。对许多囊性纤维化患者来说，一个特殊问题是成年。从儿科到成人科室，这些患者的护理连续性并不总是有保障的。得出的结论是，囊性纤维化患者应在专科中心接受治疗，而且这种治疗无法由一人充分实施，而必须由一组护理人员共同进行。
