[Cystic fibrosis : A new disease pattern in adult medicine]
1Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland. doris.staab@charite.de.
Abstract:
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.
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