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The German cystic fibrosis quality assurance project: clinical features in children and adults

¹ Institute for Medical Informatics and Biometrics, Technical University of Dresden, ² Dr. Steinkamp Clinical Research, Hannover, and CF Centre Hamburg-Altona, ³ Centre for Quality Management in the Health Care System, Hannover, ⁴ University Children's Hospital, Tübingen, Germany

CORRESPONDENCE: M. Stern, University Children's Hospital, Hoppe-Seyler-Str. 1, 72076 Tuebingen, Germany. Fax: 49 7071295477

Keywords: cystic fibrosis, epidemiology, patient registry, quality management

Received: June 19, 2000
Accepted January 3, 2001

This work was supported by Christiane Herzog Stiftung, Mukoviszidose e.V., and Niedersächsischer Verein zur Förderung der Qualität im Gesundheitswesen.

Cystic fibrosis (CF) is a complex disease which requires interdisciplinary care in specialized CF centres. In Germany, 97 paediatric and adult outpatient clinics agreed to report clinical data of their patients to a newly established registry, the Cystic Fibrosis Quality Assurance (CFQA) project. This article characterizes the design of the CFQA and the health status of the patients enrolled by the end of 1997.

Data from 4,306 patients reported to the CFQA project were analysed. Nutritional status and lung function of the patients were examined as well as the use of specific therapeutic interventions.

Mean age of all 4,182 patients alive by the end of 1997 was 15.7 yrs (maximum, 58 yrs), and 35.8% of patients were >18.0 yrs of age. One-third of the CF population were treated in the nine largest centres (each caring for >100 patients). Abnormal nutritional status (weight-for-height >90% of predicted or body mass index <19.0 kg·m², respectively) was observed in 26.8% of children and adolescents and in 38.3% of adults. Lung function was abnormal (forced expiratory volume in one second <80% predicted) in the majority of adults (83.9%) and in 42.5% of the younger patients. The mortality rate was 1.4 of 100 patients in 1997. No clear association of clinical status with centre size was observed.

The clinical features of patients treated in German cystic fibrosis centres were generally comparable to those reported from other countries, although improvements are certainly warranted. The Cystic Fibrosis Quality Assurance project represents an important tool for future progress in the quality of cystic fibrosis care.