Success of the German Cystic Fibrosis Registry

Abstract

The German Cystic Fibrosis (CF) Registry (GCFR) is a national General Data Protection Regulation–compliant centralised database sponsored by the German Cystic Fibrosis Association (Mukoviszidose e.V.) and based on informed consent for each participating patient, ethical approval, and data protection votes. The aims of the GCFR are to optimise quality of care for CF at the centres, generate epidemiologic overviews, address research questions related to improved CF care, and inform caregivers, patients (aimed at patient empowerment), and health authorities and industry (aimed at care planning and pharmacovigilance). Established in 1995, the Registry has captured data on > 9600 individuals with a combined total of more than 140 000 annual assessments with an estimated coverage rate of > 90%. Patient data are collected after informed consent and confirmed diagnosis of CF, or a CFTR-related disorder, or a screening-positive inconclusive diagnosis of CF (i.e., CFSPID). The registry collects core, encounter, and annual health data. Data include demographics, anthropometrics, lung function, microbiology, CF-specific complications and chronic medications, hospitalisations, demand-oriented antibiotic therapies, and outcomes (death and transplants). Real world and pharmacovigilance studies have been published and additional research underway; there is a formal process for requesting access to the GCFR.