ACSQHC-ARCR-292

The ACFDR is a population-level clinical registry that aims to accurately characterise the demographics, changing patterns of disease and treatments, morbidity and mortality of the population of patients with cystic fibrosis (CF) over time. The registry captures all people diagnosed with CF who attend participating specialist CF treatment centres in Australia (including outreach services), which is estimated to cover over 90% of patients diagnosed with CF nationally. The ACFDR uses either opt-in consent (mainly for paediatric patients) or opt-out consent (for some adult patients) to collect baseline and longitudinal information. Information is collected annually and per hospital or clinic encounter for each patient.

The ACFDR has contributed significantly to our understanding of CF in Australia through monitoring trends in measures such as age at diagnosis, method of diagnosis, lung function and weight over time, CF-related complications and treatments, transplantation, mortality and expected age of survival.

The registry aims to improve quality of care by monitoring and benchmarking patient outcomes in individual CF centres compared to each other, and compared to their own site over time. The ACFDR is increasingly being used to monitor the impact of new therapies, and to support clinical trials. The ACFDR collaborates with international CF registries via an International CF Registries Harmonisation Committee and with the John Hopkins-led CFTR2 genetic mutation database.