Conclusions: CF remains a progressive respiratory disease and is associated with multisystem complications. The acceleration in disease severity in adolescence and early adulthood suggests that better treatment at these stages is required to further improve survival.

The Australian Cystic Fibrosis Data Registry is positioning itself as an exemplar of a rare disease registry for the future. While it continues to inform cystic fibrosis (CF) clinicians of patterns of CF disease and quality of care, its capability is increasing as a resource for further research into CF subpopulations, as a platform for clinical trials, and as an interface for patient experiences.

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