Non-pulmonary chronic diseases in adults with cystic fibrosis: analysis of data from the Cystic Fibrosis Registry.

The international literature shows that the demography of cystic fibrosis (CF) is changing, with patients increasingly surviving into adulthood. As they age, patients with CF become more susceptible to specific non-pulmonary chronic diseases. In this study, adult data from the CF Registry of Ireland (CFRI) was used to determine the prevalence and associated features of these diseases. 104 (25.7%) adults had diabetes versus 13 (2.9%) children (p < 0.001). Liver disease was present in 47 (11.6%) adults and 26 (5.7%) children (p = 0.002). 173 (42.7%) adults had bone disease versus 25 (5.5%) children (p < 0.001). Adults with one non-pulmonary chronic disease, for example liver disease, were more likely to have another (p = 0.002), those with diabetes and bone disease had a higher number of hospital admissions in the last 12 months (p < 0.001 for both) and higher rates of depression (p = 0.046 and p = 0.049, respectively). These results highlight a number of challenges for the Irish healthcare system.