Current and emerging comorbidities in cystic fibrosis

Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed ubiquitously throughout the body. Thus, while respiratory manifestations dominate much of cystic fibrosis (CF) care, there are prominent multi-organ manifestations and comorbidities. In the general population, the number of comorbidities increases with aging. Few illnesses have experienced such a dramatic improvement in survival as CF, which has been transformed from an illness of childhood death to one of adult survival. Hence, as longevity increases in CF, it is paralleled by an increasing number of patients with multicomplex comorbidities availing of care from adult CF multi-disciplinary teams. This review gives an overview of the traditional CF associated comorbidities and those emerging in an aging adult cohort. While historically the treatment of CF focused on the consequences of CFTR dysfunction, the recent advent of CFTR modulators with the potential to enhance CFTR function represents an opportunity to potentially reverse or delay the development of some of the comorbidities associated with CF. Where evidence is available for the impact of CFTR modulatory therapy, namely ivacaftor on comorbidities in CF, this is highlighted.