Prognosis

The steady improvement in prognosis over several decades is related temporally to various advances in management strategies (figure 3). Undoubtedly these innovations have contributed to improving survival, but the less spectacular progress made by CF multidisciplinary teams making incremental gains by better application of basic treatments has also contributed greatly. Currently median survival is to the mid-30s, but a recent paper has predicted median survival into the mid-50s for men and into the mid-40s for women. The discrepancy in mortality between men and women has been described before. The gap begins after childhood and may be related to a greater mortality rate in CF women developing diabetes compared with nondiabetic women and all CF men. This gap is closing, probably due to the earlier and more aggressive use of insulin. The underlying mechanisms of the interactions between sex and insulin deficiency are not known.

A number of factors are leading to an improved prognosis, to which early detection and improved treatments contribute. Increasingly mild clinical CF phenotypes are being detected and incorporated into survival curves, thus prolonging apparent life expectancy. Nonetheless, it is anticipated that life expectancy for all patients with CF will increase over time.

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