Historically, children with CF died as infants, and as recently as 1980 the median survival was less then 20 years. However, over the past 3 decades the lifespan of CF patients has risen dramatically, and in 2006 the median survival in the United States was 37.5 years (shown by red line, figure on right). Many factors influence the health of CF patients. Older adults with CF had fewer treatment options during their childhood when compared to children born more recently.
Improving survival in CF. The red line represents the median survival (in years) for the CF registry population in the United States. Important scientific milestones and advances in treatment are depicted. (Adapted From 2005 Annual Data Report to the Center Directors. Cystic Fibrosis Patient Registry, Bethesda, MD; used with permission).
Statistics from the CF Foundation registry show that patients born in the 1990s will have a longer lifespan then patients born in the 1980s . For reasons that are not completely understood, in the past, women with CF had a significantly shorter lifespan than men. However, for women born since 1990 this “gender gap” appears to no longer be present . Thus, children born now with CF can be expected to greatly exceed today’s current average lifespan for CF patients. In addition, it is evident that individuals with less common CFTR mutations and milder forms of the disease will have a much greater life expectancy than the majority of the CF population that has the classic form of the disease.