Marked decline in cystic fibrosis-related mortality over 10 years

1. From 2000 to 2010, adjusted mortality for cystic fibrosis (CF) patients dropped by 17% (1.8% per year).

2. Median survival for those born with CF in 2010 could exceed 50 years if mortality continues to decrease at a similar pace.

Evidence Rating Level: 2 (Good)

Study Rundown: Cystic fibrosis (CF) is a genetic disease characterized by thicker mucous secretions that can damage or adversely affect organs such as the airways and pancreatic ducts. CF currently affects around 30,000 Americans, and advances in the treatment of this disease have dramatically increased patient lifespan. This study sought to examine the change in mortality rate among CF patients registered in the Cystic Fibrosis Foundation Patient Registry (CFFPR) from 2000 to 2010. During this time period, the median age increased from 14.3 to 16.7 years, and adjusted mortality dropped by 17% for the entire cohort (a decrease of around 1.8% per year). Male patients had a 19% lower adjusted risk for death than females over this period. This data was used to generate survival projections for patients diagnosed in 2010. Assuming mortality continues to drop at the rate observed between 2000 and 2010, median survival is projected to be 56 years (54 in females and 58 in males). Although these projections apply only to patients who were diagnosed before age 1 and who receive treatment at CFF-accredited care centers, the results of this study still suggests that the number of adult CF patients in the United States will continue to increase in the near future.

Click to read the study in Annals of Internal Medicine

Relevant Reading: Adult cystic fibrosis

In-Depth [retrospective cohort]: The authors examined survival trends among CF patients in the CFFPR between 2000 and 2010. Trends in mortality were analyzed using multivariable Cox proportional hazards models, and the data was adjusted for gender, race/ethnicity, F508delta mutation status, presence of symptoms at diagnosis and age at diagnosis. Patients with missing genotype or demographic data were excluded from the analyses. The adjusted hazard ratio for the 10-year period was 0.83 (CI 0.75-0.93), which translates to a 17% reduction in mortality from 2000 to 2010, or a 1.8% (CI 0.5%-2.7%) reduction per year. Three different survival projections for patients diagnosed with CF in 2010 were derived based on the following assumptions: mortality does not change from the 2010 rate, mortality continues to decrease at the rate observed between 2000 and 2010 (1.8%), and mortality decreases at half of the rate observed between 2000 and 2010 (0.9%). For the first assumption, median overall survival was projected to be 39 years (CI, 38-40 years), while the overall survival projections based on the latter two assumptions were 56 years (CI 54-58 years) and 45 years (CI 44 to 46 years), respectively.

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