The overall burden of cancer in CF patients remains low; however they have an increased risk of digestive tract cancer, particularly following transplantation. They also have increased risk of lymphoid leukemia and testicular cancer, and decreased risk of melanoma.
Background
Advances in treatments for cystic fibrosis (CF) continue to extend survival. An updated estimate of survival is needed for better prognostication and to anticipate evolving adult care needs.
Objective
To characterize trends in CF survival between 2000 and 2010 and to project survival for children born and diagnosed with the disease in 2010.
Design
Registry-based study.
Setting
110 Cystic Fibrosis Foundation–accredited care centers in the United States.
Patients
All patients represented in the Cystic Fibrosis Foundation Patient Registry (CFFPR) between 2000 and 2010.
Measurements
Survival was modeled with respect to age, age at diagnosis, gender, race or ethnicity, F508del mutation status, and symptoms at diagnosis.
Results
Between 2000 and 2010, the number of patients in the CFFPR increased from 21 000 to 26 000, median age increased from 14.3 to 16.7 years, and adjusted mortality decreased by 1.8% per year (95% CI, 0.5% to 2.7%). Males had a 19% (CI, 13% to 24%) lower adjusted risk for death than females. Median survival of children born and diagnosed with CF in 2010 is projected to be 37 years (CI, 35 to 39 years) for females and 40 years (CI, 39 to 42 years) for males if mortality remains at 2010 levels and more than 50 years if mortality continues to decrease at the rate observed between 2000 and 2010.
Limitations
The CFFPR does not include all patients with CF in the United States, and loss to follow-up and missing data were observed. Additional analyses to address these limitations suggest that the survival projections are conservative.
Conclusion
Children born and diagnosed with CF in the United States in 2010 are expected to live longer than those born earlier. This has important implications for prognostic discussions and suggests that the health care system should anticipate greater numbers of adults with CF.
Primary Funding Source
Cystic Fibrosis Foundation.
Background
People with cystic fibrosis are managed differently in the US and UK providing an opportunity to learn from differences in practice patterns.
Objectives
To compare cross-sectional demographics, practice patterns and clinical outcomes between US and UK cystic fibrosis patients.
Methods
This was a cross-sectional study using 2010 data from patients in the US Cystic Fibrosis Foundation and the UK Cystic Fibrosis patient registries. The a priori outcome measures of interest were lung function and nutritional status. Descriptive statistics and two sample comparisons were performed. Stratification and multivariable linear regression was used to adjust for confounding.
Results
The study cohort included 13,777 children and 11,058 adults from the US and 3,968 children and 3,965 adults from the UK. In children, mean body mass index percentiles were similar. Lung function (FEV1 and FVC% predicted) was significantly higher in US patients ages 6 through 25 years of age. In a regression model adjusted for only age, FEV1% predicted was on average 3.31% of predicted (95% CI: 2.65, 3.96) higher in the US compared to the UK. When adjusted for age, age at diagnosis, gender, pancreatic insufficiency and genotype, FEV1% predicted was on average 3.03% of predicted (95% CI: 2.37, 3.69) higher in the US compared to the UK These differences persisted despite adjustment for possible confounders. Hypertonic saline and dornase alfa were much more commonly prescribed in US children.
Conclusions
Children and young adults with cystic fibrosis have better lung function in the US compared to the UK despite similar nutritional status.
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