Improved survival among young patients with cystic fibrosis
Michal Kulich1, Margaret Rosenfeld, Christopher H Goss
1Department of Biostatistics, University of Washington, Seattle 98195-7232, USA. kulich@u.washington.edu
Insights
Cystic fibrosis survival rates improved significantly for US children and adolescents between 1985 and 1999. However, adult survival saw minimal gains, and females consistently had poorer outcomes than males.
Area of Science:
- Medical research
- Epidemiology
- Pulmonology
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs.
- Survival rates for CF patients have historically varied by age and sex.
Purpose of the Study:
- To analyze age-specific survival trends in US CF patients from 1985 to 1999.
- To compare survival improvements between male and female CF patients.
Main Methods:
- Retrospective cohort study utilizing data from 31,012 US patients.
- Analysis of survival outcomes using the Cox proportional hazards model.
Main Results:
- Mortality decreased significantly for patients aged 2-15 years (61%-70%).
- Smaller mortality reductions were observed in patients over 15 years old.
- Female patients consistently exhibited poorer survival rates than males aged 2-20 years, with no narrowing of this gap.
Conclusions:
- US cystic fibrosis survival rates improved substantially between 1985 and 1999, primarily in younger age groups.
- While both sexes benefited, females aged 2-20 years had persistently lower survival than males.
- Further research is needed to understand the limited survival improvements in adult CF patients.
Objective:
To investigate age-specific trends in survival among US patients with cystic fibrosis between 1985 and 1999 and to assess whether survival in female patients with cystic fibrosis has improved relative to survival in male patients.
Study Design:
A retrospective cohort study of 31,012 subjects in the US Cystic Fibrosis Foundation National Patient Registry. Trends in survival outcome were evaluated by the Cox model.
Results:
Between 1985 and 1999, mortality fell 61% (95% CI, 36-76) for patients age 2 to 5 years, 70% (60-88) for patients age 6 to 10 years, and 45% (32-66) for patients age 11 to 15 years. Improvements in mortality rates among patients older than 15 years were smaller. Female patients had poorer survival rates than male patients in the age range 2 to 20 years, and this gender gap did not narrow throughout time.
Conclusions:
Survival rates of US patients with cystic fibrosis have improved remarkably since 1985. However, most of the improvement was limited to patients 2 to 15 years old. Although both genders benefitted from this trend, female patients have had consistently poorer survival rates than male patients in the age range 2 to 20 years. Further studies are needed to clarify why adult patients with cystic fibrosis had little improvement in survival rates.
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