Improved survival among young patients with cystic fibrosis

J Pediatr. 2003 Jun;142(6):631-6. doi: 10.1067/mpd.2003.197.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Child
  • Child, Preschool
  • Cystic Fibrosis / mortality*
  • Female
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Registries
  • Retrospective Studies
  • Selection Bias
  • Sex Factors
  • Survival Analysis