Critical illness among adults with cystic fibrosis in Texas, 2004–2013: Patterns of ICU utilization, characteristics, and outcomes

OBJECTIVE Available reports on critically ill adults with cystic fibrosis (CF) suggest improving short-term outcomes. However, there is marked heterogeneity in reported findings, with studies mostly based on single-centered data, limiting generalizability. We sought to examine population-level patterns of demand for critical care resources, and the characteristics, resource utilization, and outcomes of ICU-managed adults with CF. METHODS We used the Texas Inpatient Public Use Data File to identify ICU admissions with CF aged ≥18 years in Texas between 2004-2013. We examined ICU utilization at population level (using CF Foundation annual reports) and, among ICU admissions, socio-demographic characteristics, burden of comorbidities, organ failure, life-support utilization and hospital disposition. Linear regression and multilevel logistic regression were used to examine temporal trends and predictors of short-term mortality (hospital death and discharge to hospice), respectively. RESULTS Of 9,579 hospitalizations of adults with CF, 1,249 (13%) were admitted to ICU. The incidence of ICU admission among adults with CF in Texas increased between 2004-2005 and 2012-2013 from 16.7 to 19.2 per 100 person-years (p = 0.0181), with ICU admissions aged ≥30 years accounting for 80.3% of the change. Among ICU admissions the following changes were noted between 2004-2005 and 2012-2013: any organ failure 30.2% vs. 56.3% (p = 0.0004), mechanical ventilation 11.5% vs. 19.2% (p = 0.0216), and hemodialysis 1.0% vs. 8.1% (p = 0.0007). Short-term mortality for the whole cohort and for those with mechanical ventilation was 11.4% and 41.8%, respectively, with corresponding home discharge among survivors 84% and 62.1%, respectively. Key predictors (adjusted odds ratios [aOR (95% CI)]) of short-term mortality included age ≥45 years (2.051 [1.231-3.415]), female gender (1.907 [1.237-2.941]), and mechanical ventilation (7.982 [5.001-12.739]). CONCLUSIONS Adults with CF had high and rising population-level burden of critical illness. Although ICU admissions were increasingly older and sicker, the majority survived hospitalization, with most discharged home, supporting short-term benefits of critical care in the present cohort.