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

Lavi Oud,Chiara Lazzeri

doi:10.1371/journal.pone.0186770

Lavi Oud, Chiara Lazzeri

Open Access

https://doi.org/10.1371/journal.pone.0186770

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Abstract

ObjectiveAvailable 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.MethodsWe 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.ResultsOf 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]).ConclusionsAdults 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.

Highlights

The incidence of ICU admission among adults with Cystic fibrosis (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
Cystic fibrosis (CF) is a heritable, life-shortening disease caused by dysfunction of the CF transmembrane conductance regulator, leading to progressive multisystem organ damage, with lung involvement being the predominant cause of morbidity and mortality
Dramatic improvement was noted in long-term survival of CF patients, with over 50% being 18 year or older, reaching recently a predicted median survival of nearly 42 years [3], with projected median survival over 50 years, if the recently observed decrease in mortality continues at the same rate [4]

Summary

Introduction

Cystic fibrosis (CF) is a heritable, life-shortening disease caused by dysfunction of the CF transmembrane conductance regulator, leading to progressive multisystem organ damage, with lung involvement being the predominant cause of morbidity and mortality. Dramatic improvement was noted in long-term survival of CF patients, with over 50% being 18 year or older, reaching recently a predicted median survival of nearly 42 years [3], with projected median survival over 50 years, if the recently observed decrease in mortality continues at the same rate [4]. The increasing long-term survival into adulthood is expected to result in patients increasingly manifesting the cumulative impact of CF-, age-, and therapy-related morbidity [4]. While earlier studies demonstrated the futility of intensive care and especially invasive mechanical ventilation in CF patients [5], more recent reports [6], showing improved outcomes among critically ill adults with CF, led to the proposition that critical care for CF “is no longer an oxymoron” [7]. A debate continues about use of critical care [8] and invasive mechanical ventilation [9, 10] in adults with CF

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