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Outcomes of Children With Cystic Fibrosis Admitted to PICUs

Michael A Smith¹, Meghan E McGarry², Ngoc P Ly²

¹Department of Pediatrics, School of Medicine, University of California, San Francisco, San Francisco, CA.

Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies

|April 19, 2020

Insights

The pediatric intensive care unit (PICU) mortality rate for children with cystic fibrosis has significantly decreased, with only 6.6% dying during admission. This updated data offers crucial insights into improved outcomes for critically ill pediatric cystic fibrosis patients.

Area of Science:

Pediatric Critical Care Medicine
Pulmonology
Genetics and Genetic Diseases

Background:

Limited and outdated data exist on outcomes for children with cystic fibrosis (CF) admitted to pediatric intensive care units (PICUs).
Previous studies reported high PICU mortality rates for CF patients, ranging from 37.5% to 100%.
Advances in CF care necessitate updated information on the prognosis of critically ill pediatric CF patients.

Purpose of the Study:

To provide an updated report on PICU mortality rates for children with CF.
To identify factors associated with death among critically ill pediatric CF patients.
To assess the impact of modern CF care on PICU outcomes.

Main Methods:

Retrospective multicenter cohort analysis using the Virtual Pediatric Systems database.
Inclusion of data from 135 PICUs between January 1, 2009, and June 20, 2018.
Analysis of 1,633 children with CF, accounting for 2,893 PICU admissions.

Main Results:

Overall PICU mortality rate was 6.6%, a significant decrease from prior reports.
Factors associated with increased mortality included hemoptysis, pneumothorax, GI bleeding, infections, malnutrition, and need for respiratory support.
Mechanical ventilation was required in 26.4% of admissions, with a 19.1% mortality rate in this subgroup.

Conclusions:

PICU mortality for pediatric CF patients is lower than previously reported.
Updated data offer valuable insights into the prognosis of CF patients requiring critical care.
The findings underscore the positive impact of advancements in CF management on critical care outcomes.

Read full analysis

Abstract

Objectives:

Data on outcomes of children with cystic fibrosis admitted to PICUs are limited and outdated. Prior studies cite PICU mortality rates ranging from 37.5% to 100%. Given the advances made in cystic fibrosis care, we expect outcomes for these patients to have changed significantly since last studied. We provide an updated report on PICU mortality and the factors associated with death among critically ill children with cystic fibrosis.

Design:

Retrospective multicenter cohort analysis utilizing data from the Virtual Pediatric Systems database.

Setting:

Data were collected from 135 PICUs from January 1, 2009, to June 20, 2018.

Patients:

One-thousand six-hundred thirty-three children with cystic fibrosis accounting for 2,893 PICU admissions were studied.

Interventions:

None.

Measurements And Main Results:

The primary outcome was mortality during PICU admission. Predictors included demographics, anthropometrics, diagnoses, clinical characteristics, and critical care interventions. Odds ratios of mortality were calculated in univariate and multivariable analyses to assess differences in mortality associated with predictor variables. Generalized estimating equation models were used to account for multiple admissions per patient. The overall PICU mortality rate was 6.6%. Factors associated with increased odds of mortality included hemoptysis/pulmonary hemorrhage, pneumothorax, gastrointestinal bleeding, bacterial/fungal infections, lower body mass index/malnutrition, and need for noninvasive or invasive respiratory support. Intubation/mechanical ventilation occurred in 26.4% of the 2,893 admissions and was associated with a 19.1% mortality rate. Of the nonsurvivors, 20.7% died without receiving mechanical ventilation.

Conclusions:

The mortality rate during PICU admissions for patients with cystic fibrosis is lower than has been reported in prior studies, both in the overall cohort and in the subset requiring invasive mechanical ventilation. These data provide updated insight into the prognosis for cystic fibrosis patients requiring critical care.

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