Outcomes of Children With Cystic Fibrosis Admitted to PICUs

Overview

Journal Pediatr Crit Care Med

Specialty Pediatrics

Date 2020 Apr 19

PMID 32304511

Citations 4

Authors

Michael A Smith

Meghan E McGarry

Ngoc P Ly

Matt S Zinter

Affiliations

Soon will be listed here.

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Objectives: Data on outcomes of children with cystic fibrosis admitted to PICUs are limited and outdated.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.

Citing Articles

Factors associated with cystic fibrosis mortality before the age of 30: retrospective analysis of a cohort in southern Brazil.

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Acute respiratory failure due to pulmonary exacerbation in children with cystic fibrosis admitted in a pediatric intensive care unit: outcomes and factors associated with mortality.

Drummond D, Roy C, Cornet M, Bucher J, Boussaud V, Le Pimpec-Barthes F Respir Res. 2024; 25(1):190.

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Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine.

Ritivoiu M, Dragoi C, Matei D, Stan I, Nicolae A, Craiu M Pharmaceutics. 2023; 15(1).

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Risk factors for air leakage during invasive mechanical ventilation in pediatric intensive care units.

Ma L, Yin M, Yang X, Xu W Eur J Med Res. 2022; 27(1):218.

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References

Ashkenazi M, Nathan N, Sarouk I, Aluma B, Dagan A, Bezalel Y . Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis. Lung. 2019; 197(3):371-376. DOI: 10.1007/s00408-019-00218-3. View

Kremer T, Zwerdling R, Michelson P, OSullivan P . Intensive care management of the patient with cystic fibrosis. J Intensive Care Med. 2008; 23(3):159-77. DOI: 10.1177/0885066608315679. View

Texereau J, Jamal D, Choukroun G, Burgel P, Diehl J, Rabbat A . Determinants of mortality for adults with cystic fibrosis admitted in Intensive Care Unit: a multicenter study. Respir Res. 2006; 7:14. PMC: 1403757. DOI: 10.1186/1465-9921-7-14. View

Pollack M, Patel K, Ruttimann U . PRISM III: an updated Pediatric Risk of Mortality score. Crit Care Med. 1996; 24(5):743-52. DOI: 10.1097/00003246-199605000-00004. View

Knapp E, Fink A, Goss C, Sewall A, Ostrenga J, Dowd C . The Cystic Fibrosis Foundation Patient Registry. Design and Methods of a National Observational Disease Registry. Ann Am Thorac Soc. 2016; 13(7):1173-9. DOI: 10.1513/AnnalsATS.201511-781OC. View

Rosenthal M . Patients with cystic fibrosis should not be intubated and ventilated. J R Soc Med. 2010; 103 Suppl 1:S25-6. PMC: 2905031. DOI: 10.1258/jrsm.2010.s11006. View

Vedam H, Moriarty C, Torzillo P, McWilliam D, Bye P . Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit. J Cyst Fibros. 2004; 3(1):8-14. DOI: 10.1016/j.jcf.2003.12.003. View

Jones A, Bilton D, Evans T, Finney S . Predictors of outcome in patients with cystic fibrosis requiring endotracheal intubation. Respirology. 2013; 18(4):630-6. DOI: 10.1111/resp.12051. View

Ellaffi M, Vinsonneau C, Coste J, Hubert D, Burgel P, Dhainaut J . One-year outcome after severe pulmonary exacerbation in adults with cystic fibrosis. Am J Respir Crit Care Med. 2004; 171(2):158-64. DOI: 10.1164/rccm.200405-667OC. View

10.

Ketchell I . Patients with cystic fibrosis should be intubated and ventilated. J R Soc Med. 2010; 103 Suppl 1:S20-4. PMC: 2905030. DOI: 10.1258/jrsm.2010.s11005. View

11.

Corriveau S, Sykes J, Stephenson A . Cystic fibrosis survival: the changing epidemiology. Curr Opin Pulm Med. 2018; 24(6):574-578. DOI: 10.1097/MCP.0000000000000520. View

12.

King C, Brown A, Aryal S, Ahmad K, Donaldson S . Critical Care of the Adult Patient With Cystic Fibrosis. Chest. 2018; 155(1):202-214. DOI: 10.1016/j.chest.2018.07.025. View

13.

Reid D, Bell S . ICU outcomes in cystic fibrosis following invasive ventilation. Respirology. 2013; 18(4):585-6. DOI: 10.1111/resp.12077. View

14.

Sprague K, Graff G, Tobias D . Noninvasive ventilation in respiratory failure due to cystic fibrosis. South Med J. 2001; 93(10):954-61. View

15.

Slieker M, van Gestel J, Heijerman H, Tramper-Stranders G, Teding van Berkhout F, van der Ent C . Outcome of assisted ventilation for acute respiratory failure in cystic fibrosis. Intensive Care Med. 2006; 32(5):754-8. DOI: 10.1007/s00134-006-0085-x. View

16.

Oud L . Critical illness among adults with cystic fibrosis in Texas, 2004-2013: Patterns of ICU utilization, characteristics, and outcomes. PLoS One. 2017; 12(10):e0186770. PMC: 5655478. DOI: 10.1371/journal.pone.0186770. View

17.

Elizur A, Sweet S, Huddleston C, Gandhi S, Boslaugh S, Kuklinski C . Pre-transplant mechanical ventilation increases short-term morbidity and mortality in pediatric patients with cystic fibrosis. J Heart Lung Transplant. 2007; 26(2):127-31. DOI: 10.1016/j.healun.2006.11.597. View

18.

Garland J, Chan Y, Kelly K, Rice T . Outcome of infants with cystic fibrosis requiring mechanical ventilation for respiratory failure. Chest. 1989; 96(1):136-8. DOI: 10.1378/chest.96.1.136. View

19.

DAVIS P, di SantAgnese P . Assisted ventilation for patients with cystic fibrosis. JAMA. 1978; 239(18):1851-4. View

20.

Hayes Jr D, Mansour H . Improved outcomes of patients with end-stage cystic fibrosis requiring invasive mechanical ventilation for acute respiratory failure. Lung. 2011; 189(5):409-15. DOI: 10.1007/s00408-011-9311-6. View