Survival in cystic fibrosis after acute respiratory failure supported by extracorporeal membrane oxygenation and/or invasive mechanical ventilation

Gibilaro, J.; Keating, Claire; Benvenuto, Luke; Kramer, Amanda; Privorotskiy, Ann; Zheng, Yuan; Leu, Cheng-Shiun; DiMango, Emily

doi:10.1016/j.jcf.2021.08.016

Cited by 4 publications

(3 citation statements)

References 21 publications

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“…Overall, the mortality rate with ECMO has decreased due to improvements in ECMO care, from 75% in 2010, to 33% in 2018. In one center's comparison of invasive ventilation versus ventilation plus ECMO as bridges to transplantation, no differences were seen for mortality prior to discharge, or at 1, or 5 years, however, those on ECMO had longer length of stay 62 . The information related to ECMO is encouraging as there have been improvements in care leading to decreases in mortality and therefore increasing the potential benefit of using ECMO as a bridge to transplantation.…”

Section: Advanced Lung Diseasementioning

confidence: 96%

Cystic fibrosis year in review 2021

Savant

2022

Preprint

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People with cystic fibrosis (CF) have an amazing outlook with the treatment availability of highly effective modulators. Unfortunately, not all PwCF are eligible for modulators leading to continued pulmonary exacerbations and advanced lung disease. Additionally, optimizing diagnosis and evaluation for CF in the newborn period continues to be an area of focus for research. This review article will work to cover articles published in 2021 with high clinical relevance related to the above topics, however due to the extensive body of research published, this review will not be comprehensive.

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Section: Advanced Lung Diseasementioning

confidence: 96%

Cystic fibrosis year in review 2021

Savant

2022

Preprint

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“…This improved outcome also reflects the progress in survival of the CF population overall as well as proactive supportive care in these severe patients. Indeed, advances in critical care interventions, including IMV strategies and extracorporeal membrane oxygenation (ECMO) as bridge to transplant likely contributed also to the improvement in the overall mortality of these critically ill CF children [ 18 ].…”

Section: Introductionmentioning

confidence: 99%

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,

Roy,

Cornet

et al. 2024

Respir Res

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Background Children with advanced pulmonary disease due to cystic fibrosis (CF) are at risk of acute respiratory failure due to pulmonary exacerbations leading to their admission to pediatric intensive care units (PICU). The objectives of this study were to determine short and medium-term outcomes of children with CF admitted to PICU for acute respiratory failure due to pulmonary exacerbation and to identify prognosis factors. Methods This retrospective monocentric study included patients less than 18 years old admitted to the PICU of a French university hospital between 2000 and 2020. Cox proportional hazard regression methods were used to determine prognosis factors of mortality or lung transplant. Results Prior to PICU admission, the 29 patients included (median age 13.5 years) had a severe lung disease (median Forced Expiratory Volume in 1 s percentage predicted at 29%). Mortality rates were respectively 17%, 31%, 34%, 41% at discharge and at 3, 12 and 36 months post-discharge. Survival rates free of lung transplant were 34%, 32%, 24% and 17% respectively. Risk factors associated with mortality or lung transplant using the univariate analysis were female sex and higher pCO2 and chloride levels at PICU admission, and following pre admission characteristics: home respiratory and nutritional support, registration on lung transplant list and Stenotrophomonas Maltophilia bronchial colonization. Conclusion Children with CF admitted to PICU for acute respiratory failure secondary to pulmonary exacerbations are at high risk of death, both in the short and medium terms. Lung transplant is their main chance of survival and should be considered early.

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“…Overall, the mortality rate with ECMO has decreased due to improvements in ECMO care, from 75% in 2010 to 33% in 2018. In one center's comparison of invasive ventilation versus ventilation plus ECMO as bridges to transplantation, no differences were seen for mortality before discharge, or at 1, or 5 years; however, those on ECMO had a longer length of stay 63 . The information related to ECMO is encouraging as there have been improvements in care leading to decreases in mortality and therefore increasing the potential benefit of using ECMO as a bridge to transplantation.…”

Section: Introductionmentioning

confidence: 99%

Cystic fibrosis year in review 2021

Savant

2022

Pediatric Pulmonology

View full text Add to dashboard Cite

People with cystic fibrosis (CF) have an amazing outlook with the treatment availability of highly effective modulators. Unfortunately, not all people with CF are eligible for modulators leading to continued pulmonary exacerbations and advanced lung disease. Additionally, optimizing diagnosis and evaluation for CF in the newborn period continues to be an area of focus for research. This review article will work to cover articles published in 2021 with high clinical relevance related to the above topics; however, due to the extensive body of research published, this review will not be comprehensive.

show abstract

Survival in cystic fibrosis after acute respiratory failure supported by extracorporeal membrane oxygenation and/or invasive mechanical ventilation

Cited by 4 publications

References 21 publications

Cystic fibrosis year in review 2021

Cystic fibrosis year in review 2021

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

Cystic fibrosis year in review 2021

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