Issues in PulmonaryOutcome of patients with cystic fibrosis admitted to the intensive care unit: Is invasive mechanical ventilation a risk factor for death in patients waiting lung transplantation?
Section snippets
Patient recruitment
We reviewed the records of all 155 patients with CF followed in the CF center of the Pulmonary Unit at the Sheba Medical Center, Tel Hashomer, Israel, between 1996 and 2006. All 48 patients with CF admitted to the ICUs in our center between 1996 and 2006 (pediatric and adult) were included in the current analysis. When a patient had multiple ICU admissions, the first admission was considered as the index hospitalization.
The study was approved by the medical ethics committee of our hospital. The
Results
From 1996 to 2006, there were 104 admissions of 48 patients with CF (aged 18 ± 9 years; range .5-43 years) to the ICU. Most patients (n = 36, 75%) had their first ICU admission before 2001. Twenty-four of the 48 patients (50%) survived the ICU stay, were discharged from hospital, and are still alive 3 to 10 years after the initial ICU admission. P. aeruginosa was isolated in 41 of 48 patients (85.4%). All patients had pancreatic insufficiency. The anthropometric data, basic lung function, clinical
Discussion
The present study evaluated the long-term outcome of 48 patients with CF admitted to the ICU and compared the outcome of patients with CF requiring invasive MV with those treated with NIPPV, during a 10-year period. All patients were followed up and treated at the same accredited CF center. Our results show a high mortality rate (94.4%) among patients admitted to the ICU and treated with MV for acute on chronic respiratory failure. In contrast, the survival rate among patients using BiPAP for
Study Limitations
Several limitations to our study should be considered. First, this was a retrospective study. However, such studies cannot be designed prospectively. In addition, we did not study the different modes of ventilation in a double-blind manner. Some of our patients who were listed for LTx did not have the opportunity to use BiPAP because at the time they were admitted to the ICU (7-10 years ago) this mode of ventilation was not frequently used. In contrast, over the past few years, BiPAP was
Conclusions
Our study indicates a poor prognosis in patients requiring MV, either for acute respiratory failure or during the period awaiting LTx. When possible, end-stage lung disease can be treated successfully with BiPAP, which can serve as a bridge while awaiting LTx. Reversible pulmonary and extrapulmonary complications can be managed appropriately in the ICU setting.
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Cited by (18)
Survival in cystic fibrosis after acute respiratory failure supported by extracorporeal membrane oxygenation and/or invasive mechanical ventilation
2022, Journal of Cystic FibrosisCitation Excerpt :Results of Cox proportional-hazards regression analyses in which potential confounders were adjusted for to estimate hazard ratios for ICU mortality, 1-year and 2-year mortality show that these outcomes were not significantly affected by comorbid conditions (diabetes, pancreatic insufficiency), respiratory microbiology, critical illness factors or lung function prior to ICU admission. Data previously published on the poor short term survival in CF patients requiring critical care support and endotracheal intubation has been discouraging [8,18,19]. However, recent advances may lead to improved outcomes for those patients with CF and acute respiratory failure requiring invasive ventilatory support.
Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease
2020, Journal of Cystic FibrosisCitation Excerpt :Survival to hospital discharge is reported as high as 55% when lung transplant is an option [49], and 10–55% when transplant is not an option [49-52]. Despite improvement from the 1970s, prognosis after admission to the ICU remains guarded in individuals requiring mechanical ventilation, especially those with non-reversible conditions [53]. Careful discussion between patients, families and healthcare teams is needed when ICU care is considered, particularly when lung transplant is not an option [54].
Managing end stage lung disease in children
2014, Paediatric Respiratory ReviewsCitation Excerpt :Forethought must be given to all possible outcomes including the child's death on a ventilator and the unsettling burden of the decision to withdraw ventilatory support.4 In a study looking at the outcomes of patients with CF aged 5 - 43 admitted to ICU, requiring mechanical ventilation for acute on chronic respiratory failure in whom NIPPV had failed, outcomes were extremely poor (whether they were being ventilated for acute respiratory failure or during the period awaiting lung transplantation).17 In a similar study focusing on children with CF admitted to ICU for mechanical ventilation, a more favourable prognosis was seen in children under 5 years of age (mortality of 22%) as compared with 5 - 34 year olds (mortality of 75%).33
Lung transplantation in children. Specific aspects
2013, Archivos de BronconeumologiaCitation Excerpt :It has been shown that mechanically ventilated infants who have received transplants have the same survival post-transplant as non-ventilated older children, and better than ventilated older children.21 Invasive mechanical ventilation in children with cystic fibrosis is a risk factor for higher post-transplant morbidity and mortality, unlike non-invasive ventilation.22–24 Table 3 summarizes the criteria for referring children to lung transplant teams.
Conflict of interest statement: There is no conflict of interest relating to the authors of this article.
Funding: None.