Elsevier

Heart & Lung

Volume 39, Issue 2, March–April 2010, Pages 153-159
Heart & Lung

Issues in Pulmonary
Outcome 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?

https://doi.org/10.1016/j.hrtlng.2009.06.014Get rights and content

Objective

The admission of patients with cystic fibrosis (CF) to the intensive care unit (ICU) is controversial. Our aim was to study the long-term outcome of patients with CF who were admitted to the ICU and the effect of ventilation modality.

Methods

The medical records of 104 admissions (1996-2006) of 48 patients with CF (age 18 ± 9 years) were reviewed. Seventeen patients were admitted with reversible conditions (group 1). Thirty-one patients were admitted for acute on chronic respiratory failure (group 2).

Results

In group 1, 16 of 17 patients survived up to 10 years from ICU admission. Conversely, in group 2, 23 of 31 patients (74%) died of respiratory failure. In group 2, 17 of 18 patients who were mechanically ventilated died within 90 days from admission, and 7 of 10 patients treated for prolonged periods with bi-level positive airway pressure are still alive up to 10 years after admission and transplantation.

Conclusion

Patients requiring mechanical ventilation may have a poor prognosis. The outcome of treatment with bi-level positive airway pressure is good, even in patients who had many episodes of acute respiratory failure.

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.

References (21)

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Cited by (18)

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    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.

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    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

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    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.

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Conflict of interest statement: There is no conflict of interest relating to the authors of this article.

Funding: None.

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