Clinical and demographic factors associated with post–lung transplantation survival in individuals with cystic fibrosis

Stephenson, Anne L.; Sykes, Jenna; Berthiaume, Yves; Singer, Lianne G.; Aaron, Shawn D.; Whitmore, G. À.; Stanojevic, Sanja

doi:10.1016/j.healun.2015.05.003

Cited by 93 publications

(82 citation statements)

References 31 publications

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“…For instance, median survival post-lung transplant is longer in Canadian CF patients compared to published studies of US CF patients. (32) (33) Although the LAS has been associated with shorter waiting times and improved transplant survival(35), and the number of patients with CF listed for lung transplantation has increased since its implementation, the proportion who have received a transplant has decreased. (36) In addition, Merlo et al showed that CF patients transplanted since the LAS was implemented had a higher risk of death compared to CF patients transplanted in the pre-LAS period.…”

Section: Discussionmentioning

confidence: 99%

Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States

et al. 2017

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Background In 2011, the median age of survival in cystic fibrosis (CF) reported in the United States (US) was 36.8 years compared to 48.5 years in Canada; however direct comparison of survival estimates between national registries is challenging because of inherent differences in methodologies used, data processing techniques, and ascertainment bias. Objectives To use a standardized approach to calculate CF survival estimates and to explore differences between Canada and the United States (US). Design Population-based study. Setting 42 Canadian CF clinics and 110 CF care centers in the US. Patients Patients followed in the Canadian CF Registry (CCFR) and the US Cystic Fibrosis Foundation (CFF) Patient Registry (CFFPR) between 1990 and 2013. Measurement Cox proportional hazards models were used to compare survival between patients followed in the CCFR (n=5,941) and CFFPR (n=45,448). Multivariable models were used to adjust for factors known to be associated with survival. Results Median age of survival in patients with CF increased in both countries between 1990 and 2013; however in 1995 and 2005, survival in Canada increased at a faster rate relative to the US (p<0.001). Using contemporary data from 2009-2013, the median age of survival in Canada was ten years greater than the US (50.9 vs. 40.6 years, respectively). The adjusted risk of death was 34% lower in Canada compared to the US (hazard ratio 0.66, 95% CI 0.54-0.81). A greater proportion of patients had transplants in Canada (10.3% vs. 6.5% respectively, SD 13.7). Differences in survival between US and Canadian patients varied according to the US patients' insurance status. Limitations Ascertainment bias as a result of missing data or non-random lost to follow-up could impact the results. Conclusions Differences in CF survival between Canada and the US persisted after adjusting for risk factors associated with survival. Differential access to transplantation, improved post-transplant survival and differences in the healthcare systems may, in part, explain the Canadian survival advantage.

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Section: Discussionmentioning

confidence: 99%

Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States

et al. 2017

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“…Multiple studies, including a recent study of Canadian patients with CF, have demonstrated that survival is significantly shortened in patients infected with B. cenocepacia who undergo LTx [16–19]. Most LTx centers in the US consider B. cenocepacia to be an absolute contraindication to LTx.…”

Section: Discussionmentioning

confidence: 99%

Cystic fibrosis physicians’ perspectives on the timing of referral for lung transplant evaluation: a survey of physicians in the United States

et al. 2017

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BackgroundPrior studies reveal that a significant proportion of patients with cystic fibrosis (CF) and advanced lung disease are not referred for lung transplant (LTx) evaluation. We sought to assess expert CF physician perspectives on the timing of LTx referral and investigate their LTx knowledge.MethodsWe developed an online anonymous survey that was distributed by the Cystic Fibrosis Foundation (CFF) to the medical directors of all CFF-accredited care centers in the United States in 2015. The survey addressed only adult patients (≥18 years old) and was sent to 119 adult CF physicians, 86 CFF-affiliated CF physicians (who see adults and children, but have smaller program sizes than adult or pediatric centers), and 127 pediatric CF physicians (who see some adults, but mostly children). The focus of the questions was on CFF-care center characteristics, physician experience and indications/contraindications to referral for LTx evaluation.ResultsThere were 114/332 (34%) total responses to the survey. The response rates were: 57/119 (48%) adult physicians, 12/86 (14%) affiliate physicians and 43/127 (34%) pediatric physicians; 2 physicians did not include their CFF center type. Despite the poor ability of FEV1 < 30% to predict death within 2 years, 94% of responding CF physicians said they would refer an adult patient for LTx evaluation if the patient’s lung function fell to FEV1 < 30% predicted. Only 54% of respondents report that pulmonary hypertension would trigger referral. Pulmonary hypertension is an internationally recommended indication to list a patient for LTx (not just for referral for evaluation). Very few physicians (N = 17, 15%) employed components of the lung allocation score (LAS) to determine the timing of referral for LTx evaluation. Interestingly, patient preference not to undergo LTx was “often” or “always” the primary patient-related reason to defer referral for LTx evaluation for 41% (47/114) of respondents.ConclusionsSome potential barriers to timely LTx referral for patients with CF include physician knowledge regarding non-lung function-based recommendations related to timing of referral and listing for LTx, and patient preference not to undergo LTx. Further exploration of physician-level and CF patient-level barriers to timely LTx referral is warranted.Electronic supplementary materialThe online version of this article (doi:10.1186/s12890-017-0367-9) contains supplementary material, which is available to authorized users.

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“…Burkholderia cenocepacia (genomovar III) sputum culture positivity is a known marker of worse prognosis regardless of transplant status and is a contraindication to LTx at most centers. (13–15) Other genomovars of Burkholderia cepacia complex have not been found to be associated with increased mortality and should not preclude referral for transplant evaluation. (16) The finding of a strong association between a positive Burkholderia cepacia complex culture and non-referral in our study highlights the possibility that some patients may be inappropriately denied referral, but this analysis does not provide further insight into this problem because genomovar subtypes were not available in the CFFPR until 2010.…”

Section: Discussionmentioning

confidence: 99%

Predictors of non-referral of patients with cystic fibrosisfor lung transplant evaluation in the United States

Ramos

Quon

Psoter

et al. 2016

Journal of Cystic Fibrosis

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Background Lung transplantation is an intervention that improves survival for adult patients with cystic fibrosis (CF). Some patients with CF are never referred for lung transplant evaluation despite meeting physiologic criteria for referral. Methods We performed a retrospective analysis of adult patients (≥ 18 years of age) in the Cystic Fibrosis Foundation Patient Registry (CFFPR), eligible for their first evaluation for lung transplantation during the years 2001–2008 based on FEV1 <30% predicted in two consecutive years. Results Within the CFFPR, 1240 patients met eligibility criteria. Eight hundred and nine (65.2%) were referred for lung transplant evaluation, and 431 (34.8%) were not referred. In a multivariable model, Medicaid insurance (OR 1.79, 95% CI 1.29–2.47), older age (per 5 year increase; OR 1.25, 95% CI 1.13–1.39), lack of high school graduate education (OR 2.27, 95% CI 1.42–3.64), and Burkholderia cepacia complex sputum culture positivity (OR 2.48, 95% CI 1.50–4.12) were associated with non-referral, while number of pulmonary exacerbations (OR 0.93, 95% CI 0.87–0.99) and supplemental oxygen use (OR 0.59, 95% CI 0.43–0.81) were associated with increased referral. Conclusions Despite meeting lung function criteria for lung transplant evaluation, 35% of patients with CF had not yet been referred to a lung transplant center. Predictors of non-referral included markers of low socioeconomic status, older age and Burkholderia cepacia complex sputum culture. Further work is needed to understand the outcomes for non-referred patients in order to refine referral recommendations in this population.

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Clinical and demographic factors associated with post–lung transplantation survival in individuals with cystic fibrosis

Abstract: After lung transplantation, 5-year survival in Canadians with CF is 67%, and 50% of patients live >10 years. Despite these impressive probabilities, age at transplant, pancreatic sufficiency and B cepacia infection remain important determinants of survival after lung transplantation.

Cited by 93 publications

References 31 publications

Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States

Survival Comparison of Patients With Cystic Fibrosis in Canada and the United States

Cystic fibrosis physicians’ perspectives on the timing of referral for lung transplant evaluation: a survey of physicians in the United States

Predictors of non-referral of patients with cystic fibrosisfor lung transplant evaluation in the United States

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