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Objective: Provide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF. Methods: The CF Foundation organized a multidisciplinary committee to develop CF Lung Transplant Referral Consensus Guidelines. Three workgroups were formed: timing for transplant referral; modifiable barriers to transplant; and transition to transplant care. A focus group of lung transplant recipients with CF and spouses of CF recipients informed guideline development. Results: The committee formulated 21 recommendation statements based on literature review, committee member practices, focus group insights, and in response to public comment. Critical approaches to optimizing access to lung transplant include early discussion of this treatment option, assessment for modifiable barriers to transplant, and open communication between the CF and lung transplant centers. Conclusions: These guidelines will help CF providers counsel their patients and may reduce the number of individuals with CF who die without consideration for lung transplant.

Axions are well motivated particles that could make up most or all of the dark matter if they have masses below 100 µeV. Microwave cavity techniques comprised of closed resonant structures immersed in solenoid magnets are sensitive to dark matter axions with masses of a few µeV, but face difficulties scaling to higher masses. We present the a novel detector architecture consisting of an open, Fabry-Pérot resonator and a series of current-carrying wire planes, and demonstrate this technique with a search for dark matter axion-like particles called Orpheus. This search excludes dark matter axion-like particles with masses between 68.2 and 76.5 µeV and axion-photon couplings greater than 4 × 10 −7 GeV −1 . We project that the fundamental sensitivity of this technique could be extended to be sensitive to couplings below 1 × 10 −15 GeV −1 , consistent with the DFSZ model of QCD axions.

Median survival was > 6.5 years for patients with CF and an FEV < 30%, exceeding prior survival estimates. There was substantial heterogeneity in survival, with some patients with CF dying soon after reaching this lung function threshold and others living for many years. For this reason, we conclude that FEV < 30% remains an important marker of disease severity for patients with CF. Patients with a supplemental oxygen requirement or frequent exacerbations should have prompt referral because of their increased risk of death.

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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.

BackgroundLittle is known about risk factors for chronic and mucoid Pseudomonas aeruginosa (Pa) infection in cystic fibrosis (CF) adults, and whether the prevalence is changing.MethodsWe employed a retrospective cohort to analyze data from a single adult CF center (2002 to 2012). Regression models were used to assess independent predictors and change in prevalence of chronic and mucoid Pa infection over time.ResultsThe odds ratio of mucoid Pa infection was significantly less in individuals with better baseline lung function (OR 0.84,95%CI:0.77–0.92) and those diagnosed after the age of 25 (OR 0.21, 95%CI:0.05–0.95). The prevalence of chronic Pa and mucoid Pa decreased during the time interval. After adjusting for confounders, the observed decrease in chronic and mucoid Pa between 2002 and 2012 was no longer significant.ConclusionsThe prevalence of chronic and mucoid Pa is decreasing. Larger studies are needed to confirm these regional trends and their significance.Electronic supplementary materialThe online version of this article (doi:10.1186/s12890-016-0333-y) contains supplementary material, which is available to authorized users.

Among individuals with CF, a significant decrease in the risk and rates of developing chronic P. aeruginosa infection between 2003 and 2012 was observed. Whether this decline results in changes in clinical outcomes warrants further exploration.