Information is lacking on the clinical impact of the novel coronavirus, SARS-CoV-2, on people with cystic fibrosis (CF). Our aim was to characterise SARS-CoV-2 infection in people with cystic fibrosis. Methods: Anonymised data submitted by each participating country to their National CF Registry was reported using a standardised template, then collated and summarised. Results: 40 cases have been reported across 8 countries. Of the 40 cases, 31 (78%) were symptomatic for SARS-CoV-2 at presentation, with 24 (60%) having a fever. 70% have recovered, 30% remain unresolved at time of reporting, and no deaths have been submitted. Conclusions: This early report shows good recovery from SARS-CoV-2 in this heterogeneous CF cohort. The disease course does not seem to differ from the general population, but the current numbers are too small to draw firm conclusions and people with CF should continue to strictly follow public health advice to protect themselves from infection.

Highlights We report 181 people with CF from 19 countries diagnosed with SARS-CoV-2 Spectrum of outcomes observed was similar to that seen in the general population 11 people were admitted to intensive care and 7 died Three of the people who died had received an organ transplant Lower lung function and having been transplanted may be linked to hospitalisation

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BackgroundPrevious research has shown that Dutch medical residents feel inadequate in certain management areas: 85% had a need for management training and reported preferences on the format of such training. Our objective was to explore if the perceived deficiencies and needs among Dutch residents were similar to those of their peers in other countries, and if a longer duration of the incorporation of the CanMEDS competency framework into curricula as well as management training had an influence on these perceptions.MethodsMedical residents from Denmark, Canada and Australia were approached for participation. The questionnaire used to survey the perceptions of Dutch residents was slightly modified, translated into English and sent by email to all international participants.ResultsResponse rates were; Denmark 719/2105 (34%), Canada 177/500 (35%) and Australia 194/1213 (16%) respectively. The Danish as well as the Canadian residents rated their negotiating skills poorly. In Australia the residents found their knowledge on how their specialist department was organized to be insufficient, while residents in the Netherlands rated their knowledge on how the healthcare system was organized as inadequate. In all of the countries, more than 70% of the residents reported a perceived need for management training.ConclusionsA majority of the residents in all countries felt the need for specific training in developing their management competencies. The adoption of the CanMEDS framework alone seems to be insufficient in meeting residents’ perceived educational needs in this area.

Background: Guideline-concordant treatment (GCT) of lung cancer has been observed to vary across geographic regions over the years. However, there is little evidence as to what extent this variation is explained by differences in patients' clinical characteristics versus contextual factors, including socioeconomic inequalities. Methods: This study evaluated the independent effects of individual- and area-level risk factors on geographic and temporal variation in receipt of GCT among patients with lung cancer. Receipt of GCT was defined on the basis of the National Comprehensive Cancer Network guidelines. We used Bayesian spatial-temporal multilevel models to combine individual and areal predictors and outcomes while accounting for geographically structured and unstructured correlation and linear and nonlinear trends. Results: Our study included 4,854 non–small cell lung cancer (NSCLC) and small cell lung cancer (SCLC) cases, reported to the Victorian Lung Cancer Registry between 2011 and 2018. Area-level data comprised socioeconomic disadvantage and remoteness data at the local government area level in Victoria, Australia. Around 60.36% of patients received GCT, and the rates varied across geographic areas over time. This variation was mainly associated with poor performance status, advanced clinical stages, NSCLC types, public hospital insurance, area-level deprivation, and comorbidities. Conclusions: This study highlights the need to address disparities in receipt of GCT among patients with lung cancer with poor performance status, NSCLC, advanced clinical stage, stage I–III SCLC, stage III NSCLC, public hospital insurance, and comorbidities, and living in socioeconomically disadvantaged areas. Impact: Two-year mortality outcomes significantly improved with GCT. Interventions aimed at reducing these inequalities could help to improve lung cancer outcomes.

Patient-reported outcome measures (PROM) are potentially useful outcome measures that may be reported at the individual clinical, health service and/or health system level. PROM require clearly defined patient populations to enable comparisons, and are most meaningful when integrated with clinical data sets. Where possible PROM should be measured pre- and post-intervention using reliable and validated tools. A variety of PROM collection methods exist which each have strengths and limitations, with selection depending on their purpose and patient factors. PROM programmes should be developed with high levels of clinician support and patient input to maximise collection of clinically relevant information.

A key measure of lung function in people with Cystic Fibrosis (CF) is Forced Expiratory Volume in the first second FEV1 percent predicted (FEV1pp). This study aimed to address challenges in identifying predictors of FEV1pp, specifically dealing with non-linearity and the censoring effect of death. Data was obtained from a large multi-centre Australian Cystic Fibrosis Data Registry (ACFDR). A linear mixed model was used to study FEV1pp as the endpoint. There were 3655 patients (52.4% male) included in our study. Restricted cubic splines were used to fit the non-linear relationship between age of visit and FEV1pp. The following predictors were found to be significant in the multivariate model: age of patient at visit, BMI z-score, age interaction with lung transplantation, insulin dependent diabetes, cirrhosis/portal hypertension, pancreatic insufficiency, Pseudomonas aeruginosa infection and baseline variability in FEV1pp. Those with P. aeruginosa infection had a lower mean difference in FEV1pp of 4.7 units, p < 0.001 compared to those who did not have the infection. Joint modelling with mortality outcome did not materially affect our findings. These models will prove useful for to study the impact of CFTR modulator therapies on rate of change of lung function among patients with CF.

Background and Objectives Patient-reported outcome measures (PROMs) captures the patient’s perspective regarding quality of life, daily functioning, symptom severity, and overall health, and how these may be impacted by health care or other interventions. PROMs are used in clinical quality registries (CQRs) for a number of diseases to assess the patient’s perspective of the impact of clinical care on quality-of-life. This scoping review aimed to identify dementia-specific PROMs, determine how the PROMs are being used, and whether they are used within dementia registries. Research Design and Methods Three electronic databases were searched using Medical Subject Heading terms for dementia, quality of life, and patient-reported outcomes. Data were extracted on the PROMs used and the methods and mode of administering the PROM. Results Seven dementia-specific PROMs were identified, however none were used in a dementia registry. All the PROMs were used at the patient level to identify patient needs and health service impacts. Three PROMs were also used at a system level to examine difference in care models. The majority of the PROMs were administered via a researcher or clinician and were predominantly completed by a proxy. Discussion and Implications PROMs provide an opportunity for a patient with dementia to share experiences and perspectives of care. A number of dementia-specific PROMs exist, yet none are used in dementia registries and the majority of studies utilize PROMs via a proxy. The use of PROM for patients with dementia, particularly in the context of dementia registries, requires further exploration and consideration.