key: cord-1031749-wzbkr7gz
authors: Cheng, S.; Le, T.; McIlwaine, M.; McIntosh, I.; Wallenburg, J.; Stephenson, A.
title: 44: Characterizing the COVID-19 pandemic among Canadians living with cystic fibrosis: A Canadian Cystic Fibrosis Registry study
date: 2021-11-30
journal: Journal of Cystic Fibrosis
DOI: 10.1016/s1569-1993(21)01469-7
sha: b134571bc00613d5801ce6244b4bdc879152c7b5
doc_id: 1031749
cord_uid: wzbkr7gz

nan

Background: The Canadian Cystic Fibrosis (CF) Registry, is one of the longest-running CF registries worldwide, with longitudinal data from all 42 Canadian CF clinics dating back to the 1970s. The Registry is uniquely positioned to assess the population-level impact of the COVID-19 pandemic on the Canadian CF community. The objectives of this study are to 1) characterize SARS-CoV-2 (the virus that causes COVID-19) testing patterns and 2) describe the demographic and clinical characteristics of Canadians with CF who had a confirmed SARS-CoV-2 infection. Methods: SARS-CoV-2 tests and infections recorded in the Registry between January 1 and December 31, 2020, were included in this study. For individuals with a confirmed SARS-CoV-2 diagnosis, follow-up data to March 31, 2021, was retrieved. Adults were defined as those aged ≥18 years at the time of testing. Continuous variables were summarized using median and interquartile range (IQR), and categorical variables using frequency and proportion. Lung function was measured using FEV1 percent predicted ( ppFEV1). Nutritional status was measured using body mass index (BMI) or BMI percentile (overweight if BMI > 24.9 or BMI percentile > 84%). Primary outcomes were mortality and hospitalization; secondary outcome was change in ppFEV1. Results: During the study period, the Registry recorded 1,315 tests for SARS-CoV-2 among 854 individuals (20% of Canadian CF population). Figure 1 shows the weekly number of tests conducted for adults and children with CF alongside the general Canadian population. Through the first half of the year, adults were routinely tested more than children. However, beginning in late summer (coinciding with the start of the school year), tests among children increased. In total, 24 people (17 adult, 7 children) tested positive for SARS-CoV-2. Median age at diagnosis was 27 years (range 2 to 65 years), 42% were male, 32% were overweight, and ≤5 were post-transplant. Median best ppFEV1 in the year prior to SARS-CoV-2 infection was 69% (IQR 44% to 95%) in adults and 104% (IQR 91% to 106%) in children. No deaths and 8 hospitalizations were recorded. Follow-up clinicals were available for 14 individuals (median 2.4 months postinfection), and among them, the median change in the first post-infection ppFEV1 was −5.6% (IQR −11.7% to −2.0%). Future analyses will include characterizing downstream effects on care, such as frequency of clinic visits, hospitalizations, and pulmonary exacerbations. These results will be available for NACFC. Conclusion: This is the first comprehensive look at SARS-CoV-2 testing and COVID-19 in the Canadian CF community. Testing patterns appear to mirror those of the general Canadian population. Compared to the initial concerns over SARS-CoV-2 infections in people living with CF, the absence of deaths in the cohort is encouraging; however, the decrease in ppFEV1 requires further monitoring to fully understand the impact of COVID-19 on lung function decline. Acknowledgements: We would like to acknowledge the involvement and continued participation of those living with cystic fibrosis who consent to having their data submitted, and the exceptional effort and contribution from CF clinic team members who collect and enter the data.

The effect of CFRD on lung function trajectory among ivacaftor users: An analysis of the CFF patient registry Background: A diagnosis of CF-related diabetes (CFRD) is associated with a greater degree of lung function decline [1] . While treatment with the CFTR modulator ivacaftor has been shown to improve percent predicted FEV1 ( ppFEV1) and decrease sweat chloride, it is unclear to what degree it affects glucose tolerance [2] . It is unknown if those with CFRD treated with ivacaftor will continue to have a more severe decline in lung function. To answer this question, we undertook an analysis of the CF Foundation Patient Registry (CFFPR). Methods: We queried the CFFPR from 2011, the year prior to FDA approval of ivacaftor, to 2018 [3] . Ivacaftor users were defined as any ivacaftor use in 2012 or 2013. CFRD status was determined on annual assessment in the year prior to ivacaftor use. Comparisons of baseline characteristics were completed using Pearson's chi-square test or Wilcoxon rank sum test as appropriate. The trend in ppFEV1 was estimated using a linear mixed model, which included CFRD status and its interaction with time. It also included covariates such as sex, baseline age, ppFEV1, BMI, pancreatic insufficiency status, and Pseudomonas aeruginosa sputum culture status. Results: Of the 732 ivacaftor users identified, 175/732 (24%) were categorized as CFRD and 577/732 (76%) were categorized as non-CFRD. During the study period, 195/577 (33%) of the non-CFRD group developed CFRD. Those with CFRD were older (27 vs 23), had a lower baseline ppFEV1 (61% vs 80%) and were more likely to have a diagnosis of pancreatic insufficiency (98% vs 86%) and sputum cultures positive for P. aeruginosa (74% vs 59%). There was no significant difference between the groups in sex, race, or BMI. After adjusting for sex, baseline age, baseline ppFEV1, BMI, pancreatic insufficiency status, and P. aeruginosa sputum culture status, the CFRD group had a significantly greater decline in ppFEV1 (coefficient −0.36, p < 0.0001). Conclusion: In this analysis of the CFFPR we found that, after adjustment for relevant covariates, CFRD remains associated with a more severe decline in lung function among ivacaftor users. This finding is consistent with lung function trajectory trends seen in the pre-modulator era. While there are still many unanswered questions regarding the link between CFRD and lung function decline in CF, this analysis confirms that highly effective modulator therapy does not alleviate this association and highlights a continuing need for research within the CF community.

Background: Designing patient-centered clinical trials improves research quality and efficiency [1] and is a priority of CF trials networks. Results from our group's qualitative work suggest that 6 trial attributes have a key Posters

Acknowledgements: The authors would like to thank the CF Foundation for use of the CFFPR to conduct this study. Additionally, we would like to thank the patients, care providers, and clinic coordinators at CF centers throughout the United States for their contributions to the CFFPR. Support: CFF (BENGTA19CO to C.B. and SALATH18I0 to M.S.) and NIH (UL1TR002366 to C.B. and R01 HL157942 to M.S.).