ABSTRACT
Objectives: To analyse characteristics of people with cystic fibrosis (PwCF) who were using home spirometry devices (HS) during 2020–2021 Methods: During the COVID-19 pandemic, the CF Foundation (CFF) partnered with a technology vendor, ZephyRx, to distribute MIR HS devices to eligible PwCF. During 04/2020–12/2021, 20,157 spirometers were shipped to PwCF. PwCF enrolled in the CFF patient Registry (CFFPR) provided an additional consent to have their HS values linked to their CFFPR data. An application programming interface (API) was built to allow transfers of HS data (FEV1, FVC, FEF25–75, sex, date of birth, height) from each device. Each record contained a CFFPR ID to enable its linkage to the CFFPR. This analysis uses CFFPR data to describe the HS cohort and the data obtained through API to characterise HS utilisation trends. Demographic and clinical characteristics between the HS cohort and the 2019–2020 CFFPR population ages 7 and older were also compared. Results: 272 (94.4%) CF programs participated in the HS program. Records of 1,537 patients, who had activated their device by January 10, 2021, or earlier were linked to CFFPR. The cohort was 69.8% adult, 89.5% Caucasian, 57.8% female, and had a mean age of 27.8, and mean FEV1 of 79.9% predicted. When compared to the CFFPR population, the HS cohort was older, contained more Caucasians and females, and had lower lung function. The median number of acceptable FEV1 measurements supplied per PwCF was 4 (IQR 2–8). 1065 (69%) PwCF in the HS cohort continued to use their device 6 months from activation. Conclusions: HS data has the potential to augment care and research databases like the CFFPR. Little is known about PwCF’s long-term usage of HS devices in a real-world setting. While the HS cohort is small and may be biased compared to the CFFPR population, we have established a reliable channel for collecting HS data and that PwCF’s usage patterns suggests that most are using the devices on a regular basis.
ABSTRACT
Background: The CFSmartReports (CFSR) web application is available to CF Foundation-accredited care programs and it is used to report data captured in the Cystic Fibrosis Patient Registry (CFFPR). CFSR was developed by the CFFPR team and deployed in 2017 to provide clinicians with a comprehensive view of the patient-level data collected in CFFPR. The original scope of CFSR has expandedover time frompatient-level reports to include new tools and reports requested by the CF medical community, such as the Clinical Trials tool, Population Management Reports (e.g., CFTR Modulator Eligibility report) and tools for checking and verifying the data entry. Knowledge of how the registry data are being used at the point of care is important for planning further enhancements in CFSR. This analyzes CFSR's backend database to provide longitudinal information on the usage of CFSR's reports and tools since its deployment Methods: Data entered to CFFPR become available in the CFSR within 24 hours. CFSR logsuser-basedoper at ionala ctionstostoreand maintainaudit trails. The audit trails are necessary for compliance and security reports, but they are also useful in understanding how the CF teams are using different parts of the application. The operational actions of CFSR users were linked to their CF programs and analyzed by the most common usage categories in each year between 2017 and 2020. Results: The number of programs using CFSR has reached 250 in 2020 versus 198 in 2017 (17% increase). Patient-level reports were downloaded for almost 50% of the CFFPR population in 2020 versus 30% in 2017. More programs were using clinical trials tools (73% vs 57%) and population management reports (86% vs 47%) in 2020 in comparison to 2017. The CFTR Modulator Eligibility report was the most popular among programs since its release in 2019. Almost all programs (98%) used CFSR in 2020 to analyze their registry data entry. CFSR usage, in general, fell in 2020 compared to 2019 (Table 1). Usage of CFSR was the highest among pediatric programs, followed by adult and affiliate programs (data not shown). (Table presented.) Conclusion: The tools and reports in CFSR are used by most care programs. The popularity of the data entry analysis tool in CFSR can be explained by CFF grant allocation, which is based on the number of records entered to CFFPR. High usage of other features shows the value that CFSR brings to care teamseitherdire ctlyatthe pointofcare orbyhelping the mtorecruit patients for clinical trials. The COVID-19 pandemic decreased usage of CFSR reports, especially for the patient-level reports, but this may change as the pandemic is controlled. Affiliate programs are likely underutilizing CFSR.
ABSTRACT
Objectives: As the novel coronavirus (SARS-CoV-2) pandemic continues, people with cystic fibrosis (CF) have been identified as being a vulnerable group. It is essential that people with CF, their families and their clinical teams have the most up-to-date information on the impact of SARS-CoV-2 on their health. This study aims to characterise the impact of SARS-CoV-2 infection in people with CF throughout 2020, identify factors that predict clinical progression of COVID-19, and to describe medium-term follow-up of people who have been infected. Methods: The ‘Cystic Fibrosis Registry Global Harmonization Group’ is a worldwide network of CF Registries that each contributed data on people with CF diagnosed with SARS-CoV-2 infection. In this analysis, we will report on cases contributed from 22 countries diagnosed between 1st February and 13th December 2020. We will present demographic, pre-infection clinical characteristics, symptoms, infection management and outcomes. We will use multivariable logistic regression to assess predictors for hospitalisation with respiratory support and intensive care admission as the outcomes of interest representing clinical progression of COVID-19. Descriptive analysis of medium-term follow-up BMI and FEV1% predicted values will also be undertaken. Results: Results pending. Expected cohort size >1,000, including the 181 previously reported in our paper “The global impact of SARS-CoV-2 in 181 people with cystic fibrosis.” Conclusion: It is expected that the findings of this study will have important implications for shielding advice, clinical care and vaccine prioritisation for people with CF.