Introduction: The coronavirus 2019 (COVID-19) pandemic has become a major world health problem. All U.S. states have advised their cystic fibrosis (CF) populations to socially isolate. Major health care payors such as Medicare and most private insurance companies have agreed to reimburse health care providers for telemedicine and telephone visits. Methods: The CF adult team at the University of Virginia (UVA) transitioned from face-to-face clinics to multidisciplinary telemedicine clinics by using WebEx Ò (Cisco Systems, San Jose, CA), a Health Insurance Portability and Accountability Act of 1996 (HIPAA) compliant platform. Interventions: Patients were contacted before scheduled visits and triaged into: (1) patients eligible for the multidisciplinary telemedicine clinic, (2) patients to be seen in clinic urgently due to acute needs, and (3) stable patients who can be rescheduled at a later time. Ineligible patients for the telemedicine clinic due to lack of access to technology were followed up via telephone. Results: A total of 63 patients were scheduled to be seen in the UVA clinic over 4 weeks, 10 clinic days. Of these patients, 20 (32%) rescheduled their appointment. In addition, 2 patients (3%) were seen in clinic for acute needs and 38 (60%) were seen by the multidisciplinary team through telemedicine. Conclusions: In the context of the COVID-19 pandemic, implementing a telemedicine clinic process that serves the needs of a multidisciplinary care team is paramount to preserving the CF care model. Through a systematic design and test process, a feasible and sustainable program was created that can be utilized by other multidisciplinary programs to adapt to their context.

Background Due to the COVID-19 pandemic, there was an uptake of telehealth in cystic fibrosis care. Previous studies show disparities in telehealth use based on socioeconomic status (SES). We aimed to: (1) understand telehealth use and perceptions and (2) identify the facilitators and barriers to telehealth use among people with CF and their families (PwCF) from diverse racial/ethnic and socioeconomic backgrounds. Methods We conducted an analysis of the 2020 Cystic Fibrosis State of Care surveys completed by PwCF (PFSoC), CF Care Programs (SoC1) and the CF Foundation Patient Registry (CFFPR). Results A total of 424 PwCF and 286 programs responded to the PFSoC and SoC1. Among PwCF, 90% self-identified as White, 6% as Hispanic/Latino, and 2% as Black. Racial/ethnic minorities were less likely to have had a telehealth visit (p=.015). This difference was pronounced among the Hispanic/Latino population (p<.01). Telehealth use did not differ by health insurance and was similarly offered independent of financial status. Compared to PwCF who denied financial constraints, those who reported financial difficulties found telehealth more difficult to use (p=.018) and were less likely to think that their concerns (p=.010) or issues that mattered most to them (p=.020) were addressed during telehealth. Programs perceived lack of technology, language barriers, and home conditions as barriers to telehealth in vulnerable populations. Conclusion PFSoC and SoC1 identified differences in telehealth use and care perceptions by ethnicity, race, and socioeconomic characteristics. Further studies are needed to understand how telehealth can change access to CF care in diverse subpopulations.

Background: Cystic fibrosis (CF) is characterized by inflammation, progressive lung disease, and respiratory failure. Although the relationship is not well understood, patients with CF are thought to have a higher prevalence of asthma than the general population. CF Foundation (CFF) annual registry data in 2017 reported a prevalence of asthma in CF of 32%. It is difficult to differentiate asthma from CF given similarities in symptoms and reversible obstructive lung function in both diseases. However, a specific asthma phenotype (type 2 inflammatory signature), is often identified in CF patients and this would suggest potential responsiveness to biologics targeting this asthma phenotype. A type 2 inflammatory condition is defined by the presence of an interleukin (IL)-4 high , IL-5 high , IL-13 high state and is suggested by the presence of an elevated total IgE, specific IgE sensitization, or an elevated absolute eosinophil count (AEC). In this manuscript we report the effects of using mepolizumab in patients with CF and type 2 inflammation. Results: We present three patients with CF (63, 34 and 24 year of age) and personal history of asthma, who displayed significant eosinophilic inflammation and high total serum IgE concentrations (type 2 inflammation) who were treated with mepolizumab. All three patients were colonized with multiple organisms including Pseudomonas aeruginosa and Aspergillus fumigatus and tested positive for specific IgE to multiple allergens. We examined the effect of mepolizumab on patients' lung function (FEV1), blood markers of type 2 inflammation, systemic corticosteroid use and frequency of CF exacerbations. One patient had a substantial increase in lung function after starting mepolizumab and all three patients had a substantial benefit in regards to reduced oral CCS use. While none of the patients showed significant changes in the exacerbation rates there was markedly reduced requirements for oral CCS with exacerbations. In addition, mepolizumab had a positive effect on type 2 inflammatory markers, reducing markers of allergic inflammation in all 3 patients. Conclusions: Mepolizumab appears to have a positive effect on clinical course in patients with CF presenting with a type 2 phenotype characterized by allergic sensitization and hyper-eosinophilia.

As the number of coronavirus disease 2019 (COVID-19) infections increases in Virginia, social distancing is encouraged in all aspects of current life. However, an entire population is left behind from our isolation planning: the homeless. Charlottesville alone, on any given night, has about 150 people staying in shelters: (1) Salvation Army, approximately 70 people; (2) People and Congregation Engaged in Ministry, approximately 50 to 60 people; and (3) Shelter for Help in Emergency (shelter for domestic violence survivors), approximately 25 people.

Background: Cystic fibrosis (CF) is associated with worsening of depression and anxiety symptoms. Elexacaftor/tezacaftor/ivacaftor (Trikafta®), a cystic fibrosis transmembrane regulator (CFTR) modulator approved in 2019, significantly improves lung function, decreases pulmonary exacerbations, and improves quality of life. Studies are needed to evaluate the effects of Trikafta on symptoms of anxiety and depression. Research Question: Do adults with CF report a change in depression and anxiety symptoms after Trikafta initiation? Study Design and Methods: A retrospective chart review was conducted of patients with CF ( n = 127) receiving care from January 2015 through February 2022. Data collected included demographics, annual PHQ-9 and GAD-7 scores, FEV1 percent predicted at each visit, BMI, consistency and timeline of Trikafta use, mental health diagnoses, counseling/psychotherapy use, psychiatric medication use, prescriber of psychiatric medications, number of psychiatric emergency department visits and psychiatric hospital admissions, and sleep disturbances. Results: Of the 127 patients screened for eligibility, 100 patients were included. Data collected yielded 563 PHQ-9, 563 GAD-7, and 560 ppFEV1 data points. No significant changes in average PHQ-9 or GAD-7 scores were found after Trikafta initiation or due to the COVID-19 pandemic. However, 22% of patients initiated or had a change in psychiatric medications, and patients with changes in psychiatric medications had significantly higher PHQ-9 and GAD-7 scores than patients not prescribed psychiatric medications. Trikafta use improved lung function by an average of 5.23% ( p = 8.56e−08). Around a quarter (23%) of all patients reported sleep issues after initiating Trikafta. Interpretation: No significant changes in average PHQ-9 and GAD-7 scores were found after Trikafta initiation. A quarter of patients required a change in psychiatric medications, and significant differences in depression and anxiety scores were found between patients with a change in psychiatric medications and those not prescribed medication. Twenty-three percent of patients reported a prevalence of sleep issues after Trikafta initiation.