key: cord-0974349-bcyr0m4k
authors: Burnett, S.; Preidt, M.; FitzMaurice, T.; Walshaw, M.; Nazareth, D.
title: P094 Facilitating cystic fibrosis research during the COVID-19 pandemic
date: 2021-12-31
journal: Journal of Cystic Fibrosis
DOI: 10.1016/s1569-1993(21)01120-6
sha: a281ab28964b744c885ccca378e600b7e261b084
doc_id: 974349
cord_uid: bcyr0m4k

Objectives: Although clinical research is an important part of the management of people with cystic fibrosis (pwCF), the COVID-19 pandemic has had a profound impact on research activity, especially that which involves such clinically extremely vulnerable groups. Methods: We describe our experience with continuing CF research activity during the pandemic crisis at our large adult unit (~350 pwCF). We use a bespoke CF single-person research suite that includes a lounge area incorporating hotel facilities, separate from the main clinical buildings. Results: At the start of the COVID-19 pandemic, 104 pwCF in 6 active trials (4 requiring physiologic tests) wished to carry on with their routine trial visits. One study (54 participants) was put on hold at the request of the sponsor. All pwCF were risk assessed before attending to comply with guidelines. From March to December 2020, we carried out 3 sweat tests,15 blood tests and 50 spirometry tests in the suite, and 50 research imaging and 10 full pulmonary function tests within another setting. All procedures were carried out using appropriate personal protective equipment (PPE), with staff fit-tested to perform spirometry safely. Feedback from pwCF indicated that they felt safe and secure in addition to being supported. Conclusion: With the implementation of measures such as face coverings, health screens, and social distancing, most research activity within CF presents a relatively low risk for individuals despite the challenges faced. Having a bespoke facility allowed us to ensure the safety of research participants, minimise the risk of COVID-19 community spread, including spread to the researchers themselves and the avoidance of interactions with non-research staff. We continue to adapt our strategies to make the best out of available resources and opportunities.

Facilitating cystic fibrosis research during the COVID-19 pandemic S. Burnett 1 , M. Preidt 1 , T. FitzMaurice 2 , M. Walshaw 2 , D. Nazareth 2 . 1 Liverpool Heart and Chest Hospital, Research Unit, Liverpool, United Kingdom; 2 Liverpool Heart and Chest Hospital, Adult CF Unit, Liverpool, United Kingdom

Objectives: Although clinical research is an important part of the management of people with cystic fibrosis ( pwCF), the COVID-19 pandemic has had a profound impact on research activity, especially that which involves such clinically extremely vulnerable groups. Methods: We describe our experience with continuing CF research activity during the pandemic crisis at our large adult unit (∼350 pwCF). We use a bespoke CF single-person research suite that includes a lounge area incorporating hotel facilities, separate from the main clinical buildings. Results: At the start of the COVID-19 pandemic, 104 pwCF in 6 active trials (4 requiring physiologic tests) wished to carry on with their routine trial visits. One study (54 participants) was put on hold at the request of the sponsor. All pwCF were risk assessed before attending to comply with guidelines. From March to December 2020, we carried out 3 sweat tests,15 blood tests and 50 spirometry tests in the suite, and 50 research imaging and 10 full pulmonary function tests within another setting. All procedures were carried out using appropriate personal protective equipment (PPE), with staff fit-tested to perform spirometry safely. Feedback from pwCF indicated that they felt safe and secure in addition to being supported. Conclusion: With the implementation of measures such as face coverings, health screens, and social distancing, most research activity within CF presents a relatively low risk for individuals despite the challenges faced. Having a bespoke facility allowed us to ensure the safety of research participants, minimise the risk of COVID-19 community spread, including spread to the researchers themselves and the avoidance of interactions with non-research staff. We continue to adapt our strategies to make the best out of available resources and opportunities.

Pulmonology/Immunology Results: In children aged 6-10 years, the median (IQR) FEV 1 (89.9(26.0)%) and FVC (92.6(24.0)%) were higher than in subsequent age (15-18 years: FEV 1 -81.0(41.8)%, FVC -87.0(35.0)%, p <0.001). In children 6-10 years old with CFTR-alleles classes I/I, I/II, II/II, II/III FEV 1 (91.0(25.0)%) was lower (p = 0.039) than with classes IV or V (94.7(28.4)%) and decreased in later age (15-18 years old -79.5(41.8)%, p < 0.001). FVC in children with classes I/I, I/II, II/II, II/III decreased with age and was lower at the age of 15-18 (87.0(32.3)%) than in patients of the same age with classes IV or V (100.0(24.0)%, p = 0,006). Conclusion: FEV 1 in patients with CFTR-alleles classes I/I, I/II, II/II, II/III aged 6-10 years was lower than with classes IV or V. A decrease of FEV 1 and FVC in children with CF with age was observed in patients with CFTR-alleles of classes I/I, I/II, II/II, II/III. The LF in children with CFTR-alleles IV or V classes did not statistically decrease with age. Objective: Cystic fibrosis (CF) is a chronic disease with autosomal recessive inheritance, chlorine duct defect, and multisystemic involvement. In this study, we evaluated the problems of our adolescent and adult patients with CF who were followed up in our unit to determine their problems at the regional level, to better observe their treatments, and to offer solutions for complications that occurred during their follow-up. Materials and methods: 65 patients with CF (50 adolescents and 15 adults) who consulted our clinic between September 2008 and November 2020 were included in this study, and their retrospective data were reviewed and saved. Results: The mean age of the patients was 17.2 years, and the mean age at diagnosis was 7.1 years. Nearly three-quarters (73.8%) of the patients were adults, 26.2% were adolescents. Their mean body mass index (BMI) was 18.81 ± 4.06 kg/m2. The mean FEV 1 was 82.94 ± 25.22% in the adolescent group and 64.47 ± 28.47% in the adult group. Pseudo-Bartter syndrome was the most common clinical presentation in adolescents (44%) and productive cough 28.6% was most common in adults. The rate of bronchiectasis was 73.6% in the adults and 29.2% in the adolescents. CFrelated diabetes was seen in 33.3% of the adults and 8.3% of the adolescents. Gastroesophageal reflux disease was present in 25.5% of the adolescents, but it was not seen in the adults. Mortality was 20.0% in the adult group and 4.1% in the adolescents. There was no significant difference between the groups regarding BMI, chronic Pseudomonas colonisation, and pulmonary exacerbation. In both groups, the most common allele (21.8%) was F508del. Conclusion: We saw that the disease complications were less in the adolescent group. We thought early diagnosis and treatment were related to this condition.

Who's talking about cystic fibrosis? The changing landscape of internet postings related to cystic fibrosis: a two-year comparative study

Methods: Key phrases relating to CF were identified by a CF multidisciplinary team and entered in Google Alerts with prospective tracking for 6 months in 2015. Alerts were also created for 3 non-orphan lung diseases (asthma, chronic obstructive pulmonary disease and lung fibrosis)