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ImportanceEarly in the COVID-19 pandemic, chronic respiratory disease was considered a risk factor for severe COVID-19 disease. Studies have confirmed a higher risk of intensive care unit admission and mortality in people with chronic pulmonary obstructive disease and cystic fibrosis, but there is little data in people with primary ciliary dyskinesia (PCD). ObjectiveTo study incidence of SARS-CoV-2 and its risk factors in people with PCD from May 2020 to May 2022. We also describe the severity of COVID-19 symptoms in this population and factors associated with severity. Design, setting, and participantsWe used data from COVID-PCD, an international participatory cohort study following people with PCD through the COVID-19 pandemic. The study is based on self-reported weekly online questionnaires, available in five languages, adapted to children, adolescents, and adults. COVID-PCD invites people with PCD of any age to participate. ExposuresSARS-CoV-2 Main OutcomesIncidence of reported positive test of SARS-CoV-2 and reported severity of symptoms. ResultsBy May 2022, 728 people with PCD participated (40% male, median age 27 years; range 0-85). The median weeks of follow-up was 60 (range 1-100). Eighty-seven (12%) reported a SARS-CoV-2 infection at baseline or during follow-up and 62 people reported an incident SARS-CoV-2 infection during 716 person-years of follow-up (incidence rate 9 per 100 person years; 95%CI 7-11). Using Poisson regression, we found that age above 14 years was associated with lower risk of infection (IRR 0.42, 95%CI 0.21-0.85) but the strongest predictors were exposure to Delta (IRR 4.52, 95%CI 1.92-10.6) and Omicron variants (IRR 13.3, 95%CI 5.2-33.8) compared to the original strain. Severity of disease was mainly mild; 12 (14%) were asymptomatic and 75 (86%) had symptoms among whom 4 were hospitalized. None needed intensive care and nobody died. Using Poisson regression, we found that comorbidity (IRR 1.93, 95%CI 1.40-2.64) and being infected during the period when the Delta variant was predominant (IRR 1.43, 95%CI 1.07-1.92) were associated with more reported symptoms. Conclusion and RelevancePeople with PCD do not seem to have a higher incidence of SARS-CoV-2 infections nor higher risk of severe COVID-19 disease than people from the general population. Key PointsO_ST_ABSQuestionC_ST_ABSWhat is the incidence and severity of COVID-19 in people with primary ciliary dyskinesia and which factors are associated with reporting a SARS-CoV-2 infection and risk of severe disease? FindingsThis international cohort of 728 people with primary ciliary dyskinesia followed for two years during the COVID-19 pandemic found a low incidence of reported SARS-CoV-2 in people with primary ciliary dyskinesia and mainly mild disease. The strongest predictor of incidence and severity was virus variant. MeaningPeople with PCD do not seem to have a higher incidence of SARS-CoV-2 infections nor higher risk of severe COVID-19 disease than people from the general population.
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Primary ciliary dyskinesia (PCD) is a rare genetic disease that causes recurrent respiratory infections. People with PCD may be at high risk of severe COVID-19 and vaccination against SARS-CoV-2 is therefore important. We studied vaccination willingness, speed of vaccination uptake, side effects, and changes in social contact behavior after vaccination in people with PCD. We used data from COVID-PCD, an international participatory cohort study. A questionnaire was e-mailed to participants in May 2021 that asked about COVID-19 vaccinations. 423 participants from 31 countries replied (median age: 30 years; 261 (62%) female). Vaccination uptake and willingness was high with 273 of 287 adults (96%) being vaccinated or willing to be in June 2021; only 4% were hesitant. The most common reasons for hesitancy were fear of side effects (reported by 88%). Mild side effects were common but no participant reported severe side effects. Half of participants changed their social contact behaviour after vaccination by seeing friends and family more often. The high vaccination willingness in the study population might reflect the extraordinary effort taken by PCD support groups to inform people about COVID-19 vaccination. Clear and specific public information and involvement of representatives is important for high vaccine uptake.
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IntroductionCOVID-PCD is a participatory study initiated by people with PCD who have an essential vote in all stages of the research from the design of the study to the recruitment of participants, and interpretation and communication of the study results. COVID-PCD aims to collect epidemiological data in real time from people with PCD throughout the pandemic to describe incidence of COVID-19, symptoms, and course of disease; identify risk factors for prognosis; and assess experiences, wishes, and needs. MethodsThe study is advertised through patient support groups and participants register online on the study website (www.covid19pcd.ispm.ch). The study invites persons of any age from anywhere in the world with a suspected or confirmed PCD. A baseline questionnaire assesses details on PCD diagnosis, habitual symptoms, and COVID-19 episodes that occurred before study entry. Afterwards, participants receive a weekly follow-up questionnaire with questions on incident SARS-CoV-2 infections, current symptoms, social contact behaviour, and physical activity. Occasional thematic questionnaires are sent out focusing on emerging questions of interest chosen by people with PCD. In case of hospitalisation, patients or family members are asked to obtain a hospital report. Results are continuously analysed and summaries put online. ConclusionThe study started recruitment on April 30, 2020, and 556 people with PCD completed the baseline questionnaire by November 2, 2020. The COVID-PCD study is a participatory study that follows people with PCD during the COVID-19 pandemic, helps to empower affected persons, and serves as a platform for communication between patients, physicians, and researchers.
