COVID-PCD – a participatory research study on the impact of COVID-19 in people with Primary Ciliary Dyskinesia

Pedersen, Eva Sophie Lunde; Collaud, Eugenie; Mozún, Rebeca; Ardura‐Garcia, Cristina; Lam, Yin Ting; Harris, Amanda; Lucas, Jane S.; Copeland, Fiona; Manion, Michele; Rindlishbacher, Bernhard; Silberschmidt, Hansruedi; Goutaki, Myrofora; Kuehni, Claudia E.

doi:10.1101/2020.11.11.20229922

Cited by 13 publications

(11 citation statements)

References 28 publications

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“…There is no evidence that patients with PCD are more at risk of contracting or suffering from more severe disease with COVID-19. 92 Surgical intervention for lung disease is generally avoided apart from select cases of severe, localized bronchiectasis where lobectomy can be considered. Retrospective data from the iPCD cohort revealed that the 5.6% of patients who had received lung resection resulted in no clinical benefit.…”

Section: Microbiologymentioning

confidence: 99%

Primary Ciliary Dyskinesia

Shoemark

Harman

2021

Semin Respir Crit Care Med

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Primary ciliary dyskinesia (PCD) is an inherited cause of bronchiectasis. The estimated PCD prevalence in children with bronchiectasis is up to 26% and in adults with bronchiectasis is 1 to 13%. Due to dysfunction of the multiple motile cilia of the respiratory tract patients suffer from poor mucociliary clearance. Clinical manifestations are heterogeneous; however, a typical patient presents with chronic productive cough and rhinosinusitis from early life. Other symptoms reflect the multiple roles of motile cilia in other organs and can include otitis media and hearing loss, infertility, situs inversus, complex congenital heart disease, and more rarely other syndromic features such as hydrocephalus and retinitis pigmentosa. Awareness, identification, and diagnosis of a patient with PCD are important for multidisciplinary care and genetic counseling. Diagnosis can be pursued through a multitest pathway which includes the measurement of nasal nitric oxide, sampling the nasal epithelium to assess ciliary function and structure, and genotyping. Diagnosis is confirmed by the identification of a hallmark ultrastructural defect or pathogenic mutations in one of > 45 PCD causing genes. When a diagnosis is established management is centered around improving mucociliary clearance through physiotherapy and treatment of infection with antibiotics. The first international randomized controlled trial in PCD has recently been conducted showing azithromycin is effective in reducing exacerbations. It is likely that evidence-based PCD-specific management guidelines and therapies will be developed in the near future. This article examines prevalence, clinical features, diagnosis, and management of PCD highlighting recent advances in basic science and clinical care.

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Section: Microbiologymentioning

confidence: 99%

Primary Ciliary Dyskinesia

Shoemark

Harman

2021

Semin Respir Crit Care Med

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“…A detailed description of the methods has been published [ 9 ]. In short, COVID-PCD is an international study advertised through PCD support groups and is open to people of any age with a confirmed or suspected diagnosis of PCD who can complete questionnaires in English, German, Spanish, Italian, or French.…”

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confidence: 99%

SARS-CoV-2 infections in people with primary ciliary dyskinesia: neither frequent, nor particularly severe

et al. 2021

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The coronavirus disease 2019 (COVID-19) pandemic caused by SARS-CoV-2 has by March 2021 infected at least 115 million people worldwide and caused over 2.5 million deaths. People with pre-existing chronic health conditions are reportedly at high risk of catching the disease and of having a severe disease course [1–4]. Primary Ciliary Dyskinesia (PCD) is a multisystem, genetic disease which affects about 1 in 10 000 people and leads to chronic upper and lower airway disease, laterality defects, including congenital heart disease, and other health problems [5–8].

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“…COVID-PCD is a participatory research project where people with PCD have an active role in all stages of research from the design of the study, its content, the piloting, and communication of results. Details about the study methods have been published [24, 25]. In short, the COVID-PCD study includes persons of any age from anywhere in the world with a confirmed or suspected diagnosis of PCD.…”

Section: Methodsmentioning

confidence: 99%

Facemask usage during the COVID-19 pandemic among people with primary ciliary dyskinesia: a participatory project

Pedersen

Collaud

Mozún

et al. 2021

Preprint

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Background Facemasks help prevent transmission of SARS-CoV-2 and are particularly important for people with chronic respiratory disease such as primary ciliary dyskinesia (PCD). We studied the usage of facemasks and its consequences among people with PCD in an international context. Methods We used data from COVID-PCD, an international observational cohort study which collects longitudinal data from people with PCD during the COVID-19 pandemic via weekly online questionnaires. An online questionnaire investigating use of facemasks was posted in October 2020. It asked about frequency of facemask usage in different public places, problems experienced with use of facemasks, affordability of masks, and beliefs regarding their effectiveness. Results 282 participants (50%) completed the questionnaire. Participants came from 27 different countries; median age was 32 years (interquartile range 17-48), and 63% were female. Almost all wore a facemask whenever they left their house. In addition, many avoided public places altogether. For example, 159 (57%) did not use public transport at all, 108 (39%) always wore a facemask in public transport, 4 (1%) sometimes, and 7 (3%) never. 81% agreed with the statement that facemasks protect the person who wears the mask, and 91% agreed that facemasks protect others. One third reported that it was uncomfortable to wear a mask because of their runny nose, 31% because of cough, and 22% because of difficulty breathing. Participants less often wore facemasks in public when there was no national requirement. Only two persons had a personal exemption from wearing a facemask prescribed by a physician. Conclusions This international study found that people with PCD carefully shield themselves, and most wear facemasks everywhere in public. People who did not wear facemasks in public came from countries without a national facemask requirement. National policies mandating facemask use in public are important for universal use to protect high-risk populations from SARS-CoV-2 infections.

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COVID-PCD – a participatory research study on the impact of COVID-19 in people with Primary Ciliary Dyskinesia

Cited by 13 publications

References 28 publications

Primary Ciliary Dyskinesia

Primary Ciliary Dyskinesia

SARS-CoV-2 infections in people with primary ciliary dyskinesia: neither frequent, nor particularly severe

Facemask usage during the COVID-19 pandemic among people with primary ciliary dyskinesia: a participatory project

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