Mapping challenges in the delivery of care for primary ciliary dyskinesia, an international survey on barriers and priorities

Background Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder causing chronic, multisystemic symptoms due to impaired motile ciliary function. Diagnosis and care of PCD requires specialised, resource-intensive laboratory and clinical services, which is often unavailable in settings with limited resources. We compared the opinions of PCD healthcare professionals on the barriers and priorities affecting the delivery of care for people with PCD to inform strategies for improvement. Methods We conducted a mixed-methods study comprising semi-structured interviews with PCD healthcare professionals, followed by an electronic survey guided by interview findings. We classified participants’ countries by Gross National Income (GNI) to enable comparisons across settings. Results We conducted 15 interviews across 13 countries, identifying key barriers and priorities through content analysis. 128 participants from 35 countries completed the survey. Common barriers included lack of PCD awareness among non-specialist clinicians, underestimation of disease severity, insufficient funding, and limited time for clinical and research activities. Priorities were raising awareness, disseminating referral guidelines, and enhancing collaboration between centres. High-GNI country participants highlighted lack of evidence-based guidelines for clinical management as an important barrier, and need for multidisciplinary teams as a priority. Low-GNI country participants emphasised limited prioritisation of PCD by adult clinicians and high diagnostic cost as barriers, and access to experienced diagnostic personnel and tests as priorities. Conclusion Our findings inform a shared roadmap for improving PCD care. We highlight the need for future research to identify systemic, contextual, and modifiable factors influencing access, management, and funding for PCD across service delivery models.

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Publication Details

Journal
ERJ Open Research
Published
2026-03-12
DOI
https://doi.org/10.1183/23120541.01624-2025
Primary Topic
Cystic Fibrosis Research Advances
Type
article
Field-Weighted Citation Impact
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article

Mapping challenges in the delivery of care for primary ciliary dyskinesia, an international survey on barriers and priorities

Corine Driessens, Bruna Rubbo, Lydia Newman, Laura Behan et al.
ERJ Open Research
Cystic Fibrosis Research Advances
article

Mapping challenges in the delivery of care for primary ciliary dyskinesia, an international survey on barriers and priorities

Corine Driessens, Bruna Rubbo, Lydia Newman, Laura Behan, Janice L. Coles, Myrofora Goutaki, Jane S. Lucas, John Goodwin, Claire L. Jackson, Robert A. Hirst, Claudia E. Kuehni
article en

Abstract

Background Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder causing chronic, multisystemic symptoms due to impaired motile ciliary function. Diagnosis and care of PCD requires specialised, resource-intensive laboratory and clinical services, which is often unavailable in settings with limited resources. We compared the opinions of PCD healthcare professionals on the barriers and priorities affecting the delivery of care for people with PCD to inform strategies for improvement. Methods We conducted a mixed-methods study comprising semi-structured interviews with PCD healthcare professionals, followed by an electronic survey guided by interview findings. We classified participants’ countries by Gross National Income (GNI) to enable comparisons across settings. Results We conducted 15 interviews across 13 countries, identifying key barriers and priorities through content analysis. 128 participants from 35 countries completed the survey. Common barriers included lack of PCD awareness among non-specialist clinicians, underestimation of disease severity, insufficient funding, and limited time for clinical and research activities. Priorities were raising awareness, disseminating referral guidelines, and enhancing collaboration between centres. High-GNI country participants highlighted lack of evidence-based guidelines for clinical management as an important barrier, and need for multidisciplinary teams as a priority. Low-GNI country participants emphasised limited prioritisation of PCD by adult clinicians and high diagnostic cost as barriers, and access to experienced diagnostic personnel and tests as priorities. Conclusion Our findings inform a shared roadmap for improving PCD care. We highlight the need for future research to identify systemic, contextual, and modifiable factors influencing access, management, and funding for PCD across service delivery models.

ERJ Open Research
University of Leicester (GB), University College Cork (IE), Institute of Social and Preventive Medicine (CH), University of Southampton (GB), Wessex Institute of Technology (GB)
Openalex Percentile: Top 25%
Cystic Fibrosis Research Advances
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