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Diagnosing primary ciliary dyskinesia; the patient perspective

Laura Behan, Audrey Dunn Galvin, Sarah Masefield, Fiona Copeland, Michele Manion, Bernhard Rindlisbacher, Beatrice Redfern, Jane S. Lucas
European Respiratory Journal 2015 46: PA4520; DOI: 10.1183/13993003.congress-2015.PA4520
Laura Behan
1Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton Faculty of Medicine and University Hospital Southampton NHS Foundation Trust, Southamptom, United Kingdom
3School of Applied Psychology, University College Cork, Cork, Ireland
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Audrey Dunn Galvin
3School of Applied Psychology, University College Cork, Cork, Ireland
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Sarah Masefield
4European Lung Foundation, Patient Relations, Sheffield, United Kingdom
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Fiona Copeland
5Primary Ciliary Dyskinesia Family Support Group, PCD Family Support Group, Milton Keynes, United Kingdom
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Michele Manion
6Primary Ciliary Dyskinesia Foundation, PCD Foundation, Minneapolis, MN United States
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Bernhard Rindlisbacher
7Kartagener's Syndrome and Primary Ciliary Dyskinesia Foundation, Kartagener's Syndrome and Primary Ciliary Dyskinesia Foundation, Steffisburg, Switzerland
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Beatrice Redfern
5Primary Ciliary Dyskinesia Family Support Group, PCD Family Support Group, Milton Keynes, United Kingdom
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Jane S. Lucas
1Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
2NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton Faculty of Medicine and University Hospital Southampton NHS Foundation Trust, Southamptom, United Kingdom
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Abstract

Introduction: Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by progressive sino-pulmonary disease, with symptoms starting soon after birth. An ERS PCD Task Force aims to address diagnostic disparities that exist across Europe by providing an evidence-based practice guideline. We aimed to investigate patient opinions about the PCD diagnostic process.

Methodology: A patient survey was developed by patient representatives and healthcare specialists to capture diagnostic experience and opinions. It was translated into 9 European languages and electronically distributed globally through patient organisations and ELF. Patients at different stages of testing and with a variety of outcomes were included.

Results: 352 patients/parents completed the survey from 25 EU and non EU countries, 76% PCD positive, 6% negative and 18% uncertain for PCD. 35% had >40 visits to their doctor for symptoms prior to referral for PCD testing. Age at testing was significantly lower for patients with situs inversus (median=4 years, range 0-58) than situs solitus (median=7 years, range 0-70). Nasal brushing biopsy was the most common procedure (77%) followed by nasal nitric oxide (45%). Improved PCD awareness among medical practitioners (97%), access to a PCD expert to analyse results (97%) and improved information on PCD testing (96%) were reported as important or very important. Travelling long distances to specialist centres and repeat testing were not highlighted as problematic.

Conclusion: Improved knowledge among medical practitioners and access to PCD specialist services were the most important issues as rated by patients. Patient opinions will contribute to the ERS PCD Taskforce practice guidelines for diagnosis.

  • Health policy
  • Chronic disease
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Diagnosing primary ciliary dyskinesia; the patient perspective
Laura Behan, Audrey Dunn Galvin, Sarah Masefield, Fiona Copeland, Michele Manion, Bernhard Rindlisbacher, Beatrice Redfern, Jane S. Lucas
European Respiratory Journal Sep 2015, 46 (suppl 59) PA4520; DOI: 10.1183/13993003.congress-2015.PA4520

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Diagnosing primary ciliary dyskinesia; the patient perspective
Laura Behan, Audrey Dunn Galvin, Sarah Masefield, Fiona Copeland, Michele Manion, Bernhard Rindlisbacher, Beatrice Redfern, Jane S. Lucas
European Respiratory Journal Sep 2015, 46 (suppl 59) PA4520; DOI: 10.1183/13993003.congress-2015.PA4520
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