Assessing ventilation pre and post airway clearance techniques in PCD

  • Research type

    Research Study

  • Full title

    Assessing the effects of airway clearance interventions in young people with Primary Ciliary Dyskinesia using ventilation MRI scans: a proof of concept study.

  • IRAS ID

    198572

  • Contact name

    Jim Wild

  • Contact email

    j.m.wild@sheffield.ac.uk

  • Sponsor organisation

    Sheffield Children's Hospital

  • Duration of Study in the UK

    0 years, 3 months, 1 days

  • Research summary

    Background to the research
    Primary Ciliary Dyskinesia (PCD) is an inherited condition affecting the lungs from birth. In PCD mucus builds up and blocks small airways causing repeated chest infections, progressive lung damage, and failure of their respiratory system. To manage PCD and prevent deterioration airway clearance techniques (ACTs) are used
    to move and clear mucus from the airways. ACTs include breathing techniques, blowing through devices to open the airways and inhaling medicines to make mucus less sticky. Each person with PCD is recommended an ACT regimen tailored to their needs, to complete twice daily at home. There is minimal research into ACTs in PCD,
    patient and public involvement (PPI) work has identified understanding and optimising the effects of ACTs as a research priority. Currently the measures available to assess ACTs are limited. This study uses a new and safe technique; magnetic resonance imaging (MRI) to take pictures of gas inside the lungs. In PCD, this technique has
    shown areas where mucus is blocking the airways even in people with mild disease. In other conditions MRI has been able to show changes caused by ACTs.
    Aims of the research
    This study aims to measure the effects of ACTs in young people with PCD using the most advanced new tests of lung function.
    Design and methods used
    The study aims to observe patient’s usual care with the additional information provided by lung imaging. People aged over 5 with PCD will be invited.
    This will involve attending one appointment in Sheffield. On the day they will have:
    • Simple breathing tests and the first MRI (usual care)
    • They will complete their usual ACT
    • We will repeat the MRI and breathing tests again (extra assessments)

  • REC name

    North West - Liverpool Central Research Ethics Committee

  • REC reference

    18/NW/0595

  • Date of REC Opinion

    24 Oct 2018

  • REC opinion

    Further Information Favourable Opinion