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Original Article|Articles in Press

Short-term structural and functional changes after airway clearance therapy in cystic fibrosis

  • Michael E. West
    Affiliations
    Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States
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  • David R. Spielberg
    Affiliations
    Division of Pulmonary Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Ave, Chicago, Illinois, 60611, United States
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  • David J. Roach
    Affiliations
    Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States
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  • Matthew M. Willmering
    Affiliations
    Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States
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  • Abdullah S. Bdaiwi
    Affiliations
    Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Department of Biomedical Engineering, University of Cincinnati, Cincinnati, OH, 45229, United States
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  • Zackary I. Cleveland
    Affiliations
    Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Department of Biomedical Engineering, University of Cincinnati, Cincinnati, OH, 45229, United States

    Department of Pediatrics, University of Cincinnati Medical Center, Cincinnati, OH, 45229, United States

    Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States
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  • Jason C. Woods
    Correspondence
    Corresponding author.
    Affiliations
    Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Department of Pediatrics, University of Cincinnati Medical Center, Cincinnati, OH, 45229, United States

    Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 45229, United States

    Department of Physics, University of Cincinnati, Cincinnati, OH, 45229, United States
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Published:February 03, 2023DOI:https://doi.org/10.1016/j.jcf.2023.01.017

      Highlights

      • Xe ventilation MRI and spirometry are sensitive to changes post ACT.
      • Large structural and associated functional defects are mostly unchanged by ACT.
      • Xe ventilation defects improved most in areas without obvious structural defects.

      Abstract

      Background

      Airway clearance therapy (ACT) with a high-frequency chest wall oscillation (HFCWO) vest is a common but time-consuming treatment. Its benefit to quality of life for cystic fibrosis (CF) patients is well established but has been questioned recently as new highly-effective modulator therapies begin to change the treatment landscape. 129Xe ventilation MRI has been shown to be very sensitive to lung obstruction in mild CF disease, making it an ideal tool to identify and quantify subtle, regional changes.

      Methods

      20 CF patients (ages 20.7 ± 5.1 years) refrained from performing ACT before arriving for a single-day visit. Multiple-breath washout (MBW), spirometry, Xe MRI, and ultrashort echo-time (UTE) MRI were obtained twice—before and after patients performed ACT using their prescribed HFCWO vests (average 4.7 ± 0.5 h). UTE MRIs were scored for structural abnormalities, and standard functional metrics were obtained from MBW, spirometry, and Xe MRI—FEV1,pp, LCI2.5, and VDPN4, respectively.

      Results

      Spirometry and Xe MRI detected significant improvements in lung function post-ACT. 15/20 patients showed improvements from a baseline median of 92% FEV1,pp. Similarly, 16/20 patients showed improvements in Xe MRI from a baseline median of 15.2% VDPN4. Average individual changes were +2.6% in FEV1,pp and -1.3% in VDPN4, but without spatial correlations to easily-identifiable causative structural defects (e.g. mucus plugs or bronchiectasis) on UTE MRI.

      Conclusions

      Lung function improved after a single instance of HFCWO-vest ACT and was detectable by spirometry and Xe MRI. The only common structural abnormalities were mucus plugs, which corresponded to ventilation defects, but ventilation defects were often present without visible abnormalities.
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