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No patient left behind! Therapeutic options for cystic fibrosis patients living with lung transplantation

  • Adrien Tissot
    Affiliations
    CHU Nantes, Service de Pneumologie, Institut du Thorax, Nantes, France

    Nantes Université, INSERM, Center for Research in Transplantation and Translational Immunology, UMR 1064, F-44000 Nantes, France
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  • Pierre-Régis Burgel
    Correspondence
    Corresponding author at: 27 rue du Faubourg Saint Jacques, 75014 Paris, France
    Affiliations
    Université Paris Cité, Inserm U1016, Institut Cochin, Paris, France

    Pulmonary Department and National Cystic Fibrosis Reference Centre, Cochin Hospital; Assistance Publique Hôpitaux de Paris, Paris, France
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      Lung transplantation (LT) has the potential of improving survival and quality of life in people with cystic fibrosis (pwCF) and terminal respiratory failure [
      • Thabut G
      • Christie JD
      • Mal H
      • Fournier M
      • Brugière O
      • Leseche G
      • et al.
      Survival benefit of lung transplant for cystic fibrosis since lung allocation score implementation.
      ], and cystic fibrosis (CF) represents one of the major underlying diagnosis for LT worldwide [
      • Chambers DC
      • Perch M
      • Zuckermann A
      • Cherikh WS
      • Harhay MO
      • Hayes Jr., D
      • et al.
      The international thoracic organ transplant registry of the international society for heart and lung transplantation: thirty-eighth adult lung transplantation report - 2021; focus on recipient characteristics.
      ]. PwCF are closely monitored in multiple countries by CF registries; however, pwCF living with LT are primarily followed by transplant centers, which are not systematically connected with CF registries, often resulting in underreporting their data. Nevertheless, the European Cystic Fibrosis Patient Registry identified 2845 pwCF living with transplant corresponding to 5.7% of all pwCF seen in 2019 [

      Orenti A, Zolin A, Jung A, van Rens J, et al. ECFSPR Annual Report 2019, www.ecfs.eu/ecfspr.2021.

      ]. The French CF registry reported that pwCF living with LT represented 7.5% of all pwCF and up to 20% of adult pwCF [

      Vaincre la Mucoviscidose et Ined. Registre Français de la Mucoviscidose – Bilan des données 2020 Paris [Available from: http://www.vaincrelamuco.org/face-la-mucoviscidose/registre-et-muco-en-chiffres/valorisation-des-donnees.

      ]. These data show that a relatively large number of pwCF are currently living with LT, although numbers may vary among countries, depending on access to LT.
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