Journal of Cystic Fibrosis
Volume 8, Issue 5 , Pages 295-315, September 2009

Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: A European consensus☆

  • Harry Heijerman

      Affiliations

    • Corresponding Author InformationCorresponding author. Tel.: +31 702100000; fax: +31 702102150.
    • ,
  • Elsbeth Westerman
    • ,
  • Steven Conway
    • ,
  • Daan Touw
    • ,
  • Gerd Döring for the consensus working group

      Affiliations

    • Baroukh Assael, Cystic Fibrosis Center, Verona, Italy; Ian Balfour-Lynn, Royal Brompton & Harefield NHS Trust, Sydney Street, London, United Kingdom; Gabriel Bellon, Hôpital Debrousse, Lyon, France; Celeste Barreto, Hospital de Santa Maria, Lisbon, Portugal; Cesare Braggion, Ospedale Civile Maggiore, Verona, Italy; Steven Conway, St James University Hospital, Leeds, UK; Christiane De Boeck, University Hospital Gasthuiberg, Leuven, Belgium; Gerd Döring, Institute of Medical Microbiology and Hygiene, Eberhard-Karls-Universität, Tübingen, Germany; Jean-Christophe Dubus, Faculté de Médecine, Marseille, France; Irmgard Eichler, EMEA, London; Mark Elkins, Royal Prince Alfred Hospital, Sydney Australia; Henderik Frijlink, University of Groningen, Groningen, The Netherlands; Charles Gallagher, St Vincent's Hospital, Dublin, Ireland; Silvia Gartner, University Hospital Vall d'Hebron, Barcelona, Spain; David Geller, Nemours Children's Clinic, Orlando, USA; Matthias Griese, University of Munich, Munich, Germany; Harry Heijerman, Haga Teaching Hospital, The Hague, The Netherlands; Lena Hjelte, Karolinska University Hospital Huddinge, Stockholm, Sweden; Margaret Hodson, Royal Brompton Hospital, London, United Kingdom; Niels Høiby, Rigshospitalet, Copenhagen, Denmark; James Littlewood, St. James's University Hospital, Leeds, UK; Anne Malfroot, Academisch Ziekenhuis, Vrije Universiteit Brussel, Brussels, Belgium; Alexander Möller, University Children's Hospital, Zurich, Switzerland; Petr Pohunek, Charles University 2nd Medical School, Prague, Czech Republic; Tanja Pressler, Rigshospitalet, Copenhagen, Denmark; Alexandra Quittner, University of Miami, Miami, USA; Felix Ratjen, Hospital for Sick Children, Toronto, Canada; Martin Schöni, University of Berne, Berne, Switzerland; Giovanni Taccetti, Ospedale Meyer, Florence, Italy; Harm Tiddens, Erasmus University Medical Center, Rotterdam, The Netherlands; Daan Touw, Apotheek Haagse Ziekenhuizen, The Hague, The Netherlands; Elsbeth Westerman, Apotheek Haagse Ziekenhuizen, The Hague, The Netherlands.

Haga Teaching Hospital, Department of Pulmonology, Leyweg 275, 2545 CH The Hague, The Netherlands

Received 31 January 2009; received in revised form 5 April 2009; accepted 8 April 2009.

Abstract 

In cystic fibrosis inhalation of drugs for the treatment of CF related lung disease has been proven to be highly effective. Consequently, an increasing number of drugs and devices have been developed for CF lung disease or are currently under development. In this European consensus document we review the current status of inhaled medication in CF, including the mechanisms of action of the various drugs, their modes of administration and indications, their effects on lung function, exacerbation rates, survival and quality of life, as well as side effects. Specifically we address antibiotics, mucolytics/mucous mobilizers, anti-inflammatory drugs, bronchodilators and combinations of solutions. Additionally, we review the current knowledge on devices for inhalation therapy with regard to optimal particle sizes and characteristics of wet nebulisers, dry powder and metered dose inhalers. Finally, we address the subject of testing new devices before market introduction.

Keywords: Cystic fibrosis, Inhaled medication, Inhalation devices

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☆ This meeting, held on April 4 to 6, 2008 in Artimino, Tuscany, Italy, was organized by the European Cystic Fibrosis Society. Supported by AOP Orphan Pharmaceuticals AG, Austria; Aradigm Corporation, USA; Bayer Healthcare, Germany; Boehringer Ingelheim GmbH, Germany; Chiesi Farmaceutici, Italy; Forest Pharmaceuticals Inc., USA; Gilead Sciences, USA; Grünenthal GmbH, Germany; Novartis UK, Profile Pharma Limited/Respironics, UK; Roche, Switzerland.

PII: S1569-1993(09)00035-6

doi:10.1016/j.jcf.2009.04.005

Journal of Cystic Fibrosis
Volume 8, Issue 5 , Pages 295-315, September 2009

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