title: Optimising inhaled mannitol for cystic fibrosis in an adult population.
abstract: There has been remarkable progress in the treatment of cystic fibrosis (CF) patients over the past 20 years. However, limitations of standard therapies have highlighted the need for a convenient alternative treatment to effectively target the pathophysiologic basis of CF-related disease by improving mucociliary clearance of airway secretions and consequently improve lung function and reduce respiratory exacerbations. Mannitol is an osmotic agent available as a dry powder, dispensed in a convenient disposable inhaler device for the treatment of adult patients with CF. Inhalation of mannitol as a dry powder is thought to change the viscoelastic properties of airway secretions, increase the hydration of the airway surface liquid and contribute to increased mucociliary and cough clearance of retained secretions. In two large phase 3 studies [1, 2], long-term use of inhaled mannitol resulted in a significant and clinically meaningful improvement in lung function relative to control in adult CF subjects and had an acceptable safety profile. Clinical experience with inhaled mannitol confirms that it is safe and effective. A minority of patients are unable to tolerate the medication. However, through training in proper inhaler technique and setting clear expectations regarding therapeutic effects, both the tolerance and adherence necessary for long term efficacy can be positively influenced.
  To discuss the importance of airway clearance treatments in the management of cystic fibrosis.To describe the clinical data that supports the use of mannitol in adult patients with cystic fibrosis.To highlight the role of mannitol tolerance testing in screening for hyperresponsiveness.To provide practical considerations for patient education in use of mannitol inhaler.
  
  
  CONCLUSIONS
  Inhaled mannitol is a safe and effective option in adult patients with cystic fibrosis.Mannitol tolerance testing effectively screens for hyperresponsiveness prior to initiation of therapy.Physiotherapists and respiratory therapists play an integral role in the introduction and maintenance of dry powder inhalation therapy.Patient training and follow-up is important for optimising longer term adherence.
authors: Flume: Pulmonary and Critical Care Medicine, Medical University of South Carolina, Charleston, SC, USA.
authors: Patrick A
authors: PA
authors: Flume
authors: Pulmonary and Critical Care Medicine, University of Washington Medical Center, Seattle, WA, USA.
authors: Moira L
authors: ML
authors: Aitken
authors: Department of Respiratory Medicine, Royal Brompton Hospital, London, UK.
authors: Diana
authors: D
authors: Bilton
authors: Rehabilitation & Therapies, Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, London, UK.
authors: Penny
authors: P
authors: Agent
authors: Pharmaxis Ltd, Frenchs Forest, NSW, Australia.
authors: Brett
authors: B
authors: Charlton
authors: Bristol Royal Infirmary, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.
authors: Emma
authors: E
authors: Forster
authors: Pharmaxis Ltd, Frenchs Forest, NSW, Australia.
authors: Howard G
authors: HG
authors: Fox
authors: Department of Pediatric Pulmonology, Allergology and Cystic Fibrosis, Universitats Kinderklinik, Wurzburg, Germany.
authors: Helge
authors: H
authors: Hebestreit
authors: Department of Medicine, University of Auckland, Auckland, New Zealand.
authors: John
authors: J
authors: Kolbe
authors: Tufts University School of Medicine, Maine Medical Centre, Portland, ME, USA.
authors: Jonathan B
authors: JB
authors: Zuckerman
authors: Faculty of Medicine, Nursing and Health Sciences, Monash University, Melbourne, VIC, Australia.
authors: Brenda M
authors: BM
authors: Button