A pilot study of inhaled dry-powder mannitol during cystic fibrosis-related pulmonary exacerbation.

Pulmonary exacerbation treatment aims to eradicate increased respiratory symptoms and recover acute loss in lung function. Current treatment strategies remain suboptimal, with conventional intravenous antibiotics and intensive physiotherapy failing to achieve this in 25% of patients [1]. Despite this worrying statistic, optimising recovery from acute pulmonary exacerbations has not been a focus of recent cystic fibrosis (CF) research efforts. There is a lack of adjunct evidence-based therapies for use in this setting [2] and strategies to optimise airway clearance with physiotherapy have been largely overlooked, despite common use in the outpatient setting [3, 4]. Inhaled dry-powder mannitol (IDPM), a mucoactive agent, improves mucociliary clearance [5], mucus rheology, and hydration and surface properties of mucus [6]. In the CF outpatient setting, IDPM treatment improves lung function, both in the short (>2 weeks) and long (>12 months) term [3, 7]. Its utility in in-patient pulmonary exacerbation care is unclear. In this pilot study, we investigated feasibility and safety of IDPM as an adjunct therapy to standard in-patient hospital care for children with pulmonary exacerbation. Efficacy was also explored using both conventional respiratory function outcomes and additional sensitive measures of peripheral airway function.