Evaluation of tolerability, ease of use, perception of mucus encumbrance of an alternative device (FreeAspire®) for management secretions in patients with bulbar Amyotrophic Lateral Sclerosis.

Introduction
Respiratory muscle weakness with increment of pulmonary infection is one of the main causes of mortality and morbidity in patients with Amyotrophic Lateral Sclerosis (ALS). The effectiveness of cough gradually decreased with the increase of the expiratory and bulbar muscle weakness. The indication for mechanical cough assistance is given when the CoughPEF is <270 l/min (but >160 l/min) or a maximal expiratory pressure (MEP) <35%. Mechanical insufflation-exsufflation (MI-E) is used to assist cough mechanically by applying positive and negative pressure changes to the airways. MI-E can be difficult to apply in patients with the bulbar subtype of ALS for a major collapsibility of upper airways. FreeAspire® (FA), when MI-E not applicable, is an alternative non-invasive device. FA allows a soft acceleration of expiratory flow during tidal volume improving non-invasive removal of secretions.

Methods
13 patients with bulbar-ALS (5 with coughPEF < 160 L/min, 8 with intollerance to MI-E) were enrolled to use FA. With telephonic interview we evaluated after 30 days of use through VAS (Visual Analogue Scale: 0 best result, 10 minimum result): tolerability, ease of use for patient and caregiver, perception of mucus encumbrance.

Results
In all patients FA was generally well tollerated (VAS max 2) and easy to use for patients and caregivers (VAS max 1). The perception of mucus encumbrance was between 2 to 8.

Conclusion
In patients with bulbar-ALS the FA is easy to use, well tollerated for the patients and caregivers; the perception of mucus encumbrance is variable.