Mouthpiece ventilation in neuromuscular disease

Introduction: Ventilation with mouthpiece is used since 1990 and there are evidence in literature documenting effectiveness of treatment and increase compliance by the patient. Despite this, there is little knowledge and praticality of the use of non invasive mechanical ventilation (NIV) via a simple mouthpiece. Objective: To evaluate feasibility of mouthpiece ventilation (MPV) in patients with different neuromuscular disease who require NIV and were non compliant. Methods: we treated 3 patient with amyotrophic lateral sclerosis, 1 with Steiner syndrome, 3 with Duchenne dystrophy, 2 with quadriplegia due to spinal cord lesions. All patients were non compliant to NIV and previously refused NIV for claustrophobia, skin breakdowun, conjunctivitis and gastric distension. Each patient was reassessed and was proposed a new trial in NIV with the mouthpiece. Results: All patients tolerated treatment with MPV. The preferred mode was pressure control, inspiratory pressure was set beetween 10- 16, wich ensured an optimal tidal volume (8-10 mL/kg); no back up rate is needed for daytime use so no air blows into the patient9s face. Two patients presented sleep apoea syndrome with secondary respiratory failure, during night time were treated with spontaneous timed mode. Discussion: The mouthpiece should alvays be considered for patient with neuromuscular disease that has to start NIV; it is useful to promote a positive approach and a rapid acceptance of the new condition; it should be considered for patients poorly tolerant to NIV or use NIV many hours a day sometimes alternatin with the nasal or fullface mask. In our experience MPV alone or combined with other interfaces improves the quality of patient9s life and promotes greater adherence to NIV.