Mouthpiece ventilation in Duchenne muscular dystrophy

Introduction: Patient affected by Duchenne muscular dystrophy does not accept easily the noninvasive mechanical ventilation, due to claustrophobia or anxiety of not being able to call a family member. In our experience appropriate interface selection is crucial for successful NIV. Nasal and oro-nasal masks are the most practical as well as the most commonly used for the NIV. The main limitations of these interfaces are claustrophobia, discomfort and skin lesions. Methods: We evaluated six young patients with Duchenne muscular dystrophy, mean age 18,5 y, who previously had not toleraed NIV. Each patient was reassessed and was proposed a new trial in NIV with the mouthpiece (MPV). Results: All patients tolerated treatment with NIV/MPV. After an initial phase of adaptation to the ventilation mouthpiece during daytime hours, two patients also accept ventilation with nasal mask during the night hours. Conclusion: The use of this interface and MPV modality has allowed us to treat patients who had previously refused nasal, oral or oro-nasal interface for tightness or claustrophobia. Two patients who required NIV overnight, subsequently accepted also the treatment by nasal mask during the night hours. This probably because the use of ventilation mouthpiece during daytime hours improved the feeling of patient safety. This interface should always be considered for patients that has to start NIV; it is useful to promote a positive approach and rapid acceptance of the new condition. Mouthpiece can be very valuable, particularly in patients who use the NIV many hours a day and presenting skin lesions, gastric distension or eye irritation, sometimes alternating with the nasal mask mouth and nose.