Physical Medicine Interventions for Duchenne Muscular Dystrophy, the Including Avoiding Invasive Airway Tubes

18 Figure 8 – IAPV Ventilator C. NVS Complications Nasal bridge skin pressure sores are relieved by switching to nasal prongs or pillow interfaces. There can be allergies to the interface material, dry mouth, eye irritation from air leakage, nasal congestion, sinus discomfort, and aerophagia. 25 As for invasive ventila- tion, there can be pneumothoraces but we had only in 1000 ventilator users and over 5000 patient-years of ventilator use. 26 In addition, occasional patients have claustrophobia. Interface selec- tion can minimize these difficulties. Abdominal distension tends to occur sporadically in NVS users. The air usually passes as flatus once the patient gets up into a chair in the morning. When severe, it can increase ventila- tor dependence. Volume cycling can then be switched to pressure assist/control below 25 cm H2O, which is the normal integrity pressure of the valve between the esophagus and the stomach. In exceptional cases, a stomach tube can be placed to “burp”out the air. D. Glossopharyngeal Breathing (GPB) Both inspiratory and expiratory muscle function can be assisted by GPB. 16 GPB can provide an individual with weak inspiratory muscles and no VC with normal breath volumes in the absence of any respiratory muscles at all. This gives ventilator-free breathing in the event of sudden ventilator failure day or night. 16,27 The technique involves the use of the glottis to add to an inspiratory effort by projecting (gulping) boluses of air into the lungs. The glottis closes with each “bolus”. One breath usually consists of 6 to 9 gulps of 40 to 200 ml each. During the training period, the efficiency of the GPB can be monitored by spirometrically measuring milliliters of air per gulp per breath, and breaths per minute (Figure 9). Training manuals 28 and numerous videos are available (scan QR code), 29 the best of which was in 1999. 30 Although severe oropharyngeal (throat) muscle weakness may limit the usefulness of GPB, in one center 13 DMD CNVS users with no breathing muscle function could only breathe without the ventilator by GPB.31 Approximately 60% of ventilator users with no ability to breath and good throat muscle function can