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

16 IV. STAGE 3- PROLONGING LIFE WITH QUALITY ANDWITHOUT AIRWAY TUBES Continuously Maintain Normal Pulmonary Ventilation When symptoms such as morning headaches, fatigue, sleep disturbances, and daytime sleepiness develop, 20 and there is evidence of weak respiratory muscles such as VC less when lying down than when sitting, the third stage begins with sleep NVS. The Inspiratory Muscle Aids A. Noninvasive Positive Pressure Ventilatory Support (NVS) For the majority of patients, NVS is introduced in the clinic or home settings. It can be delivered via mouthpieces, nasal, and oral-nasal interfaces for daytime or sleep NVS. The mouthpiece (Figure 6) and nasal NVS (Figure 7) are open systems used for daytime support while nasal NVS is also used for sleep. These open systems require reflex brain activity to prevent excessive insufflation air leakage, especially during sleep. Thus, it is critical to avoid supplemental O2 and sedatives 21 which can render NVS ineffective. There are 15 mm angled mouthpieces like that seen in Figure 6 and straw-style mouth interfaces equally effective for daytime NVS. To use mouthpiece NVS conveniently, adequate neck rotation and oral function are necessary to grab it. There are also numerous commercially available nasal interfaces like “CPAP masks” covering the nose, nasal prongs, and nasal pillows interfaces (Figure 7). Each design applies pressure differently to the paranasal area. Nasal bridge pressure and insufflation leakage into the eyes are common symptoms with several models. Such difficulties are avoided by prongs and pillows. 20,22,23 No one should be offered or expected to use only one nasal interface. Figure 6–Forty-eight-year- old man with Duchenne muscular dystrophy who used 24-hour NVS, via mouthpiece when awake and via nasal interface for sleep for 25 years, even without any measurable VC. The mouthpiece is secured adjacent to the mouth by a flexible metal support arm (microphone stand).