The standard treatment for patients with neuromuscular respiratory failure is non-invasive ventilation (NIV) as non-invasive ventilation support-setting (NVS). NVS is administered through a nasal or face mask and/or mouthpiece with the potential to cause nasal ulcers, discomfort, and/or aesthetic issues, resulting in poor compliance. We reported the observation of a 45-year-old woman with limb-girdle muscular dystrophy (LGMD), secondary to Dysferlin deficiency, who was on NVS since 2017 for nocturnal hypoventilation. In 2018, despite nocturnal ventilation, due to weight gain and daytime hypoventilation, a nasal mask was introduced. We initiated daytime intermittent abdominal pressure ventilation (IAPV) to mitigate cosmetic problems, improving in pO2 and decreasing in pCO2 versus baseline (52>84 mmHg, 46>33 mmHg respectively) at 6 (85 mmHg, 42 mmHg) and 18 months (93 mmHg, 38 mmHg), respectively. IAPV was effective, safe, and well-tolerated in our patients who did not tolerate standard daytime NVS with the known interface.
- non-invasive ventilation support-setting