P198 Non-invasive ventilation in the management of type II respiratory failure in myotonic dystrophy

Abstract

Background Myotonic dystrophy (DM) is the most common muscular dystrophy in adults. DM causes respiratory failure due to a combination of respiratory muscle weakness, upper airway obstruction, and reduced central drive. Non-invasive ventilation (NIV) is commonly used for the treatment of type II respiratory failure in patients with neuromuscular disease; however, there have been few studies assessing the efficacy of NIV in DM. The aim of this study was to investigate NIV adherence and the impact of NIV on ventilation and symptoms of hypoventilation in patients with DM. Methods Forty patients with DM type 1 were identified. Retrospective data on capillary blood gas analysis (including partial pressure of carbon dioxide (pCO2)), NIV adherence and symptoms of hypoventilation during initial assessment and follow-up were obtained from patient records. Results On admission for NIV set-up the mean capillary pCO2 was 6.81±1.17 kPa which significantly reduced to 5.93±0.82 kPa on discharge from the unit (p 6.5 kPa prior to starting NIV (n=23) had significantly higher pCO2 on follow-up (p Conclusion Referral of patients with DM type 1 to a regional ventilation service for initiation of NIV results in a sustained reduction in pCO2. However, reduction in pCO2 does not correlate with hours of NIV use. Further studies are required to explore the impact the role of specialist ventilation services and, specifically, of NIV in the management of type II respiratory failure in patients with DM type 1.