P125 The effect of glucocorticoids on rate of lung function decline in Duchenne Muscular Dystrophy patients receiving Non-Invasive Ventilation

Abstract

Abstract Background Duchenne Muscular Dystrophy (DMD) causes progressive muscle weakness as a result of a mutation in the X-linked gene dystrophin. Recently glucocorticoid treatment has delayed deterioration in muscle strength prolonging survival. When respiratory failure develops, Non-invasive ventilation (NIV) is used to improve symptoms and prolong life. Recent research suggests the use of glucocorticoids with concomitant NIV may be associated with accelerated lung function decline. Methods To evaluate this we conducted a retrospective analysis of serial lung function data in patients with DMD across two healthcare campuses in Melbourne, Australia. A database of all patients with a coded diagnosis of DMD was collated from 1980-2010. Patient records were examined to collect data on age, lung function (FEV1/FVC/FER), glucocorticoid use (y/n), daily glucocorticoid dose, date of NIV initiation, FVC at NIV commencement, date of death (where applicable) and concomitant diagnosis of scoliosis or cardiomyopathy. This data was analysed to see if a trend for accelerated lung function decline with the use of NIV was observed. Progress to date A total of 356 patients were identified of which 36 had transitioned to the Austin Hospital adult Ventilation Service with available serial lung function data. There was no statistical difference in mean rate of decline in lung function post NIV initation (n=26, p=0.527) with glucocorticoid treatment. Intended outcome and impact Our inital results are in line with the previously observed rates of decline in lung function from other studies and do not indicate a negative synergistic effect on NIV with glucorticoid therapy.