P036 A single-service audit of non-invasive ventilation in myotonic dystrophy

Abstract Introduction Myotonic Dystrophy is a heterogenous, multi-systemic disorder with no cure. Survival is 54-58 years, and the most common cause of death is respiratory insufficiency, treated principally by Non-invasive Ventilation (NIV). We aim to characterise service delivery and map to consensus-based treatment recommendations in Myotonic Dystrophy patients treated by a hospital sleep disorders centre from 2004-2024. Methods Demographic, diagnostic, treatment and outcome data was extracted for 68 patients. Service flow was described, and indications and interventions mapped to “Consensus-Based Care Recommendations for Pulmonologists Treating Adults with Myotonic Dystrophy Type 1.” Pooled, compliance-dependent and time-trend survival, and contributors to BiPAP-indication and mortality were assessed. Preliminary survival data from a subset of participants is reported here. Results Patients and their treatment pathways were heterogeneous. PAP-prescription in 62 patients (43 BiPAP, 16 CPAP) was 85% compliant with consensus-based initiation recommendations. Preliminary data indicates treatment uptake and adherence in ~50% of the cohort. Age-of-onset, lung-function, PaCO2 and nocturnal SpO2 contributed to BiPAP recommendation and mortality. Preliminary data indicates that ≥4 hours/day adherence does not improve survival and risk of mortality has not reduced over time with guideline introduction. Discussion Preliminary data suggests that ≥4 hours/day NIV adherence does not prolong survival in Myotonic Dystrophy. Whilst NIV is credited with increasing life-expectancy in other neuromuscular disorders, there was no change in time-trend survival in this study. Further analysis and additional research on treatment response is warranted to evaluate the effectiveness of NIV in this group.