Abstract
Background: Duchenne muscular dystrophy (DMD) affects 1 in 3500 births. Without intervention mean survival is 19 yrs. NIV improves survival & patients are now living into their 4th decade. American Thoracic Society has produced best practice consensus statement for the care of DMD patients which includes aspects of respiratory monitoring, secretions management, NIV etc. Home ventilation team at St. James's University Hospital, Leeds, have been looking after patients with DMD since the 1990s.
Aims: We aimed to determine whether guidelines for best practice were being followed and how NIV usage affected patient outcomes.
Methods: Retrospective data was collated from clinical notes and NIV databases.
Results: Mean age of NIV initiation was 18 yrs (n=54).Their most recent mean NIV usage was 13.3 hrs with a mean IPAP of 18. 54% have been on NIV for more than 5 years. 74% had cough-assist device, 3 patients had tracheostomy.All 24 NIV dependent patients had a 2nd ventilator. Echoes have been done for all patients where records could be traced. Median survival was 27 yrs. Patients who have been on NIV for > 5 years were initiated NIV at a younger age (17 vs 20,p=0.03), had higher IPAP (p=0.002) & usage (p<0.001) compared to patients who have either died before 5 years or have not reached 5 years from initiation yet.
Conclusion: This is the first time we have looked at our large cohort of DMD patients and our practice seem to in keeping with best practice guidelines and our median survival is comparable to reported data. Only 3/24 NIV dependent patients were tracheostomy ventilated which might represent a cultural difference from other European countries.
- Copyright ©the authors 2016
