Mechanical ventilation in Duchenne muscular dystrophy: A pilot project in Ukraine

Abstract

Background Home mechanical ventilation (HMV) prolongs survival in patients with Duchenne muscular dystrophy (DMD) until ±35 years of age. This study evaluates the implementation of a HMV pilot project in children with DMD in Ukraine. Methods Children with DMD were invited to Kirovograd Regional Children’s Clinical Hospital, Kropyvnytskyi, Ukraine, for 5 days’ training with non‐invasive ventilation. Donated equipment comprised second‐hand Covidien PB560 ventilators from Belgium. Due to the absence of carbon dioxide pressure and pulse oximetry monitoring, indications for HMV included sleep‐related symptoms, restrictive lung function test, loss of ambulation for more than 1 year, or age greater than 17 years. Master class lectures on HMV were conducted for Ukrainian doctors in conjunction with patient training. Results Twelve Ukrainian physicians took part in face‐to‐face master classes and 50 Ukrainian physicians participated in online master classes. Simultaneously, eight Duchenne inpatients, mean age 15.4 (SD: 1.8) years and body mass index 25.8 (SD: 4.0), were included in the study. All patients chose nasal masks and volume‐pressure‐assisted control mode. After 6 weeks, one patient stopped HMV, two others used HMV partially during sleep, and 5/8 used nocturnal HMV increasingly with few complaints. Follow up via phone call was organized after hospitalization. Conclusions Implementation of HMV is feasible in DMD inpatients in Ukraine. In the short term, the Ukrainian parliament should recognize official centers for HMV, and define the funding policy of equipment for HMV, and its maintenance. Local distributors should deliver equipment for HMV and devices for monitoring carbon dioxide pressure and pulse oximetry in specialized centers for HMV.