Introduction Patients with myotonic dystrophy (MD) may present acutely with respiratory failure due to muscle weakness and aspiration pneumonia. We are not aware of published data regarding the outcome of intubation and ventilation in these circumstances.
Method We performed a retrospective case notes review of patients referred to a specialist respiratory support centre with a diagnosis of MD. In this abstract we present the outcomes of patients who had received invasive mechanical ventilation and were transferred as in-patients to our unit.
Results Between 1996 and 2010, 22 patients (seven men), mean age 39 years, who had all been intubated and ventilated for a minimum of 2 weeks, were referred. At transfer, 16 patients were tracheostomy ventilated, two were on non-invasive ventilation (NIV) and five were self-ventilating but had on-going CO2 retention. Where specified, admission to ICU was precipitated by pneumonia (16), sudden collapse (2) and cardiac failure (2). The mean duration of invasive ventilatory support was 67 (range 17–196) days. One patient died in our unit. Of those discharged, 11 were on NIV, five still required tracheostomy ventilation and six were ventilator independent. Five patients required a mini-trach tube for access to secretions. The mean length of stay in the weaning unit was 35 (range 2–95) days. Mean survival post discharge was 44 (range 1–102) months. In the same period 53 patients with MD who had not been intubated and ventilated were initiated on NIV for ventilatory failure in our unit. Mean survival in this group was 56 (range 1–144) months.
Conclusion Patients with MD may require prolonged invasive ventilation when presenting with acute ventilatory decompensation. In this series the majority weaned from invasive mechanical ventilation and treatment including NIV was associated with survival on average of over 3.5 years. The prognosis may not be as good as for those patients with MD starting NIV who have not required prior intubation.