To address the disparities that exist between the various specialist centres in the UK, a neuromuscular expert group made up of pulmonologists, neurologists, physiotherapists and patient representatives has drawn up good practice recommendations for the routine and emergency respiratory management of people with Duchenne muscular dystrophy (DMD). The recommendations have been endorsed by the British Thoracic Society and highlight several key actions:
- respiratory assessment from the age of 6 with spirometry in the sitting and supine positions,
- regular assessment of coughing and swallowing;
- the introduction of non-invasive ventilation as soon as forced vital capacity (FVC) falls below 50%;
- early treatment of respiratory infections with respiratory physiotherapy and antibiotics,
- implementation of an action plan in the event of a respiratory emergency;
- easier access to emergency assisted ventilation and intensive care.
