Dr Anita Simonds, consultant in thoracic medicine and her team provide home ventilation support for adults and children with neuromuscular and chest wall conditions such as muscular dystrophy and curvature of the spine. There are more than 1,000 patients in this programme, making it the largest in Europe.
For adults and children with neuromuscular and chest wall conditions such as muscular dystrophy and curvature of the spine, the outlook was bleak until relatively recently. Non-invasive ventilation using small portable ventilators at night, extends survival, reduces hospital admissions and improves quality of life.
Even in common disorders such as chronic obstructive pulmonary disease (COPD), non-invasive ventilation can be used to improve the outcome from acute infections. One of our patients is a travel writer who has travelled the length of India with his small portable ventilator; another has spent time studying mountain ranges and climatology in Ecuador.
Many muscular dystrophy and spinal muscular atrophy patients are students away at college. Several of our patients have recently had babies despite using ventilators at home – so much is possible! We are currently exploring new types of ventilators, which may be easier to use for patients with problems such as motor neurone disease.
Royal Brompton Hospital has an active programme of research into the treatment of respiratory problems in children and adults with progressive neuromuscular disorders, such as Duchenne muscular dystrophy.
Breathing problems in these patients first appear during sleep and patients often need non-invasive ventilation to support their breathing at night-time. In a recent study, Dr Simonds and her team investigated the best time to introduce nocturnal ventilation. They showed benefit in starting treatment before the disease has progressed to cause daytime breathing problems (when treatment is routinely started). This earlier use might also reduce hospital admissions for respiratory crises.
Read about our research at the NHLI.