Aims Duchenne’s muscular dystrophy (DMD) is a progressive degenerative muscular disorder that leads to respiratory failure in early adulthood. Our aim was to assess the impact of the development of a multi-disciplinary neuro-respiratory clinic and recent guidelines upon the management of patients with DMD.
Methods In February 2009, data was collected and analysed from the hospital’s database of all children with DMD. Following on from this, a multi-disciplinary neuro-respiratory clinic was established. Data was re-collected and analysed in December 2012. Data was collected on the following: age, wheelchair confinement, ventilation support, pulmonary function test results and sleep study results.
Results In 2009, 47 patient were analysed, 22 were confined to a wheelchair, only 6 were under the care of a respiratory paediatrician. 20 patients had undergone lung function testing for one of the following reasons: prior to transition to adult services, as a work up for spinal surgery, subsequent to symptoms or at parent’s request. The 3 patients who were receiving long term-ventilation were under care of a respiratory physician, although 4 further children were suffering from respiratory symptoms. In December 2012, 41 children were on the database, 14 were confined to a wheelchair and 2 were receiving long term ventilation. 25 of the 41 patients were old enough for pulmonary function tests and all had had spirometry performed; 16 patients within the last year and 23 patients within the last 2 years. 5 patients had a FVC <50% and all had oxygen saturation/carbon dioxide sleep studies performed. 4 patient’s sleep studies were normal and the patient whose study was abnormal was commenced on a ventilator.
Conclusion The introduction of a neuro-respiratory clinic has resulted in a significant improvement in the respiratory monitoring of children with DMD, as well as providing them with broader respiratory advice.