Symptoms, clinical and physiological findings motivating home mechanical ventilation in patients with neuromuscular diseases.

OBJECTIVE To clarify the relationship between symptoms, clinical signs and physiological abnormalities that were motivating the initiation of home mechanical ventilation in patients suffering from neuromuscular diseases. METHODS From The Swedish Home Mechanical Ventilation Register we identified 352 patients with neuromuscular diseases and we looked at circumstances (acute vs elective) and clinical motives for starting ventilatory support. RESULTS Home mechanical ventilation was commenced electively in 268 patients (76%) and among these daytime sleepiness was the most common motive, being reported in 56% of the patients. In the 24 children with spinal muscular atrophy, however, 96% started ventilation electively and cough insufficiency was the most common motive. The patients were moderately hypercapnic (PaCO(2): 7.0 kPa, SD 1.3). None of the clinical motives were related to the PaCO(2) level. Average PaO(2) was above 8 kPa in all groups, but lowest in the patients with post-polio and dystrophia myotonica. Mean vital capacity was close to 40% of predicted, but significantly lower in the Duchenne patients (26% of predicted). CONCLUSION Daytime sleepiness was the most common clinical symptom motivating home mechanical ventilation in this group of patients with chronic hypercapnic respiratory insufficiency secondary to neuro/myopathies. Respiratory function testing is therefore suggested to be included in the diagnostic work up of daytime sleepiness in these patients.