[Respiratory insufficiency in patients with progressive neuromuscular disease can sometimes be treated effectively with ventilatory support]

Abstract

Three patients with myotonic dystrophy (MD) developed respiratory failure. The first was a 55-year-old man with MD who had been admitted elsewhere with pneumonia and respiratory failure. After discharge he was re-admitted with respiratory failure due to respiratory pump failure. Ultimately, he was given a tracheostomy and was ventilated during the night. The second patient was a 38-year-old man who was admitted to the intensive care unit when he could not be weaned off the ventilator following cholecystectomy for symptomatic gall stones. It appeared that he had partial respiratory failure due to MD and overweight. Ultimately he was able to be weaned during the day with nightly non-invasive ventilation. The third patient was a 55-year-old woman who was admitted with recurrent pneumonia and respiratory failure. She appeared to have chronic respiratory failure due to MD. She was ventilated at night via a tracheostomy. All three patients reported a gradual decline in physical and mental performance in the years preceding this acute episode. All three experienced a remarkable recovery of performance after the institution of mechanical ventilatory support. Respiratory failure that develops over a number of years can be easily missed in aging patients with slowly progressing neuromuscular disease. Timely recognition may lead to improved survival and quality of life by the application of non-invasive ventilatory support.