The lack of change in parameters of pulmonary function and arterial blood gas values during a period of 11 h of spontaneous breathing in four of our patients indicates that our patients remained stable off the ventilator and that blood gas levels obtained at midday or late during the period of spontaneous ventilation are representative [...]
In our series of eight patients with Duchenne or Duchenne-type muscular dystrophy with respiratory failure, we found, as others have, that nocturnal ventilatory assistance using noninvasive ventilators brought about a sustained reversal of hypoventilation and improvement in symptoms of chronic hypercarbia. The clinical course was stabilized in most patients who required infrequent hospitalizations despite minimal [...]
Following initiation of nocturnal ventilatory assistance, five of our eight patients required only one hospitalization for respiratory problems and one patient had no hospitalizations in more than five years. Patient 6 required repeated hospitalizations because of inability to clear secretions and patient 2 had multiple hospitalizations, several related to his severe underlying cardiomyopathy. Three patients [...]
Table 1 shows age at initiation of assisted ventilation, respiratory parameters at initiation of assisted ventilation and at times of early and extended follow-up and clinical outcome in the eight patients comprising our series. Most patients developed respiratory failure at approximately 20 years of age, as is typical for Duchenne muscular dystrophy. Delayed onset in [...]
Patients were referred for the management of hypercarbia from the Muscular Dystrophy Association Clinic at New England Medical Center. Patients had been diagnosed as having Duchenne or Duchenne-type muscular dystrophy using standard clinical criteria including male sex, elevation of creatine phosphokinase, and a compatible muscle biopsy and clinical picture. All patients were in functional class [...]
Duchenne and Duchenne-type muscular dystrophies are x-linked recessive hereditary disorders characterized by diffuse skeletal and cardiac muscle involvement. They lead inexorably to quadraparesis in the preteen years and death due to respiratory failure at an average age of 20 years or slightly older for the more slowly progressive Duchenne-type dystrophies. Although the use of ventilatory [...]
What’s going to happen to me?” he asked. “The lung doctor says I’m not in good enough shape to be operated on. What chance do I have of beating lung cancer if they don’t operate?” If the question had appeared on a medical board exam, it would have been quite simple: a 60-year-old male who [...]
