Long-Term Follow-up of Nocturnal Ventilatory Assistance in Patients with Respiratory Failure Due
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 support for the treatment of respiratory failure in these patients is controversial, noninvasive intermittent ventilatory assistance has been shown to effect sustained stabilization and even reversal of hypoventilation. However, the long-term efficacy of intermittent ventilatory assistance in patients with Duchenne-type muscular dystrophy has not been fully evaluated. In this report, we retrospectively examine the clinical course, further decline in pulmonary function, and changing requirements for ventilatory assistance in eight patients with Duchenne-type muscular dystrophy observed for an average of 39 months after the initiation of intermittent ventilatory assistance.
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