Home negative pressure ventilation: report of 20 years of experience in patients with neuromuscular disease.

Twenty years of experience using negative pressure devices (NPD) at home to ventilate 40 patients with neuromuscular disease is presented. The purpose of the study was to determine the costs, complications, and clinical outcome of this form of respiratory support, and to ascertain the reasons for failure to institute effective negative pressure ventilation (NPV) in nine patients. Emerson tank respirators, used mainly to rest respiratory muscles at night, and intermittent positive pressure breathing machines were used by 98% of patients at an average equipment cost of +2,700 annually. Patients in whom NPV was initiated on an elective rather than emergent basis saved an average of +12,000 during their initial hospitalization. Life table analysis shows a five-year survival of 76%, and a 10-year survival of 61%. Complications were minor and occurred at an average rate of less than one per year per patient at home on NPV. Failure to achieve satisfactory NPV in nine patients was associated with age (six patients were younger than 3 years of age), or severe thoracocervical scoliosis, which prevented proper fitting of the NPD. For reasons of safety, economy, and quality of life, NPV at home is the preferred treatment for patients having neuromuscular disease who need respiratory assistance.