Prevention of pulmonary morbidity for patients with neuromuscular disease

Study objective: To evaluate the effects of a respiratory muscle aid protoc ol on hospitalization rates for respiratory complications of neuromuscular disease. Design: A retrospective cohort study. Methods: A home protocol was developed in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive-p ressure ventilation and manually and mechanically assisted coughing as need ed. The patients who had more than one episode of respiratory failure befor e having access to the protocol were considered to have had preprotocol per iods (group 1). Other patients were given access to the protocol when their assisted peak cough flows decreased to < 270 L/min before any episodes of respiratory distress (group 2). The number of hospitalizations and days hos pitalized were compared longitudinally for preprotocol and protocol access periods (group 1). In addition, avoided hospitalizations were identified as "episodes" of need for continuous ventilatory support and desaturations re versed by assisted coughing that were managed at home. Data were segregated by access to protocol and by extent of baseline ventilator use. Results: Of the 47 group 1 patients with preprotocol periods who have subse quently had episodes, 10 had episodes before requiring ongoing ventilator u se. They had 1.06 +/- 0.84 preprotocol hospitalizations per year per patien t and 20.76 +/- 36.01 hospitalization days per year per patient over 3.42 /- 3.36 years per patient vs 0.03 +/- 0.11 hospitalizations per year per pa tient and 0.06 +/- 0.20 hospitalization days per year per patient with prot ocol use over 1.91 +/- 0.74 years per patient. Of these 47 group 1 patients , 33 eventually required part-time ventilatory aid and, using the protocol as needed, had 0.08 +/- 0.17 hospitalizations per year per patient and 1.43 +/- 3.71 hospitalization days per rear per patient over 3.91 +/- 3.50 year s per patient, as opposed to 1.40 +/- 1.96 hospitalizations per year per pa tient and 20.14 +/- 41.15 hospitalization days per year per patient preprot ocol and preventilator use over 5.89 +/- 6.89 years per patient. Twelve pat ients in group 1 eventually required continuo-us noninvasive ventilation an d, using the protocol as needed had 0.07 +/- 0.14 hospitalizations per year per patient and 0.39 +/- 0.73 hospitalization days per year per patient ov er 5.35 +/- 5.10 years per patient by comparison with 0.97 +/- 0.74 hospita lizations per year per patient and 10.39 +/- 8.66 hospitalization days per year per patient over 2.18 +/- 1.91 years per patient preprotocol and preve ntilator use. For the 94 patients overall when having access to the protoco l, 1.02 +/- 0.99 hospitalizations per year per patient were avoided by 14 I patients before requiring ongoing ventilator use over 4.82 +/- 1.61 years, 0.99 +/- 1.12 hospitalizations per year per patient were avoided by 73 par t-time ventilator users over 3.21 +/- 3.15 years, and 0.80 +/- 0.85 hospita lizations per year per patient were avoided by 31 full-time ventilator user s over 4.78 +/- 4.88 years. All preprotocol and protocol rate comparisons w ere statistically significant at p < 0.004. Conclusion: Patients have significantly fewer hospitalizations per rear and clays per year when using the protocol as needed than without the protocol . The use of inspiratory and expiratory aids can significantly decrease hos pitalization rates for respiratory complications of neuromuscular disease.