title: Decline in respiratory function and experience with long-term assisted ventilation in advanced Duchenne's muscular dystrophy.
abstract: We present 17 patients with advanced DMD who required long-term assisted ventilation. Eleven patients used part-time assisted ventilation. Five of the patients received BV and/or M-IPPV or N-IPPV between two and nine years before requiring full-time T-IPPV, while six others initially used part-time T-IPPV. One patient used all three modes before requiring full-time T-IPPV. Mean (+/- SD) FVC and rebreathe PCO2 at the outset of assisted ventilation were 0.62 +/- 0.20 L and 47.4 +/- 7.5 mm Hg, respectively. Clinical features were divided between symptoms suggesting respiratory muscle fatigue and sleep-related disordered breathing. We found that, while useful in early respiratory insufficiency, BV is associated with recurrent aspiration. In our experience, N-IPPV offers the safest and most convenient form of noninvasive ventilation. When the VC has decreased to about 300 ml, most patients will require full-time ventilation; T-IPPV is advised to provide airway access to suction secretions.
authors.affiliation: Chest Medicine and Pediatrics Services, Rancho Los Amigos Medical Center, Downey, CA.
authors.forename: A
authors.initials: A
authors.lastname: Baydur
authors.forename: I
authors.initials: I
authors.lastname: Gilgoff
authors.forename: W
authors.initials: W
authors.lastname: Prentice
authors.forename: M
authors.initials: M
authors.lastname: Carlson
authors.forename: D A
authors.initials: DA
authors.lastname: Fischer
mesh_terms: D000293:Adolescent; D000328:Adult; D006699:Home Care Services; D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications* / Q000503:physiopathology; D012121:Respiration, Artificial; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D014797:Vital Capacity