title: Noninvasive long-term ventilatory support for individuals with spinal muscular atrophy and functional bulbar musculature.
abstract: Ten individuals with spinal muscular atrophy (SMA) and chronic ventilatory insufficiency were trained in the use of assisted coughing techniques and received intermittent positive pressure ventilation (IPPV) via oral and/or nasal interfaces for a mean of 5.3 (range = 1 to 17) years. During this time they had significantly fewer respiratory complications than before introduction of noninvasive respiratory muscle aids despite the fact that 6 of the 10 went on to require more than 20 hours per day of ventilator use with less than 2 hours of ventilator-free breathing time (VFBT). All except two noninvasive IPPV users had vital capacities (VCs) less than 13% of predicted normal. They could, however, communicate verbally and take nutrition by mouth. All of the patients remained in the community. Five patients were gainfully employed and four were in school. We conclude that noninvasive respiratory muscle aids including noninvasive IPPV and assisted coughing techniques are effective and practical alternatives to tracheostomy for SMA patients with ventilatory failure but functional bulbar musculature.
authors.affiliation: UMD-New Jersey Medical School, Department of Physical Medicine and Rehabilitation, West Orange, USA.
authors.forename: J R
authors.initials: JR
authors.lastname: Bach
authors.forename: T G
authors.initials: TG
authors.lastname: Wang
mesh_terms: D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D003371:Cough / Q000209:etiology; D005260:Female; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation; D008134:Long-Term Care / Q000379:methods; D008137:Longitudinal Studies; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000150:complications*; D011014:Pneumonia / Q000209:etiology; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D014797:Vital Capacity