Introduction to rehabilitation of neuromuscular disorders.
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Biomedical subjects
Publications and source records attributed to J R Bach.
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The great majority of individuals with ventilatory failure due to paralytic syndromes can be managed entirely by noninvasive inspiratory and expiratory muscle aids. When the equipment is properly used, individuals may eventually become dependent on noninvasive IPPV 24 hours a day without ever being hospitalized, intubated, tracheostomized, or bronchoscoped. The use of noninvasive respiratory muscle aids eliminates the need for "crisis" decision making about whether or not to "go on a respirator." Use of respiratory muscle aids by ALS patients familiarizes them with ventilator dependency before any decision about tracheostomy needs to be made. These methods enhance quality of life in many ways, including permitting the use of GPB for security in the event of ventilator failure. They can also drastically decrease cost and optimize psychosocial functioning. These techniques should become part of the therapeutic armamentarium of every physician who treats patients with generalized neuromuscular disorders who have the potential to develop ventilatory insufficiency, mucus plugging, or both.
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Intermittent positive pressure ventilation (IPPV) delivered via nasal access can normalize alveolar ventilation for individuals with chronic alveolar hypoventilation (CAH) due to neuromuscular disease, spinal cord injury, or skeletal deformity. The purpose of this study was to evaluate the effect of nasal IPPV (NIPPV) air leakage-associated oxyhemoglobin desaturations (dSATs) on the sleep efficiency of kyphoscoliotic individuals with severe pretreatment nocturnal dSATs. Only individuals using nocturnal NIPPV without supplemental oxygen therapy were studied. Seven such individuals were able to maintain PaO2 greater than 60 mm Hg without supplemental oxygen therapy (five had been using oxygen therapy in the pretreatment period), had fewer hospitalizations, and had improvements in symptoms, arterial blood gas values, and nocturnal oxyhemoglobin saturation (SAT) by nocturnal NIPPV. This occurred despite polysomnographically observed sleep disruption and sleep stage changes associated with frequent transient dSATs and massive insufflation leakage. Arousals and dSATs were most frequent during rapid eye movement (REM) sleep with the latter occurring at a frequency of 10/h. The dSATs resulted in brief arousals or lightening of sleep stage 76% of the time. With or without arousal, central nervous system mediated reflex muscular activity occurred to diminish leak and normalize SAT. We conclude that the effectiveness of nocturnal NIPPV is dependent in part on central mediated muscular activity.
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Ventilator users whose airway secretions can be effectively cleared do not require intubation or tracheostomy for ventilatory support, despite possibly having no measurable vital capacity and no significant ventilator-free breathing time (VFBT). Likewise, ventilator users receiving intermittent positive pressure ventilation (IPPV) via an indwelling tracheostomy can be safely decannulated and converted to the use of noninvasive ventilatory support methods provided that a minimum of 3 L.s-1 of peak cough expiratory flow (PCEF) can be achieved by unassisted coughing or by the use of manually- or mechanically-assisted coughing techniques. The use of up to 24 h.day-1 noninvasive ventilatory support is preferred by patients and caregivers over tracheostomy IPPV, and is less costly, and appears to be associated with fewer long-term complications.
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The purpose of this study was to determine longitudinal changes in nocturnal blood gas tensions in individuals with stable traumatic tetraplegia. Ten individuals initially evaluated at least 6 months postinjury were reevaluated 5 years later. When initially evaluated, all the patients had normal daytime blood gases. Six (60%), however, had nocturnal oxyhemoglobin desaturations (dSATs) below 90%, 3 (30%) had mean nocturnal oxyhemoglobin saturation (SAT) below 90% for 10% or more of at least 1 hour, and 4 had maximum end-tidal carbon dioxide tensions (EtCO2) greater than 45mmHg. At 5-year follow-up evaluation, then a mean of 11.6 +/- 5.7 years postinjury, the vital capacities (VCs) of these individuals had improved significantly by a mean of 1242 +/- 545mL without treatment, and daytime blood gases were still normal for 9 out of 10 patients. Five of the individuals (50%), however, had an increased number of transient nocturnal dSATs and 8 of 9 (88%) re-studied by capnography were hypercapnic. We conclude that nocturnal dSAT and hypercapnia appear to be common in tetraplegia and may increase as the patient ages, despite significant increases in VC. Oximetry and capnography are useful in diagnosing nocturnal dSAT and in monitoring treatment.