Resp Care
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Most children with neuromuscular disease eventually require assistance with airway clearance and with breathing, especially during sleep. Techniques and devices for airway clearance and noninvasive ventilation that are commonly used in adults have been successfully adapted for use in infants and young children. Both physiological differences and small size of young patients with neuromuscular disease, however, can limit the applicability of such interventions or require special consideration. ⋯ The paucity of nasal interfaces and headgear commercially made for small patients can reduce patient tolerance of noninvasive ventilation and exacerbate patient-ventilator dyssynchrony. Despite these issues, a greater number of children with neuromuscular diseases are living well past their second decade. Strategies to transition these patients to appropriate adult-care providers, to secure cost-effective health care for them, and to help integrate them into adult society must be developed.
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Noninvasive support of ventilation is commonly needed in patients with neuromuscular disease. Body ventilators, which are used rarely, function by applying intermittent negative pressure to the thorax or abdomen. More commonly, noninvasive positive-pressure ventilation (NPPV) is used. ⋯ Selection of settings can also be based on the results of physiologic studies or sleep studies. The use of NPPV in this patient population is likely to expand, particularly with increasing evidence that it is life-prolonging in patients with diseases such as amyotrophic lateral sclerosis. Appropriate selection of equipment and settings for NPPV is paramount to the success of this therapy.
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The clearance of airway secretions from the lungs is normally supported by the mucociliary escalator and by cough. These protective mechanisms provide an effective means of pulmonary-hygiene maintenance in healthy individuals. Patients with neuromuscular disease that affects the respiratory pump (the muscles of breathing) can experience mild to profound limitation in both ventilation and cough. ⋯ Unfortunately, the need to support cough clearance is not always addressed, and few guidelines for the management of cough insufficiency have existed until relatively recently. An understanding of neuromuscular respiratory pathophysiology and the modes of effective noninvasive cough support are key in the evaluation and management of neuromuscular diseases. This review is meant to provide a basic understanding of cough mechanics, and the pathophysiology and management of neuromuscular cough insufficiency.
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Respiratory dysfunction is a major cause of morbidity and mortality in spinal cord injury (SCI), which causes impairment of respiratory muscles, reduced vital capacity, ineffective cough, reduction in lung and chest wall compliance, and excess oxygen cost of breathing due to distortion of the respiratory system. Severely affected individuals may require assisted ventilation, which can cause problems with speech production. Appropriate candidates can sometimes be liberated from mechanical ventilation by phrenic-nerve pacing and pacing of the external intercostal muscles. ⋯ An important clinical problem in SCI is weak cough, which causes retention of secretions during infections. Methods for secretion clearance include chest physical therapy, spontaneous cough, suctioning, cough assistance by forced compression of the abdomen ("quad cough"), and mechanical insufflation-exsufflation. Recently described but not yet available for general use is activation of the abdominal muscles via an epidural electrode placed at spinal cord level T9-L1.