Abstract
The increased morbidity and mortality resulting from respiratory failure in patients with neuromuscular disorders and/or kyphoscoliosis can be reversed with non-invasive ventilation. Spontaneous mode bilevel pressure ventilation is preferred to other modes of ventilation, due to relative ease of use, but may not be suitable for all patients. We report a 27-year old woman with Multi-minicore disease whose respiratory failure was refractory to spontaneous mode bilevel pressure ventilation. When we altered settings and provided mandatory inspiratory rise time and respiratory rate, it augmented her respiratory efforts and improved ventilation. Our case report describes the benefit of individualising non-invasive ventilation in the management of respiratory failure due to neuromuscular weakness and kyphoscoliosis.
Highlights
Chronic respiratory failure develops in patients with restrictive ventilatory disorders due to a combination of decrease in inspiratory efforts, reduced minute ventilation and hypoventilation during sleep [1,2].NIV is recommended in these patients as it improves pulmonary mechanics, oxygen saturation, sleep parameters and quality of life [2,3]
We report a patient with Multi-minicore disease, a rare disorder characterized by neuromuscular weakness and kyphoscoliosis with respiratory failure unresponsive to spontaneous mode bilevel pressure ventilation
Many patients progress to respiratory failure and require NIV [4]
Summary
Chronic respiratory failure develops in patients with restrictive ventilatory disorders due to a combination of decrease in inspiratory efforts, reduced minute ventilation and hypoventilation during sleep [1,2].NIV is recommended in these patients as it improves pulmonary mechanics, oxygen saturation, sleep parameters and quality of life [2,3]. Chronic respiratory failure develops in patients with restrictive ventilatory disorders due to a combination of decrease in inspiratory efforts, reduced minute ventilation and hypoventilation during sleep [1,2]. NIV is recommended in these patients as it improves pulmonary mechanics, oxygen saturation, sleep parameters and quality of life [2,3].
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