Objective: To report the use of inspiratory muscle strength training to treat repeated ventilatory insufficiency in a child with nemaline myopathy who underwent cardiac and renal transplantation.
Design: Case report.
Setting: Pediatric intensive care unit of a tertiary care university teaching hospital.
Patient: A 16-yr-old female with nemaline myopathy affecting cardiorespiratory function, recent organ transplantation, and delayed postoperative ventilatory recovery.
Intervention: Inspiratory muscle strength training was provided 5 days weekly for 2 wks, accompanied by progressive weaning from noninvasive ventilation.
Measurements and main results: Maximal inspiratory pressure increased from -36.7 cm H2O to -77.8 cm H2O, accompanied by improved inspiratory flow, volume, pressure activation and power. During the training period, the patient was weaned from continuous noninvasive ventilatory assist to her preoperative level of ventilatory function.
Conclusion: Inspiratory muscle training may be a beneficial component of care for children with nemaline myopathy who experience acute ventilatory insufficiency.