Enhancing pulmonary function in women with lymphangioleiomyomatosis through exercise training (VAC9P.1109)

Abstract

Abstract Lymphangioleiomyomatosis (LAM) is a rare disease affecting women almost exclusively. There is no cure for LAM and few treatment options other than immunosuppressive drugs (e.g. sirolimus, everolimus) exist. Rather than attempting to cure LAM, our lab is focused on reversing declines in pulmonary function through aerobic exercise training. A total of 11 patients are enrolled (age from 27 to 59). Following initial assessments of pulmonary function testing, exercise capacity, and bone mineral density, patients underwent individualized, supervised exercise training (2d/wk). Every 3mo patients were reassessed for changes in the above measures. All patients improved in exercise tolerance (time to fatigue, increased work capacity) and in oxygen consumption (VO2max) following training. Most patients showed an improvement in pulmonary function measures (FEV1, FEV1/FVC) and in bone mineral density, both whole body and lumbar vertebrae. FVC increased in several patients. Anecdotally patients reported improvements in daily living tasks (energy levels, decreased dyspnea upon exertion) and quality of life measures (lower levels of depression, greater outlook on life, improved overall mood). This is the first non-drug intervention to show improvements in pulmonary function in this patient population. Although this study is not yet complete, preliminary results suggest that exercise training only two days per week can result in substantial improvements in patients with LAM.