Abstract
While exercise has been shown to improve respiratory symptoms, exercise tolerance, and bone mineral density in many populations, no supervised exercise training interventions have been undertaken in patients with lymphangioleiomyo-matosis (LAM). One patient with TSC-LAM (tuberous sclerosis complex lymphangioleiomyomatosis) participated in two weekly sessions (50-60 min) of supervised aerobic exercise at 80-85% heart rate max for one year. Treadmill ergometry (VO2peak), spirometry (FEV1, FVC, FEV1/FVC, peak flow), and bone mineral density testing were performed prior to every 3 months. After one year of supervised aerobic exercise training we saw dramatic increases in the patient's VO2max (20%), FEV1 (9.5%), FEV1/FVC (9.1%) and peak flow (47%). The results from this study indicate that supervised exercise training can improve exercise tolerance and pulmonary function in a patient with lymphangioleiomyomatosis. Further research is needed, including longitudinal studies with larger sample sizes, to determine long-term effects and consistency of these findings. Aerobic exercise may offer a viable alternative or com-pliment to pharmacological interventions in the treatment of lymphangioleiomyomatosis. We show that high-intensity exercise training can markedly and safely improve pulmonary function in a patient with TSC-LAM. While we did not record quality of life or mood states, our patient did report improved self-confidence as well as enhanced mood.
Highlights
IntroductionApproval was obtained from the Committee for Protection of Human Subjects at the University of Houston and the Institutional Review Board at the University of Texas Health Science Center (UTHSC) and at the University of Central Arkansas
While exercise has been shown to improve respiratory symptoms, exercise tolerance, and bone mineral density in many populations, no supervised exercise training interventions have been undertaken in patients with lymphangioleiomyomatosis (LAM)
Sirolimus targets the mammalian target of rapamycin pathway and has been tremendously effective in improving pulmonary function in the majority of patients with LAM [4]
Summary
Approval was obtained from the Committee for Protection of Human Subjects at the University of Houston and the Institutional Review Board at the University of Texas Health Science Center (UTHSC) and at the University of Central Arkansas. Written informed consent was obtained from our patient prior to data collection. A 29-year-old female with tuberous sclerosis complex lymphangioleiomyomatosis (TSC-LAM; TSC diagnosed in 1988, age 5; LAM in 2004, age 21) with a history of related complications (more than twenty pneumothoraces, resulting in bilateral pleurodesis and pleurectomy; angiomyolipomas resulting in partial nephrecotmy) was referred from the UTHSC LAM Clinic by her LAM specialist. Her last pneumothorax was approximately five years prior to her inclusion in this trial (age 23), when she received her bilateral pleurectomy/pleurodesis. The patient did not have any brain lesions, nor had she ever experienced any episodes of epilepsy
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