Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease characterized by pulmonary cysts, benign extrapulmonary tumors, and a progressive decline ...
An exercise intervention clinical trial in patients with lymphangioleiomyomatosis (LAM) Thomas W. Lowder, PhD Laura S. Kabiri, PT, MS Background Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease characterized by pulmonary cysts, benign extrapulmonary tumors, and a progressive decline in lung function. There is no cure for LAM and treatment options are limited. Patients are advised to exercise, yet there have been no clinical trials examining how exercise training might improve function in patients with LAM. We have begun a year-long exercise intervention protocol to determine how exercise training can enhance pulmonary function, exercise tolerance, bone mineral density, and body composition in a group of individuals with LAM. The following preliminary results are from the first 12 week follow-up.
Objective
Summary Points
To assess the effects of a supervised exercise intervention on pulmonary function, exercise tolerance, body composition, and bone mineral density in patients with LAM.
• Supervised exercise among patients with LAM improved group mean scores in all outcome measures after just 12 weeks • Per-patient percent change varied widely
Methods
Group Means by Outcome
Three subjects with LAM (sporadic LAM n=2; TSCLAM n=1) were enrolled in the study. Subjects participated in two weekly 45-60 minute sessions of supervised aerobic exercise at 80-85% heart rate max for 12 weeks. Heart rate and oxygen saturation levels were monitored throughout the intervention. Treadmill ergometry (VO2max), spirometry (FEV1, FVC, FEV1/FVC), bone mineral density (BMD), and body composition (% fat) testing was performed prior to the study and after 12 weeks of intervention. Per-Patient Percent Change by Outcome
Results
20 15 10
Percent Change
After 12 weeks of supervised aerobic exercise training, subjects experienced a group mean increase in all areas. The highest group mean increases were seen in FEV1 (+4.2%), VO2max (+2.92%), FEV1/FVC (+2.85%), and whole body BMD (+2.52%). The group mean also showed a decrease in % fat (-5.2%). Per-patient percent change varied widely among individuals.
5 0 -5 -10 -15 -20 Subject 1 Subject 2 Subject 3
VO2max 12.2 17.8 -16.1
FEV1 9.5 1.2 -1.1
FVC 2.8 1.6 -0.8
FEV1/FVC 8.7 0.2 -0.1
BMD 9.7 1.8 -0.4
% Fat -15.2 -0.9 1.2
Conclusions This study indicates that supervised exercise training can improve lung function, exercise tolerance, and bone mineral density in patients with LAM. Further research is needed, including longitudinal studies with larger sample sizes, to determine long-term effects and consistency of these findings. However, aerobic exercise can offer a viable alternative or compliment to pharmacological intervention in the treatment of lymphangioleiomyomatosis.
