1. Physical Exercise in Patients with Fabry Disease – a Pilot Study
- Author
-
Boris Schmitz, Jörg Stypmann, Lothar Thorwesten, Eva Brand, Thomas Duning, Malte Lenders, and Stefan-Martin Brand
- Subjects
Adult ,Male ,medicine.medical_specialty ,Future studies ,Adolescent ,Pilot Projects ,Physical Therapy, Sports Therapy and Rehabilitation ,Physical exercise ,Exercise intolerance ,030204 cardiovascular system & hematology ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Activities of Daily Living ,medicine ,Humans ,Orthopedics and Sports Medicine ,In patient ,Lactic Acid ,Muscle Strength ,Young adult ,Fatigue ,Aged ,Exercise Tolerance ,business.industry ,Resistance Training ,030229 sport sciences ,Middle Aged ,Exercise capacity ,medicine.disease ,Fabry disease ,Exercise Therapy ,Physical therapy ,Muscle strength ,Fabry Disease ,Female ,medicine.symptom ,business - Abstract
The aim of this study was to assess the extent of exercise intolerance in Fabry disease (FD) patients and to report individual effects of physical exercise. Exercise capacity and strength of 14 patients (mean age 46 years, 6 females) were determined using cycle ergometry and isokinetic measurements. Patients performed a strength/circuit exercise training protocol for 12 months. The mean relative maximum performance of the group was low at baseline and increased by 12.1% (baseline: 1.9 [0.9−3.4] W·kg −1 vs . re-test: 2.1 [1.1–3.8] W·kg −1 ; p =0.035) during the study. Patients’ mean baseline maximum performance blood lactate of 5.4 [1.3–9.9] mmol·L −1 increased to a mean of 7.2 (2.4–10.2) mmol·L −1 ( p =0.038). Mean strength of the lower limbs (left/right extensors and flexors, total work of 5 sets) changed from 2269 (1017–2913) kg·m 2 ·s - 2 to 2325 (1359–3107) kg·m 2 ·s -2 (not significant). Patients reported increased well-being, daily activity and reduced fatigue during the study. Our results indicate that exercise intolerance in FD patients often results from physical inactivity. FD patients may perform exercise training to improve exercise capacity and muscle strength. Future studies will address the clinical benefits of exercise in FD.
- Published
- 2016