How Sickle Cell Disease Impairs Skeletal Muscle Function: Implications in Daily Life

Sickle cell disease (SCD) is the most frequent life-threatening genetic hemoglobinopathy in the world and occurs due to the synthesis of abnormal hemoglobin S (HbS). HbS-containing red blood cells (RBCs) are fragile, leading to hemolysis and anemia, and adhere to the endothelium, leading to hemorheological and hemodynamical disturbances. In its deoxygenated form, HbS may polymerize, leading to sickling of RBCs and potentially to vaso-occlusive crises. Recent findings observed that sickle cell disease patients demonstrate significant skeletal muscle remodeling and display reduced muscle functional capacities, contributing to exercise intolerance and poor quality of life. While acute high-intensity exercise is not recommended for sickle cell disease patients as it may increase the risk of sickling, regular moderate-intensity physical activity could have beneficial effects on skeletal muscle and more generally on the well-being of sickle cell disease patients. This paper reviews the literature regarding the impact of the disease on muscular tissue characteristics and function, as well as the corresponding implications for SCD patients' quality of life. *These authors contributed equally to this study Corresponding author: Laurent A. Messonnier, Université Savoie Mont Blanc, Campus Universitaire Savoie Technolac, F-73376 Le Bourget du Lac Cedex, France. Phone: (33) 4-79-75-81-15, Fax: (33) 4-79-75-81-85. e-mail: laurent.messonnier@univ-smb.fr The results of the studies referenced in this review are presented clearly, honestly, and without fabrication, falsification, or inappropriate data manipulation. The results of the present study do not constitute endorsement by the American College of Sports Medicine. This study was supported by a grant from the University Savoie Mont Blanc (n° DrépH_AAP2017). The funder had no role in study, decision to publish, or preparation of the manuscript. No conflicts of interest, financial or otherwise, are declared by the authors. Accepted for Publication: 30 July 2018. © 2018 American College of Sports Medicine

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