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How Sickle Cell Disease Impairs Skeletal Muscle Function

Implications in Daily Life

MERLET, ANGÈLE N.1; CHATEL, BENJAMIN2,3; HOURDÉ, CHRISTOPHE2; RAVELOJAONA, MARION1,4; BENDAHAN, DAVID3; FÉASSON, LÉONARD1,2,3,4; MESSONNIER, LAURENT A.2

Medicine & Science in Sports & Exercise: January 2019 - Volume 51 - Issue 1 - p 4–11
doi: 10.1249/MSS.0000000000001757
CLINICAL SCIENCES

ABSTRACT 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). hemoglobin S-containing red blood cells (RBC) 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 red blood cells and potentially to vasoocclusive crises. Recent findings observed that SCD patients demonstrate significant skeletal muscle remodeling and display reduced muscle functional capacities, contributing to exercise intolerance and poor quality of life. Although acute high-intensity exercise is not recommended for SCD patients because 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 SCD patients. This article 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.

1Inter-university Laboratory of Human Movement Sciences, University of Lyon, UJM Saint-Etienne, Saint-Etienne, FRANCE;

2Inter-university Laboratory of Human Movement Sciences, University of Savoie Mont Blanc, Chambéry, FRANCE;

3Aix-Marseille University, Marseille, FRANCE; and

4Myology Unit, Department of Clinical and Exercise Physiology, University Hospital of Saint-Etienne, Saint-Etienne, FRANCE

Address for correspondence: Laurent A. Messonnier, Ph.D., Université Savoie Mont Blanc, Campus Universitaire Savoie Technolac, F-73376 Le Bourget du Lac Cedex, France; E-mail: laurent.messonnier@univ-smb.fr.

A. N. M., B. C., L. F., and L. A. M. contributed equally to this study.

Submitted for publication March 2018.

Accepted for publication July 2018.

© 2019 American College of Sports Medicine