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

Medicine and Science in Sports and Exercise
Angèle N MerletLaurent André Messonnier


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.


Apr 15, 1978·European Journal of Applied Physiology and Occupational Physiology·E A BorgholsA P Hollander
Feb 1, 1991·The American Review of Respiratory Disease·P PianosiA L Coates
Jul 15, 1991·Annals of Internal Medicine·J Valeriano-Marcet, L D Kerr
Jul 1, 1989·Journal of Clinical Pathology·B J HuntM Brozovic
Sep 24, 1987·The New England Journal of Medicine·J A KarkC J Ruehle
Nov 1, 1993·The American Journal of Medicine·S Mani, T P Duffy
Jan 1, 1994·Physiological Reviews·R H Fitts
Feb 13, 1999·The New England Journal of Medicine·B Malekgoudarzi, S Feffer
Jan 15, 2000·The Journal of Pediatrics·Elizabeth M BardenVirginia A Stallings
Aug 31, 2001·Medicine and Science in Sports and Exercise·F CostesC Denis
May 7, 2002·American Journal of Respiratory and Critical Care Medicine·Leigh A CallahanBernard Gutin
Jun 26, 2002·The American Journal of Clinical Nutrition·Elizabeth M BardenBabette S Zemel
Feb 7, 2004·European Journal of Clinical Investigation·M NahavandiO Castro
Nov 4, 2004·Clinical Journal of Sport Medicine : Official Journal of the Canadian Academy of Sport Medicine·Michael F BergeronAbdullah Kutlar
Oct 8, 2005·Journal of Applied Physiology·Raymond C BrowningRodger Kram
Mar 1, 2006·European Journal of Applied Physiology·Philippe ConnesOlivier Hue
Sep 21, 2006·American Journal of Hematology·Hisham MoheebMahmoud S El-Sayed
Mar 24, 2007·American Journal of Respiratory and Critical Care Medicine·Anastasia AnthiMark T Gladwin
May 7, 2008·Journal of Pediatric Hematology/oncology·Bibhuti B DasAshok Raj
Nov 21, 2008·The New England Journal of Medicine·Mark T Gladwin, Elliott Vichinsky
Nov 17, 2009·American Journal of Physiology. Heart and Circulatory Physiology·Lucile VincentLaurent André Messonnier
Mar 24, 2010·Genetics and Molecular Research : GMR·G TintiC R B Domingos
Jan 14, 2011·Journal of Pediatric Hematology/oncology·Kelly A DoughertyBabette S Zemel
Feb 24, 2011·Journal of Nursing Scholarship : an Official Publication of Sigma Theta Tau International Honor Society of Nursing·Suzanne Ameringer, Wally Smith
May 17, 2011·British Journal of Haematology·Edwige Balayssac-SiransyPascal Bogui
Nov 9, 2011·Nature Medicine·Holger K Eltzschig, Tobias Eckle
Dec 2, 2011·IUBMB Life·Erica N Chirico, Vincent Pialoux
Feb 11, 2012·Journal of Applied Physiology·Erica N ChiricoVincent Pialoux
Mar 15, 2012·Clinical Hemorheology and Microcirculation·Xavier WaltzPhilippe Connes
Nov 6, 2012·American Journal of Physiology. Heart and Circulatory Physiology·Dhananjay K KaulXiaoqin Zhang
Mar 19, 2013·British Journal of Haematology·Philippe ConnesFrançoise Bernaudin
Apr 6, 2013·Journal of Applied Physiology·Laurent André MessonnierGeorge A Brooks
Jul 23, 2013·British Journal of Haematology·Bruce A OngJulian L Allen
Jan 1, 2014·Journal of Pediatric Oncology Nursing : Official Journal of the Association of Pediatric Oncology Nurses·Suzanne AmeringerWally Smith
Mar 26, 2014·Blood·Carol A RowleyHans Ackerman
Mar 29, 2014·Brazilian Journal of Physical Therapy·Daniela G OharaMauricio Jamami
Mar 17, 2015·The American Journal of Pathology·Marion RavelojaonaLaurent André Messonnier
Mar 31, 2015·Revista brasileira de hematologia e hemoterapia·Camila Tatiana ZanoniSara Teresinha Olalla Saad
Jun 20, 2015·Journal of Nutritional Science·Patrice L CapersJacqueline M Hibbert
Dec 17, 2015·Sports Medicine·Erica N ChiricoVincent Pialoux
May 12, 2016·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Lindsay S CahillJohn G Sled
Jan 4, 2017·Sports Medicine·Sébastien Ratel, Anthony J Blazevich
Jan 23, 2017·Blood Cells, Molecules & Diseases·Benjamin ChatelDavid Bendahan
Apr 20, 2017·The New England Journal of Medicine·Frédéric B PielDavid C Rees
Jun 10, 2017·Pediatric Blood & Cancer·Robert I LiemAlexis A Thompson
Jun 19, 2017·Blood Cells, Molecules & Diseases·Emmanuelle CharrinCyril Martin
Sep 8, 2017·American Journal of Physiology. Regulatory, Integrative and Comparative Physiology·David BendahanThomas Jue
Apr 6, 2018·Journal of Pediatric Hematology/oncology·Kelly A DoughertyVirginia A Stallings


Nov 26, 2019·Family & Community Health·Cynthia W KarlsonMichael A Welsch
Jul 19, 2020·American Journal of Hematology·Angèle N MerletEXDRE collaborative study group
Dec 11, 2020·Journal of Applied Physiology·Benjamin ChatelLaurent André Messonnier

Related Concepts

Self Care (Rehabilitation)
Anemia, Sickle Cell
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