Impact of sickle cell disease, drug treatments and endurance training on muscle tissue and patient quality of life

Background 

Sickle cell disease (SCD) is the most frequent genetic disease worldwide. SCD is due to synthesis of an abnormal haemoglobin S (HbS instead of HbA). The repercussions of SCD are numerous, sometimes fatal/lethal, and affect almost all tissues and organs. The skeletal muscle is not spared. If the structural, energetic and microvascular remodelling of this tissue is relatively well documented, all muscular alterations due to SCD are not totally identified. Hydrxyurea (hydroxycarbamide) is at the present time the only one authorized medicinal treatment allowing to reduce complications and to increase life expectancy of patients. Recently, regular moderate-intensity exercise has appeared beneficial for SCD patients. Aims. This project aims at i) studying the main mechanisms involved in muscle pH regulation in SCD patients, ii) determining the effect of hydroxyurea on muscle structural, energetic and microvascular characteristics, and iii) highlighting the benefits of an endurance training program on physical ability and quality of life of SCD patients, the ultimate goal of this latter aim is to establish recommendations of physical activity for patients and to include physical activity in the therapeutic strategy of SCD patients.