Evaluation of a Training Program for Homozygous Sickle Cell Disease Patients

Evaluation of a Training Program for Homozygous Sickle Cell Disease Patients: Benefits on Physical Ability and Skeletal Muscle. An Interventional Pilot, Multicentric, Prospective, Longitudinal Study

Sickle cell disease (SCD) is the most frequent inherited disease in the world. Literature reports that SCD patients display intolerance to exercise, important muscle weakness and profound remodeling of skeletal muscle including amyotrophy and rarefied microvascular network.

Because strenuous exercise induces acidosis, hemorheological alterations, endothelial activation and oxidative stress, it constitutes a potential triggering factor of sickling and vaso-occlusive crisis. As a consequence, physical activity is usually discouraged in patients with SCD. However, moderate and regular physical activity seems to be not only safe but also beneficial for SCD patients.

Besides, endurance training is known to induce moderate muscle hypertrophy and increase microvascular network. Therefore, adapted, moderate and regular physical activity appears as a potential strategy able to improve muscle function, decrease symptoms of the disease and improve autonomy and quality of life of patients with SCD. However, it remains necessary to define the modalities of exercise therapy in SCD and to objectively evaluate the risks, limitations and gains on physical ability, muscle function and quality of life in patients with SCD.

Inclusion Criteria:

• Sickle cell disease patient (HbSS or HbS-βthal0),
• Affiliated to a Health Security program,
• Consent form signed,
• Patients in stabilized state at the onset of the experiment: at least one month after an acute adverse event and at least 3 months after a blood transfusion.

Exclusion Criteria:

• Patients whom adhesion/compliance to the protocol appears uncertain,
• Patient involved in another clinical trial or within the exclusion period of a previous clinical trial,
• Patients known to be affected by a chronic inflammatory or infectious pathology,
• Patients having an intercurrent infection, especially inflammatory, unsolved since less than one month,
• Patients with clinical signs of heart failure or hospitalized for cardiac decompensation during the past 12 months,
• Patients with left ventricular ejection fraction < 50%, pulmonary arterial hypertension with tricuspid regurgitation velocity > 2.5 m/s, atrial fibrillation, ventricular rhythm disorders during exercise, left ventricular hypertrophy (septal to lateral wall thickness ≥ 10 mm), significant valvulopathy, established coronary disease, uncontrolled hypertension,
• Patients with a treatment against cardiac arrhythmia or altering sino-atrial node activity (beta-blockers, atropine, sympathomimetic agents…),
• Patients under anti-coagulant treatment,
• Patients with pacemaker or defibrillator,
• Body Mass Index (BMI) > 35,
• Patients with hip osteonecrosis,
• Patients with cerebral vasculopathy or history of stroke (cerebrovascular attack) with epilepsy,
• Pregnant or lactating patients,
• Homeless patients,
• Patients with the inability to understand the aims,