Muscle dysfunction is associated with poorer health-related quality of life in adults with sickle cell anaemia.

Abstract

It is known that episodes of microvascular obstruction and oxidative stress in sickle cell anaemia (SCA) can damage muscle tissue. As a consequence, deterioration in muscle function may potentially contribute to poor health-related quality of life (HRQoL) in subjects with SCA, particularly those who do not use long-term treatment. To evaluate muscle function in adults with SCA, to study the correlations between muscle function and HRQoL and to analyse the impact of hydroxyurea treatment. Twenty-two adults with SCA and 20 matched controls were subjected to Short Form-36 (SF-36), respiratory muscle strength measurement, isometric hand grip strength (iHGS) measurement and knee isokinetic dynamometry. In relation to their healthy peers, adults with SCA had lower SF-36 scores, respiratory muscle strength and iHGS. Regarding the isokinetic test, adults with SCA showed lower values, especially in the variables measured in flexion and with an angular velocity of 240∘/s. There was a significant correlation between the peak torque (PT) at 240∘/s and the physical component summary (SF-36PCS) in both extension (r= 0.77; p< 0.001) and flexion (r= 0.82; p< 0.001). Significant correlations were also observed between the agonist/antagonist ratio at 240∘/s and the SF-36PCS (r= 0.50; p< 0.001). The use of hydroxyurea led to higher scores on the SF-36 and higher values in knee isokinetic dynamometry. Adults with SCA have muscle dysfunction, especially with regard to endurance of the knee flexor muscles. In these patients, there is a significant association between muscle function and HRQoL. Moreover, the use of hydroxyurea is associated with better HRQoL and less muscle dysfunction.