Pain rate and social circumstances rather than cumulative organ damage determine the quality of life in adults with sickle cell disease

Abstract

Due to the significant morbidity associated with sickle cell disease (SCD), sickle cell patients have a reduced quality of life (QoL). Even though pain is considered an important determinant of QoL in sickle cell patients, factors such as organ damage and socioeconomic circumstances may also be important. Therefore, we determined the contribution of chronic organ damage and sickle cell-related complications to QoL and also analyzed the effect of vaso-occlusive crises and socioeconomic circumstances on QoL. Consecutive adult sickle cell patients were included. QoL was represented in a physical component scale (PCS) and a mental component scale (MCS) and assessed with SF-36 forms. Higher pain rates were related to lower QoL scores. Both occupation and the level of education were significantly related to PCS while no relation with MCS or pain rate was found. Thirty-five percent of the patients were unemployed when compared with 6% of the general population and 16% of immigrants without SCD. Neither genotype nor the presence of chronic organ damage were significantly related to QoL. In conclusion, a reduced QoL was mainly determined by pain rate, occupation, and educational level. Chronic organ damage, although a major factor determining life expectancy in SCD, was not a determinant of QoL.