Health-related quality of life measurement in adults with sickle cell disease in steady state: Experience of one French reference center

Sickle cell disease (SCD) is a genetic disease of public health concern. Adult patients face various disease-related complications, which affect their quality of life (QoL). Few studies have examined relationships between these events and health-related (HR) QoL. We conducted a study of 240 adults with SCD seen in steady state at a routine clinic visit over one year. Two self-administered questionnaires were used to determine patients’ HRQoL: the sickle cell self-efficacy scale (SCSES) and the unspecific SF-36 scoring system evaluating physical and mental components. Factors impacting HRQoL were established using univariate and multivariate regression analyses. Participants had a median age of 28 years (Sex ratio male/female 0.61; 68% SS genotype). Most of them had experienced more than one SCD-related complication and more than one affected organ system. A good correlation was established between the SCD specific and the unspecific scoring systems. Using the SF-36 scoring system, energy/fatigue, general health and pain subscales showed the lowest median scores, while physical functioning had the highest median score. In univariate and multivariate analyses, hospitalization for SCD complications occurring during the last year preceding QoL evaluation was the main feature impacting HRQoL. Good compliance to hydroxyurea (HU) therapy was associated with higher SCSES and higher emotional role functioning scores. Our study confirms substantial impairment of HRQoL in adults with SCD. HRQoL was mainly influenced by the recent occurrence of severe SCD complications. Our study suggests that a more effective treatment through a better compliance to HU therapy would provide benefit in terms of QoL.