The Health-Related Quality of Life scores and joint health in children and young adults with hemophilia

Abstract

Background. Patients with hemophilia should be evaluated for joint health and overall health in their visits. The aims of this study were to evaluate joint health and health-related quality of life (HRQoL) in patients with mild, moderate, and severe hemophilia; determine which patient groups to focus on and whether there are any neglected patient groups. Methods. This was a single-center, cross-sectional study. Patients were evaluated by ultrasonography (Hemophilia Early Arthropathy Detection with Ultrasound [HEAD-US]), physical examination (Hemophilia Joint Health Score version 2.1 [HJHS-2.1]), and HRQoL scales (EQ-5D/EQ-VAS and Haemo-QoL). Results. Thirty-nine patients with regular follow-up were evaluated for a total of 234 joints. When hemophilia severity was compared with the HEAD-US and HJHS-2.1, a significant difference was found between severe and non-severe hemophilia. On the other hand, when patients’ total HEAD-US scores were compared with total HJHS-2.1 scores, no statistically significant correlations were found; only a statistically significant but negligible correlation was detected when HEAD-US and HJHS-2.1 scores were examined at joint level. No significant difference was found when mild, moderate or severe hemophilia were compared with the HRQoL scores. Also, HEAD-US scores and HRQoL scores were not correlated, showing that the HRQoL score did not change whether the patient has arthropathy or not. Conclusion. Despite recent advances in treatment options for hemophilia, arthropathy in patients with severe hemophilia remains challenging. For the follow-up of pediatric hemophilia, the HEAD-US and HJHS should be used together because their correlation was weak. Although patients with severe hemophilia are at higher risk in terms of arthropathy, patients with mild/moderate hemophilia should not be ignored because their HRQoL is not different from that of severe hemophilia.

Keywords

• hemophilia
• hemophilic arthropathy
• health-related quality of life
• HJHS
• HEAD-US