The impact of inhibitors on the quality of life in patients with hemophilia

Sezaneh Haghpanah; Majid Naderi; Sepideh Kamalian; Hakimeh Tavoosi; Shirin Parand; Neda Javanmardi; Mehran Karimi

doi:10.1177/20503121231182284

The impact of inhibitors on the quality of life in patients with hemophilia

SAGE Open Med. 2023 Jul 10:11:20503121231182284. doi: 10.1177/20503121231182284. eCollection 2023.

Authors

Sezaneh Haghpanah¹, Majid Naderi², Sepideh Kamalian¹, Hakimeh Tavoosi³, Shirin Parand¹, Neda Javanmardi⁴, Mehran Karimi¹

Affiliations

¹ Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
² Department of Pediatrics, School of medicine, Genetics of Non-Communicable Disease Research Center, Ali Ibne Abitaleb Hospital Research Center, Zahedan University of Medical Sciences, Zahedan, Iran.
³ Shiraz Hemophilia Comprehensive Center, Dastgheib Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
⁴ Student Research committee, Shiraz University of Medical Sciences, Shiraz, Iran.

Abstract

Objective: To investigate the association of health-related quality of life in hemophilia patients with inhibitor and clinical and demographic characteristics.

Methods: In this multi-center cross-sectional study, 41 male patients with hemophilia A were investigated from May to October 2021. All patients were registered at the Hemophilia Clinic affiliated with Shiraz and Zahedan Universities of Medical Sciences in Iran. Health-related quality of life of the patients was evaluated by the Short Form-36 questionnaire.

Results: The patients' mean ± SD of age was 36.9 ± 13.2 (range: 18-76) years. Eleven patients (26.8%) were inhibitor positive. In univariate analysis, physical function, mental health dimension, and total Short Form-36 scores were significantly lower in the inhibitor-positive patients (p < 0.001, p = 0.045, and p = 0.035, respectively). Moreover, patients with severe disease showed significantly lower scores in physical function (p < 0.001), physical health dimension (p = 0.018), and total Short Form-36 (p = 0.031) than those with mild and moderate hemophilia. Also, blood-borne infections showed a significant association with lower score in physical health dimension (p = 0.038). In addition, annual bleeding rate showed significant negative correlations with physical health dimension (r_s = -0.609, p < 0.001), mental health dimension (r = -0.317, p = 0.044), and total Short Form-36 (r = -0.455, p = 0.003) scores. In multiple linear regression analysis, disease severity revealed a significant negative relationship with scores in physical function (p = 0.001), role physical (RP) (p = 0.015), general health (GH) (p = 0.006), physical health dimension (p = 0.006), and marginally in total Short Form-36 score (p = 0.054). Also, age of the patients showed a significant negative association with physical function and GH scores (p < 0.001 and p = 0.015, respectively).

Conclusion: Disease severity and age were shown as independent factors affecting health-related quality of life, but inhibitor alone was not an independent influencing factor. Reduced health-related quality of life was also observed in hemophilia patients with higher annual bleeding rate and blood-borne infections. Therefore, it is necessary to pay more attention to these subgroups. Further studies with larger sample size are needed for more accurate results.

Keywords: SF-36 questionnaire; hemophilia; inhibitor; quality of life.