In a study published in the Journal of Pediatric Hematology/Oncology, patients with hemophilia scored lower on a self-esteem evaluation, including on all subscales except 1, compared with healthy control participants.
Investigators sought to evaluate the clinical characteristics and demographic features and changes in the self-image of adolescents with hemophilia. Eligible patients were diagnosed and had follow-up at the Dr Behcet Uz Children’s Hospital between September 1, 2017, and November 30, 2017.
Data about hemophilia type, disease severity, prophylaxis received, and annual bleeding rate (ABR) were acquired from patient files and assessed. A physiotherapist applied the Hemophilia Joint Health Score (HJHS) and the Offer Self-Image Questionnaire (OSIQ), with the latter serving as a measure of self-esteem, to patients with hemophilia. Healthy male siblings, who were used as a control group, were also administered the OSIQ.
Overall, 32 patients with hemophilia A and B (mean age, 16.2 years) and 35 control participants (mean age, 16.02 years) participated in the study. Among the patients, 87.5% had hemophilia A and 12.5% had hemophilia B. The majority of patients had severe (68.8%) or moderate (21.9%) hemophilia. Secondary prophylaxis was administered to 81.3% of patients, while on-demand therapy was administered to 18.7%.
Patients with hemophilia had lower median total OSIQ score compared with the control group (212.5 vs 250.0; P =.007). OSIQ subscale parameters, which include idealism, mental health, and body image among others, were also assessed. Patients with hemophilia scored lower on all subscale parameters compared with their peers except for 1: on the subscale of sexuality attitudes, patients with hemophilia scored higher compared with the control group (P =.028).
Patients with pathologic HJHS (16 patients) had lower median OSIQ score compared with patients with normal HJHS (16 patients; 198 vs 223; P =.010). No differences were identified between patients who received secondary or on-demand prophylaxis or when patients were stratified by ABR using a threshold of 4.
“Low self-esteem in hemophilia patients indicates the importance of lifelong psychosocial support,” the investigators concluded. “Using OSIQ with HJHS during follow-up of hemophilia patients may be useful for management.”
1. Aydin Köker S, Kaygusuz A, Oymak Y, et al. Assessment of self-image with the offer self-image questionnaire in adolescents with hemophilia: a single-center experience [published online November 12, 2019]. J Pediatr Hematol Oncol. doi:10.1097/MPH.0000000000001664