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Poster Networking

Inherited Bleeding Disorders and Inhibitors

PB0226 - Adherence and quality of life are not correlated in adolescent and adult men with hemophilia on factor replacement prophylaxis

Sunday, June 23, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall
Background: Frequent joint bleeds in hemophilia result in hemophilic arthropathy early in life, limiting mobility and causing chronic pain. Thus, adherence to prophylaxis to avoid spontaneous bleeds is important to maintain joint health and improve quality of life (QoL).

Aims: We evaluated adherence to factor replacement prophylaxis and QoL among adolescents and adults with hemophilia (AAwH).

Methods: Inhibitor-negative male AAwH aged 14 years or more on factor replacement prophylaxis during 2021 (January-December) were invited to participate at a Brazilian Hemophilia Treatment Center. We collected sociodemographic, clinical, and therapeutic data. Treatment adherence was evaluated by (1) the ratio between distributed per prescribed (rDP) factor doses for prophylaxis during 2021 (rDP < 0.85 defined as non-adherence) and (2) the self-reported Validated Hemophilia Regimen Treatment Adherence Scale-Prophylaxis (VERITAS-Pro) questionnaire (higher scores meant worse adherence; scores ≥57 defined as non-adherence). Quality of life was evaluated by the self-reported Short Form Health Survey (SF-36) questionnaire.

Results: A total of 78 AAwH were included, aged 14 to 63 years (median 30 years). There were 67 (85%) AAwH A, 70 (90%) severe, and 61 (78%) self-infused factors. Seventeen (21%) AAwH were white, 26 (33%) single, and 35 (44%) graduated. Median rDP was 0.79 (range 0.13-1.30) and 44 (57%) AAwH were adherent. rDP was not associated with any sociodemographic, clinical, or therapeutic variable. Median VERITAS-Pro was 44.0 (range 24-67) and 70 (90%) AAwH were adherent. Adherence (VERITAS-Pro) was associated with having hemophilia A (p=0.012), using an infusion diary (p=0.001), infusion frequency higher than twice weekly (p=0.008), and longer duration of prophylaxis (p=0.005). rDP and VERITAS-Pro were not correlated. Median SF-36 was 70.3 (range 25.6-96.3). Higher QoL was associated with using an infusion diary (p=0.026). SF-36 was not correlated with rDP or VERITAS-Pro.

Conclusion(s): The prevalence of adherence to prophylaxis differed based on the method of evaluation and did not correlate with QoL.