Changes in Quality of Life, Adherence, and Kinesiophobia in Patients with Hemophilia Treated with Extended Half-Life Treatment: Final Results of the LongHest Project

Abstract

(1) Background: Hemophilia is a bleeding disorder characterized by hemarthrosis. Prophylaxis is the gold standard for bleeding prevention. Extended half-life (EHL) recombinant FVIII replacement products have shown to be associated with low bleeding rates. The aim was to evaluate the efficacy of EHL prophylaxis in improving perceived quality of life, adherence to treatment, and kinesiophobia in patients with hemophilia. (2) Methods: This was a prospective cohort study. Forty-six patients from different regions, who had started EHL FVIII concentrate prophylactic treatment, were evaluated at baseline and at 12-month follow-up. The study variables were as follows: perceived quality of life (36-Item Short Form Health Survey), adherence to treatment (Validated Hemophilia Regimen Treatment Adherence Scale—Prophylaxis), and kinesiophobia (Tampa Scale of Kinesiophobia). (3) Results: There were statistically significant differences in the domains Role-Physical (p p p p = 0.04), and Social Functioning (p = 0.01) and the total scores, Physical Health (p p p p p = 0.01) in terms of adherence. There were also significant differences in kinesiophobia (p = 0.02) after the study period. (4) Conclusions: EHL prophylaxis can improve the perceived quality of life of people with hemophilia. This prophylactic regimen, which requires fewer infusions, may improve adherence to treatment in adult patients with hemophilia over a 12-month period. The administration of extended half-life factor VIII concentrates can reduce kinesiophobia in adult patients with hemophilic arthropathy.

Keywords

• hemophilia
• EHL
• quality of life
• adherence
• kinesiophobia