PB0620 - (Mis)labeling hemophilia severity: Exploring the illness cognitions of persons with hemophilia.

Background: Hemophilia varies in expression with its severity traditionally defined clinically based on the degree of coagulation factor deficiency. Illness cognitions (patterns of beliefs about one’s condition) are important, yet rarely considered, when labelling persons with hemophilia (PWH) as mild, moderate, or severe. It’s unclear whether the clinical classification aligns with the lived experiences of PWH. As newly marketed, and future, therapies permit living with coagulation factors in non-severe ranges, it becomes crucial to better understand the multifaceted influences on disease seriousness across the spectrum of hemophilia severity.

Aims: Our purpose was to explore whether current conceptualizations of disease seriousness align with the illness cognitions and lived experiences of PWH.

Methods: We interviewed 29 Canadian PWH A and B (11 men with severe hemophilia, 11 men with non-severe hemophilia, 7 women with non-severe hemophilia) using constructivist grounded theory. Themes were generated using constant comparative analysis.

Results: Regardless of their coagulation factor level, participants shared a general sense of health and well-being interspersed with experiences of suffering caused by poor societal and healthcare support. All participants described bleeding events that could objectively be perceived as serious, but individuals with severe hemophilia were equipped to manage them promptly. In contrast, those with non-severe hemophilia, particularly women, commonly experienced situations whereby the seriousness of their bleeding events was downplayed due to their “mild” or “carrier” label. Often, those labelled as having non-severe hemophilia brushed off prolonged debilitating symptoms, perceiving them as “not as bad” as events experienced by persons living with severe hemophilia.

Conclusion(s): Ironically, participants labelled as having non-severe hemophilia experience serious consequences due to difficulty accessing immediate and appropriate care. Although these labels are an important frame of references about biological behavior of hemophilia, they may not reflect the experiences of living with hemophilia and potentially biasing the illness cognitions of clinicians and PWH alike.