Issues in the ageing individual with haemophilia and other inherited bleeding disorders: understanding and responding to the patients’ perspective

Abstract

An increasing number of individuals with haemophilia and other severe bleeding disorders who are ≥40 years of age are entering uncharted territory with respect to the identification and management of medical, physical and social issues relevant to ageing with a bleeding disorder. This is because the population experienced considerable mortality during the HIV/AIDs and hepatitis C epidemic due to exposure to tainted blood products for treatment of bleeding. As a result, few older individuals with this disorder survive today. To provide insight for how the comprehensive care team can adapt to the changing needs of the adult haemophiliac we evaluated the patient perspective. The objective of this study was to identify key themes of importance in the ageing population with haemophilia and other inherited bleeding disorders. For this study all subjects with a diagnosis of haemophilia A or B, von Willebrand disease or rare bleeding disorders 40 years or older from a single clinic were invited to participate. Audio-recordings of groups of six to eight participants were conducted by an independent investigator without content expertise. Transcripts were analysed using N*vivo (v. 8) software using thematic content analysis. Overall, 32 subjects, 18 men/14 women, with a mean age of 57.5 years (median 56.0 years) and range of 40-77 years, participated. Three major themes emerged: (i) reflection on living an active life, (ii) 'normal' ageing vs. disease-specific impacts and (iii) the health system, and its ability to respond to their needs as ageing individuals with bleeding disorders. We found some anticipated themes and some unexpected ones, confirming that true perspective can only be provided by the patients themselves. Knowledge of these important themes has informed the development of new programmes aimed at this growing segment of the patient population.