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Introduction: Improvements in haemophilia care have increased life expectancy in persons with haemophilia (PWH). This ageing population presents clinicians with management challenges as they develop age-related comorbidities such as cardiovascular disease (CVD)., Aims: To assess the epidemiology of CVD risk factors and events in an ageing Canadian haemophilia population., Methods: A retrospective, multicentre chart review was carried out at five Canadian Hemophilia Treatment Centres. PWH (A and B) ≥35 years old were included and data were extracted on CVD risk factors and events., Results: Data from 294 patients' charts were analysed including 222 (75.5%) patients with haemophilia A and 72 (24.5%) patients with haemophilia B with a median age at end of follow-up of 54 years (range = 36-90). Mean follow-up duration was 5.86 years. Cardiovascular risk factors were common: hypertension 31.3% (n = 90), diabetes mellitus 10.5% (n = 29), smoking 21.8% (n = 61), obesity 27.6% (n = 69), dyslipidaemia 22.4% (n = 65), family history 8.5% (n = 24), antiretroviral therapy 12.2% (n = 36). There were 24 CVD events (8.2% of the population) with a median age at event of 63 years (range = 46-83). Events consisted of coronary artery disease (CAD), 14; cerebrovascular disease, 4; and atrial fibrillation, 7. CAD was treated with coronary artery bypass grafting in three patients and percutaneous coronary intervention in nine patients. CVD events were complicated by six bleeding events (three minor and three major)., Conclusion: Cardiovascular disease risk factors and events are relatively common in PWH. PWH can be safely treated for CVD events with similar procedures as the non-PWH populations, though specific clotting factor prophylaxis protocols are not well defined., (© 2015 John Wiley & Sons Ltd.)

The prevalence of cardiovascular disease (CVD) risk and events in patients with haemophilia (PWH) is expected to increase as the longevity of this cohort increases due to treatment advances since the 1950s. The aims of this study were to assess publications of CVD and haemophilia for robustness, determine if the increasing longevity of PWH and associated age-related CVD risk factors result in CVD events; assess the need for an extension of the circle of care for ageing PWH due to the shift in comorbidities. A scoping review was conducted, resulting in a final pool of 30 articles which were organized based on publication dates. A matrix was created to illustrate which articles cited articles published prior to its own publication. This led to the identification of the primary articles, receiving the highest number of citations by other publications, which drive the research pertaining to the study of age-related risk factors of CVD in PWH. The scoping review revealed 14 original articles, four of which indicated a protective effect of haemophilia toward CVD. Twelve articles demonstrated a similar prevalence of CVD in PWH compared to the general population while seven articles concluded a difference in the prevalence of CVD in the ageing haemophilia population. The existing literature presented conflicting evidence regarding the possibility of a protective effect of haemophilia against CVD. The scoping review was not able to finalize whether the longevity of PWH and their associated age-related CVD risk factors result in CVD events because the articles assessed reported conflicting results., (© 2014 John Wiley & Sons Ltd.)

The first generation of young men using primary prophylaxis is coming of age. Important questions regarding the management of severe haemophilia with prophylaxis persist: Can prophylaxis be stopped? At what age? To what effect? Can the regimen be individualized? The reasons why some individuals discontinue or poorly comply with prophylaxis are not well understood. These issues have been explored using predominantly quantitative research approaches, yielding little insight into treatment decision-making from the perspectives of persons with haemophilia (PWH). Positioning the PWH as a source of expertise about their condition and its management, we undertook a qualitative study: (i) to explore and understand the lived experience of young men with severe haemophilia A or B and (ii) to identify the factors and inter-relationships between factors that affect young men's treatment decision-making. This manuscript reports primarily on the second objective. A modified Straussian, grounded theory methodology was used for data collection (interviews) and preliminary analysis. The study sample, youth aged 15-29, with severe haemophilia A or B, was chosen selectively and recruited through three Canadian Haemophilia Treatment Centres. We found treatment decision-making to be multi-factorial and used the Framework method to analyze the inter-relationships between factors. A typology of four distinct approaches to treatment was identified: lifestyle routine prophylaxis, situational prophylaxis, strict routine prophylaxis and no prophylaxis. Standardized treatment definitions (i.e.: 'primary' and 'secondary', 'prophylaxis') do not adequately describe the ways participants treat. Naming the variation of approaches documented in this study can improve PWH/provider communication, treatment planning and education., (© 2014 John Wiley & Sons Ltd.)

Desmopressin (DDAVP) is commonly used in the treatment of patients with type 1 von Willebrand disease (VWD) and mild hemophilia A. A patient's responsiveness to DDAVP based on a 0.3  μg/kg dose determines future therapeutic efficacy of the drug. The aim of the study was to determine whether a capped dose of 15 μg subcutaneous DDAVP is able to achieve the same level of DDAVP responsiveness as previously reported. This is a retrospective chart review of patients from 1995 to 2013 in adults and children with type 1 VWD and hemophilia A weighing more than 50 kg. Levels of factor VIII, ristocetin cofactor, and von Willebrand factor antigen were measured before and after 1 h of administration of 15 μg of DDAVP. In patients with type 1 VWD, the complete response rate was 82.5% with a partial response rate of 12.5% and 5% nonresponders. In patients with mild hemophilia A, the complete response rate was 53.8% with a partial response rate of 38.5% and 7.7% nonresponders. These results using a capped 15-μg dose of DDAVP are similar to previously published reports using the 0.3-μg/kg dose.