Published in Patient preference and adherence
Background The phase ½ hemophilia B clinical trial (AMT-060) demonstrated stable endogenous FIX levels after 3.5 years (mean FIX activity between 5.1% and 7.5%) with continued reductions in annualized bleeds to near zero with the higher dose, and a 78–96% reduction by year in exogenous FIX use. Objective The views of all the three participants from...
Published in Haemophilia : the official journal of the World Federation of Hemophilia
Despite significant advances in morbidity and mortality outcomes, quality of life for people with haemophilia (PWH) remains compromised. Underrecognized and undertreated mental health disorders decrease quality of life; however, reports are inconsistent regarding the true prevalence of mental health disorders in PWH. We conducted a systematic liter...
Published in Therapeutic Advances in Hematology
Background: Joint bleeds are the hallmark of haemophilia, and can lead to disabling haemophilic arthropathy. Consequently, the movement behaviour of adults with haemophilia differs from that of healthy adults. It seems unlikely that a single outcome is able to reflect all relevant information regarding movement behaviour. The aim of the current stu...
Published in Haemophilia : the official journal of the World Federation of Hemophilia
Qualitative interviews when developing the haemophilia caregiver impact measure© (HCI) documented the importance of capturing the positive aspects of caregiving, not just the negative. The present study thus investigates the construct underlying the positive emotions HCI subscale and tests models proposing a more comprehensive way of thinking about...
Published in Postgraduate medical journal
Published in The Journal of Haemophilia Practice
The prevalence and impact of bleeding disorders in women is not sufficiently acknowledged, with the organisation of care traditionally biased towards boys and men with haemophilia. In 2017, the European Haemophilia Consortium surveyed women with bleeding disorders, national member organisations (NMOs) and treatment centres to assess the impact of b...
Published in The Journal of Haemophilia Practice
Haemophilia is an X-linked inherited disorder that affects males and females, though the bleeding risk in girls and women has traditionally been under-recognised. About one third of haemophilia cases occur in individuals where there is no known family history. The gene mutations for rare bleeding disorders are not carried on the X chromosome and ar...
Published in International journal of laboratory hematology
Chromogenic substrate assay (CSA) reagents Revohem™ FVIII and Revohem™ FIX are now available as in vitro diagnostic reagents for autoanalysers in Japan. In this study, we evaluated the performance of these reagents in the CS-5100 automated coagulation analyser. We assessed within-run and between-day imprecision, on-board stability and frozen-storag...
Published in Haemophilia : the official journal of the World Federation of Hemophilia
To compare subjective and objective measures of adherence to prophylaxis in haemophilia. In this cross-sectional study, we compared participants' self-perceived adherence and their estimate of the number of clotting factor concentrates (CFCs) that had been missed over the last period of CFC dispensation with an objective measure of adherence based ...
Published in Haemophilia : the official journal of the World Federation of Hemophilia
Although the clinical manifestations of severe haemophilia A (HA) are well studied, the challenges, if any, of living with mild HA are not clearly delineated to date. To assess available evidence of clinical risks and societal/economic impacts of disease in adult patients with mild HA using a systematic literature review. Prespecified study selecti...
