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Khair K.,Haemophilia Center
Orthopaedic nursing / National Association of Orthopaedic Nurses | Year: 2010

Hemophilia is a genetic disorder characterized by spontaneous or excessive bleeding predominantly into the weight-bearing joints or muscles. Synovial bleeding can lead to joint damage and disability. Recent studies have shown that prophylactic therapy may prevent joint damage in children with severe hemophilia. However, adherence to these regimens is often suboptimal. Barriers to adherence include lack of understanding of the disease and the time-consuming nature of infusions. Nurses play a critical role in promoting adherence to prophylactic regimens, particularly for adolescent patients. By providing individualized education and support, nurses can help promote joint therapies and minimize disability in patients with severe hemophilia. Source

Dolan G.,University of Nottingham | Makris M.,University of Sheffield | Bolton-Maggs P.H.B.,Manchester Blood Center | Rowell J.A.,Haemophilia Center
Haemophilia | Year: 2014

Clinical registries or databases have an increasing role in the management of inherited bleeding disorders. Initially, research-based registries provided valuable data and now national databases are increasingly being developed with multiple stakeholders, including persons with haemophilia (PWH) and payers, to enable improvements and efficiencies in care. Registries are extending to international collaborations to collect adverse event data and comparisons of national approaches to the management of haemophilia to improve the availability of product to PWH. © 2014 John Wiley & Sons Ltd. Source

Khair K.,Haemophilia Center
Haemophilia : the official journal of the World Federation of Hemophilia | Year: 2014

Adherence or compliance to prescribed treatment regimens is an important and much debated area of haemophilia care. Many patients are labelled as 'non-adherent' because they don't do what we say in terms of self- treatment and factor administration. However, do we engage patients in developing mutually acceptable treatment programmes which work for them as individuals? If we do, does this affect self-care and treatment uptake through a supportive relationship which enhances treatment concordance? Once we have agreed treatment regimens, how do we measure the success or outcomes of them? This paper discusses these issues, and some of the tools that are available to assess adherence in a systematic way. © 2014 John Wiley & Sons Ltd. Source

Lentz S.R.,University of Iowa | Ehrenforth S.,Novo Nordisk AS | Abdul Karim F.,Haemophilia Center | Matsushita T.,Nagoya University | And 3 more authors.
Journal of Thrombosis and Haemostasis | Year: 2014

Background: Vatreptacog alfa, a recombinant factor VIIa (rFVIIa) analog with three amino acid substitutions and 99% identity to native FVIIa, was developed to improve the treatment of hemophilic patients with inhibitors. Objectives: To confirm the safety and assess the efficacy of vatreptacog alfa in treating bleeding episodes in hemophilic patients with inhibitors. Patients and methods: In this international, multicenter, randomized, double-blind, active-controlled, crossover, confirmatory phase III trial (adept™2) in patients with hemophilia A or B and inhibitors, bleeds were randomized 3 2 to treatment with vatreptacog alfa (one to three doses at 80 μg kg-1) or rFVIIa (one to three doses at 90 μg kg-1). Treatment failures after three doses of trial product (TP) were managed according to the local standard of care. Results: In the 72 patients enrolled, 567 bleeds were treated with TP. Both vatreptacog alfa and rFVIIa gave 93% effective bleeding control at 12 h. Vatreptacog alfa was superior to rFVIIa in secondary efficacy outcomes, including the number of doses used to treat a bleed and sustained bleeding control 24-48 h after the first dose. Eight patients (11%) developed antibodies against vatreptacog alfa, including four with cross-reactivity against rFVIIa and one with an in vitro neutralizing effect to vatreptacog alfa. Conclusions: This large randomized controlled trial confirmed the well-established efficacy and safety profile of rFVIIa, and showed that vatreptacog alfa had similar or better efficacy than rFVIIa. However, because of the development of anti-drug antibodies, a positive benefit-risk profile is unlikely to be achieved with vatreptacog alfa. © 2014 The Authors. Journal of Thrombosis and Haemostasis. Source

Khair K.,Haemophilia Center | Holland M.,Driftwood Publishing Ltd | Pollard D.,Haemophilia Center Royal Free Hospital
Haemophilia | Year: 2013

Haemophilia carriers and women with inherited bleeding disorders (IBD) experience menorrhagia, bleed following dentistry, surgery, injury or childbirth. Symptoms are easily treated leading to full and active lives. Nevertheless, some girls and women suffer with abnormal bleeding for many years before diagnosis. We explored the experiences of girls and young women (aged 9-34 years) with IBD by means of focus groups which consisted of moderated discussion addressing specific aspects of bleeding, management and coping strategies. Subsequently, these issues were explored further though a paper-based questionnaire distributed via five specialist haemophilia centres in the UK. The study suggested that young women with IBD who are managed at haemophilia centres receive appropriate care and feel well supported. Although the clinic-based literature available to these women is "fit for purpose", it does not fully address the perceived needs specifically regarding sex, menorrhagia, conception and childbirth, the Pill, tattoos/piercings and so on, leading many to turn to other information sources. Most of those who responded to our survey are confident in their lives, able to manage their IBD and take pragmatic views towards the inherited nature of their condition. But there is a substantial subgroup of women who experience stigmatization, isolation and bullying and express concerns relating to fertility and conception. Overall, this cohort would benefit from opportunities for mutual support. This could be via Internet-based social networking and may be of particular value to those who are unable to seek help from traditional medical services due to religious or other cultural barriers. © 2013 John Wiley & Sons Ltd. Source

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