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Introduction Contemporary haemophilia care demands Patient-Reported Outcomes. SO- FIT is a UK multi-centre study, assessing self-reported function, health-related quality of life ( HRQoL) and joint health in boys with severe haemophilia. Methods Subjective physical function (PedHAL, HEP-Test-Q) and HRQoL (Haemo-QoL Short Form [SF]) were assessed alongside joint health using the objective Haemophilia Joint Health Score (HJHS v2.1). Demographic and clinical data were collected. Results Data from 127 boys mean age 12.38 ± 2.5 (range 8-17) treated at 16 sites were analysed. One-hundred-and-thirteen had haemophilia A, 25/9 past/current inhibitor, 124 were treated prophylactically (46.8% primary) and three on-demand. In the preceding 6 months, boys reported median 0 joint bleeds (range 0-8) with a median HJHS score of 1 (range 0-30). Boys reported good physical functioning; HEP-Test-Q (M = 80.32 ± 16.1) showed the highest impairments in the domain 'endurance' (72.53 ± 19.1), in Ped HAL (M = 85.44 ± 18.9) highest impairments were in the domains 'leisure activities & sports' (M = 82.43 ± 23.4) and 'lying/sitting/kneeling/standing' (M = 83.22 ± 20.3). Boys reported generally good HRQoL in Haemo-QoL SF SF (M = 22.81 ± 15.0) with highest impairments in the domains 'friends' (M = 28.81 ± 30.5) and 'sports & school' (M = 26.14 ± 25.1). HJHS revealed low correlations with the Haemo-QoL SF ( r = .251, P < .006), the PedHAL ( r = −.397, P < .0001) and the HEP-Test-Q ( r = −.323, P < .0001). A moderate correlation was seen between HEP-Test-Q and Haemo-QoL SF of r = −.575 ( P < .0001) and between Ped HAL and Haemo-QoL SF r = −.561 ( P < .0001) implying that good perceived physical function is related to good HRQoL. Conclusions The SO- FIT study has demonstrated that children with severe haemophilia in the UK report good HRQoL and have good joint health as reflected in low HJHS scores. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Link nurses are practising nurses with an expressed interest in a given specialty, with formal links to clinical nurse specialists and other specialist staff. The role involves attending meetings to discuss ideas and new developments, and relaying findings to other ward nurses to improve their practice. Such nurses are common in many specialties such as diabetes and tissue viability. In haemophilia, the role has the potential to enhance the care of haemophilia patients on general hospital wards. In April 2012, a focus group of five haemophilia nurses was convened to discuss their experiences of 'link nurse' programmes within district general hospitals and the potential value of developing the haemophilia link nurse role, and to consider the materials needed to support such role development. It was agreed to test whether other haemophilia nurses perceived such a need by means of a short five-item questionnaire devised by the group and made available to all members of the UK's Haemophilia Nurse's Association via Survey- Monkey. Final responses from 59 haemophilia nurses across the UK have been analysed. Most nurses agreed that there was value in the development of a haemophilia link nurse role within UK hospitals and thought their trusts would support it. While barriers and potential downsides were acknowledged, this was seen as a useful way of sharing information and knowledge with colleagues from different specialties and of raising awareness of bleeding disorders among the general nursing community. Haemophilia nurses should coordinate the development of a Haemophilia Link Nurse training and education pack. [ABSTRACT FROM AUTHOR]

. Clinical research should form a core component of the role of haemophilia nurse specialists. The UK Haemophilia Nurses Association sought to determine the barriers that prevent nurse specialists from engaging in research and to seek ways to promote clinical research by haemophilia nurses in the UK. Web-based survey with subsequent workshop discussion was conducted. Responses were received from 32 nurses (a 50% response rate), all of whom agreed that haemophilia nurses should be actively involved in nursing research although only 21 had actually participated in research specifically related to haemophilia practice. Of these, most research had been related to educational programmes or (less commonly) was limited to data collection as part of multidisciplinary studies. Involvement in research rarely resulted in publication. Some barriers to involvement in nursing research and subsequent publication were suggested by survey respondents. They also indentified key practice areas that warranted nurse-based research including carriership and antenatal decision-making, along with the role and impact on care of the specialist haemophilia nurse, education and empowerment. To overcome the barriers to engaging in research and publishing, nurses require dedicated research time, mentorship and collaboration with more experienced haemophilia nurse researchers. [ABSTRACT FROM AUTHOR]

. Access to modern treatments allows adolescents with haemophilia to manage their haemophilia at home, with improved treatment outcomes and quality of life, but has reduced peer support and the potential for experiential learning from older peers. Social networking, aided by modern communication technologies, may offer health benefits through peer support. We sought to assess whether or not disease-specific social networking could benefit adolescents with severe haemophilia. A total of 150 adolescents (aged 10-18) with severe haemophilia A or B from 11 UK treatment centres or those who had attended focus groups to explore the potential for a social network designed specifically for their use were surveyed. Teenage boys with severe haemophilia in the UK who responded to an online and paper questionnaire ( n = 47; 31% response rate) rarely knew of or socialized with others with haemophilia outside their families. Two-thirds of respondents said they would like to meet others. For 70% of boys, parents were the major source of information about haemophilia, yet more than half said they often had trouble finding answers to their questions. These boys frequently used online social networks to chat with friends. Adolescents with severe haemophilia frequently have limited contact with others and many wish to have greater contact. They may benefit from peer support and experiential learning gained through online social networking. The SixVibe restricted access social network is to be launched in 2011. It includes features designed to promote and facilitate the development of peer-to peer disease management skills for adolescents with severe haemophilia. [ABSTRACT FROM AUTHOR]