Simon Fletcher, S. Chaplin, C. Harrison, K. Juusola, N. Collins
Abstract Background: Guidance from the European Association for Haemophilia and Allied Disorders (EAHAD) sets out the educational milestones haemophilia nurses should aim to achieve. However, little is known about the resources nurses use for education and current awareness. Aims: To assess the current educational level of haemophilia nurses, how and where they access ongoing education, where they feel they need extra support, and how best this teaching could be delivered. Methods: Haemophilia nurses in the Haemnet Horizons group devised and piloted a questionnaire. This was distributed in hard copy to nurses attending the 2019 EAHAD Congress and promoted as an online survey hosted by Survey Monkey. Results: Seventy-five replies were received from nurses in Europe (46 in the UK), and two from nurses in Chile and the Philippines. Most described their role as ‘specialist nurse’, with the majority having worked in haemophilia care for up to ten years. Half had a nursing degree and one quarter had a nursing diploma. Three quarters had attended at least one course specifically related to haemophilia nursing. Almost all used academic sources, study days and the websites of health profession organisations as information sources. Most also used Google or Wikipedia, but fewer used Twitter. Patient association websites were more popular among non-UK nurses. About half attended sponsored professional meetings and three quarters reported that educational meetings were available in their workplace. A clear majority preferred interactive and face-to-face activities using patient-focused content. Conclusions: The study shows that nurses, predominantly in Western Europe, access a range of educational resources, most of which are ‘traditional’. Use of online sources is high, but social media are less popular than Google or Wikipedia. Further research is needed to explore the potential of new media for haemophilia nurse education, and whether the current educational levels and needs highlighted in the survey remains the same across the whole of Europe.
{"title":"Professional education among haemophilia nurses: a survey of current practices","authors":"Simon Fletcher, S. Chaplin, C. Harrison, K. Juusola, N. Collins","doi":"10.17225/JHP00150","DOIUrl":"https://doi.org/10.17225/JHP00150","url":null,"abstract":"Abstract Background: Guidance from the European Association for Haemophilia and Allied Disorders (EAHAD) sets out the educational milestones haemophilia nurses should aim to achieve. However, little is known about the resources nurses use for education and current awareness. Aims: To assess the current educational level of haemophilia nurses, how and where they access ongoing education, where they feel they need extra support, and how best this teaching could be delivered. Methods: Haemophilia nurses in the Haemnet Horizons group devised and piloted a questionnaire. This was distributed in hard copy to nurses attending the 2019 EAHAD Congress and promoted as an online survey hosted by Survey Monkey. Results: Seventy-five replies were received from nurses in Europe (46 in the UK), and two from nurses in Chile and the Philippines. Most described their role as ‘specialist nurse’, with the majority having worked in haemophilia care for up to ten years. Half had a nursing degree and one quarter had a nursing diploma. Three quarters had attended at least one course specifically related to haemophilia nursing. Almost all used academic sources, study days and the websites of health profession organisations as information sources. Most also used Google or Wikipedia, but fewer used Twitter. Patient association websites were more popular among non-UK nurses. About half attended sponsored professional meetings and three quarters reported that educational meetings were available in their workplace. A clear majority preferred interactive and face-to-face activities using patient-focused content. Conclusions: The study shows that nurses, predominantly in Western Europe, access a range of educational resources, most of which are ‘traditional’. Use of online sources is high, but social media are less popular than Google or Wikipedia. Further research is needed to explore the potential of new media for haemophilia nurse education, and whether the current educational levels and needs highlighted in the survey remains the same across the whole of Europe.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"207 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121031293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C. Dodd, Alis Trivelli, D. Stephensen, G. Evans, Miranda Foord
Abstract Background The ankle joint is the most common site of bleeding for people with haemophilia (PWH) in the developed world. Recent surveys suggest that PWH do not always have access to non-surgical musculoskeletal interventions and that when provided; there is considerable heterogeneity in clinical practice. Aims To determine patient perceptions and the potential benefits of a new combined multidisciplinary physiotherapy-podiatry haemophilia clinic, and to observe the effect on frequency of bleeds and ankle joint Haemophilia Joint Health Scores (HJHS). Materials and methods PWH with a history of ankle bleeds, pain, foot and/or ankle deformities from a single UK haemophilia centre were referred to the clinic from December 2017 to December 2018. Pre- and post-intervention ankle joint HJHS data and ankle annualised joint bleed rate (AJBR) were collected together with a satisfaction questionnaire asking patients their views on the clinic's value, usefulness and their satisfaction after the initial appointment. Results Twenty-seven PWH (16 children and 11 adults) attended the clinic. All patients agreed or strongly agreed that they were satisfied with the new clinic. The combined multidisciplinary nature of the clinic meant that patients only needed to attend one appointment with the expertise of two professionals, rather than attending two separate appointments. All patients reported it “more useful to see the physiotherapist and podiatrist together”. There were no statistically significant differences in ankle AJBR or HJHS scores post-intervention compared to pre-intervention. Conclusion Establishing a multidisciplinary physiotherapy-podiatry clinic for PWH with a history of ankle bleeds, pain, foot and/or ankle deformities appears to increase patient satisfaction. We did not observe a significant change in ankle AJBR or ankle HJHS scores, suggesting they might not be sufficient to evaluate potential benefits to patients. A larger study incorporating validated tools, focusing on patient-reported foot function, pain, activity and quality of life is needed to confirm if there is any effect of a combined physiotherapy-podiatry intervention on ankle joint AJBR and function.
{"title":"Outcome of a combined physiotherapy and podiatry haemophilia clinic: patient perceptions and the effect on ankle bleeds and joint health","authors":"C. Dodd, Alis Trivelli, D. Stephensen, G. Evans, Miranda Foord","doi":"10.17225/jhp00153","DOIUrl":"https://doi.org/10.17225/jhp00153","url":null,"abstract":"Abstract Background The ankle joint is the most common site of bleeding for people with haemophilia (PWH) in the developed world. Recent surveys suggest that PWH do not always have access to non-surgical musculoskeletal interventions and that when provided; there is considerable heterogeneity in clinical practice. Aims To determine patient perceptions and the potential benefits of a new combined multidisciplinary physiotherapy-podiatry haemophilia clinic, and to observe the effect on frequency of bleeds and ankle joint Haemophilia Joint Health Scores (HJHS). Materials and methods PWH with a history of ankle bleeds, pain, foot and/or ankle deformities from a single UK haemophilia centre were referred to the clinic from December 2017 to December 2018. Pre- and post-intervention ankle joint HJHS data and ankle annualised joint bleed rate (AJBR) were collected together with a satisfaction questionnaire asking patients their views on the clinic's value, usefulness and their satisfaction after the initial appointment. Results Twenty-seven PWH (16 children and 11 adults) attended the clinic. All patients agreed or strongly agreed that they were satisfied with the new clinic. The combined multidisciplinary nature of the clinic meant that patients only needed to attend one appointment with the expertise of two professionals, rather than attending two separate appointments. All patients reported it “more useful to see the physiotherapist and podiatrist together”. There were no statistically significant differences in ankle AJBR or HJHS scores post-intervention compared to pre-intervention. Conclusion Establishing a multidisciplinary physiotherapy-podiatry clinic for PWH with a history of ankle bleeds, pain, foot and/or ankle deformities appears to increase patient satisfaction. We did not observe a significant change in ankle AJBR or ankle HJHS scores, suggesting they might not be sufficient to evaluate potential benefits to patients. A larger study incorporating validated tools, focusing on patient-reported foot function, pain, activity and quality of life is needed to confirm if there is any effect of a combined physiotherapy-podiatry intervention on ankle joint AJBR and function.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125065107","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
T. Hughes, Mikkel Brok-Kristensen, Yosha Gargeya, Anne Mette Worsøe Lottrup, Ask Bo Larsen, A. Torres-Ortuño, N. Mackett, J. Stevens
Abstract Background With the major advances in treatment of haemophilia in recent decades, people with haemophilia (PwH) are more protected in their daily lives than ever before. However, recent studies point to persisting or increasing patient experience of uncertainty. Aims The aim of this article is to further investigate findings related to how PwH understand and cope with uncertainty around their protection in their everyday life, one of the main themes identified in a large-scale ethnographic study of the everyday life of PwH, including beliefs and experiences related to their condition, their treatment, and their personal ways of managing the condition. Methods The study used ethnographic research methods. Five haemophilia experts provided historical and disease area context prior to the initiation of field research. During field research, study researchers collected data through 8–12 hours of participant observation, semi-structured interviews, written exercises, facilitated group dialogues, and on-site observations of the interactions of PwH with friends, family, and health care professionals (HCPs). Study researchers also conducted on-site observation at haemophilia treatment centres (HTCs) and interviewed HCPs. The study employed a multi-tiered grounded theory approach and combined data were analysed using techniques such as inductive and deductive analysis, cross-case analysis, challenge mapping, and clustering exercises. This article explores findings related to uncertainty and thus focuses on a subset of the data from the study. Results Fifty-one PwH in Italy, Germany, Spain, UK, and Ireland were interviewed and followed in their daily lives, and 18 HCPs were interviewed. Fifty-two per cent (n=26/50) of PwH in the study experience difficulties translating clinical understanding of protection into specific activities in everyday life. Many have developed their own mental models and care adaptations to navigate treatment uncertainy: these seldom match the medical community's view. These mental models of protection among PwH can cause distress and influence behaviour in a way that can limit possibilities, and/or increase risk. There is also a prevalent tension in the strategies PwH have for managing their protection in terms of day-to-day vs. long-term ambitions. Conclusions These findings on PwH's experience of treatment uncertainty suggest a need to develop tools and communication materials to help PwH better understand the protection provided by their treatment regimen and what that means practically for everyday life.
{"title":"Navigating uncertainty: an examination of how people with haemophilia understand and cope with uncertainty in protection in an ethnographic study","authors":"T. Hughes, Mikkel Brok-Kristensen, Yosha Gargeya, Anne Mette Worsøe Lottrup, Ask Bo Larsen, A. Torres-Ortuño, N. Mackett, J. Stevens","doi":"10.17225/jhp00168","DOIUrl":"https://doi.org/10.17225/jhp00168","url":null,"abstract":"Abstract Background With the major advances in treatment of haemophilia in recent decades, people with haemophilia (PwH) are more protected in their daily lives than ever before. However, recent studies point to persisting or increasing patient experience of uncertainty. Aims The aim of this article is to further investigate findings related to how PwH understand and cope with uncertainty around their protection in their everyday life, one of the main themes identified in a large-scale ethnographic study of the everyday life of PwH, including beliefs and experiences related to their condition, their treatment, and their personal ways of managing the condition. Methods The study used ethnographic research methods. Five haemophilia experts provided historical and disease area context prior to the initiation of field research. During field research, study researchers collected data through 8–12 hours of participant observation, semi-structured interviews, written exercises, facilitated group dialogues, and on-site observations of the interactions of PwH with friends, family, and health care professionals (HCPs). Study researchers also conducted on-site observation at haemophilia treatment centres (HTCs) and interviewed HCPs. The study employed a multi-tiered grounded theory approach and combined data were analysed using techniques such as inductive and deductive analysis, cross-case analysis, challenge mapping, and clustering exercises. This article explores findings related to uncertainty and thus focuses on a subset of the data from the study. Results Fifty-one PwH in Italy, Germany, Spain, UK, and Ireland were interviewed and followed in their daily lives, and 18 HCPs were interviewed. Fifty-two per cent (n=26/50) of PwH in the study experience difficulties translating clinical understanding of protection into specific activities in everyday life. Many have developed their own mental models and care adaptations to navigate treatment uncertainy: these seldom match the medical community's view. These mental models of protection among PwH can cause distress and influence behaviour in a way that can limit possibilities, and/or increase risk. There is also a prevalent tension in the strategies PwH have for managing their protection in terms of day-to-day vs. long-term ambitions. Conclusions These findings on PwH's experience of treatment uncertainty suggest a need to develop tools and communication materials to help PwH better understand the protection provided by their treatment regimen and what that means practically for everyday life.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"33 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114534371","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abstract Factor VII deficiency is a rare inheritable bleeding disorder that can be challenging to manage. Blood activity levels do not correlate with bleeding risk, and prophylaxis is a more difficult treatment option than for people with haemophilia due to the short half-life of factor VII. Acute bleeding manifestations and long-term complications are similar to those associated with haemophilia. This case study illustrates the psychological and physical impact of severe factor VII deficiency on a woman with impaired mobility due to haemarthropathy who must retain her independence to provide care for her elderly parents. She self-manages her joint pain and bleeding risk, but her life is limited by the need to avoid injury and her reluctance to engage fully with health services.
{"title":"Fiona’s FVII footprint","authors":"G. Mulders, M. Tuinhout","doi":"10.17225/jhp00154","DOIUrl":"https://doi.org/10.17225/jhp00154","url":null,"abstract":"Abstract Factor VII deficiency is a rare inheritable bleeding disorder that can be challenging to manage. Blood activity levels do not correlate with bleeding risk, and prophylaxis is a more difficult treatment option than for people with haemophilia due to the short half-life of factor VII. Acute bleeding manifestations and long-term complications are similar to those associated with haemophilia. This case study illustrates the psychological and physical impact of severe factor VII deficiency on a woman with impaired mobility due to haemarthropathy who must retain her independence to provide care for her elderly parents. She self-manages her joint pain and bleeding risk, but her life is limited by the need to avoid injury and her reluctance to engage fully with health services.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"41 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"117278110","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
I t is fascinating for a person born with haemophilia almost 70 years ago to read the very insightful paper ‘What more can we ask for?: an ethnographic study of challenges and possibilities for people living with haemophilia’ by Hughes et al. in this issue of the Journal of Haemophilia Practice. While medical treatments have advanced miraculously over the last seven decades, from fresh frozen plasma to cryoprecipitate and a whole range of factor concentrates, to non-factor therapies and now to the cusp of gene therapy, the
{"title":"Uncertainty to normality using ethnographic and qualitative research: a personal view","authors":"D. Page","doi":"10.17225/jhp00170","DOIUrl":"https://doi.org/10.17225/jhp00170","url":null,"abstract":"I t is fascinating for a person born with haemophilia almost 70 years ago to read the very insightful paper ‘What more can we ask for?: an ethnographic study of challenges and possibilities for people living with haemophilia’ by Hughes et al. in this issue of the Journal of Haemophilia Practice. While medical treatments have advanced miraculously over the last seven decades, from fresh frozen plasma to cryoprecipitate and a whole range of factor concentrates, to non-factor therapies and now to the cusp of gene therapy, the","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"39 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116386759","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
G. Mulders, N. Uitslager, Sharon Alavian, A. Wareing, Kathi Stein Oldenburg
Abstract Introduction Switching between clotting factor products is becoming increasingly common as product choice increases and financial pressure grows to choose the most cost-effective options. Guidance on carrying out the switch recommends a complex and long process that may benefit from being defined in a protocol. Haemophilia nurses may be responsible for managing product switches; anecdotal evidence suggests that clinical practice is variable. Aim To explore the role of specialist nurses in switching between clotting factor products and their use of a protocol. Method Nurses attending the 2018 World Federation of Hemophilia Congress were surveyed about clinical practice at their treatment centre and use of a protocol for switching clotting factor products. Results Of 192 nurses attending the conference, 49 nurses returned completed questionnaires, 45 of which were included in the study after exclusions. Responses were exclusively from economically developed countries. Almost all respondents (96%) had direct experience of switching. Half of those who responded to a question about protocol-based switching reported that switches were based on a protocol. When authorship was reported, the protocol was written by haemophilia nurses in about half of cases. Practice about blood testing to determine individual pharmacokinetic parameters prior to the switch was variable, but most nurses (86%) reported screening for inhibitors prior to switching. Respondents agreed to share their protocols among their peers, although only four were received by the research team. Conclusions Clinical practice in switching between clotting factor products is variable. Some nurses are switching treatments for patients without the supported of a written protocol, whereas others are involved in writing and implementing protocols. Sharing protocols is a first step in helping to establish best practice.
{"title":"Do nurses have the switch factor?","authors":"G. Mulders, N. Uitslager, Sharon Alavian, A. Wareing, Kathi Stein Oldenburg","doi":"10.17225/jhp00162","DOIUrl":"https://doi.org/10.17225/jhp00162","url":null,"abstract":"Abstract Introduction Switching between clotting factor products is becoming increasingly common as product choice increases and financial pressure grows to choose the most cost-effective options. Guidance on carrying out the switch recommends a complex and long process that may benefit from being defined in a protocol. Haemophilia nurses may be responsible for managing product switches; anecdotal evidence suggests that clinical practice is variable. Aim To explore the role of specialist nurses in switching between clotting factor products and their use of a protocol. Method Nurses attending the 2018 World Federation of Hemophilia Congress were surveyed about clinical practice at their treatment centre and use of a protocol for switching clotting factor products. Results Of 192 nurses attending the conference, 49 nurses returned completed questionnaires, 45 of which were included in the study after exclusions. Responses were exclusively from economically developed countries. Almost all respondents (96%) had direct experience of switching. Half of those who responded to a question about protocol-based switching reported that switches were based on a protocol. When authorship was reported, the protocol was written by haemophilia nurses in about half of cases. Practice about blood testing to determine individual pharmacokinetic parameters prior to the switch was variable, but most nurses (86%) reported screening for inhibitors prior to switching. Respondents agreed to share their protocols among their peers, although only four were received by the research team. Conclusions Clinical practice in switching between clotting factor products is variable. Some nurses are switching treatments for patients without the supported of a written protocol, whereas others are involved in writing and implementing protocols. Sharing protocols is a first step in helping to establish best practice.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"99 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134179802","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
T he European Haemophilia Consortium’s (EHC) conference Women and Bleeding Disorders, held in May 2019, was the first such meeting dedicated entirely to discussing women’s experience of living with a bleeding disorder, the activities of national member organisations (NMOs), and diagnosis and medical management. The conference welcomed almost 150 delegates from nearly 30 countries, including representatives from the European Association for Haemophilia and Allied Disorders (EAHAD) and the World Federation of Hemophilia (WFH). The EHC has long sought to represent and serve women with bleeding disorders and others affected by a bleeding disorder. In 2015, it organised a preconference workshop entitled Starting a European Conversation for Women with Bleeding Disorders at its annual scientific conference in Belgrade, Serbia. The work of a group of dedicated volunteers from across Europe, has since enabled the EHC to build on this foundation to increase its focus on women, organising sessions specific to issues concerning women with bleeding disorders at scientific conferences, youth debates and its annual Leadership Conference. In 2017, the EHC created a Committee for Women and Bleeding Disorders to promote awareness, recognition, support, and education about and for women in the bleeding disorder community at a European level. The Committee organised a survey of women, NMOs and treatment centres to assess the prevalence and experience of bleeding disorders and the effectiveness of the support available. The survey showed that the needs of women with bleeding disorders are being neglected and provided the evidence on which the EHC can build a campaign to improve recognition and services for women with a bleeding disorder . The Committee invited EHC members to propose the topics to be addressed in this conference. The response was so great that it was a struggle to accommodate everything in the time available. However, the Conference Programming Committee ensured that the meeting covered a wide range of topics in plenary sessions, also providing time for delegates to talk about issues that affected them in Q&A and breakout sessions. The programme covered an overview of bleeding disorders affecting women, genetic transmission, gynaecological and nongynaecological issues, managing monthly menses, the impact of bleeding disorders on quality of life, and tackling psychosocial issues. Breakout sessions focused on family planning, and support from NMOs and treatment centres. The conference marked the premiere of the EHC’s film Women and Bleeding Disorders: Untold Stories, DEBRA POLLARD Lead Nurse, Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust, London, UK
{"title":"The First European Conference on Women and Bleeding Disorders Frankfurt, Germany, 24-26 May 2019","authors":"D. Pollard, A. Bok","doi":"10.17225/jhp00145","DOIUrl":"https://doi.org/10.17225/jhp00145","url":null,"abstract":"T he European Haemophilia Consortium’s (EHC) conference Women and Bleeding Disorders, held in May 2019, was the first such meeting dedicated entirely to discussing women’s experience of living with a bleeding disorder, the activities of national member organisations (NMOs), and diagnosis and medical management. The conference welcomed almost 150 delegates from nearly 30 countries, including representatives from the European Association for Haemophilia and Allied Disorders (EAHAD) and the World Federation of Hemophilia (WFH). The EHC has long sought to represent and serve women with bleeding disorders and others affected by a bleeding disorder. In 2015, it organised a preconference workshop entitled Starting a European Conversation for Women with Bleeding Disorders at its annual scientific conference in Belgrade, Serbia. The work of a group of dedicated volunteers from across Europe, has since enabled the EHC to build on this foundation to increase its focus on women, organising sessions specific to issues concerning women with bleeding disorders at scientific conferences, youth debates and its annual Leadership Conference. In 2017, the EHC created a Committee for Women and Bleeding Disorders to promote awareness, recognition, support, and education about and for women in the bleeding disorder community at a European level. The Committee organised a survey of women, NMOs and treatment centres to assess the prevalence and experience of bleeding disorders and the effectiveness of the support available. The survey showed that the needs of women with bleeding disorders are being neglected and provided the evidence on which the EHC can build a campaign to improve recognition and services for women with a bleeding disorder . The Committee invited EHC members to propose the topics to be addressed in this conference. The response was so great that it was a struggle to accommodate everything in the time available. However, the Conference Programming Committee ensured that the meeting covered a wide range of topics in plenary sessions, also providing time for delegates to talk about issues that affected them in Q&A and breakout sessions. The programme covered an overview of bleeding disorders affecting women, genetic transmission, gynaecological and nongynaecological issues, managing monthly menses, the impact of bleeding disorders on quality of life, and tackling psychosocial issues. Breakout sessions focused on family planning, and support from NMOs and treatment centres. The conference marked the premiere of the EHC’s film Women and Bleeding Disorders: Untold Stories, DEBRA POLLARD Lead Nurse, Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust, London, UK","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115100435","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
T here are many aspects of living with haemophilia that make people feel different from others. But imagine the impact of having a bleeding disorder so rare that you feel different from almost everyone else with a bleeding disorder. Evelyn Grimberg and Minette van der Ven from The Netherlands are two such people: they have Glanzmann’s thrombasthenia, an inherited platelet function disorder. There are perhaps 2,000 – 3,000 people worldwide living with this diagnosis – a prevalence of about 1 in 4 million population worldwide. However, there are many more cases than expected in some countries – for example, in some areas of Iran, where the prevalence is estimated at 1 in 200,000. Whichever figure is correct, the odds of having two people with Glanzmann’s in the same room at random are inconceivably small. But this is not a random meeting: Evelyn and Minette are close friends who have come together, not because they share this unusual diagnosis, but because they have the same outlook on living with it. And they have also bonded as women who see services for bleeding disorders heavily geared towards boys and men with haemophilia, to the extent that the impact on women with bleeding disorders has been underestimated or ignored. For Evelyn and Minette, early life with Glanzmann’s was similar to that for many of the current generation of adults with a bleeding disorder. Suspicion first arose after an episode of uncontrolled bleeding – at age four months for Evelyn and two years for Minette. Both are the only family members known to be affected. For their parents, the uncertainty of a future living with a bleeding disorder was compounded by difficulty identifying the cause – it was two years before Minette’s diagnosis was confirmed – but both families adopted a robust approach to raising their child.
血友病患者在生活中有许多方面会让他们感觉与他人不同。但想象一下，患有出血性疾病的影响是如此罕见，以至于你觉得自己与几乎所有患有出血性疾病的人都不一样。来自荷兰的Evelyn Grimberg和Minette van der Ven就是这样的两个人:他们患有格兰兹曼血栓症，一种遗传性血小板功能紊乱。全世界可能有2000 - 3000人患有这种疾病，患病率约为400万人中有1人。然而，一些国家的病例比预期的要多得多，例如，在伊朗的一些地区，患病率估计为20万分之一。无论哪个数字是正确的，在同一个房间里随机出现两个患有格兰兹曼氏症的人的几率都小得难以想象。但这不是一次偶然的相遇:伊芙琳和米内特是亲密的朋友，她们走到了一起，不是因为她们都有这种不寻常的诊断，而是因为她们对生活的看法相同。她们也因为看到出血性疾病的服务主要面向患有血友病的男孩和男性而联系在一起，以至于对患出血性疾病的女性的影响被低估或忽视了。对于伊芙琳和米内特来说，患有格兰兹曼氏症的早期生活与当今许多患有出血性疾病的成年人的生活相似。伊芙琳四个月大的时候，米内特两岁大的时候，一次无法控制的出血事件引起了人们的怀疑。两人都是已知的唯一受影响的家庭成员。对于他们的父母来说，未来是否会患有出血性疾病的不确定性，加上难以确定病因——两年后米内特的诊断才得到证实——但两个家庭都采取了强有力的方法来抚养孩子。
{"title":"Doubly rare: Evelyn Grimberg and Minette van der Ven","authors":"S. Chaplin","doi":"10.17225/JHP00130","DOIUrl":"https://doi.org/10.17225/JHP00130","url":null,"abstract":"T here are many aspects of living with haemophilia that make people feel different from others. But imagine the impact of having a bleeding disorder so rare that you feel different from almost everyone else with a bleeding disorder. Evelyn Grimberg and Minette van der Ven from The Netherlands are two such people: they have Glanzmann’s thrombasthenia, an inherited platelet function disorder. There are perhaps 2,000 – 3,000 people worldwide living with this diagnosis – a prevalence of about 1 in 4 million population worldwide. However, there are many more cases than expected in some countries – for example, in some areas of Iran, where the prevalence is estimated at 1 in 200,000. Whichever figure is correct, the odds of having two people with Glanzmann’s in the same room at random are inconceivably small. But this is not a random meeting: Evelyn and Minette are close friends who have come together, not because they share this unusual diagnosis, but because they have the same outlook on living with it. And they have also bonded as women who see services for bleeding disorders heavily geared towards boys and men with haemophilia, to the extent that the impact on women with bleeding disorders has been underestimated or ignored. For Evelyn and Minette, early life with Glanzmann’s was similar to that for many of the current generation of adults with a bleeding disorder. Suspicion first arose after an episode of uncontrolled bleeding – at age four months for Evelyn and two years for Minette. Both are the only family members known to be affected. For their parents, the uncertainty of a future living with a bleeding disorder was compounded by difficulty identifying the cause – it was two years before Minette’s diagnosis was confirmed – but both families adopted a robust approach to raising their child.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"38 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122345772","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M onday 24th September 2018 was the first day of the preliminary hearings of the UK Infected Blood Inquiry. Two earlier inquiries, dismissed as whitewashes, failed to provide answers and committed campaigners had now been rewarded. The Inquiry would examine the circumstances in which people under the care of the National Health Service (NHS) were given blood and blood products contaminated with HIV and hepatitis viruses, and possibly Creuzfeldt-Jakob disease (CJD); the impact on those infected and affected; the nature, adequacy and timeliness of the response from agencies involved; to what extent individuals may have been misled and denied treatment; and whether those agencies and individuals covered up the facts. The first three days were held in public, given over to opening statements that included witness accounts from people with haemophilia and their families. Their stories are profoundly moving. The Inquiry is truly an historic step towards accountability for the government, the NHS, health professionals and all involved in haemophilia care in the UK decades since 1970. The man who worked for many years to make this a reality is Bruce Norval. Bruce was born in Edinburgh in 1965. At age three he was admitted to hospital with bleeding from a fall; after three months on the ward, he was diagnosed with haemophilia B. It did not make much difference to him at the time.
{"title":"Redressing the balance: Bruce Norval","authors":"S. Chaplin","doi":"10.17225/JHP00119","DOIUrl":"https://doi.org/10.17225/JHP00119","url":null,"abstract":"M onday 24th September 2018 was the first day of the preliminary hearings of the UK Infected Blood Inquiry. Two earlier inquiries, dismissed as whitewashes, failed to provide answers and committed campaigners had now been rewarded. The Inquiry would examine the circumstances in which people under the care of the National Health Service (NHS) were given blood and blood products contaminated with HIV and hepatitis viruses, and possibly Creuzfeldt-Jakob disease (CJD); the impact on those infected and affected; the nature, adequacy and timeliness of the response from agencies involved; to what extent individuals may have been misled and denied treatment; and whether those agencies and individuals covered up the facts. The first three days were held in public, given over to opening statements that included witness accounts from people with haemophilia and their families. Their stories are profoundly moving. The Inquiry is truly an historic step towards accountability for the government, the NHS, health professionals and all involved in haemophilia care in the UK decades since 1970. The man who worked for many years to make this a reality is Bruce Norval. Bruce was born in Edinburgh in 1965. At age three he was admitted to hospital with bleeding from a fall; after three months on the ward, he was diagnosed with haemophilia B. It did not make much difference to him at the time.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"79 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129420043","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Srila Gopal, R. Barnes, D. Quon, H. Sun, S. Jackson, A. von Drygalski
Abstract Background Patients with haemophilia have a higher prevalence of hypertension than the general population that cannot be explained by traditional cardiovascular risk factors such as age, race, diabetes or obesity. Patients with severe haemophilia, who are on clotting factor prophylaxis, have a higher prevalence of hypertension compared to patients with milder forms of haemophilia, who infuse clotting factor less frequently. This raises the question of whether there is a link between clotting factor usage and blood pressure in haemophilia patients. Methods Data was collected from 193 patients with severe haemophilia presenting to three haemophilia treatment centres in the United States and Canada, including age, body mass index (BMI), blood pressure (BP), Hepatitis C (HCV) and Human Immunodeficiency Virus (HIV) infection status, and clotting factor usage from pharmacy prescriptions (units/kg/year). The correlation between BP and factor usage was examined using quantile regression models. Results Systolic and diastolic BP plotted against factor use showed a cone-shaped scatter of points. There was no association between clotting factor usage and higher systolic or diastolic BP. Conclusion Our observations provide no evidence for an association between increased clotting factor usage and high BP.
{"title":"Blood pressure in haemophilia and its relation to clotting factor usage","authors":"Srila Gopal, R. Barnes, D. Quon, H. Sun, S. Jackson, A. von Drygalski","doi":"10.17225/jhp00147","DOIUrl":"https://doi.org/10.17225/jhp00147","url":null,"abstract":"Abstract Background Patients with haemophilia have a higher prevalence of hypertension than the general population that cannot be explained by traditional cardiovascular risk factors such as age, race, diabetes or obesity. Patients with severe haemophilia, who are on clotting factor prophylaxis, have a higher prevalence of hypertension compared to patients with milder forms of haemophilia, who infuse clotting factor less frequently. This raises the question of whether there is a link between clotting factor usage and blood pressure in haemophilia patients. Methods Data was collected from 193 patients with severe haemophilia presenting to three haemophilia treatment centres in the United States and Canada, including age, body mass index (BMI), blood pressure (BP), Hepatitis C (HCV) and Human Immunodeficiency Virus (HIV) infection status, and clotting factor usage from pharmacy prescriptions (units/kg/year). The correlation between BP and factor usage was examined using quantile regression models. Results Systolic and diastolic BP plotted against factor use showed a cone-shaped scatter of points. There was no association between clotting factor usage and higher systolic or diastolic BP. Conclusion Our observations provide no evidence for an association between increased clotting factor usage and high BP.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130546041","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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