The Journal of Haemophilia Practice最新文献

{"title":"Raising awareness globally for women with inherited bleeding disorders: World Federation of Hemophilia Symposium","authors":"Luisa Durante, Martin Sedmina, A. Vašková, B. Ziemele, A. Tollwé","doi":"10.17225/jhp00140","DOIUrl":"https://doi.org/10.17225/jhp00140","url":null,"abstract":"Abstract Figures in the World Federation of Hemophilia (WFH) global annual survey indicate, by their absence, that there is under-recognition of bleeding disorders in women. The WFH and its national member organisations (NMOs) are working to raise awareness and improve the diagnosis of care of women with bleeding disorders globally, regionally and locally. WFH initiatives include a global programme focused on improving the diagnosis, care and treatment of women with von Willebrand disease (VWD), and programmes involving education and training in conjunction with NMOs in countries including Honduras and Malaysia. NMOs in Slovakia, Latvia and Sweden describe their local activities. The Slovak Hemophilia Society is in the process of establishing a Women’s Committee and considers peer support and network building as essential tools in addressing the issues faced by women with bleeding disorders. In Latvia, access to resources is difficult and von Willebrand factor is not available. There is concern in the Latvia Hemophilia Society that the fundamental human right of access to healthcare is not being met. It supports WFH initiatives through education and advocacy, and believes that the voices of women with bleeding disorders will be better heard through working together. The Swedish Hemophilia Society’s Women’s Project has worked since 2006 to promote better care for women with bleeding disorders and to raise public awareness. Despite resistance, their campaign to increase the identification of girls and women with VWD, improve diagnosis and care, and raise awareness has been well received by healthcare professionals and has had extensive media coverage.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"1 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":"131902130","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}

{"title":"Complementary and alternative therapy (CAM) in haemophilia pain management: a review of published literature","authors":"A. Lambing, M. Witkop, T. Humphries","doi":"10.17225/JHP00122","DOIUrl":"https://doi.org/10.17225/JHP00122","url":null,"abstract":"Abstract Pain continues to be a significant issue for people with haemophilia (PWH), whether it is acute pain from a joint bleed, a needle stick when receiving clotting factor treatment, or chronic pain from chronic synovitis and arthritic changes. In the US, there has been controversy and uncertainty regarding the use of oral medications and opioids for the management of chronic pain, including haemophilia pain. Both PWH and their clinicians have started to explore nonpharmacologic options for managing pain, with many PWH trying to limit their use of oral pain medication and using alternative methods. There is little research around the use of complementary and alternative medicine (CAM) for pain management in the haemophilia community. This paper aims to provide a comprehensive literature review of current English language research on the use of therapies that fall into four distinctive domains of CAM in PWH. English language articles were obtained via searches in Medline, Embase and CINAHL using the target search words “haemophilia” or “bleeding disorders” in combination with other search terms relating to CAM. All references were considered regardless of year. Twenty-three CAM articles that included PWH or other bleeding disorders were identified; ten were evidence based while the remainder provided case reports and descriptions of CAM use in PWH. This indicates that there is limited current research in the area of CAM use for pain management in PWH. Evidence of the benefit of CAM is seen in studies of its use to treat pain in other conditions and health-related circumstances. Additional evidenced-based CAM research is needed so that alternative pain management therapies can be utilised in a safe and beneficial manner in PWH.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"20 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":"133545192","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}

{"title":"Bleeding disorders in girls and women: setting the scene","authors":"Naja Skouw-Rasmussen, M. Lavin, Minette van der Ven","doi":"10.17225/jhp00138","DOIUrl":"https://doi.org/10.17225/jhp00138","url":null,"abstract":"Abstract 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 bleeding disorders in women in four domains: physical activity, active life, romantic and social life, and reproductive life. Most women had von Willebrand disease (VWD) or described themselves as a carrier. All reported a negative impact on physical activity, active life and romantic and social life. Up to 70% of women in all groups reported that their bleeding disorder had a significant impact on their ability or willingness to have children, or prevented it. Heavy menstrual bleeding was reported as the having the most significant impact on daily life. Women face barriers to diagnosis and care, including difficulty obtaining a referral and lack of knowledge among general practitioners and gynaecologists. While bleeding disorders share many symptoms, including bleeding after minor injury and trauma, the link between heavy menstrual bleeding and a bleeding disorder often goes unrecognised and its severity is underestimated. Screening is not offered to all eligible women despite the availability of long-established management guidelines; clinical tools to estimate severity may be unreliable. Failure to recognise a bleeding disorder in a woman is a multifactorial problem that is partly due to cultural reluctance to discuss menstruation. Public awareness campaigns are seeking to correct this, and many NMOs involve women in their initiatives and provide women-centred activities. However, a transformation in diagnosis is needed to shift the focus of treatment centres beyond boys and men with haemophilia, and to acknowledge the prevalence and severity of bleeding disorders in women.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"14 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":"131827966","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}

{"title":"The Ugandan Treatment Gap – Peter Nangalu","authors":"Luke Pembroke","doi":"10.17225/jhp00137","DOIUrl":"https://doi.org/10.17225/jhp00137","url":null,"abstract":"Many people in the haemophilia community are aware of the disparity in access to healthcare and resources between developed and economically developing countries. In Uganda, for example, it is estimated that only four out of every 100 people with haemophilia are diagnosed. Access to effective treatments is severely limited, and care for people with haemophilia is available only from Mulago Hospital in the capital, Kampala. Prophylactic factor replacement is not an option, and humanitarian aid donations have allowed for on-demand factor treatment only in recent years. According to the World Federation of Hemophilia, per capita use of factor VIII in Uganda is around 350 times lower than in the UK . Speaking with Peter Nangalu reveals what that treatment gap means for people living with haemophilia. Peter is 23 years old. Despite having severe haemophilia, he is studying computer science at Kampala University. It is an impressive achievement to have got so far: many people born with haemophilia in Uganda do not survive into adulthood. The resilience and strength Peter has shown throughout his life is truly remarkable. Problems started for Peter at age two, when he suspects an attack of malaria marked the first manifestations of haemophilia.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"43 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":"116213683","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}

{"title":"Kin Kam: resilience and enlightenment","authors":"S. Chaplin","doi":"10.17225/jhp00133","DOIUrl":"https://doi.org/10.17225/jhp00133","url":null,"abstract":"K in F Kam is an inventor and writer who has severe haemophilia A. Born in Hong Kong in 1967, at age ten he came to the UK with his mother and two brothers to join his father, who had established a successful fish and chip shop in Oldham, Greater Manchester. He went on to academic success at university, obtained a doctorate and followed a career in research before going on to develop and market his inventions. All this he recounts in his autobiography Blood, Dragons & Lions. So far, so impressive. But Kin lived with the challenges of having haemophilia and being an immigrant from a minority ethnic group – a double dose of difference that weighed heavily on a child growing up in the North West of England during the ‘70s and ‘80s. The experience of coping with haemophilia and the associated stigma was so traumatic that he couldn’t bring himself to use the word throughout most of his book, referring to his condition only as ‘H’.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"56 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":"115369056","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}

{"title":"Opioid dependence among people with haemophilia in a low-resource tropical setting: prevalence and risk factors in northern Nigeria","authors":"Sagir G. Ahmed, U. Ibrahim, M. Kagu","doi":"10.17225/jhp00132","DOIUrl":"https://doi.org/10.17225/jhp00132","url":null,"abstract":"Abstract Background In tropical countries such as Nigeria, where factor VIII (FVIII) is scarce, severe pain due to musculoskeletal bleeding complications, leading to frequent opioid prescription, is not uncommon in poorly managed people with haemophilia (PWH). The relationship between opioid use and dependence is intensively studied in other painful diseases, such as cancer and rheumatoid arthritis, but surprisingly little is known about opioid dependence in haemophilia. We hypothesise that the risk of opioid dependence among PWH in tropical countries like Nigeria is multi-factorial, encompassing demographic (age), clinical (haemophilia severity and chronic arthropathy) and biological (ABO blood groups and haemoglobin (Hb) phenotypes) factors that may directly or indirectly increase incidence of bleeding and/or pain. Aims To determine the prevalence of opioid dependence and relative risks (RR) associated with age, haemophilia severity, chronic arthropathy, ABO blood groups and Hb phenotypes, and to elucidate the pathophysiological roles of each risk factor in the development of opioid dependence among haemophilia-A patients in five hospitals in northern Nigeria. Methodology A retrospective review of the medical records of 88 PWH seen between 1996 and 2012 was used to collate data on age, sex, haemophilia severity, painful chronic haemophilic arthropathy, ABO blood group, haemoglobin phenotypes, presence or absence of opioid dependence, and the types of opioids on which the patients were dependent. The prevalence of opioid dependence among the cohort was expressed as a percentage. The frequency of each putative risk factor for opioid dependence in patients with and without opioid dependence were compared using Fisher’s exact test; RR associated with each risk factor was determined by regression analysis. P<0.05 was taken as significant. Results Of the 88 PWH studied,15 (17%) were shown to be opioid-dependent. Compared with PWH who were not opioid-dependent, this group had higher frequencies of severe haemophilia (86.7% vs. 49.3%: RR= 5.2, p=0.02), survival to adulthood (73.3% vs. 12.3%: RR= 9.5, p=0.0001), chronic arthropathy in one or more joints (86.7% vs. 21.9%: RR= 13.2, p=0.0004), blood group-O (80% vs. 49.3%: RR= 3.3, p=0.04), and HbAA phenotype (86.7% vs. 54.8%: RR= 4.3, p=0.04). Conclusion Prevalence of opioid dependence among PWH treated at five hospitals in northern Nigeria was 17% during the study period. Significant risk factors were directly or indirectly associated with increased rates of bleeding and/or pain, which can only be prevented or treated through optimal application of FVIII. There is a need for the Nigerian government to establish standard haemophilia care centres with adequate FVIII for optimal prophylaxis and treatment in order to minimise painful complications, thereby helping to prevent undue opioid use and dependence.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"187 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":"127136387","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}

{"title":"Everyday issues in women with bleeding disorders","authors":"Naja Skouw-Rasmussen, D. Pollard","doi":"10.17225/jhp00144","DOIUrl":"https://doi.org/10.17225/jhp00144","url":null,"abstract":"Abstract Men and women with bleeding disorders have similar symptoms but their experiences are different. It has been shown that women with a bleeding disorder rate their quality of life on a par with that of men with haemophilia who have HIV. Many factors determine quality of life, ranging from delay in diagnosis, to access to treatment and support from family and friends. Women should ask themselves what is important to them and recognise the barriers that determine whether they can achieve their aims in life. Quality of life instruments do not measure the impact of these disorders in a way that is specific to women. Psychosocial health – i.e. the mental, emotional, social, and spiritual aspects of what it means to be healthy – can have a major impact on quality of life. Women with bleeding disorders face a number of challenges to their psychosocial health. They struggle to be believed, they live with guilt, and they may have to fight for the best care for their children. They face obstacles to building relationships and their experiences can leave them isolated. Perhaps because of this, women with bleeding disorders are strong – but they also need to be encouraged to make time for themselves and look after their mental health.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"14 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":"132465766","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}

{"title":"Preparing for menarche: treatment and management of heavy periods in women with bleeding disorders","authors":"M. Lavin, R. Kadir, S. Mackensen, D. Pollard, A. Tollwé","doi":"10.17225/jhp00141","DOIUrl":"https://doi.org/10.17225/jhp00141","url":null,"abstract":"Abstract Prolonged menstrual bleeding interferes with daily life and causes marked blood loss, resulting in anaemia and fatigue. Treatment centres should address the issue of heavy menstrual bleeding (HMB) with pre-pubertal girls in advance of their first period, in order to best prepare them. It is common for a bleeding disorder to be overlooked in primary care and in gynaecology clinics, and women sometimes struggle to get a correct diagnosis. There are cultural taboos that inhibit open discussion of menstruation, and women tend to minimise the severity of their symptoms. Health professionals should work to destigmatise the issue and seek an accurate account of bleeding severity, with diagnosis and treatment provided in a joint clinic combining gynaecology and haematology expertise. Treatment should be individualised, taking into account personal, social and medical factors, with the aim of improving quality of life. Great care is needed with regard to choice of language when talking about treatment, and treatment centres should consider offering open access to women who need support in dealing with adverse effects. National member organisations have an important role to play in educating people with bleeding disorders, health professionals and the wider public about the burden of HMB associated with bleeding disorders.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"15 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":"128364066","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}

{"title":"Haemophilic arthropathy: a case of misdiagnosis","authors":"Nakalyango Annette Grace","doi":"10.17225/JHP00124","DOIUrl":"https://doi.org/10.17225/JHP00124","url":null,"abstract":"Abstract This article describes a case of wrongly managed haemophilic arthropathy in a 15-year-old boy in Uganda. Delayed diagnosis of haemophilia and misdiagnosis of haemophilic arthropathy resulted in unnecessary corrective surgery and potentially severe complications. After haemophilia was diagnosed, he was managed by haematologists using factor VIII and regular physiotherapy. The physiotherapy treatment approach focused in particular on functional activities, which was beneficial in achieving quicker results and made the patient’s rehabilitation more meaningful. This case indicates that there is a need for greater awareness of the presentation of haemophilia in developing countries.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"27 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123317671","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}

{"title":"Prevalence, pattern and clinical implications of transfusion transmissible viral infections among paediatric haemophiliacs in northern Nigeria","authors":"Sagir G. Ahmed, U. Ibrahim, M. Kagu","doi":"10.17225/JHP00117","DOIUrl":"https://doi.org/10.17225/JHP00117","url":null,"abstract":"Abstract Background Scarcity of FVIII concentrate compels caregivers in poor countries to use multiple transfusions of fresh whole blood (FWB), fresh frozen plasma and cryoprecipitate for managing haemophilia A. FWB is the most frequently transfused blood product due to ease of production and its ability to simultaneously stop active bleeding and treat anaemia. Iron deficiency anaemia is common among haemophiliacs in poor tropical countries such as Nigeria, due to the combined effects of bleeding, malnutrition, and haemorrhagic parasitic diseases. Multiple FWB transfusion is usually initiated at local sub-tertiary hospitals before eventual referral to tertiary hospitals. The Nigerian blood transfusion service is underdeveloped, donor screening is rudimentary and transfusion safety is poor. The prevalence of transfusion transmissible viral infections (TTVIs), including HIV, and hepatitis B and C viruses (HBV and HCV), is therefore predicted to be high among Nigerian haemophiliacs. Aims To determine prevalence and pattern of TTVIs (HIV, HBV, HCV infections) among paediatric haemophiliacs who have received multiple FWB transfusions in Nigeria. Materials and methods Retrospective analyses of demographic and clinical data, disease severity, number of previous transfusions of FWB, and prevalence and pattern of TTVIs (HIV, HBV and HCV infections) of newly referred haemophiliacs as seen in five tertiary hospitals in northern Nigeria. Prevalence rates of TTVIs were expressed as percentages. Comparisons of parameters (age, disease severity and number of previous transfusions) between patients with and without TTVIs were performed using Students t-test for mean values and Fisher’s exact test for percentages, with p-values of less than 0.05 taken as significant. Results Of 97haemophiliacs studied, 24 (24.7%) were infected with TTVIs. The pattern and frequencies of TTVIs among the infected patients revealed HBV infection in 10 (41.7%), HIV-1 infection in five (20.8%), HCV infection in four (16.7%), HBV and HIV co-infection in three (12.5%), and HBV and HCV co-infection in two (8.3%). In comparison with haemophiliacs without TTVIs, haemophiliacs with TTVIs had a significantly lower mean age (4.9 vs. 7.8; p=0.007); a higher proportion of severe disease (62.5% vs. 26%; p=0.009), and a higher mean number of transfusions per patient (27.5 vs. 15.3; p=0.006). Conclusions The prevalence of TTVIs among haemophiliacs in Nigeria is high, and the risk is correlated with disease severity and number of previous transfusions. There is need for the national transfusion service to be upgraded and for standard haemophilia care centres with an adequate supply of FVIII concentrates for optimal care to be set up. Haemophilia healthcare providers in Nigeria can minimise multiple transfusions by incorporating regular screening and treatment of haemorrhagic parasitic diseases, iron supplementation, and the use of pharmacological agents in the standard of care for haemophilia.","PeriodicalId":372940,"journal":{"name":"The Journal of Haemophilia Practice","volume":"37 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126791121","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}