Few would disagree that haemophilia presents challenges for everyone who lives with it. Perhaps the greatest of these is being denied the opportunities available to other people through fear of bleeding and joint damage. The regular infusions and clinic appointments that characterise treatment are certainly burdensome, and sometimes traumatic for children and parents alike; but being told ‘People with haemophilia can’t do this…’ is damning in its finality. It is enough to make you demand, ‘Why not?’ - which is exactly what Clive Smith and a growing number of people in the haemophilia community are asking.
The need to prepare children and young people with haemophilia for long-term care in the adult sector has long been accepted and is reflected in many national health care policies. Ideally, transition should be an individualised age- and development-appropriate process, through which each young person is empowered to self-manage, with support from their families and multidisciplinary team (MDT). It is widely recognised that young people who are not engaged in self-management frequently become non-adherent to therapy. For those with haemophilia, this can have a life-long impact on joint health and quality of life. In practice, anecdotal reports from individuals suggest that the transition process is often poorly planned, leaving them feeling lost and unsupported by the healthcare professionals who, until this point, have been core members of their support network. Rarely are the views of young people or their families sought regarding the outcome of their transition process. For those with less severe disease or rarer bleeding disorders, and for girls at the menarche, transition is often not addressed at all. This literature review forms the first part of a project that seeks to identify the outcome measures that can be used to drive a nurse-led initiative improvement in transition for young people with bleeding disorders, through changes in both working practices among health professionals and in the underpinning system, which enables them to put such changes into practice.
Tranexamic acid inhibits fibrinolysis by competitively blocking the lysine binding sites of plasminogen, inhibiting binding between fibrin and plasminogen, and activation of plasminogen. It also competitively inhibits tissue plasminogen activator and inhibits plasmininduced platelet activation. The synthesis of tranexamic acid was first reported in 1962. It is used to treat or prevent excessive blood loss from trauma, surgery, and in various medical conditions including haemophilia and heavy menstrual bleeding. As a medicine affecting coagulation, it is listed in the World Health Organization’s List of Essential Medicines. Tranexamic acid remains a versatile and inexpensive agent with potential benefit in health services from the lowest to highest income countries. This pharmacy review focuses on the evidence base to support the use of tranexamic acid in acquired and inherited bleeding disorders to reduce bleeding complications.
Long-standing inhibitors present many day-today difficulties for the affected individual; the unpredictability of bleeds, bleed management, pain and treatment efficacy all affect quality of life. This study explored these issues through focus groups of affected individuals aged 16-25 in the UK. The data from the focus groups was analysed for recurring themes, which were coded under three umbrella headings: ‘daily impact’, ‘education and future’ and ‘resilience and support’. Participants felt isolated through geography and being extra ‘rare’ within the bleeding disorders community; used pain as a gauge of bleed resolution, often without use of analgesia; described transition to adult care as particularly worrying; and explained the potential impact of living with an inhibitor on future career options. Peer-to-peer networking could provide emotional support for these young adults, who could also be role models for the future. Despite the burden of living with an inhibitor and its treatment, participants described a good quality of life from their own perspectives. With new therapeutic options for these individuals on the horizon, they look forward to a future with fewer bleeds and less pain.
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.
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.
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.
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.
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.