Sandra Dodgson, Mike Holland, Luke Pembroke and Kate Khair
Transition is the term used to describe the process of approaching and crossing the chronological boundary between paediatric and adult care services. Transfer of care describes the administrative arrangements associated with moving from a paediatric to an adult service across this boundary. Transforming Transition was a nurse-led initiative designed to facilitate the sharing of knowledge and experience to enhance transition practice in the UK. The aims of the programme were to secure changes in working practices that enabled nurses to drive improved transition outcomes, enhance self-management by people with haemophilia and develop clinical leadership. From the outset of the programme it was clear that there were differences in practice and that sharing practice would be one route to addressing some of these variations. As a result of the knowledge and experience shared through the programme, resources described in this paper are available to all haemophilia centres. Sharing between people with haemophilia, their families and carers, and the haemophilia clinical team have resulted in the development of practical resources that enhance practice within the haemophilia centre. Sharing of practice between nurses and young people with haemophilia at the programme workshops provided a focus for development of local and regional action plans, a framework for reviewing progress, the introduction of transition clinics and improved transition documentation.
Introduction: Transgender people face many obstacles to accessing healthcare but cultural changes are likely to increase provision of sex reassignment surgery in countries with sufficient resources. Haemophilia services traditionally focus on providing factor replacement therapy for males and should therefore understand how the care they provide can be adapted to meet the needs of transgender people. Haemophilia is an X-linked congenital bleeding disorder, caused by deficiency of coagulation factor VIII (haemophilia A) or factor IX (haemophilia B). The condition is passed on through carrier females, the majority of whom have a factor level high enough to allow for normal blood clotting. However, around 10% of carrier females are symptomatic and at risk of abnormal bleeding. Case presentation: This case report describes a person with mild haemophilia A who, on first presentation to the haemophilia service, stated he was a transgender person in transition to becoming a male. Haemophilia was diagnosed when heavy bleeding occurred following bilateral mastectomy approximately 25 years previously. He now requested phalloplasty. Management and outcome: Phalloplasty was performed at a hospital geographically separate from the haemophilia centre, requiring careful coordination between the two services. A haemophilia specialist nurse provided education and training about haemophilia and its management to the surgical nurses. Twenty-four-hour support was available from the nurse and a specialist doctor. Preparation and administration of clotting factor was the responsibility of the haemophilia nurse until the surgical team was confident in its use. Clotting factor replacement was managed using standard procedures, successfully maintaining factor VIII above a target level of 100% with a twice daily dose. Surgery went well, but wound healing was delayed, in part, due to persistent bleeding. Discussion: Close collaboration between the haemophilia and surgical teams provided effective prophylaxis of bleeding during a complex
procedure that presented new challenges. Both services now have better understanding of the needs of transgender people.
Haemophilia impacts on the person who has it as well as his close family and friends. The majority of healthcare provider focus is with the person with haemophilia and his carers during childhood, and then on the person himself as he becomes able to self-manage. There is a belief that the family and healthcare team support the patient equally. In this study, which was designed to understand the patient/ carer/healthcare provider relationship, we investigated support mechanisms from the patient’s perspective, using narrative stories from those we call ‘witnesses’. Carers, family and friends rarely feature in haemophilia research, yet can provide in-depth insight into the life of the person with haemophilia. Three key areas were identified which underlie and cause tension in the world of haemophilia. These are described as ‘identity and tackling the lion’, ‘the haemophilia team’ and ‘the unique perspective and influence of the ‘outside-in’. Support from the ‘outside-in’ as well as healthcare providers is important for people with haemophilia for both physical and psychological health. Working together we can better support individuals with haemophilia as well as other members of their extended families. As haemophilia care changes in a new era of therapeutic options; we need to re-evaluate the supportive role of family and carers to ensure that the patient and family voice is heard in decision-making at an individual and national level.
People with haemophilia and other bleeding disorders, such as von Willebrand’s Disease (vWD), are frequently diagnosed in childhood. There is, therefore, a recognised need to prepare children and young people with these conditions for transfer to long-term care in the adult sector. The Transforming Transition programme was designed to facilitate the sharing of knowledge and experience to enhance transition practice in the UK. The aim of the programme was to secure change in working practices to enable nurses to drive improved transition outcomes, enhance self-management by people with haemophilia and develop clinical leadership. As part of the programme, we captured young people’s views of their transition experiences by means of a series of structured interviews conducted with young people identified through the patient association. Interviews revealed that transition tended to take place around the age of 16 but could be delayed in response to individual circumstances. The transition process did not always appear to be planned, with many participants reporting an abrupt or sudden change between paediatric and adult teams and/or centres. Those with severe haemophilia tended to be well educated and prepared by their paediatric teams to be confident in managing their condition from an early age. They would learn to self-infuse between the ages of 5-11, and reported feeling confident in tailoring their treatment around their daily routine. Experience of transition to adult services varied, with about half describing it as fairly well planned. For those with bleeding disorders such as vWD, there was rarely a transition process. These individuals and people with mild haemophilia described having less exposure to opportunities to learn self-management and appeared to experience issues which coincided with significant lifestyle changes, such as leaving home and attending university. This highlights the importance of ensuring that patients across the spectrum of bleeding disorders are adequately supported in the lead-up to and transfer to adult services. The interviews also reinforce the need to continue to address the specific needs of women with bleeding disorders as they transfer to adult services. The learning from this phase of the Transforming Transition programme was submitted to NICE as part of the consultation on its guideline.
Sharon Alavian, Wendy Hutchinson, Abigail Morris, Heather Williams and Debra Pollard
Introduction and objective: In the UK, the National Service Specification for haemophilia stipulates that all patients with mild inherited bleeding disorders must be reviewed annually by their haemophilia centre. For those patients who rarely experience problems relating to their bleeding disorder, attending a yearly hospital-based appointment may be viewed as a low priority. This can result in missed appointments and disconnection from their haemophilia centre, leading to poor understanding of how to manage their condition in emergencies, or when surgical or other invasive procedures may be necessary. The inherited nature of these conditions also has implications for reproduction, and it is of vital importance that the risk of bleeding around labour, delivery and the neonatal period are fully understood and mitigated against. The introduction of a structured, nurse-led telephone clinics across the North London Adult Haemophilia Network (NLAHN) offered an alternative method for patients to be reviewed. This strategy was then evaluated to assess whether the needs of patients were being fulfilled. Materials and methods: Clinical nurse specialists (CNS) from the NLAHN devised a short service evaluation questionnaire with Likert scales and one open question. Patients across the NLAHN sites who had received a telephone review in 2016 were sent an anonymised questionnaire, with a stamped addressed envelope and a six-week return date. Results: 514 questionnaires were distributed, 174 were returned, and 18 were excluded as returned incomplete, giving a return rate of 28%. Overall, 89% (139/156) of patients rated the new service between excellent and very good; 89% (139/156) reported that they were very satisfied with the information received in the review; and 95% (149/156) were happy to continue to receive telephone reviews. Conclusion: Patients found the telephone reviews a viable alternative to traditional hospital-based appointments. The telephone clinics are more convenient for patients in terms of time and resources; they also helped those surveyed to re-engage with their haemophilia centre, ensuring continued education about their condition and the services offered. Overall attendance rates for the follow-up of patients with mild bleeding disorders have improved, with a reduction in traditional clinic appointments for this group. This has an ongoing positive impact on waiting lists and the financial burden of missed hospital appointments without impacting patient care.
Masoume Rambod, Farkondeh Sharif, Zahra Molazem and Kate Khair
Background: Pain management can prevent long-term burdens in haemophilia patients and improve their quality of life. The present study aimed to describe and interpret pain experiences in haemophilia patients, focusing on pain self-management in their lives. Methods: This was a qualitative study undertaken using a hermeneutic phenomenological approach. The study involved 14 haemophilia patients referred to a haemophilia clinic affiliated to Shiraz University of Medical Sciences in Iran. Data were collected using semi-structured interviews and field notes. Thematic analysis with van Manen’s methodological framework was applied. Data analysis was performed using MAX. QDA qualitative software (2010). Results: Four themes emerged: a sense of self-awareness and recognition of pain and the factors that affect it, the ability to control and self-manage pain, gradually achieving self-efficacy in pain control, and using cognitive and spiritual strategies for pain relief. Conclusions: The study highlighted the essence of the lived experience of pain self-management and generated its linguistic description. By providing complementary therapy interventions, healthcare providers and family members could increase patients’ self-awareness, recognition, ability to self-manage and control pain effectively, and competence in developing cognitive and spiritual strategies for pain relief.
People with Inherited Bleeding Disorders (IBD) are often prescribed a course of Tranexamic Acid (TXA) mouthwash for five to seven days following dental procedures to reduce the risk of bleeding. Informal discussions with patients suggested that many do not complete the prescribed course of treatment. A literature review indicated that TXA was prescribed inappropriately for procedures with a low bleeding risk, and that there are inconsistencies in the recommended dose, mode of administration and duration of TXA for this patient group. A new protocol was implemented in the haemophilia centre at St George’s University Hospital NHS Foundation Trust, London, to rationalise the prescribing of TXA in dental procedures. A study was conducted to explore patients’ experience of this new guideline in the form of a service evaluation. Structured telephone interviews were completed following 39 dental procedures to collect data on concerns about bleeding; whether TXA was taken as prescribed and reasons for non-adherence; and any unplanned post-operative treatment. The financial impact of the new guideline was also explored. Patients were supportive of the new regimen, although almost half (46%) did not complete the prescribed course of TXA. The majority (37/39) were prescribed tablets rather than mouthwash. No patients required additional unplanned haemostasis support to control haemorrhage. Cost savings were made by replacing a five- to seven-day course of TXA mouthwash with a three-day course of TXA tablets. Although the data collected from patient interviews supports the new guideline, patients appear to be making decisions about taking TXA based on their own experience rather than following the prescribed regimen. Prescribers should support patients to make informed decisions about their medicines and incorporate patient experience into individualised regimens. Given the lack of bleeding complications experienced in this cohort of patients, it is possible that TXA is being overprescribed. Further work exploring how patients with IBDs make decisions about taking medicines is needed.
Laura Savini, Radoslaw Kaczmarek, Declan Noone, Paul Giangrande, Geoffrey Dusheiko and Brian O’Mahony
In the 1980s and 1990s, thousands of people with bleeding disorders (PWBD) across the world were infected with HIV and hepatitis C virus (HCV) through contaminated treatment products. The extent of the infection, as well as the needs of those still living with HCV, were never properly assessed. The purpose of our survey was to identify how many PWBD were infected with HCV in Europe, as well as their health status and needs. HCV infection was defined as any person with a bleeding disorder who was exposed to the virus and seroconverted to become anti-HCV antibody positive. The survey also looked at testing and treatment availability. Between December 2016 and March 2017, the survey was distributed to 45 national patient organisations in the European Haemophilia Consortium (EHC), who were encouraged to respond with the support of a local hepatologist. The data gathered led us to estimate that some 15,000 people with bleeding disorders were infected with HCV in the 30 countries that responded. Although some countries have detailed records of patients with HCV, most - including some with national haemophilia registries - were unable to provide exact numbers of initial infections, HIV coinfection, survival and SVR rates. Responding countries reported varying degrees of monitoring for disease progression, as well as extremely divergent access to new direct-acting antivirals, with only eight countries prioritising PWBD for treatment. With liver disease and hepatocellular carcinoma being among the main causes of death in an aging bleeding disorders population, this survey identifies a clear gap in care. It is a frustrating paradox that today, in many European countries PWBD, such as haemophilia, may live long and productive lives due to much-improved access to factor replacement therapy, yet die prematurely of a curable disease such as hepatitis C. It has been demonstrated that HCV eradication in PWBD can be achieved through national commitment, especially when the patient population is limited and HCV eradication could be achieved in the short-term. The eradication of HCV in PWBD in Europe is an idea whose time has come.
Debra Pollard, Kate Khair, Cléa Percier, Yen Wong and Robyn Shoemark
Management of haemophilia involves on-demand or prophylactic intravenous administration of recombinant or plasma-derived replacement clotting factors or bypassing agents. These products are provided as lyophilised powder and diluent, which need to be mixed to produce a solution for infusion. While this process has previously involved multiple time-consuming steps, several reconstitution systems are now available to make mixing easier and more convenient. This study aimed to investigate experience of use and perceptions of the Novo Nordisk MixPro® mixing device among patients and carers using activated recombinant factor VII (rFVIIa) or recombinant factor VIII (rFVIII) with MixPro, and nurse specialists who were either familiar or unfamiliar with MixPro. Nurses were asked to simulate the preparation of an inactive solution using MixPro. Semi-structured interviews were used to gain insight into participants’ opinions of mixing systems in general and their perceptions of MixPro. Likert scales were used to rate the performance of MixPro against predefined characteristics of mixing systems, and the importance of the predefined characteristics to the design of a mixing system. Patients/carers and unfamiliar nurses identified low contamination risk when mixing as the most important characteristic of a mixing system; the most important criterion for familiar nurses was confidence that patients/carers could prepare the system correctly. MixPro was perceived to perform very well overall, particularly in parameters identified as most important. It was described as being user-friendly, simple and quick; its compactness and portability were highlighted as advantages for storage and travel. The main disadvantages reported related to its small components. The majority of nurses said that they were highly likely to recommend MixPro to their patients.
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.