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2055-3390
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22 Dec 2017
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Volumen 5 (2018): Edición 1 (April 2018)

Detalles de la revista
Formato
Revista
eISSN
2055-3390
Publicado por primera vez
22 Dec 2017
Periodo de publicación
1 tiempo por año
Idiomas
Inglés

Buscar

21 Artículos

Clinical Report

Acceso abierto

Head injury in a haemophilia patient – Nursing considerations: a case report

Publicado en línea: 14 Apr 2018
Páginas: 1 - 3

Resumen

Abstract

A person with haemophilia is at risk of intracranial haemorrhage caused by spontaneous or traumatic events, potentially resulting in long-term disability. Early initiation of timely treatment is essential to prevent this. In developing countries, morbidity and mortality is common in intracerebral haemorrhage (ICH) cases with haemophilia as a result of patients and family members having inadequate knowledge, lack of awareness regarding referral, lack of availability of treatment products, and delays in the treatment decision. These barriers can potentially be managed by the haemophilia nurse taking responsibility for the coordination of care. This case report addresses the nurse-coordinated care of a haemophilic patient with ICH in a developing country, and may provide insight into the benefits of a haemophilia nurse undertaking a coordinating role.

Palabras clave

  • Bleeding
  • Brain injury
  • Haemophilia
  • Haemorrhage
  • Head injury
  • Nursing care
Acceso abierto

Haemostasis action of VELSEAL-T in a haemophilia A patient with external bleeding

Publicado en línea: 14 Apr 2018
Páginas: 4 - 7

Resumen

Abstract

VELSEAL-Tis an innovative haemostatic medical device for the control of bleeding. Incorporating a clotting agent (thrombin) and anti-fibrinolytic agent (tranexamic acid), it enables rapid coagulation when blood flows into the dressing, leading to sealing and stabilisation of wound surfaces. A 36-year-old known to have mild haemophilia A presented with profuse bleeding from the forehead after injury following a fall on concrete surface. He attended hospital after 18 hours of injury as bleeding continued as soon as pressure was released from the injury site. A VELSEAL-T patch was applied to the injury site with the patient’s full consent. The bleeding stopped after 60 seconds, but the patient was instructed to hold the patch in place for a further 60 seconds. After two minutes of tight pressure application, there was no more oozing of blood from the injury site. This case shows that VELSEAL-T can be used as an aid in stopping external bleeding in haemophilia patients. Further trials should be undertaken to evaluate the safety and efficacy of this product.

Palabras clave

  • VELSEAL-T
  • Haemophilia
  • External bleeding
  • Haemostatic patch

Case Study

Acceso abierto

Myocardial infarction in severe haemophilia

Publicado en línea: 14 Apr 2018
Páginas: 8 - 11

Resumen

Abstract

With improved replacement therapy, people with haemophilia (PWH) live longer, with the consequent emergence of age-related chronic diseases not previously seen in haemophilia. The prevalence of cardiovascular disease and associated complications in older PWH appears to be on the rise. Management of myocardial infarction in a person living with severe haemophilia presents a particular challenge, as treatment options often include antiplatelet therapy, anti-coagulation and invasive procedures. Current guidelines for managing acute myocardial infarction are not specifically designed for people with bleeding diatheses such as haemophilia. We present a patient with severe haemophilia A on extended half-life factor VIII prophylaxis who developed an acute ST-elevation myocardial infarction (STEMI), discuss his clinical management and highlight lessons learned.

Palabras clave

  • Coagulation disorders
  • Myocardial infarction
  • Severe haemophilia
Acceso abierto

Evaluation of a pre-filled diluent syringe (MixPro®) among patient/carer users and nurses

Publicado en línea: 31 May 2018
Páginas: 12 - 23

Resumen

Abstract

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.

Palabras clave

  • Haemophilia
  • Recombinant factor VIIa
  • Recombinant factor VIII
  • Reconstitution device
  • Reconstitution system
Acceso abierto

Living with an inhibitor: Results from the Study of Haemophilia Experiences, Results and Opportunities in Children and young adults with long-standing inhibitors (the SO-HEROIC study)

Publicado en línea: 31 May 2018
Páginas: 24 - 34

Resumen

Abstract

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.

Palabras clave

  • haemophilia
  • inhibitor
  • quality of life
Acceso abierto

Subcutaneous injection: learning from experience in other specialties

Publicado en línea: 31 May 2018
Páginas: 35 - 41

Resumen

Abstract

Haemophilia treatment is characterised by intravenous infusions of clotting factor concentrates, with nurses frequently taking the lead role in administration, patient training and patient care. In recent years, a number of novel factor and non-factor-based therapies delivered by subcutaneous injection have been developed. These therapies are now undergoing clinical trials and will shortly be available in clinical practice. The coming era of at least some haemophilia treatments being delivered by subcutaneous injection clearly represents a significant change not only for patients (for whom they may be more convenient) but also for haemophilia nurse practice, particularly with respect to bleed and surgical management plans, and hence for nurse training and education. This review describes evidence-based guidance on subcutaneous injection technique and summarises the implications for nurses.

Palabras clave

  • Administration
  • Injection
  • Patient preference
  • Subcutaneous
Acceso abierto

A case study exploring the impact on family life of two genetic conditions – severe haemophilia A and sickle cell disease

Publicado en línea: 21 Dec 2018
Páginas: 138 - 142

Resumen

Abstract

Sickle cell disease and haemophilia are rare inherited disorders that require intensive management. Few cases of people with both disorders have been reported. This case study describes the impact of living with these disorders and coping with their management on a boy and his mother.

Palabras clave

  • Haemophilia A
  • sickle cell disease
  • ethnic minority group
  • social care
  • housing
  • language skills
Acceso abierto

Acquired haemophilia A in an elderly patient related to relapsed cervix carcinoma

Publicado en línea: 14 Dec 2018
Páginas: 143 - 146

Resumen

Abstract

Acquired haemophilia A (AHA) is a rare, autoimmune disease, presenting as sudden haemorrhages without any personal or family history. Anti-factor VIII (FVIII) is the most commonly recognised autoantibody resulting in decreased factor activity. The aetiology and pathophysiology of these antibodies remains unclear. Approximately 50% of reported cases are idiopathic; the rest are associated with other conditions, mainly underlying malignancies, autoimmune diseases (eg rheumatoid arthritis (RA), systemic lupus erythematosus), drug administration and postpartum period. A 74-year-old woman presented to our institution with haematochezia and haematuria. She had a medical history of cervix carcinoma; total abdominal hysterectomy and bilateral salpingo-oophorectomy with postoperative chemoradiotherapy was performed in 2011. She had also been followed up for 20 years for deforming and severe RA, which was in low-disease activity with methotrexate and corticosteroid. Laboratory investigations for abnormal bleeding revealed prolongation of activated partial thromboplastin time (APTT). APTT prolongation was not corrected by 50:50 plasma mixing studies, and a confirmatory factor assay demonstrated FVIII deficiency (1.4 IU/dL; normal range 50-150 IU/dL). Positive FVIII antibodies on Bethesda testing confirmed a diagnosis of AHA. A rectosigmoid mass and fistula between rectum and bladder were discovered by computed tomography (CT). Bleeding was controlled with recombinant activated factor VII (rFVIIa) after two weeks. Eradication of the inhibitor was achieved with high-dose pulse methylprednisolone for two days and then 2mg/kg daily over four weeks.

Palabras clave

  • Acquired haemophilia A
  • bleeding
  • cancer
  • rheumatoid arthritis
Acceso abierto

A transgender person with haemophilia

Publicado en línea: 08 Jan 2019
Páginas: 147 - 151

Resumen

AbstractIntroduction

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.

Palabras clave

  • Carrier
  • haemophilia
  • surgery
  • transgender
Acceso abierto

Haemophilic arthropathy: a case of misdiagnosis

Publicado en línea: 21 Dec 2018
Páginas: 156 - 159

Resumen

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.

Palabras clave

  • Arthrofibrosis
  • arthropathy
  • haemarthrosis
  • misdiagnosis
  • physiotherapy
  • Uganda

Clinical Research

Acceso abierto

Evaluation of a shortened course of tranexamic acid for patients with inherited bleeding disorders following dental procedures

Publicado en línea: 28 Jun 2018
Páginas: 42 - 49

Resumen

Abstract

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.

Palabras clave

  • Antifibrinolytics
  • Bleeding disorders
  • Dental procedures
  • Haemophilia
  • Tranexamic acid
  • Service evaluation
Acceso abierto

Pain self-management experiences in haemophilia patients: a qualitative study

Publicado en línea: 18 Oct 2018
Páginas: 76 - 82

Resumen

AbstractBackground

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.

Palabras clave

  • Experience
  • Haemophilia
  • Pain
  • Self-management
Acceso abierto

Effects of top rope climbing therapy on joint health in adult haemophilia patients with severe arthropathies

Publicado en línea: 14 Dec 2018
Páginas: 93 - 102

Resumen

AbstractBackground

Low impact physical activity is important for patients with haemophilic arthropathies, but is often considered boring with suboptimal adherence. There is therefore a need for physical activities that motivate patient engagement.

Aims

To evaluate the benefits of top rope climbing, increasingly used in other musculoskeletal disorders, as an engaging sports discipline in haemophilia.

Materials/Methods

Six adult arthropathic patients with haemophilia (PWH) completed 12 sessions of tailored top rope climbing training. Functional and clinical joint status, climbing skills, quality of life (QoL), annual bleed rate (ABR) and joint findings with musculoskeletal ultrasound/power doppler (MSKUS/PD) were assessed before and after climbing.

Results

Haemophilia joint health scores, dorsiflexion in arthropathic joints and climbing skills all improved. ABRs were comparable before and during climbing, and QoL remained high. MSKUS evaluation demonstrated no detrimental effects on synovial and cartilage health, with a decrease of inflammatory PD signal in some joints.

Conclusion

We conclude that top rope climbing therapy (“Haemophilia Vertical”) can improve joint health in PWH with arthropathies. Haemophilia Vertical therefore emerges as an innovative athletic concept to promote physical activity among PWH. Further study investigating the longer-term impact in a larger cohort is warranted.

Palabras clave

  • Arthropathy
  • Climbing
  • Haemophilia
  • Physical activity
  • Ultrasound

Clinical Practice

Acceso abierto

Hepatitis C and bleeding disorders in Europe

Publicado en línea: 28 Jun 2018
Páginas: 50 - 65

Resumen

Abstract

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.

Palabras clave

  • Bleeding disorders
  • Haemophilia
  • Hepatitis C
  • Monitoring
  • Treatment
Acceso abierto

Guidelines for the management of haemophilia in Egypt

Publicado en línea: 18 Jan 2019
Páginas: 83 - 92

Resumen

Abstract

These guidelines have been developed by an expert panel of haemophilia treaters to support the appropriate management of people with haemophilia in Egypt. Although the guidelines are based primarily on the World Federation of Hemophilia (WFH) Guidelines for the Management of Hemophilia, they aim to address unmet needs and local requirements in the Egyptian setting.

Palabras clave

  • Bleeding disorders
  • Egypt
  • Guidelines
  • Haemophilia
  • Management
  • Treatment
Acceso abierto

Nurse-led telephone review service for mild inherited bleeding disorders improves attendance rates, frees hospital resources and is highly rated by patients

Publicado en línea: 18 Oct 2018
Páginas: 66 - 75

Resumen

AbstractIntroduction 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.

Palabras clave

  • Haemophilia
  • Mild inherited bleeding disorders
  • Nurse-led
  • Review
  • Telephone clinics
Acceso abierto

Transforming Transition: enhancing practice in haemophilia

Publicado en línea: 14 Dec 2018
Páginas: 111 - 121

Resumen

Abstract

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.

Palabras clave

  • Transition
  • leadership
  • young people
Acceso abierto

Young people’s experience of transition from paediatric to adult haemophilia care in the UK

Publicado en línea: 14 Dec 2018
Páginas: 122 - 129

Resumen

Abstract

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.

Palabras clave

  • Transition
  • patient views
  • haemophilia
  • young people
Acceso abierto

You think you know the patient inside-out. But do you know the outside-in?

Publicado en línea: 14 Dec 2018
Páginas: 130 - 137

Resumen

Abstract

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.

Palabras clave

  • haemophilia
  • inside-out
  • outside in
  • patient experience
  • self-management

Community Focus

Acceso abierto

“Ask what can you do instead of thinking about what you can’t” Profile: Clive Smith

Publicado en línea: 21 Dec 2018
Páginas: 152 - 155

Resumen

Abstract

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.

Evidence Review

Acceso abierto

Prevalence, pattern and clinical implications of transfusion transmissible viral infections among paediatric haemophiliacs in northern Nigeria

Publicado en línea: 18 Jan 2019
Páginas: 103 - 110

Resumen

AbstractBackground

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.

Palabras clave

  • Haemophilia
  • Multiple transfusion
  • Nigeria
  • Transfusion transmissible infections
  • Tropical developing countries
21 Artículos

Clinical Report

Acceso abierto

Head injury in a haemophilia patient – Nursing considerations: a case report

Publicado en línea: 14 Apr 2018
Páginas: 1 - 3

Resumen

Abstract

A person with haemophilia is at risk of intracranial haemorrhage caused by spontaneous or traumatic events, potentially resulting in long-term disability. Early initiation of timely treatment is essential to prevent this. In developing countries, morbidity and mortality is common in intracerebral haemorrhage (ICH) cases with haemophilia as a result of patients and family members having inadequate knowledge, lack of awareness regarding referral, lack of availability of treatment products, and delays in the treatment decision. These barriers can potentially be managed by the haemophilia nurse taking responsibility for the coordination of care. This case report addresses the nurse-coordinated care of a haemophilic patient with ICH in a developing country, and may provide insight into the benefits of a haemophilia nurse undertaking a coordinating role.

Palabras clave

  • Bleeding
  • Brain injury
  • Haemophilia
  • Haemorrhage
  • Head injury
  • Nursing care
Acceso abierto

Haemostasis action of VELSEAL-T in a haemophilia A patient with external bleeding

Publicado en línea: 14 Apr 2018
Páginas: 4 - 7

Resumen

Abstract

VELSEAL-Tis an innovative haemostatic medical device for the control of bleeding. Incorporating a clotting agent (thrombin) and anti-fibrinolytic agent (tranexamic acid), it enables rapid coagulation when blood flows into the dressing, leading to sealing and stabilisation of wound surfaces. A 36-year-old known to have mild haemophilia A presented with profuse bleeding from the forehead after injury following a fall on concrete surface. He attended hospital after 18 hours of injury as bleeding continued as soon as pressure was released from the injury site. A VELSEAL-T patch was applied to the injury site with the patient’s full consent. The bleeding stopped after 60 seconds, but the patient was instructed to hold the patch in place for a further 60 seconds. After two minutes of tight pressure application, there was no more oozing of blood from the injury site. This case shows that VELSEAL-T can be used as an aid in stopping external bleeding in haemophilia patients. Further trials should be undertaken to evaluate the safety and efficacy of this product.

Palabras clave

  • VELSEAL-T
  • Haemophilia
  • External bleeding
  • Haemostatic patch

Case Study

Acceso abierto

Myocardial infarction in severe haemophilia

Publicado en línea: 14 Apr 2018
Páginas: 8 - 11

Resumen

Abstract

With improved replacement therapy, people with haemophilia (PWH) live longer, with the consequent emergence of age-related chronic diseases not previously seen in haemophilia. The prevalence of cardiovascular disease and associated complications in older PWH appears to be on the rise. Management of myocardial infarction in a person living with severe haemophilia presents a particular challenge, as treatment options often include antiplatelet therapy, anti-coagulation and invasive procedures. Current guidelines for managing acute myocardial infarction are not specifically designed for people with bleeding diatheses such as haemophilia. We present a patient with severe haemophilia A on extended half-life factor VIII prophylaxis who developed an acute ST-elevation myocardial infarction (STEMI), discuss his clinical management and highlight lessons learned.

Palabras clave

  • Coagulation disorders
  • Myocardial infarction
  • Severe haemophilia
Acceso abierto

Evaluation of a pre-filled diluent syringe (MixPro®) among patient/carer users and nurses

Publicado en línea: 31 May 2018
Páginas: 12 - 23

Resumen

Abstract

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.

Palabras clave

  • Haemophilia
  • Recombinant factor VIIa
  • Recombinant factor VIII
  • Reconstitution device
  • Reconstitution system
Acceso abierto

Living with an inhibitor: Results from the Study of Haemophilia Experiences, Results and Opportunities in Children and young adults with long-standing inhibitors (the SO-HEROIC study)

Publicado en línea: 31 May 2018
Páginas: 24 - 34

Resumen

Abstract

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.

Palabras clave

  • haemophilia
  • inhibitor
  • quality of life
Acceso abierto

Subcutaneous injection: learning from experience in other specialties

Publicado en línea: 31 May 2018
Páginas: 35 - 41

Resumen

Abstract

Haemophilia treatment is characterised by intravenous infusions of clotting factor concentrates, with nurses frequently taking the lead role in administration, patient training and patient care. In recent years, a number of novel factor and non-factor-based therapies delivered by subcutaneous injection have been developed. These therapies are now undergoing clinical trials and will shortly be available in clinical practice. The coming era of at least some haemophilia treatments being delivered by subcutaneous injection clearly represents a significant change not only for patients (for whom they may be more convenient) but also for haemophilia nurse practice, particularly with respect to bleed and surgical management plans, and hence for nurse training and education. This review describes evidence-based guidance on subcutaneous injection technique and summarises the implications for nurses.

Palabras clave

  • Administration
  • Injection
  • Patient preference
  • Subcutaneous
Acceso abierto

A case study exploring the impact on family life of two genetic conditions – severe haemophilia A and sickle cell disease

Publicado en línea: 21 Dec 2018
Páginas: 138 - 142

Resumen

Abstract

Sickle cell disease and haemophilia are rare inherited disorders that require intensive management. Few cases of people with both disorders have been reported. This case study describes the impact of living with these disorders and coping with their management on a boy and his mother.

Palabras clave

  • Haemophilia A
  • sickle cell disease
  • ethnic minority group
  • social care
  • housing
  • language skills
Acceso abierto

Acquired haemophilia A in an elderly patient related to relapsed cervix carcinoma

Publicado en línea: 14 Dec 2018
Páginas: 143 - 146

Resumen

Abstract

Acquired haemophilia A (AHA) is a rare, autoimmune disease, presenting as sudden haemorrhages without any personal or family history. Anti-factor VIII (FVIII) is the most commonly recognised autoantibody resulting in decreased factor activity. The aetiology and pathophysiology of these antibodies remains unclear. Approximately 50% of reported cases are idiopathic; the rest are associated with other conditions, mainly underlying malignancies, autoimmune diseases (eg rheumatoid arthritis (RA), systemic lupus erythematosus), drug administration and postpartum period. A 74-year-old woman presented to our institution with haematochezia and haematuria. She had a medical history of cervix carcinoma; total abdominal hysterectomy and bilateral salpingo-oophorectomy with postoperative chemoradiotherapy was performed in 2011. She had also been followed up for 20 years for deforming and severe RA, which was in low-disease activity with methotrexate and corticosteroid. Laboratory investigations for abnormal bleeding revealed prolongation of activated partial thromboplastin time (APTT). APTT prolongation was not corrected by 50:50 plasma mixing studies, and a confirmatory factor assay demonstrated FVIII deficiency (1.4 IU/dL; normal range 50-150 IU/dL). Positive FVIII antibodies on Bethesda testing confirmed a diagnosis of AHA. A rectosigmoid mass and fistula between rectum and bladder were discovered by computed tomography (CT). Bleeding was controlled with recombinant activated factor VII (rFVIIa) after two weeks. Eradication of the inhibitor was achieved with high-dose pulse methylprednisolone for two days and then 2mg/kg daily over four weeks.

Palabras clave

  • Acquired haemophilia A
  • bleeding
  • cancer
  • rheumatoid arthritis
Acceso abierto

A transgender person with haemophilia

Publicado en línea: 08 Jan 2019
Páginas: 147 - 151

Resumen

AbstractIntroduction

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.

Palabras clave

  • Carrier
  • haemophilia
  • surgery
  • transgender
Acceso abierto

Haemophilic arthropathy: a case of misdiagnosis

Publicado en línea: 21 Dec 2018
Páginas: 156 - 159

Resumen

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.

Palabras clave

  • Arthrofibrosis
  • arthropathy
  • haemarthrosis
  • misdiagnosis
  • physiotherapy
  • Uganda

Clinical Research

Acceso abierto

Evaluation of a shortened course of tranexamic acid for patients with inherited bleeding disorders following dental procedures

Publicado en línea: 28 Jun 2018
Páginas: 42 - 49

Resumen

Abstract

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.

Palabras clave

  • Antifibrinolytics
  • Bleeding disorders
  • Dental procedures
  • Haemophilia
  • Tranexamic acid
  • Service evaluation
Acceso abierto

Pain self-management experiences in haemophilia patients: a qualitative study

Publicado en línea: 18 Oct 2018
Páginas: 76 - 82

Resumen

AbstractBackground

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.

Palabras clave

  • Experience
  • Haemophilia
  • Pain
  • Self-management
Acceso abierto

Effects of top rope climbing therapy on joint health in adult haemophilia patients with severe arthropathies

Publicado en línea: 14 Dec 2018
Páginas: 93 - 102

Resumen

AbstractBackground

Low impact physical activity is important for patients with haemophilic arthropathies, but is often considered boring with suboptimal adherence. There is therefore a need for physical activities that motivate patient engagement.

Aims

To evaluate the benefits of top rope climbing, increasingly used in other musculoskeletal disorders, as an engaging sports discipline in haemophilia.

Materials/Methods

Six adult arthropathic patients with haemophilia (PWH) completed 12 sessions of tailored top rope climbing training. Functional and clinical joint status, climbing skills, quality of life (QoL), annual bleed rate (ABR) and joint findings with musculoskeletal ultrasound/power doppler (MSKUS/PD) were assessed before and after climbing.

Results

Haemophilia joint health scores, dorsiflexion in arthropathic joints and climbing skills all improved. ABRs were comparable before and during climbing, and QoL remained high. MSKUS evaluation demonstrated no detrimental effects on synovial and cartilage health, with a decrease of inflammatory PD signal in some joints.

Conclusion

We conclude that top rope climbing therapy (“Haemophilia Vertical”) can improve joint health in PWH with arthropathies. Haemophilia Vertical therefore emerges as an innovative athletic concept to promote physical activity among PWH. Further study investigating the longer-term impact in a larger cohort is warranted.

Palabras clave

  • Arthropathy
  • Climbing
  • Haemophilia
  • Physical activity
  • Ultrasound

Clinical Practice

Acceso abierto

Hepatitis C and bleeding disorders in Europe

Publicado en línea: 28 Jun 2018
Páginas: 50 - 65

Resumen

Abstract

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.

Palabras clave

  • Bleeding disorders
  • Haemophilia
  • Hepatitis C
  • Monitoring
  • Treatment
Acceso abierto

Guidelines for the management of haemophilia in Egypt

Publicado en línea: 18 Jan 2019
Páginas: 83 - 92

Resumen

Abstract

These guidelines have been developed by an expert panel of haemophilia treaters to support the appropriate management of people with haemophilia in Egypt. Although the guidelines are based primarily on the World Federation of Hemophilia (WFH) Guidelines for the Management of Hemophilia, they aim to address unmet needs and local requirements in the Egyptian setting.

Palabras clave

  • Bleeding disorders
  • Egypt
  • Guidelines
  • Haemophilia
  • Management
  • Treatment
Acceso abierto

Nurse-led telephone review service for mild inherited bleeding disorders improves attendance rates, frees hospital resources and is highly rated by patients

Publicado en línea: 18 Oct 2018
Páginas: 66 - 75

Resumen

AbstractIntroduction 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.

Palabras clave

  • Haemophilia
  • Mild inherited bleeding disorders
  • Nurse-led
  • Review
  • Telephone clinics
Acceso abierto

Transforming Transition: enhancing practice in haemophilia

Publicado en línea: 14 Dec 2018
Páginas: 111 - 121

Resumen

Abstract

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.

Palabras clave

  • Transition
  • leadership
  • young people
Acceso abierto

Young people’s experience of transition from paediatric to adult haemophilia care in the UK

Publicado en línea: 14 Dec 2018
Páginas: 122 - 129

Resumen

Abstract

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.

Palabras clave

  • Transition
  • patient views
  • haemophilia
  • young people
Acceso abierto

You think you know the patient inside-out. But do you know the outside-in?

Publicado en línea: 14 Dec 2018
Páginas: 130 - 137

Resumen

Abstract

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.

Palabras clave

  • haemophilia
  • inside-out
  • outside in
  • patient experience
  • self-management

Community Focus

Acceso abierto

“Ask what can you do instead of thinking about what you can’t” Profile: Clive Smith

Publicado en línea: 21 Dec 2018
Páginas: 152 - 155

Resumen

Abstract

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.

Evidence Review

Acceso abierto

Prevalence, pattern and clinical implications of transfusion transmissible viral infections among paediatric haemophiliacs in northern Nigeria

Publicado en línea: 18 Jan 2019
Páginas: 103 - 110

Resumen

AbstractBackground

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.

Palabras clave

  • Haemophilia
  • Multiple transfusion
  • Nigeria
  • Transfusion transmissible infections
  • Tropical developing countries

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