Risk of deep venous thrombosis after total knee arthroplasty in patients with haemophilia A.

INTRODUCTION: Venous thromboprophylaxis remains controversial in patients with haemophilia undergoing major orthopaedic surgery, due to clotting factor replacement therapy to correct the patient's inherent haemostatic defect during procedure, at least in theory. We have previously reported that deep venous thrombosis (DVT) was not detected on ultrasonography (US) after total knee arthroplasty (TKA) in 36 Japanese patients with haemophilia. AIM: To evaluate the prevalence of DVT after TKA among haemophilic A patients using not only US, but also contrast-enhanced CT prospectively. METHODS: This study enrolled 11 TKAs in 11 Japanese patients with haemophilia A not having any history of inhibitor. A pneumatic compression device was used from the beginning of the operation until the patient could perform standing exercises (day 2). US of the lower extremities was performed before and after surgery (day 2) to detect DVT. Contrast-enhanced CT was performed after surgery (day 7) to detect VTE. D-dimer was measured at pre- and postoperative time. RESULTS: Mean (±standard deviation) age at the time of operation was 50.5 ± 12.3 years. DVT was not found on either pre- or postoperative examinations by US, but contrast-enhanced CT was detected DVT in two cases. No patients showed clinical signs for VTE during hospitalization, and no additional treatment for VTE. No episodes of unexpected bleeding were encountered. CONCLUSION: Contrast-enhanced CT detected DVT in 18% of haemophilia A patients who underwent TKA despite no detection of DVT on US. We must consider how to prevent and treat VTE in patients with haemophilia scheduled to undergo orthopaedic surgery.

The UK haemophilia specialist nurse: Competencies fit for practice in the 21st century.

INTRODUCTION: Nurses play a central co-ordinating role in delivering comprehensive care for people with haemophilia and allied bleeding disorders, for which they need a broad range of competencies. The UK Haemophilia Nurses Association (HNA) published a role description in 1994 which was developed into a competency framework in 2014. This has now been updated to reflect current educational and clinical practice. AIM: To summarize the evidence supporting the nurse's advanced role within haemophilia care and develop new competencies to deliver comprehensive care within a multidisciplinary team. METHODS: Systematic reviews were identified by PubMed literature search. The HNA conducted workshops to consult its membership, and the authors incorporated this input to update its competency framework within the structure outlined by Health Education England in multiprofessional framework for advanced clinical practice in England (2017). RESULTS: The proposed framework includes five domains (Clinical knowledge, Clinical/direct care, Communication and support, Collaborative practice and Research) supported by indicators for four levels of practice (beginner, competent, proficient and expert). The framework is a tool which nurses and their managers can use to assess skills and knowledge, and identify learning needs appropriate to personal development and improve patient care and outcomes. CONCLUSION: The HNA has developed a new competency framework to provide a strong foundation for haemophilia specialist nurses to continue improving services for people living with bleeding disorders and their families, as well as supporting personal development alongside new therapeutic options, models of care and follow-up.

[Training in haemophilia aimed at private practice nurses]. / Une formation en hémophilie pour les infirmières libérales.

At the crossroads between the care delivered in the hospital and community nursing care, private practice nurses occupy a central role in supporting children with haemophilia and their family. Dedicated training helps to maintain the link with hospital structures and enables them to exchange with other professionals regarding practices, treatment difficulties and the contribution of new technologies in the care provision.

A systematic review of the association between coping strategies and quality of life among caregivers of children with chronic illness and/or disability.

BACKGROUND: Parents of children with chronic illness have reported decreased psychological and physical quality of life (QoL) relative to parents of children without such illness, which may be associated with the extent of complexity involved in the caregiving role. Given that coping strategies have been reported to influence QoL, our goal was to synthesize existing research about the association between coping strategies and QoL in caregivers of children with chronic illness. We were particularly interested in whether coping strategies may mediate the association between caregiving complexity and QoL, or may modify the association. METHODS: We developed an electronic search strategy to identify relevant citations in Medline, EMBASE, PsycINFO and CINAHL. Two reviewers independently assessed retrieved citations against pre-specified inclusion criteria in two stages of screening. One reviewer abstracted data on study characteristics, methods to address confounding, measurement tools, risk of bias, and results with respect to associations of interest. A second reviewer validated extracted data. We summarized results narratively. RESULTS: 2602 citations were screened and 185 full-text articles reviewed. The 11 articles that met inclusion criteria addressed 5 diseases and included a total of 2155 caregivers. Ten of the 11 included studies were cross-sectional. We identified some evidence that coping was associated with QoL: in three studies, coping strategies considered to be adaptive were positively associated with psychological QoL while in one study, maladaptive strategies were negatively associated with psychological QoL. Only two studies considered coping as a potential mediating variable in the association between caregiving complexity and parental QoL, with inconsistent findings and challenges in interpreting cross-sectional associations. No studies considered coping as a moderating variable. The variability among instruments used to measure key constructs, particularly coping strategies, made it difficult to synthesize results. CONCLUSIONS: We found that coping strategies may be associated with psychological QoL among parents of children with chronic illness. We also identified important research gaps related to the consistent and clear measurement of coping strategies and their prospective association with QoL. Understanding how coping strategies are associated with QoL is important to inform the development of interventions to support families of children with chronic illness.

The viability and necessity of APRN-led care models in the clinical management of haemophilia and other inherited bleeding and clotting disorders.

A growing and ageing haemophilia treatment centre (HTC) population, as well as a glaring shortage of adult haematologists available for the clinical management of persons with haemophilia (PWH), has resulted in significant care gaps in the United States. In response, various advanced practice registered nurse (APRN)-based models have been adopted across the country to counteract a deficit in haematologist resources for adult PWH. These models in the management of PWH have demonstrated competency and efficiency in the care of patients with haemophilia, offering a viable solution in areas where haematologist resources are limited and assure quality care provision, most notably in rural areas. The available literature indicates equivalence in terms of clinical outcomes, patient satisfaction and elements of healthcare service utilization. With continued support in current clinical guidelines and institutional contributions in the form of residency and fellowship programmes, the robust successes of APRN models for the clinical management of PWH can be maintained and expanded well into the foreseeable future.

A European curriculum for nurses working in haemophilia.

INTRODUCTION: Currently, there is no consensus on education required to develop haemophilia nursing. The aim was to develop a curriculum for haemophilia nurses that could be used as a resource in Europe. This could form a basis for continuous professional development and used in the preparation of specialized educational programmes. METHODS: The EAHAD nurses working group set out to describe the skills and knowledge needed for a nurse to work in this specialty. This was considered at two levels: basic requirements and at a more advanced level. The working group acted as a focus group for this project drawing on existing specialist training, national role definitions, competencies and results of the EAHAD Nurses survey (2012). A template was populated with the knowledge base and the skills required. RESULTS: Themes were analysed and information generated organized into domains: content of curriculum; learning outcomes, defined in terms of knowledge, skills, behaviour and attitudes; and suggestions for teaching methods. For curriculum content the following domains were identified: Applied biological science; treatment and management of haemophilia and associated disorders; genetic practice; care management of affected carriers and women; the impact of living with bleeding disorders; evidence base and applied research in haemophilia practice; and, the specialist role of the haemophilia nurse. Examples are given for teaching and learning process. CONCLUSION: This curriculum is intended for use as a strategic resource to outline education for the haemophilia nurse and contribute to the standardization and benchmarking of haemophilia nursing care and thus to improvement in the quality of patient care.

Care of the chronically ill patient with a bleeding disorder.

PURPOSE: This article provides an overview of hemophilia, a chronic bleeding disorder. DESIGN: The epidemiology, diagnosis, treatment, complications, and management of hemophilia are described. Collaborative care using the Hemophilia Treatment Center (HTC) Comprehensive Care Model is presented in a case study. CONCLUSIONS/CLINICAL RELEVANCE: Rehabilitation nurses may encounter patients across the lifespan with bleeding disorders and with basic hemophilia knowledge; coordination with the HTC can provide high-quality care for positive outcomes.

An overview of current trends and gaps in patient-reported outcome measures used in haemophilia.

AIM: This review summarises the importance, recent progress and issues in measuring patient-reported outcomes (PROs) in haemophilia research. METHODS: A critical review of recent advances and trends in measuring haemophilia-related PROs was conducted, using current regulatory guidelines and methodological recommendations to evaluate these instruments. RESULTS: Although regulators, payers and policymakers increasingly consider the patient's perspective to be important in treatment decision-making, to date, few haemophilia intervention studies have meaningfully applied PRO endpoints. Condition-specific PRO instruments have been developed, but most are not fully validated; sensitivity to subgroup differences and changes over time is unclear. Generic PROs and instruments developed for other conditions have been used to measure health-related quality of life (HRQL) in haemophilia patients, but little evidence of their validity for this purpose exists. Haemophilia presents a number of challenges to developing valid, reliable and responsive PRO instruments, including the rarity of the disorder; necessitating research in multiple counties to attain sufficient sample size; the chronic nature of the condition; acute exacerbations of illness; age and geographical region variations with respect to treatment; differences in treatment regimens, range of disease severity and phenotypes; and changes in patients' perceived health status over time. Given that haemophilia begins at birth, the illness has an impact on the lives of caregivers, although the extent of the impact is largely unknown. CONCLUSIONS: Patient perspectives are crucial to understanding the best and most cost-effective haemophilia treatment approaches. More research is needed on the ability of current disease-specific and generic PRO instruments to capture responsiveness to treatments over time and subgroup differences in outcomes. Inclusion of PROs in clinical trials is necessary to answer these questions.

Intervenciones de enfermería en la atención pediátrica de la hemofilia / Nursing interventions in pediatric hemophilia care

Introducción: La hemofilia es una enfermedad crónica, específicamente un trastorno de la coagulación de la sangre que causa hemartrosis y sangrado prolongado espontáneo (Murillo, 2008). La patología requiere un abordaje integral en donde el papel del profesional de enfermería es satisfacer las necesidades biopsicosociales del paciente pediátrico con este problema de salud. Método: La búsqueda sistemática de información se enfocó a documentos obtenidos acerca de la temática de intervenciones de enfermería y hemofilia en pacientes pediátricos. Se utilizaron 22 documentos para la elaboración de esta guía. Resultados: Las principales intervenciones de enfermería en el paciente pediátrico con hemofilia son: ministración del Factor VIII o IX según prescripción, control de la hemorragia, manejo del dolor, inmovilización, aplicación de frio, identificación de riesgos, monitorización de extremidades inferiores, apoyo emocional y enseñanza de los procedimientos y tratamientos. Conclusiones: La valoración de enfermería en pacientes con hemofilia se centra en las respuestas humanas (Dolor, limitación del movimiento, temor, angustia y ansiedad) y en las respuestas fisiológicas (Edema, hematomas, hemorragias internas y externas)

Background: Hemophilia is a chronic disease, specifically a disorder of blood clotting and prolonged bleeding hemarthrosis cause spontaneous (Murillo, 2008). The disease requires a comprehensive approach where the role of the nurse is to meet the biopsychosocial needs of pediatric patients with this health problem.Method: A systematic search of information obtained documents focused on the theme of nursing interventions and pediatric hemophilia patients. 22 documents for the development of this guide were used.Results: The main nursing interventions in pediatric patients with hemophilia are: ministry of Factor VIII or IX as prescribed, control of bleeding, pain management, immobilization, application of cold, risk identification, monitoring lower extremities, emotional support and teaching procedures and treatments.Conclusions: Nursing assessment in patients with hemophilia focuses on human responses (pain, limitation of movement, fear, anguish and anxiety) and physiological responses (edema, bruising, internal and external bleeding).

Introdução: A hemofilia é uma doença crônica, especificamente um distúrbio da coagulação do sangue, que causa hemartrose e sangramento prolongado espontâneo (Murillo, 2008). A doença requer uma abordagem integral onde o papel do enfermeiro é satisfazer as necessidades biopsicossociais do paciente pediátrico com este problema de saúde.Método: A pesquisa sistemática de informação foi enfocada em documentos obtidos sobre o tema das intervenções de enfermagem e hemofilia em pacientes pediátricos. Foram utilizados 22 documentos para o desenvolvimento deste guia.Resultados: As principais intervenções de enfermagem no paciente pediátrico com hemofilia são: ministração de Fator VIII ou IX, conforme prescrito, controle da hemorragia e dor, imobilização, aplicação de frio, identificação de riscos, monitoramento de extremidades inferiores, apoio emocional e ensino de procedimentos e tratamentos.Conclusões: A avaliação de enfermagem em pacientes com hemofilia concentra-se nas respostas humanas (dor, limitação de movimento, medo, angústia e ansiedade) e nas respostas fisiológicas (edema, hematomas, hemorragias internas e externas).

Multimethodology research with boys with severe haemophilia.

AIM: To describe the use of an innovative, multimethodological approach to exploring the day-to-day experiences of boys across a wide age range to better understand the effects of modern haemophilia treatment on their lives. BACKGROUND: Children and young people with severe haemophilia can now be treated with prophylaxis and potentially have a lifestyle close to that of those without haemophilia. However, boys frequently describe living with haemophilia as burdensome. DATA SOURCES: The study, based on a grounded theory approach, was conducted with boys aged four to 16 years old, using research methods that included photo-elicitation, 'draw and write' techniques, focus groups run by participant co-researchers and individual interviews. REVIEW METHODS: Grounded theory was used to enable rich data capture, through reshaping of research questions as theory developed. DISCUSSION: The effectiveness of the methods used is discussed, along with consideration of the issues raised. CONCLUSION: These methods are effective for use with children. They can result in robust data and are also fun for child participants. IMPLICATIONS FOR RESEARCH/PRACTICE: Understanding life with chronic disease from a child's perspective can improve clinical care through a better understanding of health behaviour and lifestyle implications.

Chronic care infrastructures and the home.

In this article I argue that attention to the spatial and material dimensions of chronic disease management and its place-making effects is necessary if we are to understand the implications of the increased mobilisation--technologically or otherwise--of the home in chronic disease management. Analysing home treatment in asthma and haemophilia care, I argue that in relation to chronic disease management the home is not only always connected to the clinic but moreover, what the home is in part depends on the specificities of these attachments. Drawing primarily on the work of Susan Leigh Star and scholars of human geography I propose the concept of chronic care infrastructures designating the often inconspicuous socio-material elements (such as medication, control visits, phone calls, doses and daily routines), which are embedded in everyday life (of both the clinic and the home) and participate in producing the effect of treatment but also the effect of home. These chronic care infrastructures demand the emplacement of various objects and activities in everyday life and thus relate to negotiations of 'keepings'--hat to keep and care for and where to grant it room vis á vis other 'keepings'.

Evaluation of a nurse-led haemophilia counselling service.

Genetic counselling and testing for females with a family history of haemophilia has long been advocated. However, there is little research in regard to clients' satisfaction with the existing counselling models in haemophilia, and in particular with nurse-led clinics. The purpose of this study was to evaluate whether clients were satisfied with a nurse-led carrier testing clinic and counselling service. A retrospective quantitative study of clients' satisfaction and perceived knowledge was undertaken using an anonymous questionnaire. A sample of 42 women who had attended the clinic in the last 12 months was identified. The response rate for the study was 71% (n = 30).Two thirds of the respondents were 35 years of age or younger, 93% had a family history of haemophilia and 56% were diagnosed as carriers. Perceived understanding and knowledge increased significantly between the first and second appointments (p < 0.001). Overall, the study identified a high level of client satisfaction with the nurse-led carrier testing clinic and counselling service.

Understanding of genetic inheritance among Xhosa-speaking caretakers of children with hemophilia.

Hemophilia A and B are X-linked recessive inherited bleeding disorders that have a profound impact on the family of affected individuals. Education is vital to enable women to appreciate the implications of being a carrier and the implications for a prospective child. Prior research has shown that cultural, socio-economic and linguistic issues in South Africa are major barriers to communication for first-language Xhosa-speakers. This exploratory study aimed to investigate the basic knowledge of genetic inheritance among this cultural group in order to promote culturally-sensitive, effective genetic counseling. Ten in-depth interviews were conducted with Xhosa-speaking mothers or caregivers of boys with hemophilia. Results suggest that the participants had a very limited understanding of the clinical management, genetic consequences and cause of hemophilia. While treatment and care by health care service providers was fully accepted, several participants believed that traditional methods would provide them with more satisfactory explanations. These findings suggest that there is a critical need for socio-culturally tailored, language-specific education for families with hemophilia.

Developing education tailored to clinical roles: genetics education for haemophilia nurses.

BACKGROUND: Genetics is an important component of the clinical work of haemophilia nurses, but little was known about the genetic education needs of haemophilia nurses. AIM: To develop, deliver and evaluate genetic education for haemophilia nurses, based on clinical roles. METHODS: Perceived relevance of genetics to haemophilia nursing practice was explored using electronic voting (response rate 75%, 58/77). A follow-on questionnaire to a volunteer sample of participants explored educational preferences (response rate 41%, 17/41). Results informed development of a two-hour genetics workshop session, evaluated by questionnaire (response rate 67%, 47/70). RESULTS: Genetic competences were considered relevant to the clinical practice of haemophilia nurses, and learning needs were identified. Preference was expressed for education focused on practical skills. During the subsequent workshop, participant confidence ratings significantly increased in the four areas addressed. Planned changes to clinical care and training were reported. CONCLUSION: Within new areas of advanced nursing practice, learning needs can be addressed by: identifying relevant clinical activities and associated learning needs; creating a strategy and resources using preferred forms of delivery; implementing the strategy; and evaluating its effect. This will enable development of education that addresses the real needs of practising nurses, grounded in their daily clinical practice.

Evidence of prophylaxis treatment in adult haemophiliacs. Need of this treatment strategy?

In contrast to children with haemophilia the scientific evidence of prophylaxis treatment in adults is not yet proven. Existing studies are of observational character and mostly retrospectively designed. Therefore, opinion leaders in this field postulate prospectively designed, randomized, controlled and multicentric studies to set up urgently needed guidelines. Evidence according to the Canadian task force ranking is assessed as level III with a recommendation grade C by the authors. The recognition of benefits of health care providers in accordance with the German Federal Joint Committee generally demands a Grade-Ia to Ib evidence. As long as the actual evidence of prophylaxis in adult haemophiliacs does not meet the postulated criteria of the German Federal Joint Committee, prophylactic replacement therapy of the individual case has to be well documented and reasonably explained.