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Neonatal circumcision in patients with haemophilia is safe – a single centre experience

Abstract

Consensus evidence-based guidelines regarding prophylactic treatment for circumcision in patients with haemophilia are lacking. In this study, we retrospectively examined the results of circumcision of 40 consecutive neonates with haemophilia, diagnosed after delivery. Our protocol included application of a single dose of factor replacement prior to the procedure and 3 days’ oral treatment with tranexamic acid. Only 3/40 neonates with haemophilia experienced mild postcircumcision bleeding. No major or any life-threatening bleeds were encountered. As the therapy administered was minimal and the procedure simple and feasible, we concluded that circumcision in neonates with haemophilia should be performed according to family’s and physician’s discretion. Avoidance of circumcising male babies with haemophilia due to fear of bleeding complications is not supported by the results shown within our patient cohort.

Open access
Perceived confidence for injury self‑management increases for young men with mild haemophilia with the use of the mobile app HIRT?

Abstract

Background: Young men with mild haemophilia have unique challenges pertaining to bleed management. They may not always identify musculoskeletal injury requiring medical attention as they do not bleed frequently, potentially resulting in significant health consequences. In response to these challenges, a team of clinicians, researchers and young men with mild haemophilia developed a self-assessment pathway which was converted into a mobile app. Aim: This study examined the influence of the mobile app, HIRT? (Hemophilia Injury Recognition Tool) on perceived injury self-management in young men with mild haemophilia in Canada. Methods: We used a mixed methods design. The quantitative data, through a self-report questionnaire, evaluated perceived injury self-management strategies and participant confidence levels. Non-parametric Wilcoxon signed-rank test and McNemar chi-square test were used to determine association between perceived self-management strategies when using and not using the app, with significant levels set at p<0.05. Qualitative data was created using interpretive description and inductive content analysis of recorded and transcribed interviews. Results: 12 young men, aged 18-35 years, participated. Perceived confidence levels significantly increased (p=0.004) with the use of the app. Five qualitative themes were identified: accessibility, credibility, the benefit of alarms, confidence and usefulness. Conclusion: This study provides promising evidence to support the feasibility and use of HIRT? as an injury self-management tool for young men with mild haemophilia. Future research should prospectively investigate the effect of the app on injury selfmanagement confidence.

Open access
School-based programs for promoting physical activity and fitness in children and adolescents

Abstract

Rates of obesity are rising in the general population. People with haemophilia are at high risk for being overweight or obese, and may benefit from physical activity-based interventions. The school setting is an ideal environment to implement physical activity-based interventions as it greatly influences the first two decades of life. However, there is a lack of knowledge about the benefits of exercise for managing haemophilia, as well as possible restriction of physical activity by parents or carers due to a fear of increasing the number of bleeding episodes. Furthermore, schools and teachers may be uncertain of how to integrate physical activity for children with bleeding disorders. This article summarises the Cochrane Metabolic and Endocrine Disorders Group systematic review on ‘school-based physical activity programs for promoting physical activity and fitness in children and adolescents aged 6 to 18 and considers published literature about the role of physical activity within haemophilia.’

Open access
Tightrope walking with Glanzmann’s disease

Abstract

Glanzmann’s disease or thrombasthenia is a very rare genetic platelet function disorder, caused by a quantitative or qualitative defect or absence of platelet membrane glycoprotein IIb/IIIa. Gastrointestinal bleeds are variable and may be severe, unpredictable and sometimes even untreatable. We present an 86-yearold woman with Glanzmann’s disease, who went to her local hospital with complaints of chest pain, tiredness and shortness of breath. After examination and laboratory research, she was diagnosed with severe anaemia caused by angiodysplasia in the gastrointestinal tract. The patient was followed weekly at our outpatient clinic for several months. Multiple treatments were given to correct her anaemia and its underlying cause. Unfortunately, the patient remained transfusiondependent throughout. She was treated with many blood transfusions, recombinant FVIIa infusions and tranexamic acid. When the burden of the disease and side-effects of the treatment became unbearable, the joint decision was made to stop treatment. The patient then unexpectedly recovered from her gastrointestinal bleeding. This case study highlights the dilemmas clinicians often face when dealing with fragile elderly people who suffer from various disorders.

Open access
US haemophilia centre nurses and advanced practice providers: Demographics, roles/responsibilities, training, educational barriers and employment benefits

Abstract

Introduction: As the focus on personalised treatment is refined, more products are brought to market and the life expectancy of persons with haemophilia increases, there will be an expanded need of experienced and expert healthcare providers to ensure optimal patient outcomes. Aim: This survey describes the demographics, roles/ responsibilities, practice patterns, educational opportunities/barriers and employment benefits of nurses and advanced practices providers (APPs), including advanced practice registered nurses (APRNs) and physician assistants (PAs) employed by haemophilia treatment centres (HTCs) across the United States. Methods: This one-time convenience online survey was approved by the Munson Medical Center Institutional Review Board. A survey of this type had never been attempted in the HTC nursing community; therefore. there was no opportunity to utilise a previous tool. Data was analysed using statistical tools through SurveyMonkey. Results: Gaps were identified in provider age distribution, research opportunities, and standardised educational opportunities for APPs. An aging but highly educated HTC nursing population is revealed: over 50% of respondents were over the age of 50; the majority held at least a baccalaureate degree and almost half had national board certification; most were experienced in healthcare but newer to the field of bleeding disorders. Conclusion: Development of an APP fellowship program would standardise the care and treatment of those with bleeding and clotting disorders across the United States. This fellowship should include a didactic portion, advocacy within the community, mentorship with experienced APPs and regular webinar-based case studies to review current trends in care. This survey is a call to action to begin standardised education programs for the advanced practice role.

Open access
Use of an innovative syringe pump to deliver bolus rFVIIa for patients with haemophilia and inhibitors undergoing surgery

Abstract

Aim: To evaluate the efficacy and safety of using a syringe driver perfusor pump to deliver frequent regular bolus doses of rFVIIa peri- and postoperatively to patients with severe haemophilia and inhibitors. Background: The provision of surgical haemostatic coverage with rFVIIa requires regular bolus doses, initially every two hours and subsequently three-hourly, moving to four- to six-hourly post-operatively. This has implications for safety and nursing time, and can influence clinical decision-making. We investigated the use of an infusion pump to deliver timed and accurate rFVIIa bolus doses during the postoperative period. Methods: Two patients with severe haemophilia A and inhibitors undergoing three procedures were managed with a pre-surgical bolus infusion of 120mcg/kg of rFVIIa, followed by postoperative doses of 90mcg/kg every two hours for the first 72 hours, and every three hours subsequently, with increasing dose intervals thereafter. All postoperative bolus doses were delivered via a pre-programmed infusion pump. The pump also required a constant background infusion set at 0.1ml/ hour for the first patient, and reduced to 0.01ml/hour for the second patient. Results: The pump was simple to use and readily accepted by patients and nursing staff. There were no delayed or missed doses, and good haemostasis was maintained. Overall nursing time involved in reconstituting and administering rFVIIa was reduced from three to six hours per treatment day (with conventional bolus dosing) to just one to two hours per day with the pump. Conclusions: The syringe pump successfully delivered the correct doses at the specified time intervals for all three procedures. The technique met the centre’s safety and efficacy criteria and resulted in a significant reduction in the amount of nursing time needed over a 24-hour period. This novel method of rFVIIa administration appears to be safe and effective in the elective surgery setting, and should be further investigated.

Open access
What is a nurse-led service? A discussion paper

Abstract

The terms ‘nurse-led service’ and ‘nurse-led care’ are often used, but are frequently not well defined. As health care delivery evolves across the world and struggles to cope with changes in medical care and with the rising number of people living longer with long term conditions, it is frequently suggested that more care should be delivered and coordinated by nurses who have the most day-to-day contact with affected individuals and their families. This paper addresses the notion of modern ‘nurse-led’ care in the 21st century, with a focus on haemophilia. Haemophilia is one specialty in which totally nurse-led and delivered care could become a reality, there is already evidence from both developing and developed countries of nurses providing excellent and innovative haemophilia care.

Open access
Who should care for people with bleeding disorders?

Abstract

An integrated model of specialised-delivered care is widely accepted as the standard of care for people with haemophilia in the UK. Assessment of available evidence on patient outcomes confirms this approach. But leading the specialist care for this group of patients does not require a medical qualification. Specialist nursing is well established within the haemophilia service and offers perhaps the greatest resource as health services cope with cost constraints on the specialist provision of services.

Open access
Anti-fibrinolytic agents in bleeding disorders – A clinical perspective

Abstract

Tranexamic acid (TXA) is a synthetic antifibrinolytic drug used widely used to control bleeding complications in a wide variety of clinical situations. Soon after its development in the 1960s it found use in treatment of women with menorrhagia, and in inherited bleeding disorders. Subsequently it was used in surgery and with proven efficacy to reduce transfusion requirements and bleeding complications. Recent meta-analysis have provided further evidence of efficacy and safety. Tranexamic acid is now on the World Health Organization’s (WHO) list of essential drugs, and is the focus of ongoing worldwide trials. Similarly, there is increasing evidence base in both congenital and acquired bleeding disorders. We present a clinical narrative of the antifibrinolytic system and associated drugs to accompany the pharmacy review by Chaplin et al, with the aim of highlighting the evolution of TXA use in bleeding disorders over recent decades.

Open access
Diagnosis and education for a better future in Uganda

Abstract

Since 2005, the Novo Nordisk Haemophilia Foundation (NNHF; www.nnhf.org) has worked in collaboration with local partners and internationally renowned experts to improve access to haemophilia care. The NNHF has identified three areas on which to focus its activities in order to create impact where it is most needed: capacity building, diagnosis and registry, and education and empowerment. Underpinning these focus areas are targeted awareness creation activities and the development of strategic partnerships, which enable and facilitate local impact creation through empowered community advocates and authority engagement. NNHF supports fellowships, local development projects, and recognises extraordinary achievements via the annual ‘NNHF Project of the Year Award’ and the ‘NNHF Community Award’. Raising awareness of haemophilia in Uganda was the NNHF global fundraiser in 2015. This article summarises how the donations raised were used to establish diagnosis and education for a better future for Uganda’s haemophilia patients.

Open access