Umma A. Ibrahim, Sagir G. Ahmed, Modu B. Kagu and Usman A. Abjah
We predicted that haemophilia would create a prohaemorrhagic host-parasite relationship, which would make haemophiliacs very vulnerable to haemorrhagic effects of intestinal helminths in tropical countries like Nigeria. If our prediction is correct, the frequency and risks of gastrointestinal haemorrhage and iron deficiency will be higher among haemophiliacs infected by helminths in comparison with uninfected haemophiliacs. Frequency of gastrointestinal haemorrhages and iron deficiency among haemophiliacs with and without intestinal helminth infections were retrospectively obtained and analysed, and their relative risk determined by regression analysis. Haemophiliacs with intestinal helminths had significantly higher frequencies of gastrointestinal haemorrhage (73.3% vs. 18.5%, p<0.05) and iron deficiency (60% vs. 22.2%, p<0.05) in comparison with haemophiliacs without intestinal helminths. Haemophiliacs with intestinal helminths had significantly elevated relative risks (RR) of gastrointestinal haemorrhage (RR=3.4, CI95%: 2.4- 4.3, p=0.007) and iron deficiency (RR=2.5, CI95%: 1.7-3.3, p=0.009). These results showed that helminth infections were associated with increased risks of gastrointestinal haemorrhage and iron deficiency in haemophiliacs. This is thought to be due to a pro-haemorrhagic host-parasite relationship resulting from host haemostatic abnormality, coupled with the concurrent manipulation of the host haemostatic system by anticoagulants produced by some of the parasites. Haemophiliacs in tropical countries should be regularly screened and treated for intestinal helminths.
Assaf Arie Barg, Sarina Levy Mendelovich, Shadan Lalezari, Shirley Azar Aviv, Dalia Bashari and Gili Kenet
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.
Joann Nilson, Kathy Mulder, Candice L. Schachter, Kristy Wittmeier, Richard Lomotey, Catherine M. Arnold and Sarah Oosman
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.
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.’
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.
Michelle L. Witkop, Christine Guelcher, Margaret Hall and Jennifer Maahs
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.
Debra Pollard, Barbara Subel, Pratima Chowdary and Keith Gomez
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.
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.
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.
Rafał Dubiel, Adam Perzyński, Ida Kłębukowska, Aneta Perzyńska-Starkiewicz, Iwona Chmiel-Perzyńska and Marcin Olajossy
The paper describes a patient, who has been consulted and hospitalized multiple times, due to dermatological lesions on his face. We will present his medical history and describe circumstances leading to the final diagnosis of factitious disorder. In the theoretical part we will try to answer the question, whether the extreme severity of the factitious disorders can, at some point, reach the level of delusions.