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.
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.
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.
Members of the multi-disciplinary team involved in delivering haemophilia care face a range of significant clinical and service leadership challenges. These include the developing treatment landscape, the drive towards individualised care, an uneven age structure among haemophilia nurses and constrained budgets. Faced with such challenges, the ASPIRE programme has been established to encourage and support a new generation of haemophilia leaders who are committed to improving haemophilia care across the UK, and beyond. The programme is open to healthcare professional from multiple disciplines, and is designed to support the development of a leadership community comprising members of the haemophilia care team in a way that contrasts with hierarchical leadership and management courses more typically found in the NHS.
Haemophilia caregivers face limitations in their life leading to perceived burden. This single-centre study investigates the impact of burden on caregivers’ health-related quality of life (HRQoL). Methods: Questionnaires for caregivers comprised demographic data, HRQoL (EQ-5D, SF-36) and caregiver burden (IOF: Impact on Family Scale). Children were also asked about their HRQoL (EQ-5D, Haemo-QoL Short Form). Results: 20 consecutive parent/child dyads participated. 80% were mothers (mean age of 39.80±6.2 years) with 1-3 haemophilic children aged 8-17 years and 2.5±1.2 children <18 years living in the household. Mothers did most childcare (80%), 50% worked part-time, and 55% reported that haemophilia had an economic impact on their family. 80% of boys had haemophilia A; 60% were severely affected. Most received home treatment (85%) and prophylaxis (80%). Caregivers’ and boys’ HRQoL was similarly good (EQ-5Dparents M=0.90±0.1 vs. EQ-5Dchild M=0.81±0.3); by contrast, boys reported better values in the EQ-VAS (Mchild=90.25±10.0 vs. Mparents=82.16±14.8). Caregivers reported highest impairments in the dimensions ‘vitality’ (M=60.00±20.5) and ‘emotional role’ (M=70.37±42.6) of the SF-36. In the IOF, caregivers reported highest impairments in the dimension ‘negative impact’ (M=60.08±20.7). Caregivers reporting high burden had significantly worse HRQoL in the domains ‘bodily pain’ (p<.028) and ‘social functioning’ (p<0.024) of the SF-36. Caregivers who reported that haemophilia had an economic impact on their family and those with a chronic disease showed significant higher impairments in caregiver burden and their HRQoL. Conclusions: The perceived burden of haemophilia has a direct impact on caregivers’ HRQoL. Further studies with haemophilia-specific instruments are needed to verify these findings.
The benefits of regular activity and exercise are well established, yet a third of the population of the European Union does not achieve the recommended levels of activity. For patients with haemophilia, some activity can cause bleeding into joints, leading to potential joint damage, whereas other activities can protect the joint from bleeds and further destruction. This study investigates the understanding and experience of exercise and activity in people with haemophilia (PWH). We conducted semi-structured interviews with six men with severe haemophilia using a consecutive sampling framework and Interpretative Phenomenological Analysis (IPA). We identified five themes: • “I don’t think about haemophilia, I’ve just got to deal with it” A level of acceptance of awareness of their condition, but they do not want it to rule their lives. • “I don’t let my limitations hold me back” Striving to find activities they can participate in, despite joint impairment. • ”The worst thing anyone can do is stop being active” Belief that activity helps to strengthen joints, gives confidence and improves both body and mind. • ”The best thing they did was to not wrap me up in cotton wool” Knowledge of haemophilia, how to treat and recognise bleeds, and finding activities to suit their bodies. • “Time constraints at home” Common barriers to exercise, as in the general population. Our findings provide clinicians with insight into understanding the barriers to exercise and activity in men with severe haemophilia. This can help clinicians to offer the most appropriate support and allow PWH to find an activity or exercise that suits them. Our findings demonstrate that even those with severe haemophilia wish to remain active and do not want to let their limitations prevent them from exercising. Findings also indicate that being given the education and freedom to make independent decisions about exercise and activities are valued. This suggests that clinicians need to provide a therapeutic environment where their patients can feel safe to make sensible choices about types and level of activity.
Introduction: Von Willebrand disease (VWD) is the most common hereditary coagulation abnormality. Individuals with VWD experience increased mucosal bleeding as well as gingival bleeding. Some evidence suggests that gingival bleeding is related to poor oral hygiene for fear of plaque removal methods that may cause bleeding to the gingival tissue. No studies have shown a correlation between VWD and gingival bleeding; however, these studies have not adjusted for possible confounding factors, such as presence of dental plaque, dental care utilisation, and oral hygiene habits. Aims: To determine the relationship between von Willebrand factor (VWF) levels and the amount of gingival bleeding, when controlling for possible confounding variables. Methods: This multi-site study included 44 women with VWD who completed a questionnaire to evaluate demographics, oral hygiene habits, perception of own oral health, oral health quality of life, and dental care utilisation. Clinical dental examinations were conducted to determine the presence of plaque and gingival bleeding on six teeth in each individual. Von Willebrand disease type and severity were determined through a medical chart review. Institutional review board (IRB) approval was obtained prior to data collection. Results: Paired sample t-tests revealed that VWF levels did not significantly affect the amount of gingival bleeding noted (p>0.05) when controlling for presence of plaque. Multiple linear regression models revealed that other factors, such as age and last dental visit (p=0.044), had a larger effect on the amount of gingival bleeding. Conclusion: In women with VWD, the presence of plaque and dental care utilisation determines gingival bleeding more than the severity of VWD.
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.
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.