We describe the case of a patient with severe haemophilia A and significant comorbidities who underwent surgery to remove a large intra-abdominal haematoma first diagnosed 12 years previously. The haemophilia team was instrumental in coordinating care, building a strong rapport with surgical and other medical teams to manage bleeding risk with continuous infusion of factor VIII (FVIII). Medical teams adjusted their working hours according to clinical need. Haemophilia nurses were available to offer support at all times, and developed management procedures and educated staff on haemophilia and its treatment. Perioperative complications included a thrombus occluding the right internal jugular vein, infection and paraesthesia. However, surgery was effective and the patient was pleased with the overall outcome. Haemophilia nurses established strong and rewarding relationships with other teams that will enhance the delivery of care in the future.
Eugenia Biguzzi, Alison Dougall and Olivia Romero-Lux
Iron deficiency/anaemia and periodontal disease are among the non-gynaecological issues that may present a challenge in women with bleeding disorders. Anaemia is a global health problem, affecting around 32.5% of non-pregnant women aged under 50 and over 40% of pregnant women. It causes fatigue, shortness of breath and dizziness. Anaemia is usually diagnosed by a low serum level of ferritin. Ferritin may be normal in a person who is taking an iron supplement or in the presence of inflammation, in which case the diagnosis can be confirmed by a low transferrin saturation level. A low level of iron should be corrected in a woman with a bleeding disorder, and women must recognise the importance of doing so. If a healthy diet alone does not avoid iron deficiency, oral supplementation is indicated on a low dose regimen to reduce adverse effects; intravenous administration should be used when rapid restoration of iron is indicated. Failure to respond to iron supplementation is an indication for further investigation. Periodontal disease has only recently been recognised as a modern-day epidemic and can have a major impact on quality of life. Oral health has long been ignored in people with a bleeding disorder as bleeding gums secondary to periodontitis are often attributed to the underlying condition. People with a bleeding disorder may therefore feel they can do nothing to improve their oral health. However, healthy gums do not bleed, even in people with a bleeding disorder. While bleeding gums are often accepted as a consequence of having a bleeding disorder, effective cleaning has been shown to reduce gingivitis and bleeding. Regular contact with a dentist should start at a young age and continue throughout life.
Background: In tropical countries such as Nigeria, where factor VIII (FVIII) is scarce, severe pain due to musculoskeletal bleeding complications, leading to frequent opioid prescription, is not uncommon in poorly managed people with haemophilia (PWH). The relationship between opioid use and dependence is intensively studied in other painful diseases, such as cancer and rheumatoid arthritis, but surprisingly little is known about opioid dependence in haemophilia. We hypothesise that the risk of opioid dependence among PWH in tropical countries like Nigeria is multi-factorial, encompassing demographic (age), clinical (haemophilia severity and chronic arthropathy) and biological (ABO blood groups and haemoglobin (Hb) phenotypes) factors that may directly or indirectly increase incidence of bleeding and/or pain.
Aims: To determine the prevalence of opioid dependence and relative risks (RR) associated with age, haemophilia severity, chronic arthropathy, ABO blood groups and Hb phenotypes, and to elucidate the pathophysiological roles of each risk factor in the development of opioid dependence among haemophilia-A patients in five hospitals in northern Nigeria.
Methodology: A retrospective review of the medical records of 88 PWH seen between 1996 and 2012 was used to collate data on age, sex, haemophilia severity, painful chronic haemophilic arthropathy, ABO blood group, haemoglobin phenotypes, presence or absence of opioid dependence, and the types of opioids on which the patients were dependent. The prevalence of opioid dependence among the cohort was expressed as a percentage. The frequency of each putative risk factor for opioid dependence in patients with and without opioid dependence were compared using Fisher’s exact test; RR associated with each risk factor was determined by regression analysis. P<0.05 was taken as significant.
Results: Of the 88 PWH studied,15 (17%) were shown to be opioid-dependent. Compared with PWH who were not opioid-dependent, this group had higher frequencies of severe haemophilia (86.7% vs. 49.3%: RR= 5.2, p=0.02), survival to adulthood (73.3% vs. 12.3%: RR= 9.5, p=0.0001), chronic arthropathy in one or more joints (86.7% vs. 21.9%: RR= 13.2, p=0.0004), blood group-O (80% vs. 49.3%: RR= 3.3, p=0.04), and HbAA phenotype (86.7% vs. 54.8%: RR= 4.3, p=0.04).
Conclusion: Prevalence of opioid dependence among PWH treated at five hospitals in northern Nigeria was 17% during the study period. Significant risk factors were directly or indirectly associated with increased rates of bleeding and/or pain, which can only be prevented or treated through optimal application of FVIII. There is a need for the Nigerian government to establish standard haemophilia care centres with adequate FVIII for optimal prophylaxis and treatment in order to minimise painful complications, thereby helping to prevent undue opioid use and dependence.
Experience of surgery during prophylaxis with emicizumab is currently limited, but the information available suggests that it is associated with a low risk of complications. This case study describes the surgical management of a patient with haemophilia A and inhibitors, managed with emicizumab prophylaxis, who underwent parathyroidectomy. The plan to manage bleeding risk during surgery involved prophylaxis with oral tranexamic acid 1g six-hourly and recombinant Factor VIIa (rFVIIa), prescribed at the discretion of the consultant haematologist. Preoperatively, rFVIIa 45 mcg/kg (3 mg) was administered immediately, and repeated every three to four hours after surgery depending on clinical presentation. There was no unexpected or excessive bleeding during surgery and no clinical need for additional haemostatic medication. Postoperatively, rFVIIa 3 mg was administered at three and ten hours after the first dose. Two further doses were administered on the morning and evening of the first postoperative day. There was no unexpected or excessive bleeding requiring additional treatment, and satisfactory haemostasis resulted in optimal wound healing. The patient reported no bleeding episodes and also an improved quality of life. This case study demonstrates the successful use of emicizumab in conjunction with rFVIIa.
Michelle Lavin, Rezan A Kadir, Sylvia von Mackensen, Debra Pollard and Anna Tollwé
Prolonged menstrual bleeding interferes with daily life and causes marked blood loss, resulting in anaemia and fatigue. Treatment centres should address the issue of heavy menstrual bleeding (HMB) with pre-pubertal girls in advance of their first period, in order to best prepare them. It is common for a bleeding disorder to be overlooked in primary care and in gynaecology clinics, and women sometimes struggle to get a correct diagnosis. There are cultural taboos that inhibit open discussion of menstruation, and women tend to minimise the severity of their symptoms. Health professionals should work to destigmatise the issue and seek an accurate account of bleeding severity, with diagnosis and treatment provided in a joint clinic combining gynaecology and haematology expertise. Treatment should be individualised, taking into account personal, social and medical factors, with the aim of improving quality of life. Great care is needed with regard to choice of language when talking about treatment, and treatment centres should consider offering open access to women who need support in dealing with adverse effects. National member organisations have an important role to play in educating people with bleeding disorders, health professionals and the wider public about the burden of HMB associated with bleeding disorders.
Luisa Durante, Martin Sedmina, Alexandra Vaskova, Baiba Ziemele and Anna Tollwé
Figures in the World Federation of Hemophilia (WFH) global annual survey indicate, by their absence, that there is under-recognition of bleeding disorders in women. The WFH and its national member organisations (NMOs) are working to raise awareness and improve the diagnosis of care of women with bleeding disorders globally, regionally and locally. WFH initiatives include a global programme focused on improving the diagnosis, care and treatment of women with von Willebrand disease (VWD), and programmes involving education and training in conjunction with NMOs in countries including Honduras and Malaysia. NMOs in Slovakia, Latvia and Sweden describe their local activities. The Slovak Hemophilia Society is in the process of establishing a Women’s Committee and considers peer support and network building as essential tools in addressing the issues faced by women with bleeding disorders. In Latvia, access to resources is difficult and von Willebrand factor is not available. There is concern in the Latvia Hemophilia Society that the fundamental human right of access to healthcare is not being met. It supports WFH initiatives through education and advocacy, and believes that the voices of women with bleeding disorders will be better heard through working together. The Swedish Hemophilia Society’s Women’s Project has worked since 2006 to promote better care for women with bleeding disorders and to raise public awareness. Despite resistance, their campaign to increase the identification of girls and women with VWD, improve diagnosis and care, and raise awareness has been well received by healthcare professionals and has had extensive media coverage.