<abstract xmlns="http://www.w3.org/1999/xhtml"><p>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.</p></abstract>

Management of severe haemophilia A during surgery: case report

<abstract xmlns="http://www.w3.org/1999/xhtml"><p>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.</p></abstract>

Parathyroidectomy in a patient treated with emicizumab

<abstract xmlns="http://www.w3.org/1999/xhtml"><p>Pain continues to be a significant issue for people with haemophilia (PWH), whether it is acute pain from a joint bleed, a needle stick when receiving clotting factor treatment, or chronic pain from chronic synovitis and arthritic changes. In the US, there has been controversy and uncertainty regarding the use of oral medications and opioids for the management of chronic pain, including haemophilia pain. Both PWH and their clinicians have started to explore nonpharmacologic options for managing pain, with many PWH trying to limit their use of oral pain medication and using alternative methods. There is little research around the use of complementary and alternative medicine (CAM) for pain management in the haemophilia community. This paper aims to provide a comprehensive literature review of current English language research on the use of therapies that fall into four distinctive domains of CAM in PWH. English language articles were obtained via searches in Medline, Embase and CINAHL using the target search words “haemophilia” or “bleeding disorders” in combination with other search terms relating to CAM. All references were considered regardless of year. Twenty-three CAM articles that included PWH or other bleeding disorders were identified; ten were evidence based while the remainder provided case reports and descriptions of CAM use in PWH. This indicates that there is limited current research in the area of CAM use for pain management in PWH. Evidence of the benefit of CAM is seen in studies of its use to treat pain in other conditions and health-related circumstances. Additional evidenced-based CAM research is needed so that alternative pain management therapies can be utilised in a safe and beneficial manner in PWH.</p></abstract>

Complementary and alternative therapy (CAM) in haemophilia pain management: a review of published literature

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_jhp00132_s_005_w2aab3b7c29b1b6b1aab1c14b1Aa"><h3>Background</h3><p>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.</p></sec><sec id="j_jhp00132_s_006_w2aab3b7c29b1b6b1aab1c14b2Aa"><h3>Aims</h3><p>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.</p></sec><sec id="j_jhp00132_s_007_w2aab3b7c29b1b6b1aab1c14b3Aa"><h3>Methodology</h3><p>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&lt;0.05 was taken as significant.</p></sec><sec id="j_jhp00132_s_008_w2aab3b7c29b1b6b1aab1c14b4Aa"><h3>Results</h3><p>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).</p></sec><sec id="j_jhp00132_s_009_w2aab3b7c29b1b6b1aab1c14b5Aa"><h3>Conclusion</h3><p>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.</p></sec></abstract>

University of Maiduguri Teaching Hospital

Opioid dependence among people with haemophilia in a low-resource tropical setting: prevalence and risk factors in northern Nigeria

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_jhp00146_s_006_w2aab3b7c52b1b6b1aab1c14b1Aa"><h3>Background</h3><p>In patients with haemophilia, evidence suggests that the physical examination alone is not sensitive enough to detect small amounts of blood within a joint. Attention has shifted to methods of improving the sensitivity of the physical examination through adding diagnostic modalities such as point-of-care ultrasonography (POC-US). Proficiency with the physical examination and understanding of the role of POC-US are important competencies for physiotherapists. Despite training, implementation of POC-US by physiotherapists in haemophilia treatment centres in Canada has been mixed.</p></sec><sec id="j_jhp00146_s_007_w2aab3b7c52b1b6b1aab1c14b2Aa"><h3>Aim</h3><p>Using a theory-based approach, the aim of the current study is to achieve expert consensus regarding the barriers to physiotherapy performed POC-US in haemophilia treatment centres in Canada using a modified Delphi approach.</p></sec><sec id="j_jhp00146_s_008_w2aab3b7c52b1b6b1aab1c14b3Aa"><h3>Materials and Methods</h3><p>Using the Knowledge-to-Action Framework and the Consolidated Framework for Implementation Research (CFIR), a modified Delphi approach was completed using the Modified BARRIERS Scale (MBS). Participants were blinded and consensus was reached over three rounds at the Canadian Hemophilia Society’s annual three-day conference.</p></sec><sec id="j_jhp00146_s_009_w2aab3b7c52b1b6b1aab1c14b4Aa"><h3>Results</h3><p>Twenty-two physiotherapists participated; 20 participants completed Round 1, and 21 completed Rounds 2 and 3. Four items of the MBS reached consensus: 1) The physiotherapist does not have time to read research related to POC-US; 2) The physiotherapist is isolated from knowledgeable colleagues with whom to discuss POC-US; 3) Administration will not allow POC-US implementation; 4) There is insufficient time on the job to implement new ideas. All four consensus items can be mapped to one domain of the CFIR: the inner setting.</p></sec><sec id="j_jhp00146_s_010_w2aab3b7c52b1b6b1aab1c14b5Aa"><h3>Conclusion</h3><p>The haemophilia treatment centre within a healthcare organisation appears to be an important target for addressing barriers to the implementation of physiotherapy performed POC-US.</p></sec></abstract>

School of Rehabilitation Science, Faculty of Health Science

Department of Kinesiology, Faculty of Applied Health Sciences

Barriers to the implementation of point-of-care ultrasonography by physiotherapists in haemophilia treatment centres in Canada: a modified Delphi approach

<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_jhp00147_s_006_w2aab3b7c61b1b6b1aab1c14b1Aa"><h3>Background</h3><p>Patients with haemophilia have a higher prevalence of hypertension than the general population that cannot be explained by traditional cardiovascular risk factors such as age, race, diabetes or obesity. Patients with severe haemophilia, who are on clotting factor prophylaxis, have a higher prevalence of hypertension compared to patients with milder forms of haemophilia, who infuse clotting factor less frequently. This raises the question of whether there is a link between clotting factor usage and blood pressure in haemophilia patients.</p></sec><sec id="j_jhp00147_s_007_w2aab3b7c61b1b6b1aab1c14b2Aa"><h3>Methods</h3><p>Data was collected from 193 patients with severe haemophilia presenting to three haemophilia treatment centres in the United States and Canada, including age, body mass index (BMI), blood pressure (BP), Hepatitis C (HCV) and Human Immunodeficiency Virus (HIV) infection status, and clotting factor usage from pharmacy prescriptions (units/kg/year). The correlation between BP and factor usage was examined using quantile regression models.</p></sec><sec id="j_jhp00147_s_008_w2aab3b7c61b1b6b1aab1c14b3Aa"><h3>Results</h3><p>Systolic and diastolic BP plotted against factor use showed a cone-shaped scatter of points. There was no association between clotting factor usage and higher systolic or diastolic BP.</p></sec><sec id="j_jhp00147_s_009_w2aab3b7c61b1b6b1aab1c14b4Aa"><h3>Conclusion</h3><p>Our observations provide no evidence for an association between increased clotting factor usage and high BP.</p></sec></abstract>

Hemostasis and Thrombosis, Department of Medicine

Hemophilia and Thrombosis Treatment Center, Department of Medicine

Orthopaedic Hemophilia Treatment Center, Orthopaedic Institute for Children

Division of Hematology, Department of Medicine

St. Paul’s Hospital and University of British Columbia

Department of Molecular Medicine, The Scripps Research Institute, Department of Molecular Medicine

Blood pressure in haemophilia and its relation to clotting factor usage

”Take your shots and take what comes”: Rex Jepson

Redressing the balance: Bruce Norval

Doubly rare: Evelyn Grimberg and Minette van der Ven

Kin Kam: resilience and enlightenment

Patrick de Smet: Why change a winning team?

Keeping a positive outlook: Margaret Monk

The Ugandan Treatment Gap – Peter Nangalu

Now and then, and half a world away

AHEAD OF PRINT

Volume 11 (2024): Issue 1 (January 2024)

Volume 10 (2023): Issue 1 (January 2023)

Volume 9 (2022): Issue 1 (January 2022)

Volume 8 (2021): Issue 1 (January 2021)

Volume 7 (2020): Issue 1 (January 2020)

Volume 6 (2019): Issue 2 (January 2019)

Volume 6 (2019): Issue 1 (January 2019)

Volume 5 (2018): Issue 1 (December 2018)

Volume 4 (2017): Issue 1 (January 2017)

Volume 3 (2016): Issue 2 (July 2016)

Volume 3 (2016): Issue 1 (January 2016)

Volume 2 (2015): Issue 2 (July 2015)

Volume 2 (2015): Issue 1 (March 2015)

Volume 1 (2014): Issue 3 (October 2014)

Volume 1 (2013): Issue 2 (May 2013)

Volume 1 (2013): Issue 1 (January 2013)

The Journal of Haemophilia Practice

 The Journal of Haemophilia Practice is an international, open-access, peer-reviewed journal that aims to provide a forum for all members of the multidisciplinary bleeding disorders care team.   It is now widely recognised that the care and management of people with haemophilia and other rare bleeding disorders should always be based on a multidisciplinary approach, with value placed on the specific expertise brought by allied health care professionals, including nurses, physiotherapists, data managers, social workers, and psychologists.   Any manuscript that is relevant to current bleeding disorders care and practice will be considered for review and publication. To this end, we positively welcome:        Original research    Case reports    Case series    Methodology papers   Reviews in any field of bleeding disorders of relevance to current clinical practice   Clinical Updates that describe current advances in any clinical field related to bleeding disorders   Editorials that address a particular topic of current interest.   Outcomes research written by and/or with individuals affected by bleeding disorders    Please note that case reports should meet the standards outlined in the CARE Case Report Guidelines. Authors submitting case reports are advised to consult the CARE Checklist.   Manuscripts should be submitted to The Journal of Haemophilia Practice via ScholarOne (see Submit). All manuscripts will first be subject to a technical review, including a quality check of all files submitted and including a plagiarism check. Manuscripts will then be reviewed by the Editor-in-Chief, who will decide whether or not to proceed to peer review. The journal operates a double-blind peer review process. The review procedure will result in one of four decisions:        Immediate accept   Minor revision   Major revision   Reject        The editorial decision will be communicated to the authors as soon as the review process has been finalised. In case of revisions, the revised article may be sent back to review before a decision is made on acceptance.   The estimated time from manuscript submission to first decision is 4 to 6 weeks and from the final revision to acceptance is approximately 2 weeks. The authors will receive a proof of their article in order to confirm the accuracy of the text prior to publication.   The journal has no article submission charges or article processing charges (APCs).   All articles published in The Journal of Haemophilia Practice are distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs (CC BY-NC-ND 3.0) license which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial, and no modifications or adaptations are made.       Archiving   Sciendo archives the contents of this journal in Portico - digital long-term preservation service of scholarly books, journals and collections.   Plagiarism Policy   The Journal of Haemophilia Practice is participating in a growing community of Similarity Check System users to ensure that the content published is original and trustworthy. Similarity Check is a medium that allows for comprehensive manuscripts screening, aimed to eliminate plagiarism and provide a high standard and quality peer-review process.   For further information on the journal’s editorial policies and publication ethics, please see documentation listed under the ‘For Authors’ tab. 