Riding the wave of change: Providing solid ground to support nursing with patient transitions to novel haemophilia therapies.

INTRODUCTION: Haemophilia nursing practice has experienced a shift in the past decade, as the historic chief focus on factor infusions shifted to extended half-life products, bispecific antibody therapies and other non-replacement therapies. This evolution has driven a need for changes in nursing practice in many haemophilia treatment centres. AIM: This article intends to provide insights to the haemophilia nurse to champion practice changes at their haemophilia treatment centres. METHODS: Two popular change theories, Lewin's three-step change model and Kotter's eight-step change model are discussed as a framework for haemophilia nurses to think, structure and be leaders in change. CONCLUSION: Examples of these models in practice could give guidance and examples to reflect on for haemophilia nurses needing to make changes in their practice settings. These models of change, alongside existing haemophilia nurse competencies and tools such as the shared decision-making tool from the World Federation of Hemophilia, can assist the nurse to be a capable change agent to usher in these new innovations.

Utilization of a surgical database to provide care and assess perioperative treatment and outcomes in patients with bleeding disorders.

OBJECTIVES: To describe the Indiana Hemophilia and Thrombosis Center (IHTC) surgical database, its key components, and exploratory analyses of surgeries conducted between 1998 and 2019. METHODS: Surgical data across bleeding disorders collected retrospectively (1998-2006) and prospectively (2006-2019) were analyzed. Perioperative hemostasis, complications, and surgical plan deviations were compared by bleeding disorder diagnosis and data collection period. RESULTS: Within the 21-year period, 3246 procedures were conducted in 1413 patients with a diagnosis of von Willebrand disease (vWD), hemophilia A (HA), hemophilia B (HB), and other bleeding disorders. Majority of the procedures were minor (63.3%), and median number of surgeries per patient was 1 (range: 1-22). Adequate perioperative hemostasis was achieved in 90.9%, complications occurred in 13.6%, and surgical plan deviations occurred in 31.3% of procedures. Inadequate perioperative hemostasis and surgical plan deviations occurred more frequently in procedures involving HB compared with other bleeding disorders. Complications were not significantly different across bleeding disorders (p = .164). The prospective data collection period was associated with higher rates of hemostatic efficacy (92.4% vs. 88.3%; p < .001), complications (14.3% vs. 12.3%; p < .001), and plan deviations (34.2% vs. 25.1%; p < .001). CONCLUSION: The surgical database is an important resource in surgical management in patients with bleeding disorders. Further evaluation will facilitate use for the development of predictive models and principles of care.

Management of people with haemophilia A undergoing surgery while receiving emicizumab prophylaxis: Real-world experience from a large comprehensive treatment centre in the US.

INTRODUCTION: Surgery is frequently required in persons with haemophilia A (PwHA). Emicizumab, a bispecific, humanized monoclonal antibody, bridges activated factor (F) IX and FX. Management of patients undergoing surgery while receiving emicizumab is of clinical interest due to paucity of data. AIM: Review real-world experience of PwHA with/without FVIII inhibitors who required surgery while receiving emicizumab prophylaxis. METHODS: Data regarding peri-operative management, including type of surgery, haemostatic agent use and bleeding complications, were collected for PwHA receiving emicizumab undergoing surgery between 25/10/18 and 31/12/19 at the Indiana Hemophilia and Thrombosis Center. Analyses were exploratory and descriptive. RESULTS: Twenty minor and five major surgeries were performed in 17 and five patients, respectively. Overall, 9/20 minor surgeries were planned to occur with emicizumab as the sole haemostatic agent; of these, four required additional coagulation factor (2 due to haematomas following port removals, 1 due to oozing at port removal site, 1 due to bleeding following squamous cell carcinoma removal). Three of the 11 minor surgeries with planned additional coagulation factor resulted in non-major bleeds; all were safely managed with additional coagulation factor. All five major surgeries were planned with additional haemostatic agents; there was 1 bleed in a patient undergoing elbow synovectomy with nerve transposition, likely triggered by physical/occupational therapy. There were no major bleeds, thrombotic events or deaths. CONCLUSIONS: Additional haemostatic agent use is safe in PwHA undergoing surgery while receiving emicizumab. Additional data are needed to determine the optimal dosing/length of treatment of additional haemostatic agents to lower bleeding risk.

Mixing and administration times of bypassing agents: observations from the Dosing Observational Study in Hemophilia (DOSE).

DOSE (Dosing Observational Study in Hemophilia) was a prospective, observational diary study designed to evaluate the use of bypassing agents in patients prescribed recombinant activated factor VII (rFVIIa) as first-line treatment in the home setting. Patients with congenital hemophilia with inhibitors and caregivers participated, and as part of the study, the time spent preparing and administering product was recorded for bypassing agent (BPA) infusions. The aim of this manuscript is to present the results of the analysis of the time spent preparing and administering a single dose of either rFVIIa or plasma-derived activated prothrombin complex concentrate (pd-aPCC). Diaries were completed for 18 adult patients and 19 caregivers of 21 children with 176 BPA-treated bleeding episodes and 1,350 BPA infusions (1,270 rFVIIa, 80 pd-aPCC). The median preparation and administration times were 5.0 minutes and 5.0 minutes for rFVIIa and 29.0 minutes and 24.5 minutes for pd-aPCC, respectively. Preparation and administration times were significantly shorter with rFVIIa than pd-aPCC (P<0.0001). The significantly shorter combined preparation and administration time of rFVIIa, taking into consideration the faster-than-recommended aPCC infusion rates, suggests that rFVIIa permits a rapid and safe initiation of treatment once a bleeding episode is identified and a decision is made to treat at home.

Radiosynovectomy: review of the literature and report from two haemophilia treatment centers.

Haemophilic arthropathy occurs due to recurrent bleeding into joints leading to swelling, inflammation, destruction of cartilage and bone, and development of arthritis. Although prophylactic replacement therapy assists in preventing arthropathy, it is not always adequate or affordable. Radiosynovectomy is a minimally invasive intervention for treatment of synovitis in haemophilic joints. The procedure utilises locally injected radioisotopes (Y, P, Rh) to ablate abnormal synovium with the goal of decreasing bleeding, slowing progression of cartilage and bone damage and preventing arthropathy. The objective of this review is to summarize the radiosynovectomy literature and to present patient outcomes associated with radiosynovectomy over the past 17 years from two haemophilia treatment centers (HTCs), one in the United States and one in Spain. Articles from these two centers support the current literature. A retrospective medical records review was performed by the two reporting HTCs on patients who underwent radiosynovectomy prior to 2009. Data review included: site of procedure, isotope utilized, bleeding frequency, and procedure complications. Radiosynovectomy is a cost-effective, minimally invasive, well tolerated procedure. As the paradigm for care in haemophilia shifts towards prevention of joint disease, the number of target joints with synovitis will likely decrease, except in patients who develop inhibitors. We propose early consideration of radiosynovectomy for patients with haemophilic synovitis prior to appearance of articular cartilage damage.