Management and outcomes of paediatric patients on emicizumab prophylaxis undergoing surgical procedures: Experience from a large haemophilia centre in the UK

Hassan, Ekram M.; Jayashree, Muralidharan

doi:10.1111/hae.14358

Cited by 11 publications

(12 citation statements)

References 13 publications

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“…No patient received factor concentrate before or after surgery and no bleeding complications were reported. All cases received planned pre‐ and post‐operative antifibrinolytic treatment 22 . A summary of findings from these studies is shown in Table 2.…”

Section: Discussionmentioning

confidence: 99%

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Port removal in patients receiving emicizumab prophylaxis: A single centre experience and review of the literature

2021

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Introduction Treatment of patients with Haemophilia A has improved significantly in recent years since the advent of novel therapeutic agents such as emicizumab. The low annualised bleeding rates associated with emicizumab have liberated many patients from the need for central venous access devices (CVAD). Optimal peri‐operative management of CVAD removal is not currently known and there are no specific formal recommendations available. Aim We reviewed outcomes in a paediatric cohort in our centre undergoing CVAD removal without pre‐operative factor or bypassing agent and reviewed the literature regarding port removal in patients on Emicizumab. Methods Ten male patients with severe Haemophilia A underwent CVAD removal without planned administration of factor concentrate or bypassing agent. Patients were monitored in hospital for 24 h with routine laboratory testing pre‐ and post‐operatively. Results No significant bleeding episodes occurred in any patient, no patient required factor concentrate or bypassing agent and no patients were readmitted due to bleeding within 7 days of surgery. Conclusion We propose that, in the era of emicizumab, prophylactic factor administration pre‐operatively for elective CVAD removal is not required in the majority of cases.

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Section: Discussionmentioning

confidence: 99%

“…All cases received planned pre-and postoperative antifibrinolytic treatment. 22 A summary of findings from these studies is shown in Table 2.…”

Section: Discussionmentioning

confidence: 99%

Port removal in patients receiving emicizumab prophylaxis: A single centre experience and review of the literature

2021

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“…55 Regarding surgical interventions in paediatric patients during emicizumab prophylaxis, a recent study in children whose median (range) age was 6.6 years (0.7-13.2) confirmed that minor procedures, especially central venous access device removals, can be safely done with antifibrinolytic agents without administration of additional haemostatic agents, regardless of FVIII inhibitor status. 58 Overall, the availability of emicizumab has significantly upended the traditional approach to managing children with haemophilia A. Its 59 Before recommending PUP therapy or transitioning previously treated children into emicizumab prophylaxis, a shared decision-making process with parents and caregivers is advised (see Figure 1).…”

Section: Update On Paediatric Clinical Studies and The Real-world Exp...mentioning

confidence: 99%

“…Ongoing inhibitor monitoring in tolerised patients with haemophilia A who transition to emicizumab is strongly recommended 55 . Regarding surgical interventions in paediatric patients during emicizumab prophylaxis, a recent study in children whose median (range) age was 6.6 years (0.7‐13.2) confirmed that minor procedures, especially central venous access device removals, can be safely done with antifibrinolytic agents without administration of additional haemostatic agents, regardless of FVIII inhibitor status 58 …”

Section: Update On Paediatric Clinical Studies and The Real‐world Exp...mentioning

confidence: 99%

Emicizumab state‐of‐the‐art update

2022

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Introduction:Emicizumab is a bispecific monoclonal antibody developed to address the unmet needs of clotting factor replacement therapy and has become the benchmark for optimal prophylaxis in managing patients with haemophilia A with inhibitors.We describe the emicizumab rollout and pharmacokinetic strategies and their use in paediatric patients. Methods:The evolving real-world experience in using emicizumab has confirmed its safety, efficacy and pharmacokinetic profile in paediatric, adolescent and adult patients receiving emicizumab at various prophylactic dosing regimens. The emicizumab current global rollout includes over 100 countries with 29 low to middle-income countries accessing emicizumab through the World Federation of Haemophilia (WFH) Humanitarian Aid Program. The diversity of emicizumab dosing and pharmacokinetic tools such as the Calibra® and the WAPPS-Hemo platforms make it possible to achieve prophylaxis goals in line with the WFH Haemophilia treatment guidelines recommendations, with minimal drug wastage. The emerging experience from long term clinical trials and long-term real-world follow-up confirm the safety, efficacy, and pharmacokinetic profile of emicizumab in paediatric haemophilia A patients. A few questions, including inhibitor recurrence, concurrent use of emicizumab with various replacement therapies and inhibitor eradication, are being addressed through multiple ongoing clinical studies. Conclusion:The current global rollout of emicizumab is remarkable, and versatile dosing regimens and evolving pharmacokinetic tools such as the Calibra® and WAPPS-Hemo platforms make it a treatment choice available also for pharmacokinetic guided personalised treatment. Data from paediatric studies are consistent with those seen in adolescent and adult Haemophilia A.

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“…47 Similar outcomes have been reported for a total of additional 27 CVAD removals included in the description of other case series on emicizumab use. [66][67][68][69] Of these only five received preoperative treatment 66 and only one experienced a mild postoperative bleeding. 68…”

Section: Perioperative Management Of Pwh Treated With Emicizumabmentioning

confidence: 99%

Managing invasive procedures in haemophilia patients with limited resources, extended half‐life concentrates or non‐replacement therapies in 2022

2022

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New treatment possibilities and modalities are now available globally for patients with haemophilia requiring surgery or invasive procedures. The first is the appropriate application of low‐dose protocols of clotting factor concentrates (CFC) achieving adequate perioperative haemostasis in resources constraint environments. The increasing availability of CFC through humanitarian aid programs allows more invasive surgeries to be performed for which efficacy and safety data should be more widely collected and reported. Second, extended half‐life CFC that are increasingly available in many countries represent valuable alternatives to standard half‐life products in surgical patients allowing reduced number of infusions and lower consumption, in particular for extended half‐life factor IX. Third, in the era of recently introduced nonfactor prophylaxis, some minor surgical procedures can now be performed without additional haemostatic treatment, others with few low‐dose administrations of CFC or bypassing agents. Additional factor VIII/IX or recombinant activated factor VII has proven to be safe and effective in association with emicizumab for major surgeries and it was effectively given at low doses in association with fitusiran (including activated prothrombin complex concentrate). No thrombotic complications have been reported in the surgical setting so far. A multidisciplinary team/facility remains crucial to manage major surgery in patients on prophylaxis with these new agents.

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Management and outcomes of paediatric patients on emicizumab prophylaxis undergoing surgical procedures: Experience from a large haemophilia centre in the UK

Cited by 11 publications

References 13 publications

Port removal in patients receiving emicizumab prophylaxis: A single centre experience and review of the literature

Port removal in patients receiving emicizumab prophylaxis: A single centre experience and review of the literature

Emicizumab state‐of‐the‐art update

Managing invasive procedures in haemophilia patients with limited resources, extended half‐life concentrates or non‐replacement therapies in 2022

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