Skip to main navigation menu Skip to main content Skip to site footer

Admin menu

Volume 12 (2025): Issue 1

Use of extended half-life factor IX products in the surgical setting: case series from a UK haemophilia centre

Abstract

Abstract Introduction

For people with haemophilia, surgical procedures, whether haemophilia-related or for other conditions, are associated with a risk of bleeding. Factor activity levels must be maintained for any invasive intervention. Clinical studies have shown that extended half-life (EHL) recombinant factor products are beneficial in this context. Clinical nurse specialists working in comprehensive care centres play a central role in preparing patients for surgery and in coordinating the surgical process. This case series reports on the nurse experience of using EHL-FIX products in surgery in a small UK cohort of people with haemophilia B (PwHB).

Methods

A consecutive series of PwHB who underwent minor and major surgery from 2018–2022 were identified from the Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust database. Variables retrieved included age, haemophilia severity, surgery details (type, day/inpatient, major/minor, inpatient stay length, day surgery location, EHL FIX product used and consumption. Comparative data for patients receiving standard half-life FIX during the same surgery were also sought.

Results

Twenty-two surgeries were performed during the period covered (9 major, 13 minor), 12 of which were orthopaedic. All surgeries were completed without the need for continuous infusion. For 6 of 13 minor surgeries, and 1 of 9 major surgeries, only a single infusion of EHL FIX product was required. The use of EHL FIX allowed some minor procedures to be performed at local facilities rather than within the main hospital setting. Most major surgeries required an inpatient stay of 1–4 days and fewer than three infusions of an EHL FIX product. Total EHL FIX consumption ranged from 5,000 to 46,000 IU for major surgery, and from 3,000 to 13,000 IU for minor procedures.

Conclusion

EHL FIX allows for intermittent infusions, thus avoiding the requirement for continuous infusions and making outpatient treatment possible for some minor surgeries. Reduced dosing frequency and FIX consumption, greater protection from postoperative bleeding complications enabling a better recovery and a shorter hospital stay all reduce demands on medical personnel and could significantly reduce costs associated with surgery in PwHB.

Article

View Full Article

References

  • 1. Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia 2020; 26 Suppl 6: 1–158. doi: https://doi.org/10.1111/hae.14046.
  • 2. Mannuci PM. Hemophilia therapy: the future has begun. Haematologica 2020; 105(3): 545–553. doi: https://doi.org/10.3324/haematol.2019-232132.
  • 3. Escobar MA, Brewer A, Caviglia H, et al. Recommendations on multidisciplinary management of elective surgery in people with haemophilia. Haemophilia 2018; 24(5): 693–702. doi: https://doi.org/10.1111/hae.13549.
  • 4. Pollard D, Harrison C, Dodgson S, Holland M, Khair K. The UK haemophilia specialist nurse: Competencies fit for practice in the 21st century. Haemophilia 2020; 26(4): 622–630. doi: https://doi.org/10.1111/hae.14002.
  • 5. Ragni MV, Pasi KJ, White GC, Giangrande PL, Courter SG, Tubridy KL; Recombinant FIX Surgical Study Group. Use of recombinant factor IX in subjects with haemophilia B undergoing surgery. Haemophilia 2002; 8(2): 91–7. doi: https://doi.org/10.1046/j.1365-2516.2002.00587.x.
  • 6. Ar MC, Balkan C, Kavaklı K. Extended half-life coagulation factors: a new era in the management of hemophilia patients. Turk J Haematol 2019; 36(3): 141–154. doi: https://doi.org/10.4274/tjh.galenos.2019.2018.0393.
  • 7. Roth DA, Kessler CM, Pasi KJ, et al. Human recombinant factor IX: safety and efficacy studies in hemophilia B patients previously treated with plasma-derived factor IX concentrates. Blood 2001; 98: 3600–3606. doi: https://doi.org/10.1182/blood.v98.13.3600.
  • 8. Shapiro AD, Di Paola J, Cohen A, et al. The safety and efficacy of recombinant human blood coagulation factor IX in previously untreated patients with severe or moderately severe hemophilia B. Blood 2005; 105: 518–525. doi: https://doi.org/10.1182/blood-2004-06-2283.
  • 9. Windyga J, Timofeeva M, Stasyshyn O, et al. Phase 3 clinical trial: perioperative use of nonacog gamma, a recombinant factor IX, in previously treated patients with moderate/severe hemophilia B. Clin Applied Thromb Haemost 2020; 26: 1–10. doi: https://doi.org/10.1177/1076029620946839.
  • 10. Chowdary P, Holmström M, Mahlangu JN, et al. Managing surgery in hemophilia with recombinant factor VIII Fc and factor IX Fc: Data on safety and effectiveness from phase 3 pivotal studies. Res Pract Thromb Haemost 2022; 6: e12760. doi: https://doi.org/10.1002/rth2.1276d.
  • 11. Castaman G, Matino D. Hemophilia A and B: molecular and clinical similarities and differences. Haematologica 2019; 104(9): 1702–1702. doi: https://doi.org/10.3324/haematol.2019.221093.
  • 12. Curtin J, Santagostino E, Karim FA, et al. Simplifying surgery in haemophilia B: Low factor IX consumption and infrequent infusions in surgical procedures with rIX-FP. Thromb Res 2020; 188: 85–9. doi: https://doi.org/10.1016/j.thromres.2020.02.011.
  • 13. Négrier C, Abdul Karim F, Lepatan LM, et al. Efficacy and safety of long-acting recombinant fusion protein linking factor IX with albumin in haemophilia B patients undergoing surgery. Haemophilia 2016; 22(4): e259–66. doi: https://doi.org/10.1111/hae.12972.
  • 14. Loveday HP, Wilson JA, Pratt RJ, et al. epic3: national evidence-based guidelines for preventing healthcare-associated infections in NHS hospitals in England. J Hosp Infect 2014; 86 Suppl 1: S1–70. doi: https://doi.org/10.1016/S0195-6701(13)60012-2.
  • 15. Mulders G, Uitslager N, Kavanagh M, et al. The role of the specialist nurse in comprehensive care for. Bleeding disorders in Europe: An integrative review. Haemophilia 2024; 30(3): 598–608. doi: https://doi.org/10.1111/hae.14974

PDF Download

Download PDF

Open in full-page viewer

Authors

  • Molly Ndebele

    Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free Hospital, London, UK
  • Emma Charnick

    Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free Hospital, London, UK
  • Nawal Asir

    Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free Hospital, London, UK
  • Elsa Aradom

    ORCID iD
    elsa.aradom@nhs.net
    Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free Hospital, London, UK