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Successful use of ReFacto continuous infusion in two paediatric patients with severe haemophilia A undergoing
1Department of Pediatrics, University of Virginia, Charlottesville, VA, USA.
Insights
B-domain-deleted recombinant factor VIII (ReFacto) effectively managed severe haemophilia A in children undergoing orthopaedic surgery. Continuous infusion provided safe and successful haemostatic cover during complex procedures and post-operative recovery.
Area of Science:
- Haematology
- Paediatric Orthopaedics
- Pharmacology
Background:
- Severe haemophilia A requires effective factor VIII replacement therapy for surgical procedures.
- Continuous infusion of factor VIII concentrates offers potential advantages in maintaining therapeutic levels.
- Recombinant factor VIII (ReFacto) is a B-domain-deleted variant used for haemophilia A treatment.
Observation:
- Two children with severe haemophilia A received ReFacto for orthopaedic procedures, including ankle synovectomy and knee ligament repair.
- One patient developed septic arthritis, necessitating arthroscopic irrigation and debridement.
- Surgical cover involved a bolus dose followed by continuous infusion of ReFacto for up to 9 days post-operatively.
Findings:
- Continuous infusion of ReFacto was successfully administered during complex orthopaedic surgeries and post-operative periods.
- No haemostatic complications were observed in either patient throughout the treatment course.
- Both patients achieved good long-term orthopaedic outcomes.
Implications:
- Continuous infusion of ReFacto is a viable and safe strategy for surgical management in children with severe haemophilia A.
- This approach can ensure adequate haemostasis during major orthopaedic interventions and recovery.
- Further research may explore optimal dosing and duration for similar complex cases.
Abstract:
In this report we describe the successful use of B-domain-deleted recombinant factor VIII (ReFacto) administered by continuous infusion during orthopaedic procedures in two children with severe haemophilia A. Both patients underwent ankle synovectomy and in patient 2, a medial patello-femoral ligament repair was performed in the same operative session. Patient 2 developed septic arthritis A in his knee joint and arthroscopic joint irrigation and debridement was performed 2 weeks after the initial procedure. Surgical cover was initiated with a bolus dose of ReFacto 50 IU kg(-1) followed by continuous infusion at 3.3-4.8 IU kg(-1) h(-1) which was maintained for up to 9 days postoperatively. Patient 2 received an additional bolus dose of 15 IU kg(-1) during the infusion period. All procedures were performed without haemostatic complications and long-term orthopaedic outcomes were good in both patients.
