Use of Recombinant Factor VIIa in Children with Uncontrolled Bleeding - A Single Centre Experience.

Recombinant factor VIIa (rFVIIa; NovoSeven) has been successfully used in the management of hemophilia patients with inhibitors for many years. Recombinant activated factor VIIa is being increasingly used to secure haemostasis in difficult clinical situations. rFVIIa is a novel hemostatic agent that shows promise in non-hemophiliac patents of a significant therapeutic role in variety of coagulopathic and hemorrhagic conditions in clinical situations ranging from thrombocytopenia, disseminated intravascular coagulation and transfusion-related coagulopathy, as well as in patients experiencing massive blood loss undergoing orthotopic liver transplantation, cardiac, orthopedic and genitourinary surgery. This review will explore its use in the control of platelet function disorder associated haemorrhage in patients without pre-existing coagulopathy/Dengue hemorrhagic shock/post surgery bleed/post procedural uncontrolled bleeding etc, and will highlight the growing realization that rFVIIa may have a major role not only as a treatment for hemophilia, but also as a universal hemostatic agent. The mechanism of action suggests that its enhancing effects in haemostasis are limited to the site of injury and that systemic activation of the coagulation cascade does not occur. In contrast to rising experience obtained by ongoing clinical trials in adults, poor data exists for the use of rFVIIa in acquired coagulopathies in childhood. We report on four children, 1 year to 14 years old, with severe bleeding episodes treated by recombinant factor VIIa. One patient of Dengue hemorrhagic shock (Grade IV Dengue Hemorrhagic fever) with DIC, one of Glanzmann thrombosthenia with massive upper gastrointestinal bleed, one of hemorrhagic shock with bleeding from hypopharynx 48 hr post-surgery (even after surgical correction of bleeding points), and one of gross hematuria for more than 24 hrs post renal biopsy. Manifestation of bleeding included mucosal/gastrointestinal bleeding, intra-abdominal bleeding, hematuria. Recombinant factor VIIa was used as bolus therapy. Dose regimen was calculated individually with dose of 90-μg/kg/dose. Repeat dose was used in one patient with Glanzmann thrombosthenia in view of partial response after 1^st dose and restart of bleeding after 2 hours. Prior to factor VIIa administration patients were given trial of FFP, cryoprecipitate and platelets as appropriate. We monitored the response to the therapy by maintenance of blood pressure, an estimation of blood loss and by coagulation tests. In all patients, bleeding symptoms could be markedly reduced or stopped by application of rFVIIa. No adverse effects were observed, especially no thrombembolic complications. Nevertheless one of the four patients died - 13-year-old female child with Dengue hemorrhagic shock with DIC died after 35 days of hospital stay due to resistant pseudomonas sepsis. The use of recombinant factor VIIa may be a valuable therapeutic option in children with severe acquired coagulopathies/uncontrolled bleeding post procedure since it provides a good hemostatic efficacy and probably few adverse side-effects.