"I'll never forget my first patient. When I saw a prolonged aPTT, I knew where to turn for a fast treatment."2
Actor portrayal
Congenital hemophilia with inhibitors, as seen in the adept 2 clinical trial.2,3
Acquired
hemophilia4
Congenital Factor VII deficiency2,5
Glanzmann’s thrombasthenia2
Clinical pathways to prepare your hospital for patients who may visit the emergency department
Sid has congenital
hemophilia A with inhibitors.
Inhibitors put patients at higher risk of bleeding complications
Of patients with congenital hemophilia, approximately 1 in 5 patients with hemophilia A and 3 in 100 patients with hemophilia B develop inhibitors.6 Delayed treatment of bleeds may result in cumulative damage.7
Congenital hemophilia:
- Affects men predominantly8
- Symptoms include hemarthrosis and muscle bleeds8
- Patients may develop alloantibodies which target FVIII or FIX infused into the body9
Model used for
Illustrative use only
Early diagnosis is crucial for treating acquired hemophilia
Acquired hemophilia (AH) is a rare and often idiopathic autoimmune condition that can cause severe and even fatal bleeding.10 Delayed diagnosis is common with over 60% of patients getting diagnosis up to a week after onset of bleeding symptoms.
- Affects men and women
- Symptoms include subcutaneous, mucosal, and muscle bleeds
- Autoantibodies target FVIII produced in the body
NovoSeven® RT is designed for efficiency in pharmacies
NovoSeven® RT addresses bleeds, whenever they occur.2 Learn more about the features that make this rFVIIa useful for hospitals and pharmacies.
Study design
Lentz, et al
adept™2 phase 3 trial
Patients randomized: Patients ≥12 years of age with hemophilia A with inhibitors (N=66) or hemophilia B with inhibitors (N=6) who have experienced at least 5 bleeds during treatment prior to entering the trial.
Study design: A 12-month, international, multicenter, randomized, double-blind, active-controlled, crossover, confirmatory phase 3 trial. Primarily done in the home setting, treatment of bleeding episodes were randomized, either treated with 1 to 3 doses of vatreptacog alfa (340 bleeding episodes) at 80 mcg/kg or 1 to 3 doses of NovoSeven® RT (2227 bleeding episodes) at 90 mcg/kg.
Primary endpoint: Effective bleed control, which was defined as no additional treatment needed (other than the original medication) within 12 hours after first dose.
Secondary endpoint: Effective and sustained bleed control at 1 and 2 days after initial dose, number of doses of trial product for each bleed, and changes in pain assessment.
Published literature, compassionate use trials, and the Hemophilia and Thrombosis Research Society (HTRS) Registry
Patients considered: Data was collected from published literature, compassionate use trials, and the HTRS for patients with congenital factor VII deficiency (N=70) treated with NovoSeven® RT.
Study design: NovoSeven® RT was used as treatment in 124 bleeding episodes, surgeries, or prophylaxis regimens. Dosing ranged from 6 to 98 mcg/kg administered every 2 to 12 hours (except for prophylaxis [doses administered from 2 times per day up to 2 times per week]). Patients were treated with an average of 1 to 10 doses. Treatment was effective if bleeding stopped or the physician rated the treatment as effective.
Glanzmann’s Thrombasthenia Registry (GTR)
Patients considered: Data was collected from the GTR in patients with Glanzmann’s thrombasthenia (N=218).
Study design: Adjudicator-assessed effectiveness of treatment regimens in patients with GT (N=218) in all severe bleeding episodes and all surgical procedures (N=1073) based on review of Glanzmann’s Thrombasthenia Registry (GTR) data unblinded to investigator-coded efficacy. Efficacy was evaluated on a 2-point scale (clinical assessment of success or failure of treatment regimen as a whole, blinded and unblinded to investigator-coded outcome) including 92 patients treated with NovoSeven® RT for 266 bleeding episodes and 77 patients treated for 160 surgical procedures.
Educational materials for you.
Need perioperative dosing information?
Selected Important Safety Information for NovoSeven® RT
WARNING: THROMBOSIS
- Serious arterial and venous thrombotic events following administration of NovoSeven® RT have been reported
- Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive NovoSeven® RT
- Monitor patients for signs or symptoms of activation of the coagulation system and for thrombosis
Warnings and Precautions
- Serious arterial and venous thrombotic events have been reported in clinical trials and postmarketing surveillance
- Patients with congenital hemophilia receiving concomitant treatment with aPCCs (activated prothrombin complex concentrates), older patients particularly with acquired hemophilia and receiving other hemostatic agents, and patients with a history of cardiac and vascular disease may have an increased risk of developing thrombotic events
Indications and Usage
NovoSeven® RT (coagulation Factor VIIa, recombinant) is a coagulation factor indicated for:
- Treatment of bleeding episodes and perioperative management in adults and children with hemophilia A or B with inhibitors, congenital Factor VII (FVII) deficiency, and Glanzmann’s thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets
- Treatment of bleeding episodes and perioperative management in adults with acquired hemophilia
Important Safety Information
WARNING: THROMBOSIS
- Serious arterial and venous thrombotic events following administration of NovoSeven® RT have been reported
- Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive NovoSeven® RT
- Monitor patients for signs or symptoms of activation of the coagulation system and for thrombosis
Warnings and Precautions
- Serious arterial and venous thrombotic events have been reported in clinical trials and postmarketing surveillance
- Patients with congenital hemophilia receiving concomitant treatment with aPCCs (activated prothrombin complex concentrates), older patients particularly with acquired hemophilia and receiving other hemostatic agents, and patients with a history of cardiac and vascular disease may have an increased risk of developing thrombotic events
- Hypersensitivity reactions, including anaphylaxis, can occur with NovoSeven® RT. Patients with a known hypersensitivity to mouse, hamster, or bovine proteins may be at a higher risk of hypersensitivity reactions. Discontinue infusion and administer appropriate treatment when hypersensitivity reactions occur
- Factor VII deficient patients should be monitored for prothrombin time (PT) and factor VII coagulant activity (FVII:C). If FVII:C fails to reach the expected level, or PT is not corrected, or bleeding is not controlled after treatment with the recommended doses, antibody formation may be suspected and analysis for antibodies should be performed
- Laboratory coagulation parameters (PT/INR, aPTT, FVII:C) have shown no direct correlation to achieving hemostasis
Adverse Reactions
- The most common and serious adverse reactions in clinical trials are thrombotic events. Thrombotic adverse reactions following the administration of NovoSeven® RT in clinical trials occurred in 4% of patients with acquired hemophilia and 0.2% of bleeding episodes in patients with congenital hemophilia
Drug Interactions
- Thrombosis may occur if NovoSeven® RT is administered concomitantly with Coagulation Factor XIII
Please click here for NovoSeven® RT Prescribing Information, including Boxed Warning.
References
- Data on file as of 2021. Novo Nordisk Inc; Plainsboro, NJ.
- NovoSeven RT [package insert]. Plainsboro, NJ: Novo Nordisk Inc; 2020.
- Lentz SR, Ehrenforth S, Abdul Karim F, et al; adept™2 investigators. Recombinant factor VIIa analog in the management of hemophilia with inhibitors: results from a multicenter, randomized, controlled trial of vatreptacog alfa. J Thromb Haemost. 2014;12(8):1244-1253.
- Sumner MJ, Geldziler BD, Pedersen M, Seremetis S. Treatment of acquired haemophilia with recombinant activated FVII: a critical appraisal. Haemophilia. 2007;13(5):451-461.
- Mariani G, Napolitano M, Dolce A, et al. Replacement therapy for bleeding episodes in factor VII deficiency. A prospective evaluation. Thromb Haemost. 2013;109:238–247.
- Centers for Disease Control and Prevention. Hemophilia. https://www.cdc.gov/ncbddd/hemophilia/inhibitors.html. September 6, 2018. Accessed August 2, 2019.
- Salek SZ, Benson GM, Elezovic I, et al. The need for speed in in the management of haemophilia patients with inhibitors. Haemophilia. 2011;17(1):95-102.
- Srivastava A, Brewer AK, Mauser-Bunschoten EP, et al. Guidelines for the management of hemophilia. Haemophilia. 2013;19(1):e1-e47.
- Carcao M, Goudemand J. Inhibitors in Hemophilia. Montréal, Québec, Canada: World Federation of Hemophilia; 2018.
- Huth-Kühne A, Baudo F, Collins P, et al. International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica. 2009;94(4):566-575.