An Exploratory Multi-Centre, Multi-National, Randomised, Double Blinded, Parallel Arm Trial Evaluating Safety, Pharmacokinetics and Dose-finding of prophylactic administration of Long Acting rFVIIa (LA-rFVIIa) in Haemophilia A or B Patients with Inhibitors

INTERVENTION: Product Name: LA‐rFVIIa Product Code: NN7128 Pharmaceutical Form: Powder and solvent for solution for injection INN or Proposed INN: eptacgo alfa pegol (activated) CAS Number: 944130‐77‐2 Current Sponsor code: NN7128 Other descriptive name: Long Acting recombinant activated Factor VII (LA‐rFVIIa), PEGylated rFVIIa Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 2‐ Pharmaceutical form of the placebo: Powder and solvent for solution for injection Route of administration of the placebo: Intravenous bolus use (Noncurrent) CONDITION: Haemophilia A (Factor VIII) or B with Inhibitors ; MedDRA version: 9.1 Level: LLT Classification code 10018939 Term: Haemophilia B (Factor IX) ; MedDRA version: 9.1 Level: LLT Classification code 10060612 Term: Hemophilia A PRIMARY OUTCOME: Main Objective: The primary objective is to determine the safety including the potential immunogenicity of LArFVIIa intravenously administered to haemophilia patients with inhibitors every second day for 12 weeks at three dose levels 25 µg/kg, 100 µg/kg and 200 µg/kg.; ; Primary end point(s): • Frequency of AE, SAE and Medical Events of Special Interest (MESI as defined in section 12.2.1) reported during the trial period; • Neutralising antibodies towards FVII and/or LA‐rFVIIa; • Coagulation related parameters: D‐dimers, Prothrombin Fragment 1+2, Fibrinogen, PT, aPTT and AT; • Platelet count, haematology; • Biochemistry; • ECG and Troponin T; • Urinalysis; • Vital signs, physical examination; • Injection site inspection Secondary Objective: To evaluate the preliminary efficacy of LA‐rFVIIa in reducing the bleeding frequency in; haemophilia A and B patients with inhibitors, and to identify the dose(s) suitable for further; development.; To investigate the pharmacokinetic properties of multiple intravenous doses of three dose levels of; LA‐rFVIIa.; To investigate the effect of LA‐rFVIIa on health economic (HE)/Patient Reported Outcomes (PRO) parameters INCLUSION CRITERIA: 1. Informed consent obtained before any trial‐related activities (Trial‐related activities are any procedure that would not have been performed during normal management of the patient.) 2. Male Haemophilia A or B patients with inhibitors 3. Aged between 12 and 65 years, both inclusive 4. Willing to undergo a bleeding preventive regimen of 3 months duration and a total trial length of approximately 8 months 5. Historical or ongoing high titer inhibitor (= 5 BU) based on either medical records, laboratory report reviews, patient and/or care provider interviews 6. At least 2 bleeding episodes requiring bypassing haemostatic‐drug‐based treatment within the last month or 12 bleeding episodes within the last 6 months prior to Observation period 7. Body weight between 30 and 100 kg (both inclusive) 8. Patient has adequate venous access at the Screening Visit 9. Patient or caregiver is capable of assessing a bleed, capable of home treatment of