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Phase 3 N=74 Treatment

Safety, Efficacy and PK of BIVV001 in Pediatric Patients With Hemophilia A

Hemophilia A

Enrolled (actual)
74
Serious AEs
12.2%
Results posted
Feb 2024
Primary outcome: Primary: Number of Participants With Neutralising Antibodies (Development of Inhibitors) Directed Against Factor VIII — 0; 0 Participants

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
efanesoctocog alfa (BIVV001) (Drug)
Age
Pediatric
Sex
Male
Sponsor
Bioverativ, a Sanofi company
Primary completion
Jan 2023

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Participants With Neutralising Antibodies (Development of Inhibitors) Directed Against Factor VIII
0; 0
SECONDARY
Annualized Bleeding Rate (ABR): For Treated Bleeds
0.48; 1.33
SECONDARY
Sensitivity Analysis: Annualized Bleeding Rate: For Treated Bleeds
0.48; 0.75
SECONDARY
Annualized Bleeding Rate for All Bleeding Episodes
2.78; 2.85
SECONDARY
Sensitivity Analysis: Annualized Bleeding Rate for All Bleeding Episodes
2.78; 2.32
SECONDARY
Annualized Bleeding Rate by Type of Bleed (Spontaneous, Traumatic and Unknown Type)
0.17; 0.14; 0.28; 0.59; 0.03; 0.59
SECONDARY
Sensitivity Analysis: Annualized Bleeding Rate by Type of Bleed (Spontaneous, Traumatic and Unknown Type)
0.17; 0.15; 0.28; 0.52; 0.03; 0.09
SECONDARY
Annualized Bleeding Rate by Location of Bleed (Joint, Muscle, Internal and Skin/Mucosa)
0.19; 0.99; 0.03; 0.17; 0.08; 0.06
SECONDARY
Sensitivity Analysis: Annualized Bleeding Rate by Location of Bleed (Joint, Muscle, Internal and Skin/Mucosa)
0.19; 0.40; 0.03; 0.17; 0.08; 0.06
SECONDARY
Percentage of Participants Achieving FVIII Activity Levels Above 1%, 3%, 5%, 10%, 15%, and 20%
100; 100; 100; 100; 75.0; 100
SECONDARY
Number of Injections of BIVV001 Required to Treat a Bleeding Episode
1.12; 1.30
SECONDARY
Sensitivity Analysis: Number of Injections of BIVV001 Required to Treat a Bleeding Episode
1.12; 1.00
SECONDARY
Percentage of Bleeding Episodes Treated With a Single Injection of BIVV001
88.2; 78.7
SECONDARY
Sensitivity Analysis: Percentage of Bleeding Episodes Treated With a Single Injection of BIVV001
88.2; 100
SECONDARY
Total Dose of BIVV001 Required to Treat a Bleeding Episode
52.29; 66.90
SECONDARY
Sensitivity Analysis: Total Dose of BIVV001 Required to Treat a Bleeding Episode
52.29; 50.39
SECONDARY
Physicians' Global Assessment (PGA) of Participant's Response to BIVV001 Treatment Based on a 4-point Response Scale
37; 34; 0; 2; 0; 0
SECONDARY
Participant's Response to BIVV001 Treatment Based on the International Society on Thrombosis and Haemostasis (ISTH) 4-point Response Scale
14; 22; 1; 2; 1; 0
SECONDARY
Total Annualized BIVV001 Consumption Per Participant
3115.57; 2884.67
SECONDARY
Annualized Joint Bleeding Rate (AJBR)
0.19; 0.99
SECONDARY
Sensitivity Analysis: Annualized Joint Bleeding Rate (AJBR)
0.19; 0.41
SECONDARY
Change From Baseline in Hemophilia Joint Health Score Domain Score at Week 52
0.0; -0.1; 0.1; -0.0; 0.0; -0.1
SECONDARY
Change From Baseline in Hemophilia Quality of Life Questionnaire (Haemo-QoL) Kids Short Version Total Score at Week 52 for Children Participants (Aged 4 to 7 and 8 to <12 Years)
-5.31; 4.69; -9.79
SECONDARY
Change From Baseline in Hemophilia Quality of Life Questionnaire Parent Proxy Short Version Total Score at Week 52 for Children Participants (Aged 4 to 7 and 8 to <12 Years): Parent's Evaluation
-3.21; -1.17; -4.05
SECONDARY
Change From Baseline in Hemophilia Quality of Life Questionnaire Kids Short Version Physical Health Domain Score at Week 52 for Children Participants (Aged 8 to <12 Years)
-10.63
SECONDARY
Change From Baseline in Hemophilia Quality of Life Questionnaire Parent Proxy Short Version Physical Health Domain Score at Week 52 for Children Participants (Aged 8 to <12 Years): Parent's Evaluation
-7.64
SECONDARY
Total Number of Target Joints Resolved in Participants at Week 52
2
SECONDARY
Change From Baseline in Hemophilia Joint Health Score (HJHS) Total Score at Week 52
0.2; -1.1
SECONDARY
Investigators' or Surgeons' Assessment of Participant's Hemostatic Response to BIVV001 Treatment
2; 2; 0; 0; 0
SECONDARY
Number of Injections Per Surgery Required to Maintain Hemostasis During Perioperative Period for Major Surgery
2; 0; 0; 0; 0
SECONDARY
Number of Blood Component Transfusions Used During Perioperative Period for Major Surgery
2; 0; 0; 0; 0
SECONDARY
Total BIVV001 Consumption From Day -1 to 14 During Perioperative Period for Major Surgery
201.97
SECONDARY
Type of Blood Component Transfusions Used During Perioperative Period for Major Surgery
0; 0; 0; 0; 0
SECONDARY
Total Dose Required to Maintain Hemostasis From Day -1 to Day 0 During Perioperative Period for Major Surgery
61.13
SECONDARY
Estimated Blood Loss During Major Surgery
25.00
SECONDARY
Number of Participants With Treatment-emergent Adverse Events (TEAEs) and Treatment-emergent Serious Adverse (TESAEs)
33; 29; 5; 4
SECONDARY
Number of Participants With Occurrence of Embolic and Thrombotic Events
0; 0
SECONDARY
Pharmacokinetics (PK): Maximum FVIII Activity (Cmax)
128
SECONDARY
Pharmacokinetics: Clearance (CL)
0.711
SECONDARY
Pharmacokinetics: Volume of Distribution at Steady State (Vss)
37.3
SECONDARY
Pharmacokinetics: Elimination Half-life (t1/2z)
40.2
SECONDARY
Pharmacokinetics: Area Under the Plasma FVIII Activity Versus Time Curve (AUC0-tau)
7000
SECONDARY
Pharmacokinetics: Total Clearance at Steady State (CLss)
SECONDARY
Pharmacokinetics: Incremental Recovery (IR)
2.53
SECONDARY
Pharmacokinetics: Trough Concentration for BIVV001 (Ctrough)
0.00; 13.68
SECONDARY
Pharmacokinetics: Mean Residence Time (MRT)
53.0
SECONDARY
Time Above Predefined (10% and 40%) FVIII Activity Levels
160; 72.2
SECONDARY
Pharmacokinetics: Dose-normalized Area Under the Activity-time Curve (DNAUC0-tau)
139

Summary

Primary Objective: - To evaluate the safety of BIVV001 in previously treated pediatric participants with hemophilia A. Secondary Objectives: * To evaluate the efficacy of BIVV001 as a prophylaxis treatment. * To evaluate the efficacy of BIVV001 in the treatment of bleeding episodes. * To evaluate BIVV001 consumption for prevention and treatment of bleeding episodes. * To evaluate the effect of BIVV001 prophylaxis on joint health outcomes. * To evaluate the effect of BIVV001 prophylaxis on Quality of Life (QoL) outcomes. * To evaluate the efficacy of BIVV001 for perioperative management. * To evaluate the safety and tolerability of BIVV001 treatment. * To assess the pharmacokinetics (PK) of BIVV001.

Eligibility Criteria

Inclusion criteria

  • Participant must be younger than 12 years of age, at the time of signing the informed consent.
  • Severe hemophilia A defined as =) 0.6 Bethesda units (BU/mL), or any value greater than or equal to the lower sensitivity cut-off for laboratories with cut-offs for inhibitor detection between 0.7 and 1.0 BU/mL, or clinical signs or symptoms of decreased response to FVIII administrations. Family history of inhibitors would not exclude the participant.
  • Positive inhibitor test result, defined as >=0.6 BU/mL at Screening.

The above information was not intended to contain all considerations relevant to a participant's potential participation in a clinical trial.

View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT04759131). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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