Status:

WITHDRAWN

Emicizumab PUPs and Nuwiq ITI Study

Lead Sponsor:

Emory University

Collaborating Sponsors:

Genentech, Inc.

Conditions:

Hemophilia A

Eligibility:

All Genders

Up to 21 years

Phase:

PHASE3

Brief Summary

This study prospectively investigates the safety, FVIII immunogenicity, and hemostatic efficacy of prophylactic HEMLIBRA® given with a concomitant low dose recombinant factor VIII (rFVIII) known as NU...

Detailed Description

Hemophilia A (HA) is a congenital bleeding disorder caused by deficient or dysfunctional factor VIII (FVIII) which leads to bleeding correlated with severity. Management is focused on FVIII replacemen...

Eligibility Criteria

Inclusion

  • Inclusion Criteria - Part A:
  • Moderately severe (≤2% FVIII) hemophilia A
  • \<3 Years of age at the time of informed consent
  • Caregiver (parent or legal guardian) has provided written informed consent
  • ≤2 EDs to pdFVIII, rFVIII, or a single dose of FFP, Cryoprecipitate or PRBCs.
  • Adequate hematologic function (HgB \>8 g/dL and platelet count \>100,000 µL)
  • Adequate hepatic function (total bilirubin ≤1.5x ULN and both AST/ALT ≤3x ULN at screening (excluding known Gilbert's)
  • Adequate renal function (≤2.5 x ULN and CrCl ≥30 mL/min)
  • Negative test for inhibitor (\<0.6 BU/mL) with a 72-hour washout within 4 weeks of enrollment
  • No documented FVIII inhibitor since birth \*Participants will be encouraged to co-enroll in the ATHN 8 Study
  • Inclusion Criteria - Part B
  • Moderately severe (≤2% FVIII) hemophilia A
  • \<21 Years of age at the time of informed consent
  • Documented on 2 occasions a persistent low (\>0.6 BU/mL) titer inhibitor with a 72-hour washout within 24 weeks of enrollment or historical high titer inhibitor (\>5 BU/mL) and a single occasion of a low titer inhibitor (\>0.6 BU/mL) with a 72-hour washout within 24 weeks of enrollment after either the first time ITI or after single attempt of \<6 months of continuous 3x/week factor ITI
  • Has completed loading doses of HEMLIBRA® (weekly for 4 weeks, dose 3 mg/kg, a total of 12 mg/kg/dose will also be allowed)
  • Caregiver and/or participant provided written informed consent
  • Adequate hematologic function (HgB \>8 g/dL and platelet count \>100,000 µL)
  • Adequate hepatic function (total bilirubin ≤1.5x ULN and both AST/ALT ≤3x ULN at screening (excluding known Gilbert's)
  • Adequate renal function (≤2.5 x ULN and CrCl ≥30 mL/min)
  • Exclusion Criteria - Part A and B
  • Inherited or acquired bleeding disorder other than severe hemophilia A (participants with previous documentation of low von Willebrand factor (vWF) defined as vWF antigen and vWF ristocetin cofactor both between 40-50 will be permitted)
  • Previous or current treatment for thromboembolic disease or signs of thromboembolic disease
  • Conditions that may increase the risk of bleeding or thrombosis. Will not require or request a thrombophilia evaluation
  • History of clinically significant hypersensitivity associated with monoclonal antibody therapies or components of the HEMLIBRA® injection (with the exception of rituximab)
  • Known HIV infection with CD4 count \<200 cells/µL within 24 weeks prior to screening. Testing is not required if can demonstrate negative testing in the mother prior to pregnancy
  • Use of systemic immunomodulators at enrollment or planned use during the study
  • Participants who are at high risk for thrombotic microangiopathy (TMA) (for example, have a previous medical or family history of TMA), in the investigator's judgment
  • Concurrent disease, treatment, or abnormality in clinical laboratory tests that could interfere with the conduct of the study, may pose an additional risk, or would, in the opinion of the investigator, preclude the participant's safe participation in and completion of the study
  • Planned surgery (excluding minor procedures or central line placement) during the study
  • Receipt of HEMLIBRA® as part of a prior investigational study; an investigational drug to treat or reduce the risk of hemophilic bleeds within 5 half-lives of the last drug administration; a non-hemophilia-related investigational drug concurrently, within the last 30 days or 5 half-lives, whichever is shorter

Exclusion

    Key Trial Info

    Start Date :

    February 17 2022

    Trial Type :

    INTERVENTIONAL

    Allocation :

    ACTUAL

    End Date :

    January 19 2023

    Estimated Enrollment :

    Patients enrolled

    Trial Details

    Trial ID

    NCT04030052

    Start Date

    February 17 2022

    End Date

    January 19 2023

    Last Update

    January 5 2024

    Active Locations (8)

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    Page 1 of 2 (8 locations)

    1

    Children's Hospital Los Angeles

    Los Angeles, California, United States, 90027

    2

    Emory University/Children's Healthcare of Atlanta

    Atlanta, Georgia, United States, 30322

    3

    Rush University Medical Center

    Chicago, Illinois, United States, 60612

    4

    Mindy_L_Simpson@rush.edu

    Indianapolis, Indiana, United States, 46260