NCT04023019

Brief Summary

This is a non-interventional, multicenter, observational, international study in male persons with haemophilia A who have developed inhibitors to any replacement coagulation factor VIII (FVIII) product. The purpose of the study is to capture different approaches in the management of persons with haemophilia A and FVIII inhibitors, document current immune tolerance induction approaches, and evaluate the efficacy and safety of immune tolerance induction, including the combination of FVIII and emicizumab. Patients will be assigned to 1 of 3 groups based on the treatments they receive, and may switch to another group if their treatment is changed. Participants will be followed after a maximum observational period of 5 years.

Trial Health

80
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
120

participants targeted

Target at P50-P75 for all trials

Timeline
37mo left

Started Mar 2020

Longer than P75 for all trials

Geographic Reach
2 countries

2 active sites

Status
recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress67%
Mar 2020Jun 2029

First Submitted

Initial submission to the registry

July 12, 2019

Completed
5 days until next milestone

First Posted

Study publicly available on registry

July 17, 2019

Completed
8 months until next milestone

Study Start

First participant enrolled

March 17, 2020

Completed
8.7 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 1, 2028

Expected
6 months until next milestone

Study Completion

Last participant's last visit for all outcomes

June 1, 2029

Last Updated

September 5, 2025

Status Verified

August 1, 2025

Enrollment Period

8.7 years

First QC Date

July 12, 2019

Last Update Submit

August 28, 2025

Conditions

Keywords

Blood disorders

Outcome Measures

Primary Outcomes (4)

  • Proportion of participants achieving inhibitor titer < 0.6 Bethesda units (BU)/mL L for at least 2 consecutive measurements

    The proportion of participants in Groups 1 and 2 achieving inhibitor titer \< 0.6 Bethesda units (BU)/mL L for at least 2 consecutive measurements will be determined. FVIII inhibitor titer is measured at baseline and throughout the study, according to standard of care.

    Up to 5 years

  • Proportion of participants achieving FVIII recovery ≥ 66% of the predefined reference value of 1.5% IU/kg body weight (Groups 1 and 2)

    The proportion of participants in Groups 1 and 2 achieving FVIII recovery ≥ 66% of the predefined reference value of 1.5% IU/kg body weight will be determined. Once inhibitor has become negative (\< 0.6 BU/mL), FVIII plasma levels are measured prior to and approximately 15 to 30 minutes after FVIII to evaluate FVIII recovery.

    Up to 5 years

  • Proportion of participants achieving FVIII half-life ≥ 6 h (Groups 1 and 2)

    The proportion of participants in Groups 1 and 2 achieving FVIII half-life ≥ 6 h will be determined. Once inhibitor has become negative (\< 0.6 BU/mL), FVIII plasma levels are measured prior to and at 15-30 minutes and 2, 4, 8-12, and 24 hours after administration of the immune tolerance induction (or prophylactic FVIII) to evaluate half-life; when FVIII trough levels are \> 1% during regular prophylaxis, half-life can be evaluated from fewer samples or using a population pharmacokinetic model.

    Up to 5 years

  • Annualized bleeding rate

    Annualized rate of all bleeding episodes will be reported and compared between all 3 study groups.

    Up to 5 years

Secondary Outcomes (11)

  • Time to achieve immune tolerance induction outcome

    Up to 5 years

  • Frequency of emicizumab, aPCC, and rFVIIa use during immune tolerance induction

    Up to 5 years

  • Rate of FVIII inhibitor relapse

    Up to 5 years

  • Frequency of bleeding episodes

    Up to 5 years

  • Severity of bleeding episodes

    Up to 5 years

  • +6 more secondary outcomes

Study Arms (3)

Group 1: ITI with Nuwiq, octanate, or wilate

Participants receiving immune tolerance induction with either Nuwiq, octanate, or wilate. As needed, aPCC/rFVIIa will be administered to treat bleeding episodes or during surgery and for prophylaxis.

Biological: NuwiqBiological: OctanateBiological: WilateBiological: Recombinant factor VIIa (rFVIIa)Biological: Activated prothrombin complex concentrate (aPCC)

Group 2: ITI with Nuwiq, octanate, or wilate with emicizumab

Participants receiving immune tolerance induction with either Nuwiq, octanate, or wilate, in combination with emicizumab prophylaxis. As needed, aPCC/rFVIIa will be administered to treat bleeding episodes or during surgery.

Biological: NuwiqBiological: OctanateBiological: WilateBiological: EmicizumabBiological: Recombinant factor VIIa (rFVIIa)Biological: Activated prothrombin complex concentrate (aPCC)

Group 3: Prophylaxis with emicizumab, aPCC, or rFVIIa

Participants receiving routine prophylaxis with emicizumab, aPCC, or rFVIIa without immune tolerance induction. On-demand aPCC/rFVIIa can be used as needed to treat bleeding episodes or during surgery.

Biological: EmicizumabBiological: Recombinant factor VIIa (rFVIIa)Biological: Activated prothrombin complex concentrate (aPCC)

Interventions

NuwiqBIOLOGICAL

Nuwiq is a recombinant FVIII concentrate from a human cell line. It is administered via intravenous injection at a dosage determined by the investigator's discretion, in consideration of the participants' clinical condition and prescribing information.

Group 1: ITI with Nuwiq, octanate, or wilateGroup 2: ITI with Nuwiq, octanate, or wilate with emicizumab
OctanateBIOLOGICAL

Octanate is a high-purity human Factor VIII / von Willebrand Factor (VWF) concentrate. It is administered via intravenous injection at a dosage determined by the investigator's discretion, in consideration of the participants' clinical condition and prescribing information.

Group 1: ITI with Nuwiq, octanate, or wilateGroup 2: ITI with Nuwiq, octanate, or wilate with emicizumab
WilateBIOLOGICAL

Wilate is a high-purity human von Willebrand Factor (VWF)/Factor VIII concentrate. It is administered via intravenous injection at a dosage determined by the investigator's discretion, in consideration of the participants' clinical condition and prescribing information.

Group 1: ITI with Nuwiq, octanate, or wilateGroup 2: ITI with Nuwiq, octanate, or wilate with emicizumab
EmicizumabBIOLOGICAL

Emicizumab is a therapeutic antibody which brings activated factor IX and factor X together It is administered via subcutaneous injection at a dosage determined by the investigator's discretion, in consideration of the participants' clinical condition and prescribing information.

Also known as: Hemlibra
Group 2: ITI with Nuwiq, octanate, or wilate with emicizumabGroup 3: Prophylaxis with emicizumab, aPCC, or rFVIIa

Recombinant factor VIIa (rFVIIa) is a blood factor VII manufactured using recombinant technology. It is administered via intravenous injection at a dosage determined by the investigator's discretion, in consideration of the participants' clinical condition and prescribing information.

Also known as: NovoSeven
Group 1: ITI with Nuwiq, octanate, or wilateGroup 2: ITI with Nuwiq, octanate, or wilate with emicizumabGroup 3: Prophylaxis with emicizumab, aPCC, or rFVIIa

Activated prothrombin complex concentrate (aPCC) is an anti-inhibitor coagulant complex acting on multiple pathways to facilitate coagulation. It is administered via intravenous injection at a dosage determined by the investigator's discretion, in consideration of the participants' clinical condition and prescribing information.

Also known as: FEIBA
Group 1: ITI with Nuwiq, octanate, or wilateGroup 2: ITI with Nuwiq, octanate, or wilate with emicizumabGroup 3: Prophylaxis with emicizumab, aPCC, or rFVIIa

Eligibility Criteria

Sexmale
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

The study population includes males with haemophilia A who have delveloped inhibitors to any FVIII product. Participants starting treatment after study inclusion will have all data recorded prospectively while those who have already started treatment can enter if detailed retrospective documentation is available. Participants will be observed for a maximum of 5 years.

You may qualify if:

  • Male persons with haemophilia A, of any severity, who have a historical inhibitor titer ≥ 0.6 BU/mL, including those who have failed previous immune tolerance induction (ITI) attempt(s)
  • Persons undergoing ITI with Nuwiq, octanate, or wilateor undergoing ITI with Nuwiq®, octanate® or wilate® and receiving prophylactic therapy with emicizumab, activated prothrombin complex concentrate (aPCC), or activated recombinant factor VII (rFVIIa)
  • Participants or participants' parent(s)/legal guardian(s) must be capable of giving signed informed consent and be able to understand the trial documents

You may not qualify if:

  • Participants are excluded from the trial if any coagulation disorder other than haemophilia A is diagnosed
  • Partly retrospective patients will be excluded if detailed documentation on treatment, all bleeding episodes, inhibitor titers, and FVIII levels is not available for the retrospective period

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (2)

Arthur M. Blank Hospital

Atlanta, Georgia, 30329, United States

RECRUITING

HZRM Hämophilie-Zentrum Rhein Main

Mörfelden-Walldorf, 64546, Germany

RECRUITING

Related Publications (1)

  • Escuriola Ettingshausen C, Sidonio RF Jr. Design of an international investigator-initiated study on MOdern Treatment of Inhibitor-positiVe pATiEnts with haemophilia A (MOTIVATE). Ther Adv Hematol. 2021 Sep 23;12:20406207211032452. doi: 10.1177/20406207211032452. eCollection 2021.

MeSH Terms

Conditions

Hemophilia AHematologic Diseases

Interventions

Factor VIIIvon Willebrand Factoremicizumabrecombinant FVIIaanti-inhibitor coagulant complex

Condition Hierarchy (Ancestors)

Blood Coagulation Disorders, InheritedBlood Coagulation DisordersHemic and Lymphatic DiseasesCoagulation Protein DisordersHemorrhagic DisordersGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and Abnormalities

Intervention Hierarchy (Ancestors)

Blood Coagulation FactorsBlood ProteinsProteinsAmino Acids, Peptides, and ProteinsProtein PrecursorsBiological Factors

Study Officials

  • Robert Sidonio, MD, MSc

    Emory University

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Robert Sidonio, MD, MSc

CONTACT

Carmen Escuriola-Ettingshausen, MD

CONTACT

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
Associate Professor

Study Record Dates

First Submitted

July 12, 2019

First Posted

July 17, 2019

Study Start

March 17, 2020

Primary Completion (Estimated)

December 1, 2028

Study Completion (Estimated)

June 1, 2029

Last Updated

September 5, 2025

Record last verified: 2025-08

Data Sharing

IPD Sharing
Will not share

Locations