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Von Willebrand DiseasePhase 3NCT02932618

A Study of Recombinant Von Willebrand Factor (rVWF) With or Without ADVATE in Children With Severe Von Willebrand Disease (VWD)

A Phase 3 study testing Vonicog alfa and Antihemophilic Factor (Recombinant) in people with Von Willebrand Disease, run by Baxalta now part of Shire at 46 sites in 13 countries.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
Search all trials

Trial summary

The main aim of the study is to check effectiveness, side effects, and tolerability of vonicog alfa (recombinant von Willebrand factor [rVWF]), with or without ADVATE, in the treatment and control of nonsurgical bleeding events in pediatric participants (less than (<)18 years of age) with severe hereditary von… Read more about this study

Who is it for?
Ages Up to 17 years
Conditions the study is for
Von Willebrand Disease
What is being tested?
Vonicog alfa and Antihemophilic Factor (Recombinant)
Could I receive a placebo?
No, everyone receives an active treatment
How many people
47 participants
Where
46 sitesin 13 countries
Study dates
6 Nov 2017to 17 Apr 2026
Phase
Phase 3
Are there results?
None posted in the registry

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages Up to 17 years
  • All sexes
  • No healthy volunteers
  • Child

Who may be able to join

  • Diagnosis of severe von Willebrand disease (VWD) (defined as von Willebrand factor: ristocetin cofactor [VWF:RCo] less than [<] 20 percent [%]):
  • Type 1 (VWF:RCo <20 International Units per deciliter [IU/dL]); or
  • Type 2A (VWF:RCo <20 IU/dL), Type 2B (as diagnosed by genotype), Type 2N (Factor VIII coagulation activity [FVIII:C] <10 % and historically documented genetics), Type 2M; or
  • Type 3 (VWF:Ag less than or equal to [=<] 3 IU/dL).
  • Age 0 to <18 years at the time of Screening.
  • The participant has provided assent (if appropriate) and legally authorized representative(s) has provided informed consent.
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  • If female of childbearing potential, participant presents with a negative serum pregnancy test.
  • If applicable, participant agrees to employ adequate birth control measures for the duration of the study.
  • The participant and/or the legally authorized representative are willing and able to comply with the requirements of the protocol, which should also be confirmed based on a pre-screening evaluation held between the Investigator and the Sponsor, to ensure no eminent risk is present that could challenge the participants compliance with the study requirements.
  • Additional inclusion criteria for both previously treated participants and participants undergoing surgery are as follows:
  • Unable to tolerate are inadequately responsive to, or not a good candidate for 1-deamino-8-D-arginine vasopressin (DDAVP). Examples of participants who are not good candidates for DDAVP include participants with type 2B or type 3 VWD.
  • The participant has had a minimum of 1 documented bleed requiring VWF coagulation factor replacement therapy (i.e. treatment with a VWF product) during the previous 12 months prior to enrollment and overall historically 3 or more exposure days (EDs) to VWF replacement therapy.
  • Additional inclusion criterion for previously untreated participants are as follows:
  • The participant has not received prior VWF coagulation factor replacement therapy.

Who cannot join

  • Diagnosis of pseudo-VWD or another hereditary or acquired coagulation disorder (eg, qualitative and quantitative platelet disorders or elevated prothrombin time [PT]/international normalized ratio [INR] greater than [>] 1.4).
  • History or presence of a VWF inhibitor at Screening.
  • History or presence of a Factor VIII (FVIII) inhibitor with a titer greater than or equal [>=] 0.4 Bethesda units (BU) (by Nijmegen assay) or >=0.6 BU (by Bethesda assay).
  • Documented history of a VWF: RCo half-life <6 hours.
  • Known hypersensitivity to any of the components of the study drug, such as mouse or hamster proteins.
  • Medical history of immunological disorders, excluding seasonal allergic rhinitis/conjunctivitis/asthma, food allergies, or animal allergies.
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  • Medical history of a thromboembolic event.
  • Human immunodeficiency virus (HIV) positive, with an absolute CD4 count <200/ cubic millimeter (mm^3).
  • In the judgment of the Investigator, the participant has another clinically significant concomitant disease (e.g. uncontrolled hypertension, cancer) that may pose additional risks for the participant.
  • Diagnosis of significant liver disease, as evidenced by, but not limited to, any of the following: serum alanine aminotransferase (ALT) of 5 times the upper limit of normal; hypoalbuminemia; portal vein hypertension (e.g. presence of otherwise unexplained splenomegaly, history of esophageal varices) or liver cirrhosis classified as Child B or C.
  • Diagnosis of renal disease, with a serum creatinine level >=2.5 milligram per deciliter (mg/dL).
  • Immunomodulatory drug treatment other than anti-retroviral chemotherapy (e.g. α-interferon, or corticosteroid agents at a dose equivalent to hydrocortisone greater than 10 milligram per day [mg/day] (excluding topical treatment [e.g. ointments, nasal sprays]), within 30 days prior to signing the informed consent (or assent, if appropriate).
  • If female, participant is pregnant or lactating at the time informed consent (or assent, if appropriate) is obtained.
  • Participant has participated in another clinical study involving an investigational product (IP), other than vonicog alfa with or without ADVATE, or investigational device within 30 days prior to enrollment or is scheduled to participate in another clinical study involving an IP other than vonicog alfa or investigational device during the course of this study.
  • Participant's legal representative is a family member or employee of the Investigator.

Trial locations and contacts

46 sites in 13 countries.

46 sites in 13 countries

United States16 sites
  • University of Colorado Hemophilia & Thrombosis Center

    Aurora, Colorado, 80045

  • Medical University of South Carolina

    Charleston, South Carolina, 29425

  • St. Jude Affiliate Clinic at Novant Health

    Charlotte, North Carolina, 28204

  • Cincinnati Children's Hospital Medical Center

    Cincinnati, Ohio, 45229

  • Rainbow Babies and Children's Hospital

    Cleveland, Ohio, 44106

  • Nationwide Children's Hospital

    Columbus, Ohio, 43205

  • Texas Children's Cancer and Hematology Center

    Houston, Texas, 77030

  • Texas Children's Hospital

    Houston, Texas, 77030

  • Indiana Hemophilia and Thrombosis Center

    Indianapolis, Indiana, 46260

  • University of Florida College of Medicine

    Jacksonville, Florida, 32610

  • Comprehensive Center for Bleeding Disorders

    Milwaukee, Wisconsin, 53225

  • University of Nebraska Medical Center

    Omaha, Nebraska, 68198

  • Bleeding and Clotting Disorders Institute

    Peoria, Illinois, 61615

  • Children's Hospital of Philadelphia

    Philadelphia, Pennsylvania, 19104

  • Children's National Medical Center

    Washington D.C., District of Columbia, 20010

  • Comprehensive Cancer Center of Wake Forest Unversity

    Winston-Salem, North Carolina, 27157

Other trials in United States

France8 sites
  • Groupe Hospitalier Pellegrin - Hôpital Pellegrin

    Bordeaux, 33000

  • Hôpital Morvan

    Brest, Finistere, 29609

  • Groupement Hospitalier Est- Hôpital Louis Pradel

    Bron, 69677

  • CHU CAEN - Hôpital de la Côte de Nacre

    Caen, 14033

  • Groupement Hospitalier Sud - Hôpital Bicêtre

    Le Kremlin-Bicêtre, 94270

  • Hopital Cardiologique - CHU Lille

    Lille, 59037

  • CHU de Nantes Site Hotel Dieu

    Nantes, 44093

  • Hôpital Necker - Enfants Malades

    Paris, 75743

Other trials in France

Italy4 sites
  • Azienda Ospedaliera Universitaria Careggi

    Florence, 50134

  • Fondazione IRCCS CA' Granda Ospedale Maggiore Policlinico

    Milan, 20122

  • Azienda Ospedaliera Pediatrica Santobono Pausillipon

    Naples, 80122

  • Ospedale Pediatrico Bambino Gesù

    Roma, 00165

Other trials in Italy

Germany3 sites
  • Universitaetsklinikum Hamburg-Eppendorf

    Hamburg, 20246

  • Werlhof-Institut GmbH

    Hanover, 30159

  • Medizinische Hochschule Hannover

    Hanover, 30625

Other trials in Germany

Russia3 sites
  • SBEI HPE Altai State Medical University of MoH and SD

    Barnaul, 656038

  • SAIH "Kemerovo Regional Clinical Hospital"

    Kemerovo, 650066

  • FSBI of Science "Kirov Scientific and Research Institute of Hematology and Blood Transfusion of FMBA

    Kirov, 610027

Other trials in Russia

Turkey (Türkiye)3 sites
  • Istanbul University Cerrahpasa Medical Faculty

    Istanbul, 34098

  • Ege University Medical Faculty

    Izmir, 35100

  • Ondokuz Mayis Univ. Med. Fac.

    Samsun, 55139

Other trials in Turkey (Türkiye)

Austria2 sites
  • Medizinische Universität Innsbruck

    Innsbruck, 6020

  • AKH - Medizinische Universität Wien

    Vienna, 1090

Other trials in Austria

Spain2 sites
  • Hospital General Universitario de Alicante

    Alicante, 03010

  • Hospital Universitari i Politecnic La Fe

    Valencia, 46026

Other trials in Spain

Belgium1 site
  • UZ Leuven

    Leuven, 3000

Other trials in Belgium

Czechia1 site
  • Fakultni nemocnice Brno

    Brno, 613 00

Other trials in Czechia

Netherlands1 site
  • Erasmus Medisch Centrum

    Rotterdam, 3015 CN

Other trials in Netherlands

Ukraine1 site
  • SI Institute of Blood Pathology and Transfusion Medicine of NAMSU

    Lviv, 79044

Other trials in Ukraine

United Kingdom1 site
  • Royal Manchester Children's Hospital

    Manchester, Greater Manchester, M13 9WL

Other trials in United Kingdom

About this study

Official title: A Phase 3, Prospective, Multicenter, Uncontrolled, Open-Label Clinical Study to Determine the Efficacy, Safety, and Tolerability of rVWF With or Without ADVATE in the Treatment and Control of Bleeding Episodes, the Efficacy and Safety of rVWF in Elective and Emergency Surgeries, and the Pharmacokinetics (PK) of rVWF in Children Diagnosed With Severe Von Willebrand Disease

The main aim of the study is to check effectiveness, side effects, and tolerability of vonicog alfa (recombinant von Willebrand factor [rVWF]), with or without ADVATE, in the treatment and control of nonsurgical bleeding events in pediatric participants (less than (<)18 years of age) with severe hereditary von Willebrand disease (VWD).

The participants will be treated with vonicog alfa for 12-18 months. Their von Willebrand Disease will be treated by their doctor according to their doctor's usual clinical practice. During the study, participants will be followed up at clinics or over telephone calls.

Being tested
Vonicog alfa, Antihemophilic Factor (Recombinant)
Medical subject headings
von Willebrand Diseases

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • Vonicog alfaBiological

    Also called VONVENDI, rVWF, BAX 111, TAK-577

    Lyophilized powder and solvent to prepare solution for injection.

  • Antihemophilic Factor (Recombinant)Biological

    Also called Recombinant Factor VIII, rFVIII, Octocog alfa

    Packaged in single boxes with 2 glass vials, with one vial containing the lyophilized ADVATE and the second vial containing the diluent.

Study groups in NCT02932618
Study groupRoleWhat they receive
On-demand TreatmentExperimentalParticipants will receive vonicog alfa (recombinant von Willebrand factor [rVWF]) treatment for non-surgical bleeding episodes over a 12 to 18-month period.
Receives: Biological: Vonicog alfa; Biological: Antihemophilic Factor (Recombinant)
Elective SurgeryExperimental12-24 hours prior to surgery and within 3 hours of surgery. Minor surgery: infuse every 12-24 hours for at least 48 hours based on post-operative dosing. Oral Surgery: infuse at least once within first 8-12 hours post-surgery based on post-operative dosing. Major Surgery: infuse every 12-24 hours for at least first 96 hours post-surgery based on post-operative dosing.
Receives: Biological: Vonicog alfa; Biological: Antihemophilic Factor (Recombinant)
Emergency SurgeryExperimentalWithin 3 hours prior to surgery. Minor surgery: infuse every 12-24 hours for at least 48 hours based on post-operative dosing. Oral Surgery: infuse at least once within first 8-12 hours post-surgery based on post-operative dosing. Major Surgery: infuse every 12-24 hours for at least first 96 hours post-surgery based on post-operative dosing.
Receives: Biological: Vonicog alfa; Biological: Antihemophilic Factor (Recombinant)

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. Hemostatic Efficacy

    Treatment success for vonicog alfa-treated nonsurgical bleeding episodes (using a 4-point scale: Excellent, Good, Moderate, None).

    Measured: Within 24 hours after the last infusion of study drug following the onset of the bleeding episode (if/when the severity and/or duration of the bleeding requires the infusion of the study drug)

Secondary outcomes (25)
  1. Number of Treated Nonsurgical Bleeding Episodes With an Efficacy Rating of 'Excellent' or 'Good'

    If/when the severity and/or duration of the bleeding requires the infusion of the study drug.

    Measured: Throughout the study duration of approximately 8.5 years

  2. Number of Infusions per Bleeding Episode

    Measured: Throughout the study duration of approximately 8.5 years

  3. Number of Vonicog Alfa Units per Bleeding Episode

    Measured: Throughout the study duration of approximately 8.5 years

  4. Number of ADVATE Units (if needed) per Bleeding Episode

    Measured: Throughout the study duration of approximately 8.5 years

  5. Elective or Emergency Surgery: Assessment of Hemostatic Efficacy - Immediately After Surgery

    Assessed by the operating surgeon, based on a 4-point ordinal scale: Excellent, Good, Moderate, None.

    Measured: Immediately after surgery

  6. Elective or Emergency Surgery: Overall Assessment of Hemostatic Efficacy 24 Hours After the Last Perioperative Infusion of rVWF

    Assessed by the Investigator (hematologist) on a 4-point ordinal scale: Excellent, Good, Moderate, None.

    Measured: 24 hours after last perioperative rVWF infusion

  7. Elective or Emergency surgery: Overall Assessment of Hemostatic Efficacy Day 7 Post-operative

    Assessed by the Investigator (hematologist) on a 4-point ordinal scale: Excellent, Good, Moderate, None.

    Measured: Post-operative Day 7

  8. Elective or Emergency surgery: Overall Assessment of Hemostatic Efficacy Day 14 Post-operative

    Assessed by the Investigator (hematologist) on a 4-point ordinal scale: Excellent, Good, Moderate, None.

    Measured: Post-operative Day 14

  9. Incidence and Severity of Adverse Events (AEs)

    Measured: Throughout the study period of approximately 8.5 years

  10. Incidence of Thromboembolic Events

    Measured: Throughout the study period of approximately 8.5 years

  11. Incidence of Severe Hypersensitivity Reactions

    Measured: Throughout the study period of approximately 8.5 years

  12. Development of Neutralizing Antibodies to von Willebrand Factor (VWF) and Factor VIII (FVIII)

    Measured: Throughout the study period of approximately 8.5 years

  13. Development of Total Binding Antibodies to von Willebrand Factor (VWF)

    Measured: Throughout the study period of approximately 8.5 years

  14. Development of Antibodies to Chinese Hamster Ovary (CHO) Proteins, Murine Immunoglobulin G (IgG), and rFurin

    Measured: Throughout the study period of approximately 8.5 years

  15. Area Under the Plasma Concentration/Time Curve From 0 to 96 Hours Post-Infusion (AUC0-96h) for Von Willebrand factor: ristocetin cofactor (VWF:Rco), von Willebrand factor: antigen (VWF:Ag) and von Willebrand factor: collagen binding capacity (VWF:CB)

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  16. Area Under the Plasma Concentration/Time Curve From Time 0 to Infinity (AUC0-inf) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  17. Mean Residence Time (MRT) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  18. Time to Reach Maximal Plasma Concentration (Tmax) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  19. Maximal Plasma Concentration (Cmax) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  20. Clearance (CL) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  21. Incremental Recovery (IR) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  22. In-vivo Recovery (IVR) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  23. Elimination Phase Half-life (T1/2) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  24. Volume of Distribution at Steady State (Vss) for VWF:RCo, VWF:Ag and VWF:CB

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

  25. Area Under the Plasma Concentration/Time Curve From 0 to 96 Hours Post-infusion (AUC0-96h) for Factor VIII (FVIII) Activity

    Measured: Within 30 minutes prior to infusion and post infusion at: SEQUENCE 1: 1, 24, 72 hours; SEQUENCE 2: 0.25, 12, and 48 hours; SEQUENCE 3: 6, 30, and 96 hours

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted13 Oct 2016 (estimated)
  2. Study start6 Nov 2017
  3. Primary completion17 Apr 2026
  4. Study completion17 Apr 2026

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 3
Primary purpose
Treatment
Allocation
Non-randomised
Intervention model
Parallel groups
Masking
None (open label)
Enrolment
47 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT02932618
Sponsor's protocol ID
071102
Other IDs
2016-001477-33, 2023-509769-18-00, 2023, 2016
Status last verified
May 2026
First submitted
12 Oct 2016
Last updated
15 May 2026
Results posted
No
Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
Individual data sharing
Yes: Takeda provides access to the de-identified individual participant data (IPD) for eligible studies to aid qualified researchers in addressing legitimate scientific objectives (Takeda's data sharing commitment is available on https://clinicaltrials.takeda.com/takedas-commitment?commitment=5). These IPDs will be provided in a secure research environment following approval of a data sharing request, and under the terms of a data sharing agreement.

Questions about this trial

What is this clinical trial studying?

A Phase 3 study testing Vonicog alfa and Antihemophilic Factor (Recombinant) in people with Von Willebrand Disease, run by Baxalta now part of Shire at 46 sites in 13 countries.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 14 inclusion and 15 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 15 May 2026. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 46 sites in United States, France, Italy, Germany, Russia and 8 more countries. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 3 studies compare the treatment with the current standard treatment or a placebo in a large group, to confirm how well it works and watch for side effects.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

Hemostatic Efficacy (time frame: Within 24 hours after the last infusion of study drug following the onset of the bleeding episode (if/when the severity and/or duration of the bleeding requires the infusion of the study drug)). It is what the study was designed to measure, not a finding.

Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 15 May 2026.

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