40 Participants Needed

Emicizumab for Von Willebrand Disease

(BCDI-XII Trial)

Recruiting at 9 trial locations
SE
LW
SK
NB
Overseen ByNikki Barclay, BS
Age: Any Age
Sex: Any
Trial Phase: Phase 1
Sponsor: Bleeding and Clotting Disorders Institute Peoria, Illinois
Must be taking: Emicizumab
No Placebo GroupAll trial participants will receive the active study treatment (no placebo)
Approved in 2 JurisdictionsThis treatment is already approved in other countries

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial examines whether Emicizumab, a medication typically administered as a subcutaneous injection, can safely assist individuals with severe Von Willebrand Disease (VWD) or both VWD and hemophilia. These conditions lead to excessive bleeding, and the trial seeks to determine if Emicizumab can prevent bleeding episodes. It suits those with severe VWD or a combination of VWD and hemophilia who currently require regular treatments to manage bleeding. Participants must not have recently used Emicizumab and must be willing to switch to this treatment if they are using another. As a Phase 1 trial, the research focuses on understanding how the treatment works in people, offering participants the chance to be among the first to receive this potentially groundbreaking therapy.

Will I have to stop taking my current medications?

If you are currently on a prophylaxis treatment for Von Willebrand Disease or VWD/hemophilia A that is not emicizumab, you may need to stop your current treatment to participate in the trial. The protocol requires that you have not taken emicizumab in the last 18 months and are willing to discontinue your current prophylaxis.

Is there any evidence suggesting that Emicizumab is likely to be safe for humans?

Research has shown that Emicizumab is safe for people with bleeding disorders like hemophilia A. Studies have found that Emicizumab can help prevent bleeding with weekly injections under the skin, a method easier and less invasive than injections into a vein.

In past studies, patients who used Emicizumab reported few side effects, most of which were mild, such as skin reactions at the injection site. No serious side effects were commonly reported, suggesting that most patients tolerate Emicizumab well.

The FDA has approved Emicizumab for hemophilia A, further supporting its safety. While individual experiences may vary, current evidence suggests that Emicizumab could be a safe option for managing bleeding disorders.12345

Why do researchers think this study treatment might be promising?

Unlike the standard treatments for von Willebrand Disease, which typically involve replacing missing clotting factors, Emicizumab offers a novel approach by mimicking a protein that helps blood clotting. This gives it a unique mechanism of action that doesn't rely on factor replacement. Researchers are excited because Emicizumab is administered as a subcutaneous injection, which could be more convenient and potentially improve adherence compared to intravenous options. Additionally, Emicizumab has shown promise in other bleeding disorders, raising hopes that it might offer a more effective or convenient option for von Willebrand Disease as well.

What evidence suggests that Emicizumab might be an effective treatment for Von Willebrand Disease?

Research has shown that Emicizumab, also known as Hemlibra, is a promising treatment for bleeding disorders. It acts as a bridge between two important blood proteins, aiding proper clotting. In studies with patients who have hemophilia A, Emicizumab effectively prevented unexpected bleeding when administered as a weekly injection under the skin. This trial will evaluate Emicizumab for Von Willebrand Disease (VWD), which also involves blood clotting issues. Although direct data for VWD is limited, Emicizumab's success in similar conditions provides a strong basis for its potential in treating VWD.12467

Who Is on the Research Team?

Dr. Jonathan Roberts, MD - Bleeding ...

Jonathan C Roberts, MD

Principal Investigator

Bleeding and Clotting Disorders Institute

Are You a Good Fit for This Trial?

This trial is for individuals with severe Von Willebrand Disease (VWD) or VWD combined with Hemophilia A. Participants must be at least 2 years old, willing to adhere to the study's protocol and use emicizumab prophylaxis. They should not have used emicizumab in the past 18 months unless switching from a different prophylaxis. Menstruating participants must agree to effective contraception or abstinence.

Inclusion Criteria

I need treatment to prevent bleeding as advised by my doctor.
I have been diagnosed with severe type 3 VWD or a variant confirmed by genetic testing.
Signed informed consent
See 6 more

Exclusion Criteria

I haven't taken antibiotics for a serious infection in the last 30 days.
I have been treated for blood clots in the past year or have current signs of them, except for catheter-related ones.
Patients who are at high risk for thrombotic microangiopathy (TMA; e.g., have a previous medical or family history of TMA), in the investigator's judgment
See 12 more

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Retrospective Chart Review

One-year retrospective chart review of annualized bleed rate and hemostatic therapies collected at the time of enrollment

1-2 weeks

Treatment

Participants receive emicizumab with a 3mg/kg weekly loading dose for 4 weeks, followed by once weekly prophylaxis of 1.5mg/kg for 1 year. Dose up-titration to 3 mg/kg once weekly allowed after 24 weeks if suboptimal efficacy observed.

12 months

Follow-up

Participants are monitored for safety and effectiveness after treatment, including collection of adverse events, hypersensitivity reactions, and thrombotic events.

6 months

What Are the Treatments Tested in This Trial?

Interventions

  • Emicizumab
Trial Overview The trial is testing Emicizumab, a monoclonal antibody designed as a preventive treatment for severe bleeding in patients with VWD and those who also have Hemophilia A. It aims to show that Emicizumab can safely replace intravenous treatments by being administered through subcutaneous injections.
How Is the Trial Designed?
1Treatment groups
Experimental Treatment
Group I: Open Label EmicizumabExperimental Treatment1 Intervention

Emicizumab is already approved in United States, European Union for the following indications:

🇺🇸
Approved in United States as Hemlibra for:
🇪🇺
Approved in European Union as Hemlibra for:

Find a Clinic Near You

Who Is Running the Clinical Trial?

Bleeding and Clotting Disorders Institute Peoria, Illinois

Lead Sponsor

Trials
1
Recruited
40+

Genentech, Inc.

Industry Sponsor

Trials
1,578
Recruited
569,000+
Ashley Magargee profile image

Ashley Magargee

Genentech, Inc.

Chief Executive Officer since 2024

MBA from Harvard University, BA from Princeton University

Levi Garraway profile image

Levi Garraway

Genentech, Inc.

Chief Medical Officer since 2021

MD, PhD

Published Research Related to This Trial

Emicizumab-kxwh (Hemlibra®) is a bispecific monoclonal antibody that effectively restores the function of missing activated FVIII in patients with hemophilia A, facilitating better blood clotting.
It is approved in the USA for routine prophylaxis to prevent bleeding episodes in hemophilia A patients with FVIII inhibitors, and is currently undergoing phase 3 trials for those without FVIII inhibitors, indicating its potential broad application in hemophilia treatment.
Emicizumab-kxwh: First Global Approval.Scott, LJ., Kim, ES.[2019]
In a study of 17 elderly patients with Haemophilia A, emicizumab was found to be well tolerated and significantly reduced the annualized bleeding rate, indicating its efficacy in this population.
No serious adverse events like thrombosis or thrombotic microangiopathy were reported, suggesting that emicizumab is a safe option for elderly patients, even those with cardiovascular risk factors.
Emicizumab prophylaxis in haemophilia patients older than 50 years with cardiovascular risk factors: Real-world data.Misgav, M., Brutman-Barazani, T., Budnik, I., et al.[2021]
In a phase 3 trial with 152 participants, emicizumab significantly reduced the annualized bleeding rate in hemophilia A patients without factor VIII inhibitors, showing a 96% reduction compared to no prophylaxis.
Emicizumab was well-tolerated, with the most common side effect being low-grade injection-site reactions, and it did not lead to serious complications like thrombotic events or the development of factor VIII inhibitors.
Emicizumab Prophylaxis in Patients Who Have Hemophilia A without Inhibitors.Mahlangu, J., Oldenburg, J., Paz-Priel, I., et al.[2022]

Citations

Anti-factor IXa/X bispecific antibody ACE910 prevents joint ...ACE910 is expected to prevent spontaneous bleeds and joint damage in hemophilia A patients even with weekly SC dosing, although appropriate clinical ...
The role of emicizumab, a bispecific factor IXaAnti-factor IXa/X bispecific antibody ACE910 prevents joint bleeds in a long-term primate model of acquired hemophilia A. Blood 2014; 124 ...
Emicizumab for Severe Von Willebrand Disease (VWD) ...Emicizumab (Hemlibra) is a monoclonal bispecific antibody developed to bind activated FIX and FX and mimic FVIII cofactor functionality. Hemlibra is ...
Emicizumab, a bispecific antibody recognizing coagulation ...Here, we will provide an overview of the similarities and dissimilarities between FVIII and emicizumab from a biochemical and mechanistical perspective.
Pharmacokinetics and Pharmacodynamics of Emicizumab in ...Here we assess pharmacokinetic (PK) and pharmacodynamic (PD) biomarkers in PwHA with FVIII inhibitors in the Phase III HAVEN 1 study (NCT02622321).
Emicizumab, a bispecific antibody recognizing coagulation ...Here, we will provide an overview of the similarities and dissimilarities between FVIII and emicizumab from a biochemical and mechanistical perspective.
7.pubmed.ncbi.nlm.nih.govpubmed.ncbi.nlm.nih.gov/24738137/
Anti-factor IXa/X bispecific antibody (ACE910)Results: A single bolus of 1 or 3 mg kg⁻¹ ACE910 showed hemostatic activity comparable to that of 10 U kg⁻¹ (twice daily) rpoFVIII against ongoing bleeds. The ...
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