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Assess Safety and Efficacy of VAD044 in HHT Patients

Phase 1
Active, not recruiting
Conditions
Hereditary Hemorrhagic Telangiectasia (HHT)
Interventions
Drug: VAD044 Part I
Drug: VAD044 Part II
Registration Number
NCT05406362
Lead Sponsor
Vaderis Therapeutics AG
Brief Summary

Part I: The purpose of this Phase 1b proof of concept study, randomised, placebo controlled, double blind, multicentre study is to asssess safety and efficacy of 2 doses of VAD044 in adult HHT patients.

Part II: The purpose of this open-label extension following the completion of the randomised double blind treatment and follow-up period (Part I of the study) is to assess the long-term safetty, tolerability and efficacy of VAD044 in adult HHT patients.

Detailed Description

Part I: After being informed about the study and the potential risks, all patients giving written informed consent will undergo a two months screening and observation period to determine eligibility for study entry. At Day 0, patients who meet the eligibility requirements will be randomized in a double-blind manner (participant and investigator) in a 1:1:1 ratio to 30mg VAD044 (once daily), 40 mg (once daily) or placebo (once daily).

Part II: Patients who have completed the study Part I can participate in the open-label extension study (Part II).The patients can roll over immediately after last visit of the Part I or at any time at their convenience and according to their availability, but within a timeframe no longer than 8 months after the last visit (visit 12) of the part I. All patients in Part II will receive 30 mg of VAD044 once daily for the first 4 weeks afterwards the daily dose can be increased to 40 mg daily for up to 12 months.

Recruitment & Eligibility

Status
ACTIVE_NOT_RECRUITING
Sex
All
Target Recruitment
75
Inclusion Criteria
  • Diagnosis of HHT by the Curaçao criteria
  • Several epistaxis/week
  • Anaemia
  • COVID-19 vaccination or positive COVID-19 antibody test
  • Patient has given written informed consent to participate in Part I
Exclusion Criteria
  • Type 1 diabetes or uncontrolled type II diabetes (insulin or non-insulin dependent)
  • Active COVID-19 infection
  • active uncontrolled infection or known to be serologically positive for HIV, Hep B, Hep C infection
  • Recent procedures on nasal telangiectases (<6 weeks)
  • Requiring therapeutic anticoagulation
  • Use of drugs with anti-angiogenic properties in the past 8 weeks
  • laboratory abnormalities

Fort Part II of the study:

Inclusion Criteria:

  • Completion of Part I of the study
  • All adverse events or serious adverse events occuring during Part I of the study have resolved
  • Patient has given written informed consent to participate in Part II

Exclusion Criteria:

  • Same as in Part I.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
30 mgVAD044 Part I30 mg VAD044
30 mgVAD044 Part II30 mg VAD044
40 mgVAD044 Part I40 mg VAD044
40 mgVAD044 Part II40 mg VAD044
PlaceboVAD044 Part IPlacebo
Primary Outcome Measures
NameTimeMethod
Part I: Safety and Tolerability12 weeks

Type and severity of Adverse Events (AEs)

Part II: Safety and Tolerability12 months

Type and severity of Adverse Events (AEs)

Secondary Outcome Measures
NameTimeMethod
Part I: Change in Epistaxis episodes12 weeks

The number of Epistaxis episodes

Part I: Change in Epistaxis duration12 weeks

Epistaxis duration

Part I: Change in Epistaxis intensity12 weeks

Epistaxis flow intensity

Part I: Change in Epistaxis Severity Score (ESS)12 weeks

ESS used to evaluate the current severity of HHT patient nosebleeds (typically in the last three months) and can help health care providers to evaluate how a patient is responding to treatment. This score ranges from 0-10 and is automatically calculated after answering six simple questions. The higher the score the more severe.

Part I: Change in Haemoglobin12 weeks

Haemoglobin

Part I: Change Ferritin12 weeks

Ferritin

Part I: Change in blood Transferrin saturation level12 weeks

Transferrin saturation level

Part I: Change in Iron supplementation needs12 weeks

Iron supplementation needs

Part I: Change Blood tranfusions requirements12 weeks

Blood tranfusions requirements

Part I: Change in the Nasal Outcome for Epistaxis in Hereditary Hemorrhagic Telangiectasia score12 weeks

The Nasal Outcome for Epistaxis in Hereditary Hemorrhagic Telangiectasia (NOSE HHT questionnaire) wil be used to measure physical, social and emotional impacts of epistaxis. It is a 29-items questionnaire using a Likert scale. A higher score indicates a worse outcome

Part I: Quality of Life Scale SF-1212 weeks

The SF-12 Quality of Life Scale is a scale to evaluate quality of life using 12 questions. In the SF-12, physical (SF12-PCS) and mental (SF12-MCS) component summary scores are calculated as sub-dimensions. The total score of the physical and mental component summary of the scale varies between 0-100. An increase in the score indicates well-being, and a decrease indicates a state of disability.

Part I: Plasma concentration of VAD04412 weeks

Plasma concentration of VAD044

Part I: Maximum concentration (Cmax) of VAD0412 weeks

Maximum concentration (Cmax) of VAD044

Part I: Time of maximum concentration (Tmax) of VAD04412 weeks

Time of maximum concentration (Tmax) of VAD044

Part I: Area under the curve (exposure to drug) during 24 hours (AUC0-24h) of VAD04412 weeks

Area under the curve (exposure to drug) during 24 hours (AUC0-24h) of VAD044

Part I: Trough concentration (Ctrough) of VAD04412 weeks

Trough concentration (Ctrough) of VAD044

Part I: Pharmacodynamics (PD) of VAD04412 weeks

PRP assay to measure the levels of pAKT in the blood in a subset of patients

Part II: Change in The number of Epistaxis episodes12 months

The number of Epistaxis episodes

Part II: Change in Epistaxis duration12 months

Epistaxis duration

Part II: Change in Epistaxis flow intensity12 months

Epistaxis flow intensity

Part II: Change in Epistaxis Severity Score (ESS)12 months

ESS used to evaluate the current severity of HHT patient nosebleeds (typically in the last three months) and can help health care providers to evaluate how a patient is responding to treatment. This score ranges from 0-10 and is automatically calculated after answering six simple questions. The higher the score the more severe.

Part II: Change in Haemoglobin12 months

Haemoglobin

Part II: Change in Ferritin12 months

Ferritin

Part II: Change in Transferrin saturation level12 months

Transferrin saturation level

Part II: Change in Iron supplementation needs12 months

Iron supplementation needs

Part II: Change in blood tranfusions requirements12 months

blood tranfusions requirements

Part II: Quality of Life Scale SF-1212 months

The SF-12 Quality of Life Scale is a scale to evaluate quality of life using 12 questions. In the SF-12, physical (SF12-PCS) and mental (SF12-MCS) component summary scores are calculated as sub-dimensions. The total score of the physical and mental component summary of the scale varies between 0-100. An increase in the score indicates well-being, and a decrease indicates a state of disability.

Trial Locations

Locations (7)

Massachusetts General Hospital

🇺🇸

Boston, Massachusetts, United States

Universitair Ziekenhuis Gent

🇧🇪

Gent, Belgium

Hospices Civils de Lyon

🇫🇷

Lyon, France

Ospedale Maggiore di Crema

🇮🇹

Crema, Italy

St. Antonius Hospital

🇳🇱

Nieuwegein, Netherlands

Hospital Universiati De Bellvitge

🇪🇸

Barcelona, Spain

Hospital Universitario Ramón y Cajal

🇪🇸

Madrid, Spain

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