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A Gene Transfer Study for Hemophilia A

Phase 1
Completed
Conditions
Hemophilia A
Interventions
Genetic: SPK-8011
Registration Number
NCT03003533
Lead Sponsor
Spark Therapeutics, Inc.
Brief Summary

This clinical research study is being conducted by Spark Therapeutics, Inc. to determine the safety and efficacy of the factor VIII gene transfer treatment with SPK-8011 in individuals with hemophilia A.

Detailed Description

Hemophilia A is a condition in which blood is unable to clot effectively. It is caused by a mutation or deletion in the gene that is responsible for producing blood-clotting factor VIII protein. Individuals with hemophilia A suffer from repeated bleeding episodes, often into the joints, which can cause chronic joint disease and sometime results in death due to the inability of the blood to clot efficiently. This chronic joint disease can have significant physical, psychosocial, and quality-of-life effects, including financial burden. The current standard of care includes the use of factor-based therapies which are given either as prophylaxis or to treat bleeding, as well as new non-factor prophylaxis therapies.

Recruitment & Eligibility

Status
COMPLETED
Sex
Male
Target Recruitment
25
Inclusion Criteria
  • Males age 18 years or older
  • Confirmed diagnosis of hemophilia A as evidenced by their medical history with baseline FVIII activity levels <=2%
  • Have received >150 exposure days (EDs) to FVIII concentrates or cryoprecipitate
  • Have no prior history of allergic reaction to any FVIII product
  • Have no measurable inhibitor against FVIII as assessed by the central laboratory and have no prior history of inhibitors to FVIII protein and no clinical signs or symptoms of decreased response to FVIII administration
  • Agree to use reliable barrier contraception
Exclusion Criteria
  • Evidence of active hepatitis B or C
  • Currently on antiviral therapy for hepatitis B or C
  • Have significant underlying liver disease
  • Have serological evidence* of HIV-1 or HIV-2 with CD4 counts ≤200/mm3 and who are on an antiretroviral drug regimen (* participants who are HIV+ and stable with CD4 count >200/mm3 and undetectable viral load are eligible to enroll)
  • Have detectable antibodies reactive with AAV-Spark200 capsid
  • Participated in a gene transfer trial within the last 52 weeks or in a clinical trial with an investigational product within the last 12 weeks

Study & Design

Study Type
INTERVENTIONAL
Study Design
SEQUENTIAL
Arm && Interventions
GroupInterventionDescription
SPK-8011SPK-8011All participants who meet the eligibility criteria will receive an outpatient single intravenous (i.v.) administration of SPK-8011.
Primary Outcome Measures
NameTimeMethod
Number of Participants With Treatment-emergent Adverse Events (TEAEs)From date of first dose to Week 52/End of Study (EOS) Visit

An adverse event (AE) was defined as any untoward medical occurrence in a participant who received study drug without regard to possibility of causal relationship. Serious adverse events (SAEs) were defined as adverse events that result in death, are life-threatening, require inpatient hospitalization or prolongation of existing hospitalization, result in persistent or significant disability or incapacity, are a congenital anomaly or birth defect, or are an important medical event that jeopardized participant and required medical intervention to prevent 1 of the outcomes listed in this definition. A TEAE is defined as an AE with an onset date on or following SPK-8011 administration. A summary of other non-serious AEs and all serious AEs, regardless of causality is located in Reported AE section.

Number of Participants Who Received Corticosteroids for Presumed Immune ResponseUp to Week 52/EOS Visit
Peak Factor VIII (FVIII) Activity Levels Assessed by One-Stage Coagulation Assay (OSA)Up to Week 52/EOS visit

Median peak FVIII activity up to Week 52

Nominal FVIII Level by OSA at Week 52/EOSUp to Week 52/EOS Visit

Steady-state FVIII activity measured by median FVIII levels at week 52 by OSA.

Spontaneous Bleeds Annualized Bleeding RateWeek 5 up to Week 52/EOS Visit
Total Annualized FVIII Infusion RateWeek 5 up to Week 52/EOS Visit
Secondary Outcome Measures
NameTimeMethod
Time to Achieve Peak FVIII Activity LevelUp to Week 52/EOS Visit
Number of Participants With Vector-shedding Confirmed Below Quantifiable Limits (BQL) of SPK-8011-101 in Bodily FluidsUp to Week 52/EOS Visit
Incidence of Immune Response to the BDD-hFVIII TransgeneUp to Week 52/EOS Visit

Trial Locations

Locations (16)

Jefferson University Hospitals

🇺🇸

Philadelphia, Pennsylvania, United States

University of California Davis - Hemostasis and Thrombosis Center

🇺🇸

Sacramento, California, United States

Chaim Sheba Center

🇮🇱

Ramat Gan, Tel Hashomer, Israel

Pennsylvania State University Milton S. Hershey Medical Center

🇺🇸

Hershey, Pennsylvania, United States

Oregon Health & Science University

🇺🇸

Portland, Oregon, United States

The Alfred Hospital

🇦🇺

Melbourne, Australia

Children's Hospital of Philadelphia

🇺🇸

Philadelphia, Pennsylvania, United States

Royal Prince Alfred Hosptial

🇦🇺

Camperdown, New South Wales, Australia

Hemophilia Center of Western Pennsylvania

🇺🇸

Pittsburgh, Pennsylvania, United States

Virginia Commonwealth University School of Medicine

🇺🇸

Richmond, Virginia, United States

Mahidol University - Ramathibody Hospital

🇹🇭

Bangkok, Thailand

McMaster University Medical Centre and Juravinski Hospital

🇨🇦

Hamilton, Ontario, Canada

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

University of Florida Health

🇺🇸

Gainesville, Florida, United States

Weill Cornell Medicine-Comprehensive Center for Hemophilia and Coagulation Disorders

🇺🇸

New York, New York, United States

Mississippi Center for Advanced Medicine

🇺🇸

Madison, Mississippi, United States

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