A Study of the Safety and Efficacy of Fabrazyme (Agalsidase Beta) as Compared to Placebo in Patients With Advanced Fabry Disease

NCT ID: NCT00074984

Last Updated: 2013-12-27

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE4

Total Enrollment

82 participants

Study Classification

INTERVENTIONAL

Study Start Date

2001-02-28

Study Completion Date

2004-01-31

Brief Summary

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People with Fabry disease have an alteration in their genetic material (DNA) which causes a deficiency of the a-galactosidase A enzyme. Fabrazyme (agalsidase beta) is a drug that helps to breakdown and remove certain types of fatty substances called "glycolipids." These glycolipids are normally present within the body in most cells. In Fabry disease, glycolipids build up in various tissues such as the liver, kidney, skin, and blood vessels because a-galactosidase A is not present, or is present in small quantities. The build up of glycolipid ("globotriaosylceramide" or "GL-3") levels in these tissues in particular is thought to cause the clinical symptoms that are common to Fabry disease. This study will test the safety and efficacy of Fabrazyme in the treatment of patients with Fabry disease.

Detailed Description

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Conditions

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Fabry Disease

Keywords

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a-Galactosidase A aGAL r-haGAL Fabry GL-3 Fabrazyme

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Placebo

Patients randomized to placebo

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type BIOLOGICAL

1 mg/kg placebo intravenously every 2 weeks

Fabrazyme (agalsidase beta)

Patients randomized to Fabrazyme (agalsidase beta).

Group Type ACTIVE_COMPARATOR

Fabrazyme (agalsidase beta)

Intervention Type BIOLOGICAL

1mg/kg Fabrazyme (agalsidase beta) every 2 weeks

Interventions

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Fabrazyme (agalsidase beta)

1mg/kg Fabrazyme (agalsidase beta) every 2 weeks

Intervention Type BIOLOGICAL

Placebo

1 mg/kg placebo intravenously every 2 weeks

Intervention Type BIOLOGICAL

Other Intervention Names

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Fabrazyme

Eligibility Criteria

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Inclusion Criteria

* Patients must provide written informed consent
* Patients must be at least 16 years old
* Patients must have a current diagnosis of Fabry disease and have a clinical presentation consistent of Fabry disease (decreased sweating, Fabry pain, angiokeratoma, etc.)
* Patients may not have received enzyme replacement therapy as a treatment for Fabry disease
* Patients must have a documented plasma a-galactosidase A (aGAL) activity of \< 1.5 nmol/hr/mL or a documented leukocyte aGAL activity of \< 4 nmol/hr/mg
* Patients must have one or more of the following: a serum creatinine measurement of 1.2 to 3 mg/dL (106.1 to 265 umol/L) OR estimated creatinine clearance \< 80 mL/min only if the patient's serum creatinine measurement is \< 1.2 mg/dL
* Female patients of childbearing potential must have a negative pregnancy test prior to each dosing and all female patients must use a medically accepted form of contraception

Exclusion Criteria

* Patient has undergone or is currently scheduled for kidney transplantation or is currently on dialysis
* Patient has acute renal failure
* Patient has participated in a study employing an investigational drug within 30 days of study entry
* Patient has diabetes mellitus or presence of confounding renal disease
* Patient has a history of transient ischemic attack (TIA) or ischemic stroke within 3 months of study entry documented by mild-to-moderate neurological deficit
* Patient has critical coronary disease
* Patient has congestive heart failure
* Patient has severe residual neurological deficit that will confound the detection of new events as determined by an attending neurologist and/or Principal Investigator
* Patient is unwilling to comply with the requirements of the protocol or the patient has a medical condition, serious intercurrent illness, or extenuating circumstances that would significantly decrease study compliance, including prescribed follow-up
Minimum Eligible Age

16 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Genzyme, a Sanofi Company

INDUSTRY

Sponsor Role lead

Responsible Party

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Genzyme Coporation

Principal Investigators

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Medical Monitor

Role: STUDY_DIRECTOR

Genzyme Coorporation

Locations

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University of Alabama at Birmingham

Birmingham, Alabama, United States

Site Status

Cedars-Sinai Medical Center

Los Angeles, California, United States

Site Status

University of San Francisco

San Francisco, California, United States

Site Status

University of Connecticut Health Partners

Farmington, Connecticut, United States

Site Status

Oncology Hematology Association

Coral Springs, Florida, United States

Site Status

Emory University School of Medicine

Atlanta, Georgia, United States

Site Status

Children's Memorial Hospital

Chicago, Illinois, United States

Site Status

University of Kansas Medical Center

Kansas City, Kansas, United States

Site Status

Massachusetts General Hospital

Boston, Massachusetts, United States

Site Status

Gene Therapy Center - Dept. of Pediatrics and Institute of Human Genetics

Minneapolis, Minnesota, United States

Site Status

Children's Hospital

Buffalo, New York, United States

Site Status

Mount Sinai School of Medicine

New York, New York, United States

Site Status

University of Rochester School of Medicine

Rochester, New York, United States

Site Status

Duke University Medical Center

Durham, North Carolina, United States

Site Status

Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

University of Pittsburgh

Pittsburgh, Pennsylvania, United States

Site Status

Baylor College of Medicine

Houston, Texas, United States

Site Status

University of Washington School of Medicine

Seattle, Washington, United States

Site Status

Queen Elizabeth II Health Center

Halifax, Nova Scotia, Canada

Site Status

North York General Hospital

Toronto, Ontario, Canada

Site Status

Hopital du Sacre-Coeur de Montreal

Montreal, Quebec, Canada

Site Status

University Hospital

Prague, , Czechia

Site Status

Sopron Megyei Jogu Varos Erzsebet Korhaz

Sopron, , Hungary

Site Status

Klinika Chorob Metabolicznych Instytut

Warsaw, , Poland

Site Status

Hope Hospital

Manchester, , United Kingdom

Site Status

Countries

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United States Canada Czechia Hungary Poland United Kingdom

References

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Banikazemi M, Bultas J, Waldek S, Wilcox WR, Whitley CB, McDonald M, Finkel R, Packman S, Bichet DG, Warnock DG, Desnick RJ; Fabry Disease Clinical Trial Study Group. Agalsidase-beta therapy for advanced Fabry disease: a randomized trial. Ann Intern Med. 2007 Jan 16;146(2):77-86. doi: 10.7326/0003-4819-146-2-200701160-00148. Epub 2006 Dec 18.

Reference Type DERIVED
PMID: 17179052 (View on PubMed)

Other Identifiers

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AGAL-008-00

Identifier Type: -

Identifier Source: org_study_id