Treatment Protocol of Velaglucerase Alfa for Patients With Type 1 Gaucher Disease

NCT ID: NCT00954460

Last Updated: 2021-05-21

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

APPROVED_FOR_MARKETING

Study Classification

EXPANDED_ACCESS

Brief Summary

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Gaucher disease is a rare lysosomal storage disorder caused by the deficiency of the enzyme glucocerebrosidase (GCB). Due to the deficiency of functional GCB, glucocerebroside accumulates within macrophages leading to cellular engorgement, organomegaly, and organ system dysfunction. The purpose of this treatment protocol is to observe the safety of velaglucerase alfa in patients with type 1 Gaucher disease who are either treatment naive (newly diagnosed) or who are currently being treated with the Enzyme Replacement Therapy (ERT) imiglucerase.

Detailed Description

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Type 1 Gaucher disease, the most common form, accounts for more than 90% of all cases of Gaucher disease and does not involve the CNS. Typical manifestations of type 1 Gaucher disease include hepatomegaly, splenomegaly, thrombocytopenia, bleeding tendencies, anemia, hypermetabolism, skeletal pathology, growth retardation, pulmonary disease, and decreased quality of life. Velaglucerase alfa (Gene-Activated™ human glucocerebrosidase;GA-GCB) is produced in a continuous human cell line using proprietary gene-activation technology and has an identical amino acid sequence to the naturally occurring human enzyme. Velaglucerase alfa contains terminal mannose residues that target the enzyme to the macrophages-the primary target cells in Gaucher disease. This treatment protocol will observe the safety of velaglucerase alfa in patients with type 1 Gaucher disease who are either treatment naive (newly diagnosed) or who are currently being treated with the Enzyme Replacement Therapy (ERT) imiglucerase. Patients currently being treated with ERT for their Gaucher disease will receive the same number of units of velaglucerase alfa per month as their imiglucerase dose for doses between 30-120 U/kg/month. For patients who experienced dose reductions in their imiglucerase treatment due to supply constraints the pre-reduction monthly dose may be used to determine the monthly dose of velaglucerase alfa.

Conditions

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Gaucher Disease, Type 1

Interventions

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velaglucerase alfa

up to 60 U/kg, every other week via intravenous infusion

Intervention Type DRUG

Other Intervention Names

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VPRIV Gene activated human glucocerebrosidase GA-GCB

Eligibility Criteria

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Inclusion Criteria

1. The patient has a documented diagnosis of type 1 Gaucher disease
2. The patient is \> 2 years of age
3. The patient has NOT previously experienced an anaphylactic or anaphylactoid reaction to another ERT including imiglucerase
4. Women of child-bearing potential must agree to use a medically acceptable method of contraception at all times during the study; and must have a negative result to a pregnancy test as required throughout their participation in the study. Male patients must use a medically acceptable method of birth control throughout their participation in the study and must report their partner's pregnancy.
5. The patient is sufficiently cooperative to participate in this treatment plan as judged by the Investigator
6. If the patient is naïve or new to treatment, the patient has one or more of the following (in absence of the following criteria, please call the sponsor for treatment justification):

* Gaucher disease-related anemia
* Moderate splenomegaly (2 to 3 cm below the left costal margin), by palpation
* Gaucher disease-related thrombocytopenia
* Gaucher disease-related palpable enlarged liver
Minimum Eligible Age

3 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Shire

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Study Director

Role: STUDY_DIRECTOR

Takeda

Locations

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St Joseph's Hospital & Medical Center

Phoenix, Arizona, United States

Site Status

Tower Hematology Oncology

Beverly Hills, California, United States

Site Status

Rady's Children's Hospital of San Diego

La Jolla, California, United States

Site Status

Southern California Permanente Medical Group

Los Angeles, California, United States

Site Status

The Permanente Medical Group

Sacramento, California, United States

Site Status

Stanford University Medical Genetics

Stanford, California, United States

Site Status

Rocky Mountain Cancer Centers

Denver, Colorado, United States

Site Status

Yale University

New Haven, Connecticut, United States

Site Status

University Research Foundation for Lysosomal Storage Diseases

Coral Springs, Florida, United States

Site Status

Gainesville Hematology Oncology Associates

Gainesville, Florida, United States

Site Status

Adventis Healthcare System dba Florida Hospital

Orlando, Florida, United States

Site Status

East Lake Oncology

Palm Harbor, Florida, United States

Site Status

Emory Genetics

Decatur, Georgia, United States

Site Status

Children's Memorial Hospital

Chicago, Illinois, United States

Site Status

University of Iowa Hospitals and Clinics

Iowa City, Iowa, United States

Site Status

Annapolis Oncology Center

Annapolis, Maryland, United States

Site Status

Sinai Hospital of Baltimore

Baltimore, Maryland, United States

Site Status

University of Massachusetts

Shrewsbury, Massachusetts, United States

Site Status

Children's Hospitals and Clinics of Minnesota

Minneapolis, Minnesota, United States

Site Status

The University Research Foundation for Lysosomal Storage Diseases

Kansas City, Missouri, United States

Site Status

St. Joseph's

Paterson, New Jersey, United States

Site Status

Hemophilia Center of Western New York Incorporated

Buffalo, New York, United States

Site Status

North Shore Hematology/Oncology - Manhasset

Manhasset, New York, United States

Site Status

New York University School of Medicine

New York, New York, United States

Site Status

Mount Sinai School of Medicine

New York, New York, United States

Site Status

Fullerton Genetic

Asheville, North Carolina, United States

Site Status

Duke Medical Center

Durham, North Carolina, United States

Site Status

Akron Children's Hospital

Akron, Ohio, United States

Site Status

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

University of Virginia Health Systems

Charlottesville, Virginia, United States

Site Status

O & O Alpan, LLC

Springfield, Virginia, United States

Site Status

Countries

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United States

References

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Pastores GM, Rosenbloom B, Weinreb N, Goker-Alpan O, Grabowski G, Cohn GM, Zahrieh D. A multicenter open-label treatment protocol (HGT-GCB-058) of velaglucerase alfa enzyme replacement therapy in patients with Gaucher disease type 1: safety and tolerability. Genet Med. 2014 May;16(5):359-66. doi: 10.1038/gim.2013.154. Epub 2013 Nov 21.

Reference Type DERIVED
PMID: 24263462 (View on PubMed)

Other Identifiers

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HGT-GCB-058

Identifier Type: -

Identifier Source: org_study_id

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