Elfabrio
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rithish Nimmagadda,MBBS.[2]
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Black Box Warning
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HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS
See full prescribing information for complete Boxed Warning.
Patients treated with pegunigalsidase alfa-iwxj have experienced hypersensitivity reactions, including anaphylaxis.
Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available. If a severe hypersensitivity reaction occurs, discontinue pegunigalsidase alfa-iwxj immediately and initiate appropriate medical treatment. In patients with severe hypersensitivity reaction, a desensitization procedure to pegunigalsidase alfa-iwxj may be considered |
Overview
Elfabrio is {{{aOrAn}}} a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme, is a pegylated recombinant form of human α-galactosidase A that is FDA approved for the treatment of ELFABRIO is an FDA approved drug that is used for treatment of adults with confirmed Fabry disease.. There is a Black Box Warning for this drug as shown here. Common adverse reactions include Most common adverse reactions (≥15%) are infusion-associated reactions, nasopharyngitis, headache, diarrhea, fatigue, nausea, back pain, pain in extremity, and sinusitis..
Adult Indications and Dosage
FDA-Labeled Indications and Dosage (Adult)
-Recommended dosage is 1 mg/kg every 2 weeks administered as an IV infusion.
-Pegunigalsidase alfa-iwxj is available in the following dosage form(s) and strength(s): Injection: 20 mg/10 mL (2 mg/mL) solution concentrate in a single-dose vial for dilution and IV infusion.
Off-Label Use and Dosage (Adult)
Pediatric Indications and Dosage
FDA-Labeled Indications and Dosage (Pediatric)
There is limited information regarding Elfabrio FDA-Labeled Indications and Dosage (Pediatric) in the drug label.
Off-Label Use and Dosage (Pediatric)
Contraindications
None
Warnings
|
HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS
See full prescribing information for complete Boxed Warning.
Patients treated with pegunigalsidase alfa-iwxj have experienced hypersensitivity reactions, including anaphylaxis.
Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available. If a severe hypersensitivity reaction occurs, discontinue pegunigalsidase alfa-iwxj immediately and initiate appropriate medical treatment. In patients with severe hypersensitivity reaction, a desensitization procedure to pegunigalsidase alfa-iwxj may be considered |
Hypersensitivity Reactions
-Hypersensitivity reactions, including anaphylaxis, have been reported in pegunigalsidase alfa-treated patients. In clinical trials, 14% of patients who received pegunigalsidase alfa-iwxj experienced hypersensitivity reactions. In these trials, 4 patients treated with the drug (3%; 1 naive to enzyme replacement therapy (ERT) and 3 ERT-experienced patients) experienced anaphylaxis during the initial infusion and were positive for anti-pegunigalsidase alfa-iwxj IgE antibodies (referred to as IgE ADA). The risk of pegunigalsidase alfa-related hypersensitivity may be increased in certain patients with pre-existing ADA from prior ERT.
-Anaphylaxis (reported as Type I hypersensitivity reaction, hypersensitivity reaction, or bronchospasm) occurred within 5 to 40 minutes of the start of the initial infusion. Signs and symptoms included headache, nausea, vomiting, throat tightness, facial and oral edema, truncal rash, tachycardia, hypotension, rigors, urticaria, intense pruritus, moderate upper airway obstructions, macroglossia, and mild lip edema.
-Membranoproliferative Glomerulonephritis
A case of membranoproliferative glomerulonephritis with immune depositions in the kidney was reported during clinical trials. This event led to a decline in renal function that slowly improved upon discontinuation of pegunigalsidase alfa-iwxj but did not return to baseline by the end of the trial.
Adverse Reactions
Clinical Trials Experience
Most common adverse reactions (≥15%) are infusion-associated reactions, nasopharyngitis, headache, diarrhea, fatigue, nausea, back pain, pain in extremity, and sinusitis.
Postmarketing Experience
There is limited information regarding Elfabrio Postmarketing Experience in the drug label.
Drug Interactions
There is limited information regarding Elfabrio Drug Interactions in the drug label.
Use in Specific Populations
Pregnancy
Pregnancy Category (FDA):
There is no FDA guidance on usage of Elfabrio in women who are pregnant.
Pregnancy Category (AUS):
There is no Australian Drug Evaluation Committee (ADEC) guidance on usage of Elfabrio in women who are pregnant.
Labor and Delivery
There is no FDA guidance on use of Elfabrio during labor and delivery.
Nursing Mothers
There is no FDA guidance on the use of Elfabrio in women who are nursing.
Pediatric Use
There is no FDA guidance on the use of Elfabrio in pediatric settings.
Geriatic Use
There is no FDA guidance on the use of Elfabrio in geriatric settings.
Gender
There is no FDA guidance on the use of Elfabrio with respect to specific gender populations.
Race
There is no FDA guidance on the use of Elfabrio with respect to specific racial populations.
Renal Impairment
There is no FDA guidance on the use of Elfabrio in patients with renal impairment.
Hepatic Impairment
There is no FDA guidance on the use of Elfabrio in patients with hepatic impairment.
Females of Reproductive Potential and Males
There is no FDA guidance on the use of Elfabrio in women of reproductive potentials and males.
Immunocompromised Patients
There is no FDA guidance one the use of Elfabrio in patients who are immunocompromised.
Administration and Monitoring
Administration
egunigalsidase alfa-iwxj is available in the following dosage form(s) and strength(s):
Injection: 20 mg/10 mL (2 mg/mL) solution concentrate in a single-dose vial for dilution and IV infusion.
Monitoring
There is limited information regarding Elfabrio Monitoring in the drug label.
IV Compatibility
There is limited information regarding the compatibility of Elfabrio and IV administrations.
Overdosage
There is limited information regarding Elfabrio overdosage. If you suspect drug poisoning or overdose, please contact the National Poison Help hotline (1-800-222-1222) immediately.
Pharmacology
There is limited information regarding Elfabrio Pharmacology in the drug label.
Mechanism of Action
There is limited information regarding Elfabrio Mechanism of Action in the drug label.
Structure
There is limited information regarding Elfabrio Structure in the drug label.
Pharmacodynamics
There is limited information regarding Elfabrio Pharmacodynamics in the drug label.
Pharmacokinetics
There is limited information regarding Elfabrio Pharmacokinetics in the drug label.
Nonclinical Toxicology
There is limited information regarding Elfabrio Nonclinical Toxicology in the drug label.
Clinical Studies
There is limited information regarding Elfabrio Clinical Studies in the drug label.
How Supplied
There is limited information regarding Elfabrio How Supplied in the drug label.
Storage
There is limited information regarding Elfabrio Storage in the drug label.
Images
Drug Images
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Package and Label Display Panel
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Patient Counseling Information
There is limited information regarding Elfabrio Patient Counseling Information in the drug label.
Precautions with Alcohol
Alcohol-Elfabrio interaction has not been established. Talk to your doctor regarding the effects of taking alcohol with this medication.
Brand Names
Elfabrio®
Look-Alike Drug Names
There is limited information regarding Elfabrio Look-Alike Drug Names in the drug label.
Drug Shortage Status
Price
References
The contents of this FDA label are provided by the National Library of Medicine.