Pegunigalsidase alfa-iwxj
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alen Antony Pathil, M.D.[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 may experience hypersensitivity reaction including anaphylaxis during the treatment course with ELFABRIO. Therefore, a cardiopulmonary resuscitation kit should always available while administering the treatment.
Patients who develop hypersensitivity reaction symptoms should immediately discontinue the treatment of ELFABRIO. Furthermore, they should undergo desensitization procedure to ELFABRIO.
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Overview
Pegunigalsidase alfa-iwxj is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme that is FDA approved for the treatment of Fabry's disease in adults. There is a Black Box Warning for this drug as shown here. Common adverse reactions include 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)
ELFABRIO is used to treat fabrys disease in adults.
These patients who are treated with ELFABRIO may require pre-treatment with antihistamines, antipyretics, and/or corticosteroids prior to ERT administration. They may require these pre-treatment for 4-6 cycles of ELFABRIO infusion, after which it is step-wise declined or discontinued if ELFABRIO is tolerated.
The recommended dosage of ELFABRIO is based on actual body weight which is 1 mg/kg administered by intravenous infusion every 2 weeks.
The initial recommended ELFABRIO infusion rates for ERT (enzyme replacement therapy)-experienced or ERT-naïve patients are based on actual body weight:
- ˂ 70 kg - total infusion rate=150 mL - infusion volume=0.83 mL/min (50 mL/h)
- 70 -100 kg - total infusion rate=250 mL - infusion volume=1.39 mL/min (83 mL/h)
- > 100 kg - total infusion rate=500 mL - infusion volume=2.78 mL/min (167 mL/h)
The Infusion rate may be increased if the patient tolerates the initial 4-6 infusions and may be slowed in case of a hypersensitivity reaction or an IAR (infusion associated reaction).
Appropriate medical support measures including cardiopulmonary resuscitation equipment should be readily available during ELFABRIO administration.
Off-Label Use and Dosage (Adult)
Pediatric Indications and Dosage
FDA-Labeled Indications and Dosage (Pediatric)
There is limited information regarding Pegunigalsidase alfa-iwxj FDA-Labeled Indications and Dosage (Pediatric) in the drug label.
Off-Label Use and Dosage (Pediatric)
Contraindications
There is limited information regarding Pegunigalsidase alfa-iwxj Contraindications in the drug label.
Warnings
|
HYPERSENSITIVITY REACTIONS including Anaphylaxis
See full prescribing information for complete Boxed Warning.
Patients may experience hypersensitivity reaction including anaphylaxis during the treatment course with ELFABRIO. Therefore, a cardiopulmonary resuscitation kit should always available while administering the treatment.
Patients who develop hypersensitivity reaction symptoms should immediately discontinue the treatment of ELFABRIO. Furthermore, they should undergo desensitization procedure to ELFABRIO.
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Hypersensitivity Reactions Including Anaphylaxis In clinical trials, 20 (14%) of ELFABRIO-treated patients experienced hypersensitivity reactions. the reactions included Type I hypersensitivity reaction, hypersensitivity reaction, or bronchospasm which occurred within 5 to 40 minutes of the start of the initial infusion. During thesereaction patients presented with 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. And these symptoms were treated with antihistamines, epinephrine and systemic corticosteroids. There it is essential patients should receive pretreatment with antihistamines, antipyretics, and/or corticosteroids prior to ELFABRIO administration.
- If the patient has severe hypersensitivity reaction like anaphylaxis, immediately discontinue ELFABRIO and initiate appropriate medical treatment.
- If a mild or moderate hypersensitivity reaction occurs, consider temporarily holding the infusion or slowing the infusion rate.
Furthermore, consider monitoring the presence of IgG and IgE anti-drug antibodies (ADA) in patients who demonstrate hypersensitivity reactions during ELFABRIO treatment.
Infusion-Associated Reactions (IAR) In clinical trials, 41 (29%) of ELFABRIO-treated patients experienced one or more IARs, defined as any adverse reaction with onset after start of the infusion and up to 24 hours after the end of infusion. Besides the hypersenstivity reactions, other IAR symptoms include nausea, chills, pruritus, rash, chest pain, dizziness, vomiting, asthenia, pain, sneezing, dyspnea, nasal congestion, throat irritation, abdominal pain, erythema, diarrhea, burning sensation, neuralgia, headache, paresthesia, tremor, agitation, increased body temperature, flushing, bradycardia, myalgia, hypertension, and hypotension.
Membranoproliferative Glomerulonephritis Immune deposits in the glomerulus, indicating membraneous glomerulonephritis was observed in the clinical trials. The eventually leading to decline in renal function which slowly improved upon discontinuation of ELFABRIO. Thereofore it is essential to monitor serum creatinine and urinary protein to creatinine ratio.
Adverse Reactions
Clinical Trials Experience
There is limited information regarding Pegunigalsidase alfa-iwxj Clinical Trials Experience in the drug label.
Postmarketing Experience
There is limited information regarding Pegunigalsidase alfa-iwxj Postmarketing Experience in the drug label.
Drug Interactions
There is limited information regarding Pegunigalsidase alfa-iwxj Drug Interactions in the drug label.
Use in Specific Populations
Pregnancy
Pregnancy Category (FDA): B
There is no available data on ELFABRIO use in pregnant females to evaluate a drug-associated risk of major birth defects, miscarriage or other adverse maternal or fetal outcomes; however, as an enzyme replacement, ELFABRIO is not expected to cause adverse outcomes. Animal reproduction studies have been conducted with pegunigalsidase alfa-iwxj in pregnant rats and rabbits and no adverse effects on embryofetal development were observed in pregnant rats.
Pregnancy Category (AUS):
There is no Australian Drug Evaluation Committee (ADEC) guidance on usage of Pegunigalsidase alfa-iwxj in women who are pregnant.
Labor and Delivery
There is no FDA guidance on use of Pegunigalsidase alfa-iwxj during labor and delivery.
Nursing Mothers
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj in women who are nursing.
Pediatric Use
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj in pediatric settings.
Geriatic Use
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj in geriatric settings.
Gender
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj with respect to specific gender populations.
Race
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj with respect to specific racial populations.
Renal Impairment
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj in patients with renal impairment.
Hepatic Impairment
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj in patients with hepatic impairment.
Females of Reproductive Potential and Males
There is no FDA guidance on the use of Pegunigalsidase alfa-iwxj in women of reproductive potentials and males.
Immunocompromised Patients
There is no FDA guidance one the use of Pegunigalsidase alfa-iwxj in patients who are immunocompromised.
Administration and Monitoring
Administration
There is limited information regarding Pegunigalsidase alfa-iwxj Administration in the drug label.
Monitoring
There is limited information regarding Pegunigalsidase alfa-iwxj Monitoring in the drug label.
IV Compatibility
There is limited information regarding the compatibility of Pegunigalsidase alfa-iwxj and IV administrations.
Overdosage
There is limited information regarding Pegunigalsidase alfa-iwxj 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 Pegunigalsidase alfa-iwxj Pharmacology in the drug label.
Mechanism of Action
ELFABRIO provides an exogenous source of alpha-galactosidase A, which is an enzyme deficient in Fabry's disease. This ELFABRIO is internalized and transported into lysosomes where it is thought to exert enzymatic activity and reduce accumulated globotriaosylceramide (Gb3).
Structure
There is limited information regarding Pegunigalsidase alfa-iwxj Structure in the drug label.
Pharmacodynamics
There is limited information regarding Pegunigalsidase alfa-iwxj Pharmacodynamics in the drug label.
Pharmacokinetics
There is limited information regarding Pegunigalsidase alfa-iwxj Pharmacokinetics in the drug label.
Nonclinical Toxicology
There is limited information regarding Pegunigalsidase alfa-iwxj Nonclinical Toxicology in the drug label.
Clinical Studies
There is limited information regarding Pegunigalsidase alfa-iwxj Clinical Studies in the drug label.
How Supplied
ELFABRIO (pegunigalsidase alfa-iwxj) injection is a sterile, preservative-free, clear and colorless solution supplied in a single-dose vial. Each vial contains 20 mg/10 mL (2 mg/mL) of pegunigalsidase alfa-iwxj. ELFABRIO is available as:
- One single-dose vial in a carton
- Five single-dose vials in a carton
- Ten single-dose vials in a carton
Storage
It is stored refrigerated at 2°C to 8°C (36°F to 46°F). Do not freeze. Do not shake.
Images
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Patient Counseling Information
There is limited information regarding Pegunigalsidase alfa-iwxj Patient Counseling Information in the drug label.
Precautions with Alcohol
Alcohol-Pegunigalsidase alfa-iwxj 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 Pegunigalsidase alfa-iwxj 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.