Avalglucosidase alfa-ngpt

Avalglucosidase alfa-ngpt
Black Box Warning
Adult Indications & Dosage
Pediatric Indications & Dosage
Contraindications
Warnings & Precautions
Adverse Reactions
Drug Interactions
Use in Specific Populations
Administration & Monitoring
Overdosage
Pharmacology
Clinical Studies
How Supplied
Images
Patient Counseling Information
Precautions with Alcohol
Brand Names
Look-Alike Names

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Tejasvi Aryaputra

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Black Box Warning

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Overview

Avalglucosidase alfa-ngpt is a hydrolytic lysosomal glycogen-specific enzyme that is FDA approved for the treatment of late-onset Pompe disease. There is a Black Box Warning for this drug as shown here. Common adverse reactions include erythema, pruritus, urticaria, nausea, headache, dizziness, paresthesia, myalgia, arthralgia, diarrhea, dyspnea, and vomiting.

Adult Indications and Dosage

FDA-Labeled Indications and Dosage (Adult)

Recommended Dosage

• Avalglucosidase alfa-ngpt dosage is given through an intravenous infusion and is based on a patients body weight.
• Avalglucosidase alfa-ngpt should be both diluted and reconstituted before administering dosage into patients.
• Use of antipyretics, corticosteroids, or antihistamines may be used and considered before the administration of Avalglucosidase alfa-ngpt dosage into patients.
• The recommended dosage of patients ≥30 kg is 20 mg/kg every 2 weeks.
• The recommended dosage of patients <30 kg is 40 mg/kg every 2 weeks.
• 1 mg/kg/hour is the initial infusion rate that should be gradually increased every 30 minutes if there are no IARs seen in patients.

Off-Label Use and Dosage (Adult)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Avalglucosidase alfa-ngpt in adult patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Avalglucosidase alfa-ngpt in adult patients.

Pediatric Indications and Dosage

FDA-Labeled Indications and Dosage (Pediatric)

There is limited information regarding Avalglucosidase alfa-ngpt FDA-Labeled Indications and Dosage (Pediatric) in the drug label.

Off-Label Use and Dosage (Pediatric)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Avalglucosidase alfa-ngpt in pediatric patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Avalglucosidase alfa-ngpt in pediatric patients.

Contraindications

There are no contraindications associated with Avalglucosidase alfa-ngpt.

Warnings

TITLE See full prescribing information for complete Boxed Warning. Condition Name: (Content)

Hypersensitivity Reactions Including Anaphylaxis

• Cardiopulmonary resuscitation equipment and other medical support should be on the ready during Avalglucosidase alfa-ngpt dosage administration.
• Stop Avalglucosidase alfa-ngpt treatment and seek proper medical attention if patient experiences a severe hypersensitivity reaction.
• Advise patients to consider both the risks and benefits prior to restarting Avalglucosidase alfa-ngpt treatment.
• A desensitization procedure during Avalglucosidase alfa-ngpt may be considered in patients who experienced a severe hypersensitivity reaction.
• Monitor patients during infusion if restarting Avalglucosidase alfa-ngpt treatment.
• Patients may be returned to recommended dosage of Avalglucosidase alfa-ngpt if they can tolerate the infusion.
• Advise patients to either slow or temporary stop infusion rate if patient experiences a mild or moderate hypersensitivity reaction.
• 48% of patients treated with Avalglucosidase alfa-ngpt reported experiencing hypersensitivity reactions during clinical studies.
• 4% of patients treated with Avalglucosidase alfa-ngpt reported experiencing severe hypersensitivity reactions during clinical studies.
• 2% of patients treated with Avalglucosidase alfa-ngpt reported experiencing anaphylaxis during clinical studies.
• 1% of patients treated with Avalglucosidase alfa-ngpt reported experiencing anaphylaxis that led to discontinuation from the clinical study.
• Chest discomfort, dysphagia, lip swelling, respiratory distress, rash, swollen tongue, flushing, erythema, pruritus, and cough are symptoms of anaphylaxis in patients.
• Tongue edema, erythema, rash, respiratory distress, and urticaria are symptoms of severe hypersensitivity reactions in patients.
• Patients with higher antidrug antibody titers are more likely to experience hypersensitivity reactions when treated with Avalglucosidase alfa-ngpt.

Infusion-Associated Reactions

• Patients receiving pretreatment such as antipyretics, antihistamines, or corticosteroids may still experience infusion-associated reactions.
• Stop Avalglucosidase alfa-ngpt treatment and seek proper medical attention if patient experiences an infusion-associated reaction.
• Advise patients to consider both the risks and benefits prior to restarting Avalglucosidase alfa-ngpt treatment.
• Decrease or stop the infusion rate if patient is experiencing a mild or moderate infusion-associated reaction.
• 34% of patients treated with Avalglucosidase alfa-ngpt reported experiencing infusion-associated reaction during clinical studies.
• 4% of patients treated with Avalglucosidase alfa-ngpt reported experiencing 10 severe infusion-associated reaction during clinical studies.
• Dysphagia, tongue edema, increased blood pressure, respiratory distress, nausea, urticaria, erythema, and chest discomfort are some of the symptoms reported by patients experiencing a severe infusion-associated reaction.
• Cough, ocular hyperemia, dizziness, chest discomfort, flushing, respiratory distress, nausea, and erythema are the symptoms reported by patients with infusion-associated reactions that led to discontinuation of Avalglucosidase alfa-ngpt treatment.
• Patients with higher ADA titers were more likely to experience an infusion-associated reaction during Avalglucosidase alfa-ngpt treatment.
• Acute underlying illness in patients could pose a greater risk to experiencing an infusion-associated reaction during Avalglucosidase alfa-ngpt treatment.
• Severe complications of infusion-associated reactions increase in patients with advanced Pompe disease.

Risk of Acute Cardiorespiratory Failure in Susceptible Patients

• The risk of serious exacerbation of cardiac or respiratory status in patients may increase in patients with acute underlying respiratory illness, susceptible to fluid volume overload, and compromised cardiac or respiratory function.
• Monitor patients for serious exacerbation of cardiac or respiratory status.

Adverse Reactions

Clinical Trials Experience

Clinical Trial Experience

• Because clinical trials are conducted under widely varying conditions and durations of follow up, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice.

Adverse Reactions from Clinical Trials in the Pompe Disease Population

• 118 adult and 23 pediatric patients were treated with Avalglucosidase_alfa-ngpt in 4 clinical trials that tested the safety of NEXVIAZYME treatment.
• Chills, respiratory distress, and pyrexia were some of the adverse reactions reported in at least 2 patients treated with NEXVIAZYME.
• Discontinuation of NEXVIAZYME treatment due to adverse reactions occurred in 5 patients with 2 of them experiencing serious adverse reactions.
• Arthralgia, myalgia, dizziness, rash, cough, urticaria, dyspnea, hypertension, vomiting, pyrexia, abdominal pain, pruritus, erythema, abdominal pain upper, chills, headache, diarrhea, nausea, fatigue, and hypotension were the most common adverse reactions reported in the clinical trials.
• 34% of patients experienced infusion-associated reactions when treated with NEXVIAZYME in the clinical trials.
• Pruritus, rash, rash erythematous, tachycardia, urticaria, vomiting, chest discomfort, dizziness, hyperhidrosis, lip swelling, diarrhea, erythema, fatigue, headache, influenza-like illness, nausea, ocular hyperemia, pain in extremity, oxygen saturation decreased, pain, palmar erythema, swollen tongue, abdominal pain upper, burning sensation, eyelid edema, chills, cough, feeling cold, flushing, respiratory distress, throat irritation, and tremor are some of the infusion-associated reactions reported in more than 1 patient treated with NEXVIAZYME.

Adverse Reactions from Clinical Trials in Late-Onset Pompe Disease (LOPD)

• Study 1 looked into the effects of giving 20 mg/kg of NEXVIAZYME or 20 mg/kg of Alglucosidase alfa in 100 patients with LOPD.
• 49 patients were given 20 mg/kg of Alglucosidase alfa while 51 patients were given 20 mg/kg of NEXVIAZYME.
• Each dosage was given every other week for 49 weeks that would be followed by an open-label extension period.
• 6% of patients reported serious adverse reactions when treated with Alglucosidase alfa.
• 2% of patients reported serious adverse reactions when treated with NEXVIAZYME.
• Diarrhea, nausea, arthralgia, dizziness, myalgia, paresthesia, urticaria, headache, fatigue, pruritus, vomiting, dyspnea, and erythema were the most common adverse reactions reported in the study.
• 25% of patients reported mild or moderate infusion-associated reactions when treated with NEXVIAZYME.
• No patient treated with NEXVIAZYME reported severe infusion-associated reactions.
• Pruritus, rash, headache, urticaria, and diarrhea were the most common infusion-associated reactions in patients treated with NEXVIAZYME.
• 33% of patients reported infusion-associated reactions when treated with Alglucosidase alfa.
• Pruritis, rash, erythema, dizziness, flushing, dyspnea, chills, nausea, and feeling hot were the most common mild to severe infusion-associated reactions in patients treated with Alglucosidase alfa.

Table 2 summarizes the Adverse Reactions Reported in Study 1.

Insert Table 2

Immunogenicity

• 62% was the incidence of IAR in patients treated with NEXVIAZYME.
• Patients with higher ADA titer were more likely to experience hypersensitive reactions in comparison to patients with a low ADA titer.
• Patients who developed ADA were more likely to experience hypersensitive reactions and IAR compared to patients who are ADA negative.
• 8 weeks is the median time to seroconversion.

Table 3 Incidence of Anti-avalglucosidase alfa-ngpt Antibodies in Pompe Disease Patients.

Insert Table 3

Postmarketing Experience

There is limited information regarding Avalglucosidase alfa-ngpt Postmarketing Experience in the drug label.

Drug Interactions

There is limited information regarding Avalglucosidase alfa-ngpt Drug Interactions in the drug label.

Use in Specific Populations

Pregnancy

Pregnancy Category (FDA): There is no FDA guidance on usage of Avalglucosidase alfa-ngpt in women who are pregnant.
Pregnancy Category (AUS): There is no Australian Drug Evaluation Committee (ADEC) guidance on usage of Avalglucosidase alfa-ngpt in women who are pregnant.

Labor and Delivery

There is no FDA guidance on use of Avalglucosidase alfa-ngpt during labor and delivery.

Nursing Mothers

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt in women who are nursing.

Pediatric Use

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt in pediatric settings.

Geriatic Use

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt in geriatric settings.

Gender

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt with respect to specific gender populations.

Race

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt with respect to specific racial populations.

Renal Impairment

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt in patients with renal impairment.

Hepatic Impairment

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt in patients with hepatic impairment.

Females of Reproductive Potential and Males

There is no FDA guidance on the use of Avalglucosidase alfa-ngpt in women of reproductive potentials and males.

Immunocompromised Patients

There is no FDA guidance one the use of Avalglucosidase alfa-ngpt in patients who are immunocompromised.

Administration and Monitoring

Administration

There is limited information regarding Avalglucosidase alfa-ngpt Administration in the drug label.

Monitoring

There is limited information regarding Avalglucosidase alfa-ngpt Monitoring in the drug label.

IV Compatibility

There is limited information regarding the compatibility of Avalglucosidase alfa-ngpt and IV administrations.

Overdosage

There is limited information regarding Avalglucosidase alfa-ngpt 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 Avalglucosidase alfa-ngpt Pharmacology in the drug label.

Mechanism of Action

There is limited information regarding Avalglucosidase alfa-ngpt Mechanism of Action in the drug label.

Structure

There is limited information regarding Avalglucosidase alfa-ngpt Structure in the drug label.

Pharmacodynamics

There is limited information regarding Avalglucosidase alfa-ngpt Pharmacodynamics in the drug label.

Pharmacokinetics

There is limited information regarding Avalglucosidase alfa-ngpt Pharmacokinetics in the drug label.

Nonclinical Toxicology

There is limited information regarding Avalglucosidase alfa-ngpt Nonclinical Toxicology in the drug label.

Clinical Studies

There is limited information regarding Avalglucosidase alfa-ngpt Clinical Studies in the drug label.

How Supplied

There is limited information regarding Avalglucosidase alfa-ngpt How Supplied in the drug label.

Storage

There is limited information regarding Avalglucosidase alfa-ngpt 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 Avalglucosidase alfa-ngpt Patient Counseling Information in the drug label.

Precautions with Alcohol

Alcohol-Avalglucosidase alfa-ngpt interaction has not been established. Talk to your doctor about the effects of taking alcohol with this medication.

Brand Names

There is limited information regarding Avalglucosidase alfa-ngpt Brand Names in the drug label.

Look-Alike Drug Names

There is limited information regarding Avalglucosidase alfa-ngpt 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.