Drug Guide
Avalglucosidase Alfa-ngpt
Classification
Therapeutic: Enzyme Replacement Therapy
Pharmacological: Hydrolase enzyme
FDA Approved Indications
- Pompe disease in pediatric and adult patients
Mechanism of Action
Avalglucosidase alfa is a recombinant human GAA (acid alpha-glucosidase) enzyme that replaces the deficient enzyme in patients with Pompe disease, helping to break down glycogen accumulated in tissues.
Dosage and Administration
Adult: The recommended dosage varies; clinical approval typically involves weight-based dosing administered via intravenous infusion every two weeks.
Pediatric: Dosing in pediatric patients is typically weight-based and administered every two weeks, following specific guidelines per FDA approval.
Geriatric: Dosing adjustments are generally based on clinical response and tolerability; no specific age-related dose recommended.
Renal Impairment: No specific adjustments are generally necessary; monitor renal function.
Hepatic Impairment: No specific adjustments required; monitor liver function as appropriate.
Pharmacokinetics
Absorption: Administered intravenously; absorption not applicable.
Distribution: Widely distributed in body tissues, with a large volume of distribution.
Metabolism: Metabolized by cellular pathways, typical for recombinant enzymes.
Excretion: Excreted via lysosomal degradation pathways.
Half Life: Approximately 30 to 40 hours, depending on the patient.
Contraindications
- Hypersensitivity to avalglucosidase alfa or any component of the formulation.
Precautions
- Monitor for infusion-associated reactions, allergic reactions, and hypersensitivity. Use caution in patients with a history of allergies.
Adverse Reactions - Common
- Infusion site reactions (Common)
- Headache, fever, back pain (Common)
- Nausea, vomiting (Common)
Adverse Reactions - Serious
- Anaphylaxis (Rare)
- Severe allergic reactions or hypersensitivity (Rare)
Drug-Drug Interactions
- Concurrent use of other immunomodulatory drugs may increase risk of hypersensitivity.
Drug-Food Interactions
- No specific interactions noted.
Drug-Herb Interactions
- No known interactions.
Nursing Implications
Assessment: Monitor for infusion reactions, hypersensitivity, and respiratory status during and after infusion.
Diagnoses:
- Risk for allergic reaction related to hypersensitivity.
- Impaired tissue perfusion related to adverse reactions.
Implementation: Administer via certified infusion protocols, premedicate as necessary, and monitor vital signs closely during infusion.
Evaluation: Assess patient response, monitor for adverse reactions, and adjust therapy accordingly.
Patient/Family Teaching
- Report any signs of allergic reactions such as rash, itching, swelling, or difficulty breathing.
- Take the medication exactly as prescribed, and attend all scheduled infusions.
- Notify healthcare provider of any new or unusual symptoms.
Special Considerations
Black Box Warnings:
- Potential for severe allergic (hypersensitivity) reactions.
- Monitor closely during infusions.
Genetic Factors: Patients with GAA gene mutations may exhibit variable responses.
Lab Test Interference: May interfere with assays measuring GAA activity.
Overdose Management
Signs/Symptoms: Signs of overdose may include allergic reactions, hypersensitivity, or infusion reactions.
Treatment: Discontinue infusion, provide supportive care, administer antihistamines or epinephrine as indicated, and provide emergency interventions if needed.
Storage and Handling
Storage: Store vials refrigerated at 2-8°C; do not freeze.
Stability: Stable until expiration date when stored properly. After reconstitution, use immediately or store refrigerated and use within specified time.