Share on Facebook
Share on Twitter
Share on LinkedIn
Share by Email
Print
Opfolda Generic Name & Formulations
Legal Class
General Description
Pharmacological Class
How Supplied
Caps—4, 24, 100
Manufacturer
Generic Availability
NO
Mechanism of Action
Miglustat binds with, stabilizes, and reduces inactivation of cipaglucosidase alfa-atga in the blood after infusion. The bound miglustat is dissociated from cipaglucosidase alfa-atga after it is internalized and transported into lysosomes. Miglustat alone has no pharmacological activity in cleaving glycogen.
Opfolda Indications
Indications
In combination with Pombiliti, for adults with late-onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency) weighing ≥40kg and who are not improving on their current enzyme replacement therapy (ERT).
Opfolda Dosage and Administration
Adult
Start in combination with Pombiliti 2 weeks after the last ERT dose. Take caps ~1hr prior to Pombiliti IV administration. Swallow whole only with unsweetened beverages (eg, water, tea/coffee without cream, sugar, or sweeteners). Do not consume other beverages or food for at least 2hrs before and 2hrs after. 40–<50kg: 195mg every other week; ≥50kg: 260mg every other week. Moderate (CrCl 30–59mL/min) or severe (CrCl 15–29mL/min) renal impairment: (40–<50kg): 130mg; (≥50kg): 195mg.
Children
Not established.
Opfolda Contraindications
Contraindications
Pregnancy.
Opfolda Boxed Warnings
Not Applicable
Opfolda Warnings/Precautions
Warnings/Precautions
Must be administered in combination with Pombiliti (see full labeling). Moderate or severe renal impairment: reduce dose (see Adult dose). May impair fertility. Embryo-fetal toxicity. Exclude pregnancy status prior to initiation. Advise females of reproductive potential to use effective contraception during and for at least 60 days after the last dose. Nursing mothers: not recommended.
Opfolda Pharmacokinetics
Absorption
Mean time to reach the maximum concentration (Tmax): ranged from 2–3 hours. Co-administration with food is predicted to result in delayed absorption and decreased Cmax.
Distribution
Apparent volume of distribution: ~94 L.
Elimination
Apparent clearance: ~10 L/hr. Half-life: ~6 hours.
Opfolda Interactions
Not Applicable
Opfolda Adverse Reactions
Adverse Reactions
Headache, diarrhea, fatigue, nausea, abdominal pain, pyrexia.
Opfolda Clinical Trials
See Literature
Opfolda Note
Not Applicable
Opfolda Patient Counseling
See Literature
