Medical Uses
Avlayah (tividenofusp alfa-eknm) is a hydrolytic lysosomal glycosaminoglycan (GAG)-specific enzyme indicated for the treatment of neurologic manifestations of Hunter syndrome (Mucopolysaccharidosis type II, MPS II) when initiated in presymptomatic or symptomatic pediatric patients weighing at least 5 kg before advanced neurologic impairment.
This indication is approved under accelerated approval depending on reduction of cerebrospinal fluid heparan sulfate noticed in patients treated with Avlayah.
Limitations of Use: This therapeutic drug is not recommended for use in combination with other enzyme replacement therapies.
Recommended Dosage:
The recommended starting dosage of Avlayah for pediatric patients weighing at least 5 kg is 3 mg/kg administered once weekly via intravenous infusion. Administer each dosage level for at least 4 weeks before escalating to the next dosage level.
The recommended maintenance dosage of Avlayah for pediatric patients who weigh at least 5 kg is 15 mg/kg administered once weekly via intravenous infusion.
The recommended dosage of Avlayah for pediatric patients weighing ≥5 kg is as follows:
- Week 1 to Week 4: 3 mg/kg once weekly
- Week 5 to Week 8: 7.5 mg/kg once weekly
- Week 9 and beyond: 15 mg/kg once weekly (maintenance dosage)
Do not escalate the dosage level of Avlayah (tividenofusp alfa-eknm) if the current dosage level is not tolerated.
If any dose of Avlayah is missed, skip the missed dose. Avoid taking a double dose to compensate for a missed dose. Restart treatment as soon as possible, maintaining the one-week interval between infusions thereafter. Resume dosing at the last administered dosage following the recommended infusion rate.
