Livmarli is the only medicine approved by the FDA and an ileal bile acid transporter (IBAT) inhibitor widely used to treat cholestatic pruritus (itch) in patients with Alagille syndrome (ALGS) who are aged 12 months and older.
Recommended Dosage: For those with bile acid-binding resins, this oral medication should be taken at least four hours prior to or four hours after taking a bile acid-binding resin. The recommended Livmarli dosage is 380 mcg per kg once in a day, taken 30 minutes prior to the initial meal of the day.
Start Livmarli dosing at 190 mcg per kg taken orally (by mouth) once in a day; after 1-week, increase to 380 mcg per kg once in a day, as tolerated. The maximum daily Livmarli dose volume for those above 70 kg is 3mL or 28.5 mg/daily.
If any Livmarli dose is puked or missed, it needs to be taken as promptly within 12 hours of the time it is typically taken, and the normal dosing schedule needs to be continued. In case any dose is missed or skipped beyond 12 hours, the missed dose can be omitted and the standard dosing schedule continued.