Livmarli Dosage

Generic name: maralixibat chloride 9.5mg in 1mL
Dosage form: oral solution
Drug class: Miscellaneous GI agents

Medically reviewed by Drugs.com. Last updated on Nov 20, 2024.

Recommended Dosage for Alagille Syndrome

Use LIVMARLI Oral Solution 9.5 mg/mL for the treatment of ALGS.

The recommended dosage is 380 mcg/kg once daily, taken 30 minutes before a meal in the morning. Start dosing at 190 mcg/kg administered orally once daily; after one week, increase to 380 mcg/kg once daily, as tolerated. The maximum daily dose should not exceed 28.5 mg (3 mL) per day. Refer to the dosing by weight guidelines presented in Table 1.

Table 1: 9.5 mg/mL Solution for Patients with ALGS: Volume per Dose (mL) by Weight
Patient Weight (kg)	Days 1-7 (190 mcg/kg once daily)	Beginning Day 8 (380 mcg/kg once daily)
Patient Weight (kg)	9.5 mg/mL Solution (for ALGS) Volume per Dose (mL)
5 to 6	0.1	0.2
7 to 9	0.15	0.3
10 to 12	0.2	0.45
13 to 15	0.3	0.6
16 to 19	0.35	0.7
20 to 24	0.45	0.9
25 to 29	0.5	1
30 to 34	0.6	1.25
35 to 39	0.7	1.5
40 to 49	0.9	1.75
50 to 59	1	2.25
60 to 69	1.25	2.5
70 or higher	1.5	3

Recommended Dosage for Progressive Familial Intrahepatic Cholestasis

Use LIVMARLI Oral Solution 19 mg/mL for the treatment of PFIC.

The two strengths of LIVMARLI, 9.5 mg/mL and 19 mg/mL, should not be substituted for one another when treating PFIC patients. Special attention should be given to the accurate calculation of the dose volume of LIVMARLI. This is especially important for pediatric patients less than 5 years old as LIVMARLI oral solution contains the excipient propylene glycol (364.5 mg/mL).

The recommended dosage is 570 mcg/kg twice daily 30 minutes before a meal. The starting dose is 285 mcg/kg orally once daily in the morning, and should be increased to 285 mcg/kg twice daily, 428 mcg/kg twice daily, and then to 570 mcg/kg twice daily, as tolerated. The maximum daily dose should not exceed 38 mg (2 mL) per day. Refer to the dosing by weight guidelines presented in Table 2.

Table 2: 19 mg/mL Solution for Patients with PFIC: Volume per Dose (mL) by Weight
Patient Weight (kg)	285 mcg/kg (once daily titrated to twice daily)	428 mcg/kg (twice daily)	570 mcg/kg (twice daily as tolerated)
Patient Weight (kg)	19 mg/mL Solution (for PFIC) Volume per Dose (mL)
5	0.1	0.1	0.15
6 to 7	0.1	0.15	0.2
8	0.1	0.2	0.25
9	0.15	0.2	0.25
10 to 12	0.15	0.25	0.3
13 to 15	0.2	0.3	0.4
16 to 19	0.25	0.4	0.5
20 to 24	0.3	0.5	0.6
25 to 29	0.4	0.6	0.8
30 to 34	0.45	0.7	0.9
35 to 39	0.6	0.8	1
40 to 49	0.6	0.9	1
50 to 59	0.8	1	1
60 or higher	0.9	1	1

Missed Dose

For once daily dosing: If a dose is missed, it should be taken as soon as possible within 12 hours of the time it is usually taken, and the original dosing schedule should be resumed. If a dose is missed by more than 12 hours, the dose can be omitted and the original dosing schedule resumed.

For twice daily dosing: If a dose is missed, it should be taken as soon as possible within 6 hours of the time it is usually taken, and the original dosing schedule should be resumed. If a dose is missed by more than 6 hours, the dose can be omitted and the original dosing schedule resumed.

Important Administration Instructions

Administer LIVMARLI 30 minutes before a meal.

A calibrated measuring device (0.5 mL, 1 mL or 3 mL oral dosing dispenser) will be provided by the pharmacy to measure and deliver the prescribed dose accurately.

After opening the LIVMARLI bottle, store below 30°C (86°F) and discard any remaining LIVMARLI after 100 days.

Dosage Modification for Management of Adverse Events

Establish the baseline pattern of variability of liver tests prior to starting LIVMARLI, so that potential signs of liver injury can be identified. Monitor liver tests (e.g., ALT [alanine aminotransferase], AST [aspartate aminotransferase], TB [total bilirubin]), DB [direct bilirubin], and International Normalized Ratio [INR]) during treatment with LIVMARLI. Reduce the dosage or interrupt LIVMARLI if new onset liver test abnormalities occur. Once the liver test abnormalities either return back to baseline values or stabilize at a new baseline value, consider restarting LIVMARLI at the last tolerated dose, and increase the dose as tolerated. Consider discontinuing LIVMARLI permanently if liver test abnormalities recur or symptoms consistent with clinical hepatitis are observed.

LIVMARLI has not been studied in patients with hepatic decompensation. Discontinue LIVMARLI permanently if a patient experiences a hepatic decompensation event (e.g., variceal hemorrhage, ascites, hepatic encephalopathy).