Dosing | LIVMARLI® (maralixibat) oral solution and tablets

The only IBAT inhibitor with oral solution and tablet formulation options

Oral solution

A dosing dispenser is provided to measure and deliver the prescribed dose accurately¹*

Clear, colorless to yellow grape-flavored liquid.¹

There are 2 strengths of LIVMARLI oral solution, 9.5 mg/mL and 19 mg/mL.

LIVMARLI bottle and syringe not actual size.

Tablets

Patients can be transitioned from oral solution to tablets without interruption.

Tablets and dime not actual size.

Tablets can be used in patients ≥25 kg who are able to swallow them. They are available in 4 strengths.¹

Dime shown for scale.

*Please advise patients that household teaspoons and tablespoons are not adequate measuring devices.²

Simple dosing

One dose, once per day

30 minutes before a meal

for patients with Alagille syndrome (ALGS)

One dose, twice per day

30 minutes before a meal

for patients with progressive familial intrahepatic cholestasis (PFIC)

Dosing calculator

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ALGS PFIC

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Full dosing information

A table of individual daily dose by patient weight for ALGS.

The recommended dosage is 380 mcg/kg once daily, taken 30 minutes before a meal in the morning
Starting dose is 190 mcg/kg orally once daily and should be increased to 380 mcg/kg daily after 1 week, as tolerated, and not to exceed a maximum daily dose of 28.5 mg (3 mL) per day for the oral solution and 30 mg per day for the tablets

Patients should be regularly evaluated for changes in weight to ensure they are receiving the appropriate dose

The 2 strengths of LIVMARLI, 9.5 mg/mL and 19 mg/mL, should not be
substituted for one another when treating patients with LIVMARLI

^†Select the appropriate product based on the patient's weight and ability to swallow tablets.

IBAT=ileal bile acid transporter.

References:

1. LIVMARLI® (maralixibat). Prescribing Information. Mirum Pharmaceuticals, Inc. 2. LIVMARLI® (maralixibat). Instructions for Use. Mirum Pharmaceuticals, Inc.

Indications

LIVMARLI (maralixibat) oral solution and tablets are indicated for the treatment of cholestatic pruritus in patients who are 3 months of age and older with Alagille syndrome.

LIVMARLI (maralixibat) oral solution and tablets are indicated for the treatment of cholestatic pruritus in patients who are 12 months of age and older with progressive familial intrahepatic cholestasis (PFIC).

Limitations of Use: LIVMARLI is not recommended in a subgroup of PFIC type 2 patients with specific ABCB11 variants resulting in nonfunctional or complete absence of bile salt export pump (BSEP) protein.

Important Safety Information

CONTRAINDICATIONS

LIVMARLI (maralixibat) oral solution and tablets are contraindicated in patients with prior or active hepatic decompensation events (eg, variceal hemorrhage, ascites, or hepatic encephalopathy).

WARNINGS AND PRECAUTIONS

Hepatotoxicity: LIVMARLI treatment is associated with a potential for drug-induced liver injury (DILI). In the PFIC trial, treatment-emergent hepatic decompensation events and elevations of liver tests or worsening of liver tests occurred. Two patients experienced DILI attributable to LIVMARLI. Two additional patients experienced DILI in the open-label extension portion of the trial. Of these 4 patients, 1 patient required liver transplant and another patient died.

In the Alagille syndrome trial, treatment-emergent elevations of liver tests or worsening of liver tests occurred.

Obtain baseline liver tests and monitor during treatment. Liver-related adverse reactions and physical signs of hepatic decompensation should also be monitored. Dose reduction or treatment interruption may be considered if abnormalities occur in the absence of other causes. For persistent or recurrent liver test abnormalities, consider treatment discontinuation. Permanently discontinue LIVMARLI if a patient experiences the following: persistent or recurrent liver test abnormalities, clinical hepatitis, or a hepatic decompensation event.

Gastrointestinal Adverse Reactions: Diarrhea and abdominal pain were reported as the most common adverse reactions. Monitor for dehydration and treat promptly. Consider reducing the dosage or interrupting LIVMARLI treatment if a patient experiences persistent diarrhea or diarrhea with bloody stool, vomiting, dehydration requiring treatment, or fever.

Fat-Soluble Vitamin (FSV) Deficiency: Patients can have FSV deficiency (vitamins A, D, E, and K) at baseline, and LIVMARLI may adversely affect absorption of FSVs. Treatment-emergent bone fracture events have been observed more frequently with patients treated with LIVMARLI compared with patients treated with placebo. If bone fractures or bleeding occurs, consider interrupting LIVMARLI and supplement with FSVs. LIVMARLI can be restarted once FSV deficiency is corrected and maintained at corrected levels.

Risk of Propylene Glycol Toxicity (Pediatric Patients Less Than 5 Years of Age): LIVMARLI oral solution contains propylene glycol. Total daily intake of propylene glycol should be considered for managing the risk of propylene glycol toxicity. Monitor patients for signs of propylene glycol toxicity. Discontinue LIVMARLI if toxicity is suspected.

ADVERSE REACTIONS

Alagille syndrome: The most common adverse reactions are diarrhea, abdominal pain, vomiting, FSV deficiency, liver test abnormalities, and bone fractures.

PFIC: The most common adverse reactions are diarrhea, FSV deficiency, abdominal pain, liver test abnormalities, hematochezia, and bone fractures.

DRUG INTERACTIONS

Administer LIVMARLI at least 4 hours before or 4 hours after administration of bile acid binding resins.

A decrease in the absorption of OATP2B1 substrates (eg, statins) due to OATP2B1 inhibition by LIVMARLI in the GI tract cannot be ruled out. Consider monitoring the drug effects of OATP2B1 substrates as needed.

DOSING INFORMATION

In patients with Alagille syndrome, LIVMARLI is taken once daily, 30 minutes before a meal in the morning. In patients with PFIC, LIVMARLI is taken twice daily, 30 minutes before a meal. Refer to the dosing by weight guidelines in the full Prescribing Information for complete details on dosing for the oral solution and tablet formulations. The provided oral solution dosing dispenser must be used to accurately measure the dose. Any remaining LIVMARLI oral solution should be discarded 100 days after first opening the bottle. LIVMARLI tablets can be used in patients weighing ≥25 kg who can swallow tablets.

Please see full Prescribing Information for LIVMARLI.

Dosing & administration