Explore LIVMARLI® (maralixibat)

What is LIVMARLI?

LIVMARLI is an FDA-approved medicine specifically designed to treat cholestatic pruritus (itch) in patients 12 months of age and older with progressive familial intrahepatic cholestasis (PFIC). LIVMARLI helps relieve cholestatic pruritus and lower bile acid buildup.

LIVMARLI is not for use in PFIC type 2 patients who have a severe defect in the bile salt export pump (BSEP) protein.

How does LIVMARLI work?

Liver with intestines, number 1 shows LIVMARLI targeting IBAT, number 2 shows LIVMARLI removing bile acids, number 3 shows reductions in bile acids

Graphic of the liver and intestines with a number 1 label representing how LIVMARLI targets and temporarily blocks IBAT.

Graphic of the liver and intestines, with a number 2 label representing how LIVMARLI blocks bile acids and increases bile acid removal.

Graphic of the liver and intestines, with a number 3 label representing reductions in bile acids that are associated with less intense cholestatic pruritus.

LIVMARLI is a type of medicine called an ileal bile acid transporter (IBAT) inhibitor.

LIVMARLI targets and temporarily blocks something called the IBAT. In doing so, LIVMARLI lowers bile acids in the body (as measured by levels in the blood).

LIVMARLI blocks bile acids from going back into the liver and increases the amount of bile acids removed from the body in feces.

In the clinical study for LIVMARLI, reductions in bile acid buildup were associated with decreases in intensity of cholestatic pruritus.

IBAT inhibitor medicines like LIVMARLI work differently than many medications used to treat symptoms of PFIC, which may not be FDA approved to treat cholestatic pruritus.

The way LIVMARLI improves cholestatic pruritus is not completely known. It may involve inhibition of the IBAT, which interrupts bile acid recycling to the liver and decreases serum bile acids.

What are the possible side effects?

Side effects can happen as your body is adjusting to LIVMARLI and working to get rid of excess bile. Talk with your doctor about what to expect and how to manage any side effects.

The most common* side effects in clinical studies included:

Diarrhea (around 6 in 10 people)
Stomach pain (around 3 in 10 people)
Fat-soluble vitamin (FSV) deficiency (around 3 in 10 people)

Liver test abnormalities (around 2 in 10 people)
Rectal bleeding (around 1 in 10 people)
Bone fractures (around 1 in 10 people)

About gastrointestinal (GI) side effects GI events like diarrhea and stomach pain may occur as your body adjusts to LIVMARLI. In clinical studies, most GI side effects were mild to moderate and temporary, lasting 5.5 days, on average.
About fat-soluble vitamin (FSV) deficiency
Though LIVMARLI may affect absorption of FSVs, patients with PFIC can have FSV deficiency at the start of treatment and are often supplemented with FSVs.

In clinical studies, FSV deficiency was more common with placebo than with LIVMARLI (35% vs 28%, respectively), after 26 weeks of treatment.

Because PFIC can affect vitamin absorption, bone weakness and fractures may occur.
About liver test abnormalities High levels of certain liver enzymes called alanine aminotransferase (ALT) and aspartate aminotransferase (AST) can be a sign of liver injury. Your doctor should monitor liver tests during treatment with LIVMARLI.

*“Common” defined as occurring in ≥5% of patients treated with LIVMARLI.

Navigating side effects

Many side effects can improve with proper management
Your doctor is your treatment partner. If side effects occur, always talk with your doctor before stopping treatment
They may adjust your dose, pause treatment briefly, or suggest supportive steps (like diet changes, fluids, or over-the-counter medicines) to ease GI side effects

Indication

LIVMARLI (maralixibat) oral solution and tablets are a prescription medicine used to treat cholestatic pruritus (itch) in patients 12 months of age and older with progressive familial intrahepatic cholestasis (PFIC).

LIVMARLI is not for use in PFIC type 2 patients who have a severe defect in the bile salt export pump (BSEP) protein.

It is not known if LIVMARLI is safe and effective in children with PFIC who are under 12 months of age. It is not known if LIVMARLI is safe and effective in adults who are 65 years of age and older.

Please see full Prescribing Information, including Patient Information and Instructions for Use, for LIVMARLI.

Important Safety Information

What are the possible side effects of LIVMARLI (maralixibat) oral solution and tablets?

LIVMARLI can cause serious side effects, including:

Liver injury. Changes in certain liver tests are common in patients with PFIC but may worsen during treatment. These changes may be a sign of liver injury and can be serious or may lead to liver transplant or death. Your health care provider should do blood tests and physical exams before starting and during treatment with LIVMARLI to check your liver function. Tell your health care provider right away if you get any signs or symptoms of liver problems, including:
- nausea or vomiting
- your skin or the white part of your eye turns yellow
- dark or brown urine
- pain on the right side of your stomach (abdomen)
- fullness, bloating, or fluid in your stomach area (ascites)
- loss of appetite
- bleeding or bruising more easily than normal, including vomiting blood
Stomach and intestinal (gastrointestinal) problems. LIVMARLI can cause stomach and intestinal problems, including diarrhea and stomach pain during treatment. Diarrhea can also cause the loss of too much body fluid (severe dehydration). Your health care provider may advise you to monitor for new or worsening stomach problems, including stomach pain, diarrhea, blood in your stool, or vomiting
Tell your health care provider right away if you have any new or worsening signs or symptoms of stomach and intestinal problems, including:
- diarrhea
- more frequent bowel movements than usual
- stools that are black, tarry, or sticky, or have blood or mucous
- severe stomach-area pain or tenderness
- vomiting
- urinating less often than usual
- dizziness
- headache
A condition called Fat-Soluble Vitamin (FSV) Deficiency caused by low levels of certain vitamins (vitamin A, D, E, and K) stored in body fat. FSV deficiency is common in patients with PFIC but may worsen during treatment. Your health care provider should do blood tests before starting and during treatment, and may monitor for bone fractures and bleeding, which are common side effects

Tell your health care provider about all medicines that you take, as LIVMARLI may interact with other medicines. If you take a medicine that lowers cholesterol by binding bile acids, such as cholestyramine, colesevelam, or colestipol, take LIVMARLI at least 4 hours before or 4 hours after you take that medicine.

Your health care provider may change your dose, or temporarily or permanently stop treatment with LIVMARLI if you have certain side effects.

LIVMARLI is available in oral solution and tablet formulations. Take LIVMARLI exactly as your health care provider tells you to and do not change your dose unless you are instructed to. In patients with PFIC, LIVMARLI is taken by mouth, 2 times each day, 30 minutes before a meal. For the oral solution, be sure to use the provided oral dosing dispenser to accurately measure the dose of medicine.

These are not all of the possible side effects of LIVMARLI. For more information, ask your health care provider or pharmacist. Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Please see full Prescribing Information, including Patient Information and Instructions for Use, for LIVMARLI.