Pronunciation: LIV-mar-lee
Generic name: maralixibat
Dosage form: oral solution (9.5 mg/mL, 19 mg/mL), oral tablet (10 mg, 15 mg, 20 mg, 30 mg)
Drug class: Miscellaneous GI agents

Medically reviewed by Carmen Pope, BPharm. Last updated on Jun 26, 2025.

What is Livmarli?

Livmarli is used to treat cholestatic pruritus (itch) in people with Alagille syndrome (ALGS) or progressive familial intrahepatic cholestasis (PFIC). It is an oral solution or tablet that is taken 1 or 2 times a day.

Livmarli works by blocking the ileal bile acid transporter (IBAT, a protein responsible for the reabsorption of bile acids from the ileum back into the bloodstream), preventing bile acid reabsorption in the terminal ileum. This reduces serum bile acid levels, which may alleviate pruritus (itching) in patients with ALGS or PFIC, though the exact mechanism for itch relief remains incompletely understood. Livmarli belongs to the drug class called ileal bile acid transporter (IBAT) inhibitors.

Livmarli (maralixibat) gained FDA approval on September 29, 2021. There is no generic. Approval was based on the results of clinical trials, which reported:

• Livmarli significantly improved cholestatic pruritus in ALGS patients from Week 3, with full effect by Week 18. At one year, 84% experienced clinically meaningful improvements. Benefits were durable through nearly 4 years, with patients achieving 95% of days having minimal/no pruritus during extended treatment. (ICONIC trial; NCT02160782)
• In PFIC trials (MARCH study; NCT03905330), 64% of patients experienced meaningful improvements in cholestatic pruritus within the first six months.

FDA approvals and uses

Livmarli is approved to treat cholestatic pruritus in adults and children:

• 3 months of age and older with Alagille syndrome (ALGS)
• 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 ALGS who are under 3 months of age
• children with PFIC who are under 12 months of age
• adults 65 years of age and older. 

Side effects

The most common side effects of Livmarli in ALGS are:

• diarrhea
• abdominal (stomach) pain
• vomiting
• fat-soluble vitamin deficiency
• liver-test abnormalities
• bone fractures.

The most common side effects of Livmarli in PFIC are:

• diarrhea
• fat-soluble vitamin deficiency
• abdominal (stomach) pain
• liver-test abnormalities
• hematochezia (vomiting up blood)
• bone fractures.

Serious side effects and warnings

Livmarli can cause the following serious side effects:

• Liver injury. Changes in certain liver tests are common in patients with ALGS and PFIC but may worsen during treatment with Livmarli. These changes may be a sign of liver injury and can be serious, or may lead to liver transplant or death. Your healthcare provider should do blood tests and physical exams before starting and during treatment to check your liver function. Tell your healthcare 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 healthcare provider should check your stool for blood and monitor you for too much body fluid loss. Tell your healthcare 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, sticky, or have blood or mucous
  • severe stomach-area pain or tenderness.

Tell your healthcare provider if you have any signs and symptoms of a loss of too much body fluid, including:

• • vomiting
  • diarrhea
  • urinating less often than usual
  • headache
  • dizziness.
• 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 ALGS and PFIC but may worsen during treatment with Livmarli. Your healthcare provider should do blood tests before starting and during treatment with Livmarli.

Other common side effects of FSV deficiency reported during treatment with Livmarli were bone fractures and bleeding. Tell your healthcare provider if you have signs or symptoms of bleeding or any fractures.

Children less than 5 years old are given the Livmarli solution. There is a risk of propylene glycol toxicity with higher dosages of Livmarli oral solution. Tell your healthcare provider if you suspect your child may have propylene glycol toxicity. Symptoms may include episodes of unresponsiveness, a fast heartbeat or fast breathing, excessive sweating, or low blood glucose levels. 

Your healthcare provider may change your dose or temporarily or permanently stop treatment if you have certain side effects.

These are not all of the possible side effects. Call your healthcare provider for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Related/similar drugs

Before taking

Do not take Livmarli if you have or currently have active liver decompensation, such as variceal hemorrhage, ascites, or hepatic encephalopathy.

Before taking Livmarli, tell your healthcare provider about all of your medical conditions, including if you:

• are pregnant or plan to become pregnant
• are breastfeeding or plan to breastfeed.

Pregnancy

It is not known if Livmarli will harm your unborn baby. Tell your healthcare provider right away if you think that you are pregnant.

Breastfeeding

It is not known if Livmarli passes into your breast milk. Talk with your healthcare provider about the best way to feed your baby if you take Livmarli.

How do I take Livmarli?

Livmarli is available as an oral solution or a tablet.

• Read the Instructions for Use that come with the Livmarli Package Insert for information about the right way to prepare and take the Livmarli oral solution.
• Take Livmarli exactly as your healthcare provider tells you to.
• Your healthcare provider may start you on a low dose and then increase the dose, especially if you have not taken Livmarli before.
• Do not change you dose unless your healthcare provider tells you to.

Livmarli is taken by mouth, 1 or 2 times each day.

• If you take it 2 times a day, take a dose in the morning and a dose in the evening.
• Take Livmarli 30 minutes before a meal.

If you take a medicine that lowers cholesterol by binding bile acids, such as cholestyramine, colesevelam, or colestipol, take it at least 4 hours before or 4 hours after you take Livmarli. Ask your healthcare provider if you are not sure if you take these medicines.

Livmarli oral solution

Livmarli oral solution comes in 2 different strengths:

• 9.5 mg/mL for use in ALGS
• 19 mg/mL for use in PFIC.

Use the calibrated oral dispenser provided by your pharmacist to accurately measure and deliver you or your child's dose of Livmarli.

Before you take the oral solution for the first time, talk to your healthcare provider or pharmacist about how to measure the prescribed dose.

After opening the Livmarli oral solution bottle, store it below 30°C (86°F). Throw away any remaining Livmarli
oral solution 100 days after first opening the bottle.

Dosing information

Dose of Livmarli for Alagille Syndrome (ALGS)

The dose depends on weight. Your healthcare provider will calculate this for you.

• Initial: 190 mcg/kg orally once daily in the morning, 30 minutes before breakfast.
• After 1 week: Increase to 380 mcg/kg daily after one week, as tolerated.
• Usual dosage: 380 mcg/kg daily.
• Maximum dose: Do not exceed 28.5 mg/day (oral solution) or 30 mg/day (tablets).

Note: Only use Livmarli Oral Solution 9.5 mg/ mL or Livmarli Tablets for the treatment of ALGS. 

Dose of Livmarli for Progressive Familial Intrahepatic Cholestasis (PFIC)

The dose depends on weight. Your healthcare provider will calculate this for you.

• Initial: 285 mcg/kg orally once daily in the morning, 30 minutes before breakfast.
• If tolerated: Increase to 285 mcg/kg twice daily, as tolerated. Then increase to 428 mcg/kg twice daily, and then to 570 mcg/kg twice daily as tolerated.
• Usual dosage: 285 mcg/kg twice daily to 570 mcg/kg twice daily.
• Maximum dose: Do not exceed 38 mg/day (oral solution) or 40 mg/day (tablets).

Note: Only use Livmarli Oral Solution 19 mg/mL or Livmarli Tablets for the treatment of PFIC.

What happens if I miss a dose?

If you miss a dose and you usually take Livmarli 1 time a day:

• If it is 12 hours or less from the time you usually take Livmarli, take the missed dose as soon as possible. Then take your next dose at the usual time.
• If it is more than 12 hours from the time you usually take Livmarli, do not take the missed dose. Take your next dose at the usual time.

If you miss a dose and you usually take Livmarli 2 times a day:

• If it is 6 hours or less from the time you usually take Livmarli, take the missed dose as soon as possible. Then take your next dose at the usual time.
• If it is more than 6 hours from the time you usually take Livmarli, do not take the missed dose. Take your next dose at the usual time.

What happens if I overdose?

If you take too much Livmarli, call your healthcare provider or go to the nearest emergency room right away.

What other drugs will affect Livmarli?

Tell your healthcare provider about all medicines that you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements. Know the medicines you take. Keep a list of them to show your healthcare provider and pharmacist when you get a new medicine. Livmarli may affect the way some other medicines work, and some other medicines may affect the way Livmarli works.

Especially tell your healthcare provider if you take a medicine that lowers cholesterol by binding bile acids, such as cholestyramine, colesevelam, or colestipol. If you do, you should take the cholesterol-lowering medicine at least 4 hours before or 4 hours after you take Livmarli. Ask your healthcare provider if you are not sure if you take these medicines.

Not all possible drug interactions are listed here. Refer to the Livmarli Prescribing Information for a list of interactions.

Storage

Store unopened Livmarli oral solution and tablets at room temperature between 68ºF and 77ºF (20ºC and
25ºC).

After opening the Livmarli oral solution bottle, store below 86°F (30ºC). Throw away any remaining Livmarli oral solution 100 days after first opening the bottle.

Keep all medicines out of the reach of children and pets.

Ingredients

Active ingredients: maralixibat chloride 

Oral solution inactive ingredients: edetate disodium, grape flavor, propylene glycol, purified water, and sucralose.

Oral tablets inactive ingredients: crospovidone, glyceryl distearate, lactose monohydrate, microcrystalline cellulose, and silicon dioxide.

Livmarli oral solution is available in 2 strengths, depending on the condition being treated:

• Treatment of ALGS: maralixibat 9.5 mg/mL.
• Treatment of PFIC: maralixibat 19 mg/mL.

Livmarli tablets are available in 4 strengths: 10 mg, 15 mg, 20 mg, and 30 mg.

Manufacturer

Livmarli (maralixibat) is made by Mirum Pharmaceuticals Inc., Foster City, CA 94404. 

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

Medical Disclaimer