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

Medically reviewed by Carmen Pope, BPharm. Last updated on Apr 15, 2025.

What is Livmarli?

Livmarli is used to treat Alagille syndrome (ALGS) in people 3 months and older and progressive familial intrahepatic cholestasis (PFIC) in people 12 months and older, except for those with certain ABCB11 variants. It is available as an oral solution or tablet and is taken once or twice a day.

• Livmarli treats cholestatic pruritus (skin itching caused by a build-up of bile acids).

Livmarli (maralixibat) gained FDA approval on September 29, 2021. There is no generic.

Mechanism

Livmrali is thought to work by blocking the ileal bile acid transporter, which is crucial for the reabsorption of bile acids from the terminal ileum (the last part of the small intestine). This causes a decrease in the amount of bile salts in the blood and a reduction in skin itching.

Livmarli belongs to the drug class called ileal bile acid transporter (IBAT) inhibitors.

Side effects

The most common side effects of Livmarli are:

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

Vomiting up blood (hematochezia) is also a common side effect in adults and children with PFIC taking Livmarli.

Serious side effects and warnings

Livmarli can cause the following serious side effects, including:

• Changes in liver tests. Changes in certain liver tests are common in patients with Alagille syndrome but may worsen during treatment. These changes may be a sign of liver injury and can be serious. Your healthcare provider should do blood tests 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)
  • loss of appetite
• Stomach and intestinal (gastrointestinal) problems. Livmarli can cause stomach and intestinal problems, including diarrhea, stomach pain, and vomiting during treatment. Tell your healthcare provider right away if you have any of these symptoms more often or more severely than normal for you.
• 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 Alagille syndrome but may worsen during treatment. Your healthcare provider should do blood tests before starting and during treatment.
• Other common side effects reported during treatment were bone fractures and gastrointestinal bleeding.

There is a risk of propylene glycol toxicity when Livmarli oral solution is given to children aged less than 5 years. Your healthcare provider will consider this when prescribing the dose of Livmarli for your child. 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. 

It is not known if this medicine is safe and effective for:

• Children with ALGS under 3 months of age
• Children with PFIC under the age of 12 months
• Adults with ALGS or PFIC aged 65 years of age and older.

Livmarli is not recommended for PFIC type 2 patients with specific ABCB11 variants that result in nonfunctional or complete absence of bile salt export pump (BSEP) protein. 

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 you take Livmarli

Before you start treatment, tell your healthcare provider about all of your medical conditions, including if you:

• have liver problems.
• 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?

Read the Instructions for Use that comes with the Livmarli Package Insert for information about the right way to take Livmarli oral solution or tablets.

• Take Livmarli 30 minutes before a meal once or twice daily as prescribed by your healthcare provider.
• If using the oral solution, use the calibrated oral dispenser provided by your pharmacist to accurately measure and deliver the prescribed dose.
• Take Livmarli at least 4 hours before or 4 hours after taking a bile acid-binding resin (such as cholestyramine, colesevelam, or colestipol). 
• Do not change your dose unless your healthcare provider tells you to.

Dosing information

Usual Dose 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. 

Usual Dose 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 of Livmarli and you usually take it 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 of Livmarli and you usually take it 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 or overdose, 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 oral solution and tablets at room temperature.

Once opened, keep the solution or tablets at room temperature. Always leave the cap on the bottle.

• Discard any unused Livmarli oral solution 100 days after opening the bottle.

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

Ingredients

Active ingredients: maralixibat chloride 

Inactive ingredients (oral solution): edetate disodium, grape flavor, propylene glycol, purified water, and sucralose.

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.

Inactive ingredients (oral tablets): crospovidone, glyceryl distearate, lactose monohydrate, microcrystalline cellulose, and silicon dioxide.

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

Who makes Livmarli

Livmarli (maralixibat) is made by Mirum Pharmaceuticals. The company is headquartered in Foster City, California.

Further information

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

Medical Disclaimer