Pronunciation: LIV-mar-lee
Generic name: maralixibat
Dosage form: oral solution
Drug class: Miscellaneous GI agents

Medically reviewed by Carmen Pope, BPharm. Last updated on Jul 30, 2024.

What is Livmarli?

Livmarli (maralixibat) is an oral solution that may be used to treat skin itching caused by a build-up of bile salts in the blood (cholestatic pruritus) associated with:

• Alagille syndrome (ALGS) in adults and children aged 3 months and older
• Progressive familial intrahepatic cholestasis (PFIC) in adults and children 12 months and older.

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.

Livmrali (maralixibat) 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 class of medicines known as ileal bile acid transporter (IBAT) inhibitors.

Livmarli is not recommended for PFIC type 2 patients with specific genetic variants that result in a nonfunctional or absent bile salt export pump (BSEP) protein.

Livmarli gained FDA approval on September 29, 2021.

Livmarli side effects

The most common side effects of Livmarli are:

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

In addition to these side effects, in adults and children with PFIC, vomiting of blood (hematochezia) is also a common side effect.

Serious side effects and warnings

Livmarli can cause 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 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.

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. It is not known if Livmarli will harm your unborn baby. Tell your healthcare provider right away if you think that you are pregnant.
• are breastfeeding or plan to breastfeed. 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.

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 doctor if you take:

• certain medicines for high cholesterol - cholestyramine, colesevelam, or colestipol. These medicines least should be taken at least 4 hours before or 4 hours after taking Livmarli.

How should I take Livmarli?

Read the Instructions for Use that comes with Livmarli Package Insert for information about the right way to prepare and take Livmarli.

• Take Livmarli 30 minutes before a meal once or twice daily as prescribed by your healthcare provider.
• Use the calibrated measuring device (0.5 mL, 1 mL, or 3 mL oral dispenser) provided by your pharmacist to measure and deliver the prescribed dose accurately.
• Take Livmarli at least 4 hours before or 4 hours after taking a bile acid-binding resin (such as cholestyramine, colesevelam, or colestipol). See What other drugs affect Livmarli below.
• Do not change your dose unless your healthcare provider tells you to.

Livmarli dosing information

Usual adult and child dose of Livmarli for Alagille Syndrome (ALGS)

• Use Livmarli oral Solution 9.5 mg/mL.

The dose depends on you or your child's weight. Your healthcare provider will calculate this for you.

• The recommended starting dose is 190 mcg/kg orally once daily in the morning, 30 minutes before breakfast.
• Increase to 380 mcg/kg once daily after one week, as tolerated.
• The usual recommended dosage is 380 mcg/kg once daily.
• Do not exceed a maximum daily dose of 28.5 mg.

Usual adult and child dose of Livmarli for Progressive familial intrahepatic cholestasis (PFIC)

• Use Livmarli oral Solution 19 mg/mL.

The dose depends on you or your child's weight. Your healthcare provider will calculate this for you.

• The recommended starting dose is 285 mcg/kg orally once daily in the morning, 30 minutes before breakfast.
• Increase to 285 mcg/kg twice daily, 428 mcg/kg twice daily, and then to 570 mcg/kg twice daily, as tolerated.
• The usual recommended dosage is 570 mcg/kg twice daily.
• Do not exceed a maximum daily dose of 38 mg.

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?

Sometimes it is not safe to use certain medicines at the same time. Some drugs can affect your blood levels of other drugs you use, which may increase side effects or make the medicines less effective. Tell your doctor about all your current medicines.

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.

This list is not complete and many other drugs may interact with Pylera. This includes prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible drug interactions are listed here. Refer to the Livmarli Prescribing Information for a full list of interactions.

How should I store Livmarli?

Store the opened bottle at room temperature below 30ºC.

• Always store Livmarli with the cap on the bottle.

Discard any unused Livmarli 100 days after opening the bottle.

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

Livmarli ingredients

Active ingredients: maralixibat chloride 9.5 mg/mL or 19 mg/mL.
Inactive ingredients: edetate disodium, grape flavor, propylene glycol, purified water, and sucralose.

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

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

Who makes Livmarli

Mirum Pharmaceuticals, Inc., manufactures Livmarli.

Further information

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

Medical Disclaimer