Livmarli

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

Medically reviewed by Judith Stewart, BPharm. Last updated on Mar 14, 2024.

What is Livmarli?

Livmarli is an ileal bile acid transporter (IBAT) inhibitor that may be used to treat cholestatic pruritus (skin itching caused by a build-up of bile salts in the blood) associated with:

Alagille syndrome (ALGS) in adults and children aged 3 months and older
Progressive familial intrahepatic cholestasis (PFIC) in adults and children aged at least 5 years.

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 5 years
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 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 was FDA-approved on September 29, 2021.

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 for information about the right way to prepare and take Livmarli.

Before you take Livmarli for the first time, talk to your healthcare provider or pharmacist about how to measure your prescribed dose.
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.
Do not change your dose unless your healthcare provider tells you to.
Livmarli is taken by mouth, 1 time each day, 30 minutes before your first meal of the day.
If you miss a dose and 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.
- 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 take too much or overdose, call your healthcare provider or go to the nearest emergency room right away.
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.

Dosing information

Usual Adult Dose and Usual Pediatric Dose for Cholestatic Pruritus in Patients with Alagille Syndrome:

- 380 mcg/kg once daily, taken 30 minutes before the first meal of the day. Starting dose is 190 mcg/kg orally once daily, and should be increased to 380 mcg/kg once daily after one week, as tolerated.

Use: cholestatic pruritus in patients with Alagille syndrome (ALGS) 1 year of age and older.

Livmarli side effects

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.

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 FDA at 1-800-FDA-1088.

How should I store Livmarli?

Store Livmarli at room temperature between 68ºF and 77ºF (20ºC and 25ºC).
Always store Livmarli with the cap on the bottle.
Throw away any remaining Livmarli, following the steps below, 45 days after first opening the bottle.
- Mix medicine with an unappealing substance such as dirt, cat litter, or used coffee grounds;
- Place the mixture in a container such as a sealed plastic bag;
- Throw away the container in your trash at home; and
- Delete all personal information on the prescription label of the empty medicine bottle, then throw away or recycle the empty bottle.

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

What are the ingredients in Livmarli?

Active ingredients: maralixibat chloride
Inactive ingredients: edetate disodium, grape flavor, propylene glycol, purified water, and sucralose.