Maralixibat

What is Maralixibat?

• Maralixibat (Livmarli) is an ileal bile acid transporter (IBAT) inhibitor used to treat cholestatic pruritus associated with Alagille syndrome.

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Maralixibat

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Maralixibat chloride skeletal

Maralixibat chloride

What are the uses of this medicine?

• Maralixibat (Livmarli) is a prescription medicine used to treat cholestatic pruritus (itch) in patients with Alagille syndrome 1 year of age and older.
• It is not known if Livmarli is safe and effective in adults 65 years of age and older.

How does this medicine work?

• Maralixibat is a reversible inhibitor of the ileal bile acid transporter (IBAT).
• It decreases the reabsorption of bile acids (primarily the salt forms) from the terminal ileum.
• Although the complete mechanism by which maralixibat improves pruritus in ALGS patients is unknown, it may involve inhibition of the IBAT, which results in decreased reuptake of bile salts, as observed by a decrease in serum bile acids.

Who Should Not Use this medicine ?

• This medicine have no usage limitations.

What drug interactions can this medicine cause?

• Livmarli may affect the way some other medicines work, and some other medicines may affect the way Livmarli works.
• Bile acid binding resins may bind to maralixibat in the gut. Administer bile acid binding resins (e.g., cholestyramine, colesevelam, or colestipol) at least 4 hours before or 4 hours after administration of Livmarli.
• A decrease in the oral absorption of OATP2B1 substrates (e.g., statins) due to OATP2B1 inhibition in the GI tract cannot be ruled out. Consider monitoring the drug effects of OATP2B1 substrates (e.g. statins) as needed.

Is this medicine FDA approved?

• Maralixibat was approved for medical use in the United States in September 2021.

How should this medicine be used?

Recommended dosage:

• The recommended dosage is 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.

Administration:

• Take Livmarli exactly as your healthcare provider tells you to.
• Your healthcare provider may start you on a low dose of Livmarli and then increase the dose, especially if you have not taken Livmarli.
• Do not change your dose of Livmarli 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 of Livmarli 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 Livmarli, 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.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As Oral solution: 9.5 mg of maralixibat per mL.

This medicine is available in fallowing brand namesː

• Livmarli

What side effects can this medication cause?

The most common side effects of this medicine include:

• diarrhea
• abdominal pain
• vomiting
• fat-soluble vitamin deficiency
• liver test abnormalities
• gastrointestinal bleeding
• bone fractures

Livmarli can cause serious side effects, including:

• Changes in liver tests
• Stomach and intestinal (gastrointestinal) problems
• 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

What special precautions should I follow?

• Liver Test abnormalities were observed. Most abnormalities included elevation in ALT, AST, or T/DB. Obtain baseline liver tests and monitor during treatment. Dose reduction or treatment interruption may be considered if abnormalities occur. For persistent or recurrent liver test abnormalities, consider Livmarli discontinuation.
• Diarrhea, abdominal pain, and vomiting were reported as the most common adverse reactions in patients treated with Livmarli. Consider interrupting Livmarli treatment if a patient experiences persistent diarrhea, abdominal pain, vomiting, or has diarrhea with bloody stool, vomiting, dehydration requiring treatment, or fever. If diarrhea, abdominal pain, or vomiting persists and no alternate etiology is identified, consider stopping Livmarli treatment.
• Fat-soluble vitamins (FSV) include vitamin A, D, E, and K (measured using INR levels). ALGS patients can have FSV deficiency at baseline. Livmarli may affect absorption of fat-soluble vitamins. : Obtain baseline levels and monitor during treatment. Supplement if deficiency is observed. If FSV deficiency persists or worsens despite FSV supplementation, consider discontinuing Livmarli treatment.

What to do in case of emergency/overdose?

Symptoms of overdosage may include:

• Overdoses of propylene glycol may manifest with hyperosmolality, CNS, cardiovascular, and/or respiratory effects and may subside with the elimination of propylene glycol.

Management of overdosage:

• If an overdose occurs, discontinue Livmarli, monitor the patient for any signs and symptoms and institute general supportive measures if needed.

Can this medicine be used in 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.

Can this medicine be used in children?

• It is not known if Livmarli is safe and effective in children under 1 year of age.

What are the active and inactive ingredients in this medicine?

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

Who manufactures and distributes this medicine?

• Manufactured for: Mirum Pharmaceuticals, Inc., 950 Tower Lane, Suite 1050, Foster City, CA

What should I know about storage and disposal of this medication?

• 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.

• Dailymed label info on Maralixibat
• FDA Maralixibat

Maralixibat Resources
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