Livmarli (maralixibat) is used to treat itching (cholestatic pruritis) from a genetic condition called Alagille syndrome (ALGS). It's also approved to treat itching from a genetic liver condition called progressive familial intrahepatic cholestasis (PFIC). This medication belongs to the drug class called ileal bile acid transporter (IBAT) inhibitors. Livmarli (maralixibat) is a grape-flavored liquid that adults and children take by mouth on an empty stomach. Some common side effects include diarrhea and stomach pain.
Itching (cholestatic pruritis) from:
Alagille syndrome (ALGS) in people 3 months of age and older
Progressive familial intrahepatic cholestasis (PFIC) in people 5 years of age and older
People with Alagille syndrome and progressive familial intrahepatic cholestasis have too much bile acid in the body. Bile acids normally help your body digest and absorb fat. But when there's too much bile acid in the body, it can damage the liver and is thought to lead to symptoms like itchiness.
Livmarli (maralixibat) is an ileal bile acid transporter (IBAT) inhibitor. It blocks a protein in the body called IBAT. This protein typically helps keep bile acid in the body. By blocking IBAT, Livmarli (maralixibat) helps lower the amount of bile acids in your body.
Source: DailyMed
Side effects that you should report to your care team as soon as possible:
Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):
Works well to help improve itchiness from ALGS and PFIC
Can use in children
Comes as a grape-flavored liquid for better taste
Need to take 30 minutes before a meal
Might take several weeks to start working
Can cause stomach side effects and poor absorption of certain vitamins (vitamin A, D, E, and K)
Before you take Livmarli (maralixibat), be sure to read the instructions for use. Ask your care team if you have any questions about how to take the medication properly.
Take Livmarli (maralixibat) 30 minutes before a meal. Studies show that food lowers how well the medication gets absorbed into your body.
Use the dosing dispensers (oral syringes) that come with Livmarli (maralixibat) to measure your dose. Ask your care team if you're not sure how much medication to draw out for your dose. You can reuse each dosing dispenser for up to 100 days (a little more than 3 months), but make sure they're cleaned properly before each dose.
Don't use household spoons to measure out your dose of Livmarli (maralixibat). Household spoons might not be accurate and using them might cause you to take the wrong dose.
Take Livmarli (maralixibat) at least 4 hours before or 4 hours after any bile acid medications, such as cholestyramine (Prevalite), colestipol (Colestid), or colesevelam (Welchol). This will keep the medications from interacting with each other and will make sure enough Livmarli (maralixibat) gets absorbed into your body.
Tell your care team if you're taking warfarin (Coumadin). And tell the healthcare professional managing your blood thinner therapy that you're taking Livmarli (maralixibat). You might need to get your INR checked more often or have your warfarin (Coumadin) dose adjusted. Livmarli (maralixibat) can lower the amount of vitamin K in your body, which can affect how warfarin (Coumadin) works for you.
Store Livmarli (maralixibat) at room temperature. Make sure the medication is away from light by keeping it in its original container.
The Livmarli (maralixibat) bottle is good for 100 days (a little more than 3 months) once opened. After 100 days or after it expires, throw away the medication, even if there's still some left. Throw it away by mixing the medication with dirt, cat litter, or used coffee grounds. Place the mixture in a sealed plastic bag and then into your trash can at home. Make sure to remove any labels on the bottle with your personal information on it, and recycle the bottle.
If you forget to take a dose of Livmarli (maralixibat), take the missed dose as soon as you remember. If you're taking the medication once daily, you can do this as long as your next dose is more than 12 hours away. And if you're taking the medication twice daily, take your missed dose as soon as you can as long as your next dose is more than 6 hours away. If not, skip the missed dose and wait until it's time for your next dose to take the medication. Afterwards, continue your normal dosing schedule. Don't double up on the dose because this can raise your risk for side effects.
Livmarli (maralixibat) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
Risk factors: Drinking alcohol | Taking other medications that cause liver injury
Livmarli (maralixibat) can raise your liver enzymes, which can be a sign of liver damage. You'll need regular blood tests done during treatment so your care team can make sure that your liver is healthy. Avoid alcohol and other medications that might harm your liver while you're taking Livmarli (maralixibat) to prevent further liver damage. Get medical help right away if you notice symptoms of severe liver damage, such as severe nausea, vomiting blood, swelling of your stomach, or yellowing of the skin or eyes, or confusion.
Livmarli (maralixibat) commonly causes diarrhea, stomach pain, and vomiting. Make sure to stay well hydrated if you're not eating or drinking much or if you're having diarrhea. This is because you can lose too much fluid in the body, which can lead to dehydration. Symptoms of severe dehydration include being very thirsty; feeling exhausted, lightheaded, or dizzy; and not urinating as much or having dark urine.
Contact your prescriber if you have diarrhea or stomach pain that doesn't go away, bloody diarrhea, or diarrhea with vomiting while you're taking Livmarli (maralixibat). Your prescriber will consider lowering your dose. It's also possible that you might need to stop the medication for a while and then restart it at a lower dose once these side effects go away.
Livmarli (maralixibat) can lower your body's absorption of certain vitamins (A, D, E, and K). This can sometimes make you more likely to have bleeds or bone fractures. Your healthcare professional (HCP) will probably check the blood levels of these vitamins before and during treatment with Livmarli (maralixibat). If they're low, your HCP will prescribe supplements to help the levels of these vitamins go back up to a normal range.
Your prescriber will calculate your dose based on your body weight and then tell you how many milliliters (mL) of medication you should take for each dose. Each mL of the liquid contains 9.5 mg of Livmarli (maralixibat).
Alagille syndrome: The typical starting dose is 190 mcg/kg of body weight by mouth once daily, at least 30 minutes before the first meal in the morning. After 1 week, your prescriber might raise your dose to 380 mcg/kg by mouth once daily.
Progressive familial intrahepatic cholestasis: The typical starting dose is 285 mcg/kg of body weight by mouth once daily, at least 30 minutes before the first meal of the day. After 1 week, your prescriber might raise your dose to 570 mcg/kg by mouth twice daily.
Currently have or have had certain complications from liver damage (also called hepatic decompensation)
Itching (cholestatic pruritis) from:
Alagille syndrome (ALGS) in people 3 months of age and older
Progressive familial intrahepatic cholestasis (PFIC) in people 5 years of age and older
Itching in children 3 months and older with progressive familial intrahepatic cholestasis (PFIC)
By signing up, I agree to GoodRx's Terms and Privacy Policy, and to receive marketing messages from GoodRx.
Al-Dury, S., et al. (2018). Ileal bile acid transporter inhibition for the treatment of chronic constipation, cholestatic pruritus, and NASH. Frontiers in Pharmacology.
Drugs and Lactation Database (LactMed). (2023). Maralixibat. National Institute of Child Health and Human Development.
Ebhohon, E., et al. (2023). Systematic review: Efficacy of therapies for cholestatic pruritus. Therapeutic Advances in Gastroenterology.
Mansour, D., et al. (2018). Management of decompensated cirrhosis. Clinical Medicine: Journal of the Royal College of Physicians of London.
MedlinePlus. (2009). Progressive familial intrahepatic cholestasis.
MedlinePlus. (2014). Alagille syndrome.
MedlinePlus. (2023). Vitamins.
Mirium Pharmaceuticals, Inc. (2024). Mirum Pharmaceuticals’ Livmarli receives FDA approval for treatment of cholestatic pruritus in patients with progressive familial intrahepatic cholestasis. Business Wire.
Mirum Pharmaceuticals, Inc. (2024). Livmarli- maralixibat chloride solution [package insert]. DailyMed.
Mirum Pharmaceuticals, Inc. (2023). Patient information: Livmarli® (liv-MAR-lee) (maralixibat) oral solution.
National Institute of Diabetes and Digestive and Kidney Diseases. (2019). Definition & facts for Alagille syndrome.
Patel, S. P., et al. (2019). Cholestatic pruritus: Emerging mechanisms and therapeutics. Journal of the American Academy of Dermatology.
Progressive Familial Intrahepatic Cholestasis Advocacy and Resource Network, Inc. (n.d.). Learn about PFIC.
Shirley, M. (2022). Maralixibat: First approval. Drugs.
Shneider, B. L., et al. (2022). Impact of long-term administration of maralixibat on children with cholestasis secondary to Alagille syndrome. Hepatology Communications.
U.S. Food and Drug Administration. (2024). Where and how to dispose of unused medicines.
Research prescriptions and over-the-counter medications from A to Z, compare drug prices, and start saving.