This medication is used to treat skin itching caused by certain inherited liver conditions (progressive familial intrahepatic cholestasis-PFIC, Alagille syndrome-ALGS). In these conditions, a substance that your body normally makes (bilirubin) builds up in the liver and causes jaundice. Maralixibat belongs to a class of drugs known as ileal bile acid transporter inhibitors. It helps to lessen symptoms of jaundice such as itching, yellowing eyes/skin, dark urine, and pale-colored stools.

Read the Patient Information Leaflet if available from your pharmacist before you start using maralixibat and each time you get a refill. If you have any questions, ask your doctor or pharmacist.

Take this medication by mouth 30 minutes before a meal as directed by your doctor, usually either once daily (in the morning) or twice daily (once in the morning and once in the evening). The dosage is based on your medical condition, weight, and response to treatment.

To reduce your risk of side effects, your doctor may direct you to start this medication at a low dose and gradually increase your dose. Follow your doctor's instructions carefully.

Use this medication regularly to get the most benefit from it. To help you remember, take it at the same time(s) each day.

Do not increase your dose or use this drug more often or for longer than prescribed. Your condition will not improve any faster, and your risk of side effects will increase.

If you are also taking bile acid-binding resins such as cholestyramine, colesevelam, or colestipol, take this medication at least 4 hours before or at least 4 hours after taking these medications. These medications can bind to maralixibat, preventing your body from fully absorbing the drug.

Tell your doctor if your condition does not get better or if it gets worse.

Diarrhea, vomiting, or abdominal pain may occur. If any of these effects last or get worse, tell your doctor or pharmacist promptly.

Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.

Vomiting/diarrhea that doesn't stop may result in dehydration. Contact your doctor promptly if you notice any symptoms of dehydration, such as unusual decreased urination, unusual dry mouth/thirst, fast heartbeat, or dizziness/lightheadedness.

This medication may lower your fat-soluble vitamin levels, and that may increase your risk for bone fractures and bleeding. Talk to your doctor for more details.

Although this medication is used to treat liver problems, it may sometimes cause liver tests to worsen. Tell your doctor right away if you have symptoms of worsening liver problems such as nausea/vomiting that doesn't stop, loss of appetite, yellowing eyes/skin, or dark urine.

Tell your doctor right away if you have any serious side effects, including:

• abdominal pain that won't go away
• blood/mucus in stools
• fever

A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including:

• rash
• itching/swelling (especially of the face/tongue/throat)
• severe dizziness
• trouble breathing

This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.

In the US - Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.

In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

Before taking maralixibat, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.

Before using this medication, tell your doctor or pharmacist your medical history, especially of:

• other liver problems (such as ascites, encephalopathy)

Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).

During pregnancy, this medication should be used only when clearly needed. It may harm an unborn baby. Discuss the risks and benefits with your doctor.

It is unknown if this medication passes into breast milk. Consult your doctor before breastfeeding.

See also How to Use section.

Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.

If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call 1-800-222-1222. Canada residents can call 1-844-764-7669.

Do not share this medication with others.

Lab and/or medical tests (such as liver function, fat-soluble vitamin levels) should be done before starting this medication and while you are taking it. Keep all medical and lab appointments.

If you are taking this medication once daily and you miss a dose, take it as soon as you remember. If it is more than 12 hours after the missed dose, skip the missed dose. Take your next dose at the regular time. Do not double the dose to catch up.

If you are taking this medication twice daily and you miss a dose, take it as soon as you remember. If it is more than 6 hours after the missed dose, skip the missed dose. Take your next dose at the regular time. Do not double the dose to catch up.

Store at room temperature away from light and moisture. Do not store in the bathroom. Keep all medications away from children and pets.

Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.