Maralixibat

Maralixibat is a prescription medication used for the treatment of cholestatic pruritus (itching) in patients with Alagille syndrome. 

• Maralixibat is available under the following different brand names: Livmarli.

Common side effects of Maralixibat include:

• Diarrhea,
• Abdominal pain,
• Vomiting,
• Fat-soluble vitamin deficiency (a, d, e, or k deficiency), and
• Increased transaminases

Serious side effects of Maralixibat include:

• Hives,
• Difficulty breathing,
• Swelling of the face, lips, tongue, or throat,
• Changes or worsening liver test results,
• Nausea,
• Vomiting,
• Yellowing of the skin or eyes (jaundice),
• Dark or brown urine,
• Pain in the right side of the abdomen,
• Loss of appetite,
• Diarrhea,
• Stomach pain,
• Bone fractures, and
• Gastrointestinal bleeding

Rare side effects of Maralixibat include:

• None 

Seek medical care or call 911 at once if you have the following serious side effects:

• Severe headache, confusion, slurred speech, arm or leg weakness, trouble walking, loss of coordination, feeling unsteady, very stiff muscles, high fever, profuse sweating, or tremors;
• Serious eye symptoms such as sudden vision loss, blurred vision, tunnel vision, eye pain or swelling, or seeing halos around lights;
• Serious heart symptoms include fast, irregular, or pounding heartbeats; fluttering in the chest; shortness of breath; sudden dizziness, lightheadedness, or passing out.

This is not a complete list of side effects and other serious side effects or health problems that may occur because of the use of this drug. Call your doctor for medical advice about serious side effects or adverse reactions. You may report side effects or health problems to FDA at 1-800-FDA-1088.

Adult and pediatric dosage

Oral solution

• 9.5 mg/ml

Cholestatic Pruritus

Adult and pediatric dosage

• Days 1-7: 190 mcg/kg orally once a day
• Beginning Day 8: Increase to 380 mcg/kg orally once a day, as tolerated; not to exceed 28.5 mg/day for patients weighing above 70 kg.
• Administer 30 minutes before the first meal of the day.

Dosage Considerations – Should be Given as Follows: 

• See “Dosages”

If your medical doctor is using this medicine to treat your pain, your doctor or pharmacist may already be aware of any possible drug interactions and may be monitoring you for them. Do not start, stop, or change the dosage of any medicine before checking with your doctor, health care provider, or pharmacist first.

• Maralixibat has severe interactions with no other drugs.
• Maralixibat has serious interactions with no other drugs.
• Maralixibat has moderate interactions with no other drugs.
• Maralixibat has minor interactions with no other drugs.

This information does not contain all possible interactions or adverse effects. Visit the RxList Drug Interaction Checker for any drug interactions. Therefore, before using this product, tell your doctor or pharmacist about all your products. Keep a list of all your medications with you and share this information with your doctor and pharmacist. Check with your healthcare professional or doctor for additional medical advice, health questions, or concerns.

Contraindications

• None

Effects of drug abuse

• None

Short-Term Effects

• See “What Are Side Effects Associated with Using Maralixibat?”

Long-Term Effects

• See “What Are Side Effects Associated with Using Maralixibat?”

Cautions

• Diarrhea, abdominal pain and vomiting were reported as the most common adverse reactions; dosage interruption and/or discontinuation may be necessary.
• Liver test abnormalities
  • Patients with Alagille syndrome often present with jaundice or paucity of intrahepatic bile ducts.
  • Obtain baseline liver tests and monitor during treatment.
  • Consider dose reduction or treatment interruption if hepatic abnormalities occur in the absence of other causes.
  • For persistent or recurrent liver test abnormalities, consider treatment discontinuation.
• Drug interaction overview
  • Bile acid–resins
    • Administer maralixibat and bile acid binding resins 4 hours apart.
    • Bile acid binding resins (. g, cholestyramine, colesevelam, colestipol) may bind to maralixibat in the gut.
  • OATB2B1 substrates
    • Monitor the effects of OATP2B1 substrates as needed.
    • Maralixibat is an OATP2B1 inhibitor based on in vitro studies and may decrease the oral absorption of OATP2B1 substrates (. g, statins) owing to OATP2B1 inhibition in the gastrointestinal tract.
  • Fat-soluble vitamins
    • Monitor for vitamin deficiency and supplement as needed.
    • Consider discontinuing maralixibat if deficiency persists or worsens despite adequate supplementation.
    • Maralixibat may affect the absorption of fat-soluble vitamins (. g, vitamins A, D, E, K); patients with Alagille syndrome can have fat-soluble vitamin deficiency at baseline.

Pregnancy and Lactation

• Maternal use at the recommended dose is not expected to result in measurable fetal exposure owing to low systemic absorption following oral administration.
• No developmental effects were observed in animal reproduction studies.
• Clinical considerations
  • Maralixibat may inhibit fat-soluble vitamin absorption.
  • Monitor for deficiency and supplement as needed.
  • Increased supplementation of vitamins may be needed during pregnancy.
• Lactation
  • Low absorption following oral administration; breastfeeding is not expected to result in exposure to the infant at the recommended dose.
  • Data are not available on the presence of drugs in human milk, effects on breastfed infants, or effects on milk production.
  • As with pregnancy, consider supplementation with fat-soluble vitamins.