maralixibat

Contraindicated in:

Patients with progressive familial intrahepatic cholestasis type 2 with specific ABCB11 variants resulting in nonfunctional or complete absence of bile salt export pump protein;
Prior or active hepatic decompensation events (e.g., variceal hemorrhage, ascites, hepatic encephalopathy).

Use Cautiously in:

Clinically significant portal hypertension or decompensated cirrhosis;
OB: May inhibit absorption of fat soluble vitamins during pregnancy;
Pedi: Children <5 yr at ↑ risk for propylene glycol toxicity (for Alagille syndrome, at dose of 0.38 mg/kg once daily, exposure to propylene glycol is 14.6 mg/kg/day; for progressive familial intrahepatic cholestasis, at dose of 0.57 mg/kg twice daily, exposure to propylene glycol is 21.9 mg/kg/day);
Pedi: Safety and effectiveness not established in children <1 yr (progressive familial intrahepatic cholestasis) or <3 mo (Alagille syndrome);
Geri: Safety and effectiveness not established in older adults.

Adv. Reactions/Side Effects ⬆ ⬇

GI: ↑liver enzymes, abdominal pain, diarrhea, HEPATOTOXICITY, vomiting, hyperbilirubinemia, nausea

Hemat: BLEEDING

MS: fractures

Misc: fat soluble vitamin deficiency (vitamins A, D, E, and K)

Interactions ⬆ ⬇

Drug-drug:

Bile acid resins, including cholestyramine, colesevelam, and colestipol, may bind to maralixibat in the gut and ↓ its effectiveness; administer bile acid resins ≥4 hr before or after maralixibat.
May ↓ absorption of OATP2B1 substrates, including statins.

Availability ⬆ ⬇

Oral solution grape flavor (contains propylene glycol): 9.5 mg/mL; 19 mg/mL

Route/Dosage ⬆ ⬇

Use 9.5 mg/mL oral solution for treatment of Alagille syndrome and 19 mg/mL oral solution for treatment of progressive familial intrahepatic cholestasis. Do NOT interchange different concentrations of oral solution.

Alagille Syndrome

PO (Adults and Children ≥3 mo): 9.5 mg/mL oral solution: 0.19 mg/kg (max = 14.25 mg) once daily for 7 days; then ↑ to target dose of 0.38 mg/kg (max = 28.5 mg) once daily.

Progressive Familial Intrahepatic Cholestasis

PO (Adults and Children ≥1 yr): 19 mg/mL oral solution: 0.285 mg/kg once daily (max = 38 mg/day); then ↑ to 0.285 mg/kg twice daily (max = 38 mg/day), 0.428 mg/kg twice daily (max = 38 mg/day), and then to 0.57 mg/kg twice daily (max = 38 mg/day), as tolerated.

US Brand Names ⬆ ⬇

Action ⬆ ⬇

Acts as a reversible inhibitor of the ileal bile acid transporter, which results in a reduction in the reabsorption of bile acids from the terminal ileum.

Therapeutic effects:

Reduction in the severity of pruritus.

Classifications ⬆ ⬇

Therapeutic Classification: none assigned

Pharmacologic Classification: temporary class

Pharmacokinetics ⬆ ⬇

Absorption: Minimally absorbed into the systemic circulation.

Distribution: Unknown.

Protein Binding: 91%.

Metabolism/Excretion: Primarily excreted in the feces as unchanged drug.

Half-Life: 1.6 hr.

Time/Action Profile ⬆ ⬇

(plasma concentrations)

ROUTE	ONSET	PEAK	DURATION
PO	unknown	45 min	unknown

Patient/Family Teaching ⬆ ⬇

Explain purpose and side effects of medication. Counsel patient and caregiver to take drug as directed 30 min prior to a meal once or twice daily, using included calibrated measuring device. Once-daily dose: If missed, it should be taken ≤12 hr of the time it is usually taken. Twice-daily dose: If missed, it should be taken ≤6 hr of the time it is usually taken. Advise patient to read Patient Information before starting therapy.
Instruct patient and caregiver to promptly report symptoms of hepatotoxicity (fatigue, yellowing of the skin or eyes, abdominal pain, bloating, nausea, vomiting, dark urine, light-colored stools).
Advise patient and caregiver to report persistent abdominal pain, diarrhea, loss of appetite, nausea, or vomiting.
Advise patient to report any symptoms of bleeding or fractures.
Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Rep: Advise women of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. ↑ supplementation of fat-soluble vitamins may be needed during pregnancy.

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Indications ⬇

Contraind./Precautions ⬆ ⬇