LIVMARLI 9.5 MG — DIN 02539888

What is LIVMARLI?

LIVMARLI 9.5 MG contains MARALIXIBAT (MARALIXIBAT CHLORIDE) : 9.5 MG. It is listed in the Health Canada Drug Product Database under DIN 02539888, held by MIRUM PHARMACEUTICALS, INC., supplied as SOLUTION for ORAL administration. Health Canada currently lists it as marketed, status dated 11-Aug-2023. Nexara Health is a licensed Canadian wholesale distributor (DEL 3-002896-A) and can source this product for hospitals, pharmacies and clinics. Availability and lead time are confirmed per order.

What is LIVMARLI used for?

LIVMARLI (maralixibat oral solution and tablets) is indicated for: • the treatment of cholestatic pruritus in patients aged 12 months or older with Alagille syndrome (ALGS). • the treatment of cholestatic pruritus in patients aged 12 months or older with progressive familial intrahepatic cholestasis (PFIC). Limitations of Use: LIVMARLI may not be effective in a subgroup of PFIC type 2 patients with specific ABCB11 variants resulting in non-functional or complete absence of the bile salt export pump protein.

Authorized indication as written in the Health Canada product monograph. For healthcare professionals and licensed purchasers; this is not medical advice.

How is it administered?

Administer LIVMARLI oral solution or tablets 30 minutes before a meal in the morning for once daily dosing, or in the morning and evening for twice daily dosing. LIVMARLI oral solution is to be administered orally via an oral syringe by a caregiver or the patient. A calibrated measuring device (0.5 mL, 1 mL or 3 mL oral dosing dispenser) for LIVMARLI oral solution will be provided by the pharmacy to measure and deliver the prescribed dose accurately. Recommended Dose for Alagille Syndrome: The recommended maintenance dosage of LIVMARLI is 380 mcg/kg taken once daily in the morning. LIVMARLI should be initiated at a dose of 190 mcg/kg orally once daily. If tolerated, LIVMARLI may be increased to 380 mcg/kg once daily after one week. The maximum daily dose volume for patients above 70 kg is 3 mL or 28.5 mg per day for LIVMARLI oral solution. For LIVMARLI tablets, the maximum daily dose for patients above 66 kg is 30 mg. Refer to dosing by weight guidelines presented in LIVMARLI Oral Solution. Recommended Dose for Progressive Familial Intrahepatic Cholestasis: The recommended dosage is 570 mcg/kg twice daily 30 minutes before a meal. The starting dose is 285 mcg/kg orally once daily (QD) in the morning and should be increased to 285 mcg/kg twice daily (BID), 428 mcg/kg twice daily, and then to 570 mcg/kg twice daily, as tolerated. The maximum daily dose volume for patients above 35 kg is 2 ml (38 mg) for LIVMARLI oral solution. For LIVMARLI tablets the maximum daily dose for patients above 44 kg is 40 mg. Refer to the dosing by weight guidelines presented in Table 3 (19 mg/mL oral solution) and Table 4 (tablet). Missed Dose: For once daily dosing: If a dose is missed, it should be taken as soon as possible within 12 hours of the time it is usually taken, with the original dosing schedule then to be resumed. If a dose is missed by more than 12 hours, the dose can be omitted and the original dosing schedule resumed. For twice daily dosing: If a dose is missed, it should be taken as soon as possible within 6 hours of the time it is usually taken, and the original dosing schedule should be resumed. If a dose is missed by more than 6 hours, the dose can be omitted and the original dosing schedule resumed.

Included because it drives pack selection and wastage. Consult the monograph.

Contraindications

LIVMARLI is contraindicated in patients with prior or active hepatic decompensation events (e.g., variceal hemorrhage, ascites, hepatic encephalopathy). LIVMARLI is contraindicated in patients who are hypersensitive to this drug or to any ingredient in the formulation, including any non-medicinal ingredient, or component of the container.

Warnings and precautions

Hepatic/Biliary/Pancreatic: LIVMARLI is contraindicated in patients with prior or active hepatic decompensation events. The safety and efficacy of LIVMARLI have not been studied in such patients. In ALGS and PFIC clinical trials, serum ALT elevations were observed in patients receiving maralixibat treatment. Elevations in serum bilirubin beyond those at baseline were also observed. Liver function tests should be monitored in patients before and during treatment with maralixibat. Treatment with LIVMARLI was associated with an increased incidence of liver enzyme elevations compared to placebo. Evaluate liver enzymes before initiating LIVMARLI and monitor thereafter as needed and clinically required. Liver enzyme elevations should be investigated for potential cases of DILI, and if necessary, and depending on the severity of the findings, LIVMARLI should be either interrupted or the dose reduced. However, LIVMARLI should be discontinued if the findings (liver tests elevation or clinical signs) are persistent or recurrent, or if hepatitis develops when treatment is resumed, or if the patient experience hepatic decompensation. Risk of Propylene Glycol Toxicity (Pediatric Patients Less Than 5 Years of Age): LIVMARLI oral solution contains propylene glycol. Patients less than 5 years of age are at highest risk for propylene glycol toxicity, and a safe level for propylene glycol exposure with repeated administration has not been established for pediatric patients less than 5 years of age. Monitor patients for signs of potential propylene glycol toxicity, including hemolysis, hyperosmolarity with anion gap metabolic acidosis, acute kidney injury, and central nervous system toxicity. Discontinue LIVMARLI oral solution if propylene glycol toxicity is suspected.