LIVMARLI Oral solution Ref.[50565] Active ingredients: Maralixibat

Treatment with Livmarli should be initiated under the supervision of a physician experienced in the management of patients with cholestatic liver diseases.

Posology

The recommended target dose is 380 mcg/kg once daily. The starting dose is 190 mcg/kg once daily and should be increased to 380 mcg/kg once daily after one week. Table 1 provides the dose in mL of solution to be given for each weight range. In case of poor tolerability, dose reduction from 380 mcg/kg/day to 190 mcg/kg/day, or treatment interruption can be considered. Renewed dose-escalation can be attempted as tolerated. The maximum recommended daily dose for patients above 70 kg is 3 mL (28.5 mg).

Table 1. Individual dose volume by patient weight:

Alternative treatment should be considered in patients for whom no treatment benefit can be established following 3 months of continuous daily treatment with maralixibat.

Missed dose

If a dose is missed, but it can be taken within 12 hours of the regular schedule, it should be taken as soon as possible. If a dose is missed by more than 12 hours, the dose should be omitted, and the original dose schedule resumed the following day.

Special populations

Renal impairment

Maralixibat has not been studied in patients with renal impairment or end-stage renal disease (ESRD) requiring haemodialysis. However, due to the minimal plasma concentrations and negligible renal excretion, no dose adjustment is required for these patients (see section 5.2).

Hepatic impairment

Maralixibat has not been sufficiently studied in patients with liver impairment. Due to minimal absorption, no dose adjustment is required for patients with hepatic impairment. Close monitoring is, however, advised for patients with end-stage liver disease or progression to decompensation.

Paediatric population

The safety and efficacy of Livmarli in infants less than 2 months of age have not been established. No data are available.

Method of administration

Livmarli is administered orally via an oral syringe by a caregiver or the patient, before (up to 30 minutes) or with a meal, in the morning.

Mixing Livmarli oral solution directly into food or drink prior to administration has not been studied and should be avoided.

Three sizes of oral syringe (0.5 mL, 1 mL and 3 mL) are provided with each bottle of Livmarli. Table 1 provides the correct oral syringe size for each weight range.

Maralixibat is minimally absorbed from the gastrointestinal tract and overdose is not expected to result in high plasma levels of the active substance. Single doses of up to 500 mg, approximately 18-fold higher than the recommended dose, have been administered in healthy adults without any adverse consequences.

In the event of an overdose, general supportive measures should be followed and the patient should be monitored for signs and symptoms of adverse reactions (see section 4.8).

2 years.

After first opening: After the first opening of the bottle, the medicinal product must be used within 100 days stored below 30°C. Then the bottle and its contents have to be discarded, even if not empty

This medicinal product does not require any special temperature storage conditions. Store in the original package in order to protect from light.

For storage conditions after first opening of the medicinal product, see section 6.3.

30 mL amber-coloured PET bottle with a preinstalled LDPE adapter and a HDPE child-resistant closure with a foam liner, containing 30 mL oral solution.

Pack size:

Each pack contains one 30 mL bottle and is co-packaged with three oral repeated-use syringes (0.5 mL, 1 mL and 3 mL) with the following graduations:

• 0.5 mL polypropylene syringe with a white plunger: numbers for each 0.1 mL, major hash marks for 0.05 mL increments, and minor hash marks for 0.01 mL increments.
• 1 mL polypropylene syringe with a white plunger: numbers for each 0.1 mL increment.
• 3 mL polypropylene syringe with a white plunger: numbers for each 0.5 mL increment, and hash marks for each 0.25 mL increment between 0.5 mL and 3 mL.

The oral syringes may be rinsed with water, air dried and reused for 100 days.

Any unused medicinal product or waste material should be disposed of in accordance with local requirements.