Usual Adult Dose for Pruritus

Initial dose: 190 mcg/kg orally once a day
Maintenance dose: 380 mcg/kg orally once a day
Maximum dose: 28.5 mg/day (patients over 70 kg)

Comments:

• Doses should be taken 30 minutes prior to the first meal of the day.
• After the first week, the initial dose should be increased to the maintenance dose (if tolerated).
• If a missed dose is within 12 hours of the time it is usually taken, patients should take the dose as soon as possible and resume the original schedule the following day. If the missed dose is greater than 12 hours of the time it is usually taken, the dose should be omitted, and the original dosing schedule should be resumed the following day.

Use: Treatment of cholestatic pruritus in patients with Alagille syndrome (ALGS)

Usual Adult Dose for Pruritus of Partial Biliary Obstruction

Initial dose: 190 mcg/kg orally once a day
Maintenance dose: 380 mcg/kg orally once a day
Maximum dose: 28.5 mg/day (patients over 70 kg)

Comments:

• Doses should be taken 30 minutes prior to the first meal of the day.
• After the first week, the initial dose should be increased to the maintenance dose (if tolerated).
• If a missed dose is within 12 hours of the time it is usually taken, patients should take the dose as soon as possible and resume the original schedule the following day. If the missed dose is greater than 12 hours of the time it is usually taken, the dose should be omitted, and the original dosing schedule should be resumed the following day.

Use: Treatment of cholestatic pruritus in patients with Alagille syndrome (ALGS)

Usual Pediatric Dose for Pruritus

1 year and older:

• Initial dose: 190 mcg/kg orally once a day
• Maintenance dose: 380 mcg/kg orally once a day
• Maximum dose: 28.5 mg/day (patients over 70 kg)

Comments:

• Doses should be taken 30 minutes prior to the first meal of the day.
• After the first week, the initial dose should be increased to the maintenance dose (if tolerated).
• If a missed dose is within 12 hours of the time it is usually taken, patients should take the dose as soon as possible and resume the original schedule the following day. If the missed dose is greater than 12 hours of the time it is usually taken, the dose should be omitted, and the original dosing schedule should be resumed the following day.

Use: Treatment of cholestatic pruritus in patients with ALGS

Usual Pediatric Dose for Pruritus of Partial Biliary Obstruction

1 year and older:

• Initial dose: 190 mcg/kg orally once a day
• Maintenance dose: 380 mcg/kg orally once a day
• Maximum dose: 28.5 mg/day (patients over 70 kg)

Comments:

• Doses should be taken 30 minutes prior to the first meal of the day.
• After the first week, the initial dose should be increased to the maintenance dose (if tolerated).
• If a missed dose is within 12 hours of the time it is usually taken, patients should take the dose as soon as possible and resume the original schedule the following day. If the missed dose is greater than 12 hours of the time it is usually taken, the dose should be omitted, and the original dosing schedule should be resumed the following day.

Use: Treatment of cholestatic pruritus in patients with ALGS

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Patients with clinically significant portal hypertension, decompensated cirrhosis, and/or hepatic decompensation: Data not available

Baseline liver function tests (e.g., ALT, AST, direct bilirubin, INR, total bilirubin) should be obtained prior to starting treatment, and then levels should be monitored throughout treatment.

If abnormalities develop during therapy in the absence of other causes:

• Interrupt therapy until levels return to baseline.
• Treatment may be reintroduced at 190 mcg/kg orally once a day (and increase to 380 mcg/kg/day, if tolerated) if levels return to baseline or stabilize at a new baseline.
• If signs/symptoms of clinical hepatitis OR LFT abnormalities recur, discontinuation of this drug should be considered.

Hepatic decompensation events (e.g., ascites, hepatic encephalopathy, variceal hemorrhage): Treatment should be permanently discontinued.

Dose Adjustments

Gastrointestinal Adverse Events:

• Dose adjustments (e.g., a reduction in dose OR an interruption in dosing) should be considered in patients who develop gastrointestinal adverse reactions in the absence of alternative etiologies.
• Dehydration should be monitored for and treated promptly in patients with diarrhea and/or vomiting.
• An interruption of treatment should be considered in patients with persistent diarrhea or in those who have diarrhea with bloody stool, dehydration requiring treatment, fever, and/or vomiting.
• Treatment may be reintroduced at 190 mcg/kg orally once a day (and increase to 380 mcg/kg/day, if tolerated) when gastrointestinal reactions resolve.
• If signs/symptoms recur, discontinuation of this drug should be considered.

Fat-Soluble Vitamin (FSV) Deficiency:

• Baseline FSV levels should be obtained prior to starting treatment, and levels should be monitored throughout treatment.
• Patients should undergo supplementation with FSVs if a deficiency is diagnosed.
• Treatment discontinuation should be considered in patients with persistent or worsening FSV deficiency despite supplementation.

Precautions

CONTRAINDICATIONS: None.

Safety and efficacy have not been established in patients younger than 1 year.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• Administration of bile acid resins should be separated by at least 4 hours (if applicable).
• This drug should be taken 30 minutes before the first meal of the day.

General:

• Any remaining solution should be discarded after 45 days of opening.

Monitoring:

• HEPATIC: Liver function tests at baseline, then monitor routinely
• METABOLIC: Serum fat soluble vitamin levels at baseline, then monitor routinely

Patient advice:

• Advise patients to speak to their healthcare provider if they become pregnant, intend to become pregnant, or are breastfeeding.