Select a medication above to begin.
Juxtapid
lomitapide
Black Box Warnings .
Appropriate Use
restricted distribution program (JUXTAPID REMS) due to hepatotoxicity risk; prescribers, pharmacies must be certified and enroll at 1-855-898-2743 or www.juxtapidremsprogram.com; prescribe only to patients with clinical or laboratory diagnosis consistent with homozygous familial hypercholesterolemia; safety and efficacy not established in other hypercholesterolemia patients
Hepatotoxicity Risk
ALT/AST incr. >3x ULN in 34% of adult patients and 14% of pediatric patients in clinical trials; no clinically meaningful incr. in total bilirubin, INR, alk phos observed; median hepatic fat incr. from 1% to 6% in adult patients after 26wk and 78wk of tx, and from 3% to 4% in patients 5-17 yo after 24wk and 104wk of tx; lomitapide-associated hepatic steatosis may be risk factor for progressive hepatic disease, including steatohepatitis and cirrhosis; monitor ALT/AST, alk phos, and total bilirubin at baseline, then at least ALT/AST regularly as recommended; adjust dose if ALT/AST >3x ULN; D/C tx for clinically significant hepatotoxicity
Adult Dosing .
Dosage forms: CAP: 2 mg, 5 mg, 10 mg, 20 mg, 30 mg
Restricted Distribution in US
- [1-855-898-2743 or www.juxtapidremsprogram.com for more info]
homozygous familial hypercholesterolemia
- [5-60 mg PO qd]
- Start: 5 mg PO qd x2wk, then may double dose q4wk; Max: 60 mg/day; Info: see pkg insert for dose adjustments based on ALT/AST; give >2h after evening meal; may open cap, but do not crush/chew/dissolve contents; fat-soluble vitamin supplementation recommended, see pkg insert
renal dosing
- [see below]
- renal impairment: not defined
- HD: max 40 mg/day; supplement after dialysis not defined; PD: not defined
hepatic dosing
- [see below]
- Child-Pugh Class A: max 40 mg/day; Child-Pugh Class B or C: contraindicated
Peds Dosing .
- Dosage forms: CAP: 2 mg, 5 mg, 10 mg, 20 mg, 30 mg
Restricted Distribution in US
- [1-855-898-2743 or www.juxtapidremsprogram.com for more info]
homozygous familial hypercholesterolemia
- [2-10 yo]
- Dose: 2-20 mg PO qd; Start: 2 mg PO qd x8wk, then may incr. to 5 mg PO qd x4wk, then may double dose q4wk; Max: 20 mg/day; Info: see pkg insert for dose adjustments based on ALT/AST; give >2h after evening meal; may open cap, but do not crush/chew/dissolve contents; fat-soluble vitamin supplementation recommended, see pkg insert
- [11-15 yo]
- Dose: 2-40 mg PO qd; Start: 2 mg PO qd x4wk, then may incr. to 5 mg PO qd x4wk, then may double dose q4wk; Max: 40 mg/day; Info: see pkg insert for dose adjustments based on ALT/AST; give >2h after evening meal; may open cap, but do not crush/chew/dissolve contents; fat-soluble vitamin supplementation recommended, see pkg insert
- [16-17 yo]
- Dose: 5-40 mg PO qd; Start: 5 mg PO qd x4wk, then may double dose q4wk; Max: 40 mg/day; Info: see pkg insert for dose adjustments based on ALT/AST; give >2h after evening meal; may open cap, but do not crush/chew/dissolve contents; fat-soluble vitamin supplementation recommended, see pkg insert
renal dosing
- [2-10 yo]
- renal impairment: not defined
- HD: max 15 mg/day; supplement after dialysis not defined; PD: not defined
- [11-17 yo]
- renal impairment: not defined
- HD: max 30 mg/day; supplement after dialysis not defined; PD: not defined
hepatic dosing
- [2-10 yo]
- Child-Pugh Class A: max 15 mg/day; Child-Pugh Class B or C: contraindicated
- [11-17 yo]
- Child-Pugh Class A: max 30 mg/day; Child-Pugh Class B or C: contraindicated