"The U.S. Food and Drug Administration today approved Kynamro (mipomersen sodium) injection as an addition to lipid-lowering medications and diet to treat patients with a rare type of high cholesterol called homozygous familial hypercholesterolemi"...
Homozygous Familial Hypercholesterolemia
JUXTAPID is indicated as an adjunct to a low-fat diet and other lipid-lower in g treatments, including LDL apheresis where available, to reduce low-density lipoprotein cholesterol (LDLC), total cholesterol (TC), apolipoprote in B (apo B), and non-high-density lipoprotein cholesterol (non-HDL-C) in patients with homozygous familial hypercholesterolemia (HoFH).
Limitations Of Use
DOSAGE AND ADMINISTRATION
Initiation And Maintenance Of Therapy
Before beginning treatment with JUXTAPID:
- Measure transaminases (ALT, AST), alkaline phosphatase, and total bilirubin [see WARNINGS AND PRECAUTIONS];
- Obtain a negative pregnancy test in females of reproductive potential [see WARNINGS AND PRECAUTIONS]; and,
- Initiate a low-fat diet supplying < 20% of energy from fat [see WARNINGS AND PRECAUTIONS].
The recommended starting dosage of JUXTAP ID is 5 mg once daily, and the dose should be escalated gradually based on acceptable safety and tolerability. Transaminases should be measured prior to any increase in dose [see WARNINGS AND PRECAUTIONS]. The maintenance dosage of JUXTAP ID should be individualized, taking into account patient characteristics such as goal of therapy and response to treatment, to a maximum of 60 mg daily as described in Table 1. Modify dosing for patients taking concomitant weak CYP 3A4 inhibitors and for those with renal impairment or baseline hepatic impairment [see below]. Monitor transaminases during treatment with JUXTAP ID as described in WARNINGS AND PRECAUTIONS, and reduce or withhold dosing for patients who develop transaminase values ≥ 3x the upper limit of normal (ULN) [see below].
Table 1: Recommended Regimen for Titrating Dosage
|Dosage||Duration of Administration Before Cons idering Increase to Next Dosage|
|5 mg daily||At least 2 weeks|
|10 mg daily||At least 4 weeks|
|20 mg daily||At least 4 weeks|
|40 mg daily||At least 4 weeks|
|60 mg daily||Maximum recommended dosage|
To reduce the risk of developing a fat-soluble nutrient deficiency due to JUXTAPID's mechanism of action in the small intestine, patients treated with JUXTAP ID should take daily supplements that contain 400 international units vitamin E and at least 200 mg linoleic acid, 210 mg alpha-linolenic acid (ALA), 110 mg eicosapentaenoic acid (EPA), and 80 mg docosahexaenoic acid (DHA) [see WARNINGS AND PRECAUTIONS].
JUXTAPID should be taken once daily with a glass of water, without food, at least 2 hours after the evening meal because administration with food may increase the risk of gastrointestinal adverse reactions [see WARNINGS AND PRECAUTIONS]. Patients should swallow JUXTAPID capsules whole. Capsules should not be opened, crushed, dissolved, or chewed.
Dosing With Cytochrome P450 3A4 Inhibitors
The recommended maximum dosage of JUXTAPID is 30 mg daily with concomitant use of weak CYP3A4 inhibit ors (such as alprazolam, amiodarone, amlodipine, atorvastatin, bicalutamide, cilostazol, cimetidine, cyclosporine, fluoxetine, fluvoxamine, ginkgo, goldenseal, isoniazid, lapatinib, nilotinib, pazopanib, ranitidine, ranolazine, ticagrelor, zileuton). However, the recommended maximum dosage of JUXTAPID is 40 mg daily with concomitant use of oral contraceptives.
When initiatin g a weak CYP3A4 inhibit or in a patient already taking JUXTAPID 10 mg daily or more, decrease the dose of JUXTAPID by half; patients taking JUXTAPID 5 mg daily may continue with the same dosage. Careful titration of JUXTAPID may then be considered according to LDL-C response and safety/tolerabilit y to a maximum recommended dosage of 30 mg daily except when coadministered with oral contraceptives, in which case the maximum recommended lomitapide dosage is 40 mg daily [see DRUG INTERACTIONS].
Dose Modification Based on Elevated Transaminases
Table 2 summarizes recommendations for dose adjustment and monitoring for patients who develop elevated transaminases during therapy with JUXTAPID [see WARNINGS AND PRECAUTIONS].
Table 2: Dose Adjustment and Monitoring for Patients
with Elevated Transaminases
|ALT or AST||Treatment and monitoring recommendations *|
|≥ 3x and < 5x ULN||
|≥ 5x ULN||
|*Recommendations based on an ULN of approximately 30-40 international units/L.|
If transaminase elevations are accompanied by clinical symptoms of liver injury (such as nausea, vomiting, abdominal pain, fever, jaundice, lethargy, flu-like symptoms), increases in bilirubin ≥ 2x ULN, or active liver disease, discontinue treatment with JUXTAPID and investigate to identify the probable cause [see WARNINGS AND PRECAUTIONS].
Dosing In Patients With Renal Impairment
Patients with end-stage renal disease receiving dialysis should not exceed 40 mg daily. There are no data available to guide dosing in other patients with renal impairment [see Use in Specific Populations].
Dosing In Patients With Baseline Hepatic Impairment
Patients with mild hepatic impairment (Child-Pugh A) should not exceed 40 mg daily [see Use in Specific Populations].
Dosage Forms And Strengths
5 mg: Orange/orange hard gelatin capsule printed with black ink “A733” and “5 mg”
10 mg: Orange/white hard gelatin capsule printed with black ink “A733” and “10 mg”
20 mg: White/white hard gelatin capsule printed with black ink “A733” and “20 mg”
30 mg: Orange/yellow hard gelatin capsule printed with black ink “A733” and “30 mg”
40 mg: Yellow/white hard gelatin capsule printed with black ink “A733” and “40 mg”
60 mg: Yellow/yellow hard gelatin capsule printed with black ink “A733” and “60 mg”
Storage And Handling
5 mg Capsules
Orange/orange hard gelatin capsule printed with black ink “A733” and “5 mg” Bottles of 28 NDC 76431-105-01
10 mg Capsules
Orange/white hard gelatin capsule printed with black ink “A733” and “10 mg” Bottles of 28 NDC 76431-110-01
20 mg Capsules
White/white hard gelatin capsule printed with black ink “A733” and “20 mg” Bottles of 28 NDC 76431-120-01
30 mg Capsules
Orange/yellow hard gelatin capsule printed with black ink “A733” and “30 mg” Bottles of 28 NDC 76431-130-01
40 mg Capsules
Yellow/white hard gelatin capsule printed with black ink “A733” and “40 mg” Bottles of 28 NDC 76431-140-01
60 mg Capsules
Yellow/yellow hard gelatin capsule printed with black ink “A733” and “60 mg” Bottles of 28 NDC 76431-160-01
Store at 20°C to 25°C (68°F to 77°F); excursions permitted between 15°C and 30°C (between 59°F and 86°F). Brief exposure to temperatures up to 40°C (104°F) may be tolerated provided the mean kinetic temperature does not exceed 25°C (77°F); however, such exposure should be minimized. Keep container tightly closed and protect from moisture.
Manufactured for: Aegerion Pharmaceuticals, Inc., Cambridge, M A 02142. Revised: May 2016This monograph has been modified to include the generic and brand name in many instances.
Last reviewed on RxList: 5/31/2016
Additional Juxtapid Information
Report Problems to the Food and Drug Administration
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.
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