Juxtapid 10mg Capsules
WARNING: This drug may cause liver problems in some patients. Blood tests will be needed to watch for any liver problems. Talk with the doctor. @ COMMON USES: It is used to lower cholesterol.
Drug Class
Lipid-lowering agent
Pharmacologic Class
Microsomal Triglyceride Transfer Protein (MTP) Inhibitor
Pregnancy Category
X
FDA Approved
Dec 2012
DEA Schedule
Not Controlled
Overview
What is this medicine?
Juxtapid is a medicine used to lower very high cholesterol levels, especially 'bad' cholesterol (LDL-C), in people with a rare inherited condition called homozygous familial hypercholesterolemia (HoFH). It works by blocking a protein in your liver and gut that helps make cholesterol particles.
How to Use This Medicine
Taking Your Medication Correctly
To get the most benefit from your medication, follow your doctor's instructions carefully. Read all the information provided to you and follow the instructions closely.
Take your medication at least 2 hours after your evening meal to minimize the risk of an upset stomach.
Do not take your medication with food, as this can cause stomach upset.
Take your medication with a full glass of water to help it go down smoothly.
Swallow the tablet whole; do not chew, break, open, or dissolve it, as this can affect how the medication works.
Storing and Disposing of Your Medication
To keep your medication effective and safe:
Store it at room temperature in a dry place, away from moisture.
Do not store your medication in a bathroom, as the humidity can affect its potency.
Keep the lid tightly closed to maintain the medication's effectiveness.
Keep all medications in a safe place, out of the reach of children and pets, to avoid accidental ingestion.
What to Do If You Miss a Dose
If you miss a dose, skip it and go back to your normal dosing schedule.
Do not take two doses at the same time or take extra doses, as this can increase the risk of side effects.
If you stop taking your medication for more than 1 week, consult with your doctor before restarting, as this may require adjustments to your treatment plan.
To get the most benefit from your medication, follow your doctor's instructions carefully. Read all the information provided to you and follow the instructions closely.
Take your medication at least 2 hours after your evening meal to minimize the risk of an upset stomach.
Do not take your medication with food, as this can cause stomach upset.
Take your medication with a full glass of water to help it go down smoothly.
Swallow the tablet whole; do not chew, break, open, or dissolve it, as this can affect how the medication works.
Storing and Disposing of Your Medication
To keep your medication effective and safe:
Store it at room temperature in a dry place, away from moisture.
Do not store your medication in a bathroom, as the humidity can affect its potency.
Keep the lid tightly closed to maintain the medication's effectiveness.
Keep all medications in a safe place, out of the reach of children and pets, to avoid accidental ingestion.
What to Do If You Miss a Dose
If you miss a dose, skip it and go back to your normal dosing schedule.
Do not take two doses at the same time or take extra doses, as this can increase the risk of side effects.
If you stop taking your medication for more than 1 week, consult with your doctor before restarting, as this may require adjustments to your treatment plan.
Lifestyle & Tips
- Follow a strict low-fat diet (less than 20% of energy from fat) as prescribed by your doctor. This is crucial to minimize gastrointestinal side effects and liver fat accumulation.
- Take daily supplements of fat-soluble vitamins (A, D, E, K) and essential fatty acids (linoleic acid, alpha-linolenic acid, eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA)) as directed by your doctor.
- Avoid grapefruit and grapefruit juice, as they can increase the levels of Juxtapid in your body.
Available Forms & Alternatives
Available Strengths:
Dosing & Administration
Adult Dosing
Standard Dose:
Initial 5 mg once daily. Titrate gradually based on tolerability and LDL-C response.
Dose Range:
5 - 60 mg
Condition-Specific Dosing:
initialDose:
5 mg once daily
titration:
Increase to 10 mg after at least 2 weeks, then to 20 mg, 40 mg, and finally 60 mg at intervals of at least 4 weeks, based on tolerability and LDL-C response.
administration:
Take with water, at least 2 hours after the evening meal. Do not take with food.
Pediatric Dosing
Neonatal:
Not established
Infant:
Not established
Child:
Not established for children under 12 years of age.
Adolescent:
For patients 12 years and older with HoFH: Initial 5 mg once daily. Titrate gradually up to a maximum of 40 mg/day based on tolerability and LDL-C response.
Dose Adjustments
Renal Impairment:
Mild:
No dose adjustment needed.
Moderate:
No dose adjustment needed.
Severe:
Maximum dose 40 mg/day (eGFR <30 mL/min/1.73 m2, including ESRD).
Dialysis:
Maximum dose 40 mg/day for patients on dialysis.
Hepatic Impairment:
Mild:
Maximum dose 40 mg/day (Child-Pugh A).
Moderate:
Contraindicated (Child-Pugh B).
Severe:
Contraindicated (Child-Pugh C).
Pharmacology
Mechanism of Action
Lomitapide binds to and inhibits the microsomal triglyceride transfer protein (MTP), which is located in the lumen of the endoplasmic reticulum. This inhibition prevents the assembly of apolipoprotein B (apoB)-containing lipoproteins (chylomicrons and VLDL) in the enterocytes and hepatocytes, respectively. This leads to a reduction in the production of chylomicrons and VLDL, and subsequently, LDL-C levels.
Pharmacokinetics
Absorption:
Bioavailability:
Not precisely quantified, but well absorbed.
Tmax:
6 hours (range 3-12 hours)
FoodEffect:
High-fat meal delays Tmax by 3 hours and reduces Cmax by 47%. Should be taken at least 2 hours after the evening meal.
Distribution:
Vd:
985 L
ProteinBinding:
>99%
CnssPenetration:
Limited
Elimination:
HalfLife:
39.5 hours (range 28.6-49.3 hours)
Clearance:
Not readily available as a single value, primarily hepatic metabolism.
ExcretionRoute:
Fecal (59.5%), Renal (33.4%)
Unchanged:
<1% in urine, 7% in feces
Pharmacodynamics
OnsetOfAction:
Lipid-lowering effects observed within weeks.
PeakEffect:
Maximal LDL-C reduction typically seen after several weeks of stable dosing.
DurationOfAction:
Due to long half-life, effects persist.
Safety & Warnings
BLACK BOX WARNING
JUXTAPID can cause elevations in transaminases and hepatic steatosis. JUXTAPID is available only through a restricted program called the JUXTAPID REMS Program.
Side Effects
Serious Side Effects: Seek Medical Attention Immediately
Although rare, some people may experience severe and potentially life-threatening side effects while taking this medication. If you notice any of the following symptoms, contact your doctor or seek medical help right away:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Signs of liver problems, including:
+ Dark urine
+ Tiredness
+ Decreased appetite
+ Upset stomach or stomach pain
+ Light-colored stools
+ Vomiting
+ Yellow skin or eyes
Chest pain
Abnormal heartbeat
Flu-like symptoms
Severe diarrhea, which may lead to hospitalization
Signs of dehydration, such as:
+ Severe dizziness or fainting
+ Inability to urinate or changes in urine output
+ Extreme fatigue
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only mild ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects, contact your doctor or seek medical help if they bother you or persist:
Stomach cramps
Bloating
Dizziness, tiredness, or weakness
Gas
Constipation
Heartburn
Weight loss
Back pain
Nose or throat irritation
Headache
Diarrhea
Upset stomach
Vomiting
* Stomach pain
A low-fat diet may help reduce the likelihood of experiencing these side effects. If any of these side effects are severe, bothersome, or persistent, contact your doctor.
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns about side effects, contact your doctor. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Although rare, some people may experience severe and potentially life-threatening side effects while taking this medication. If you notice any of the following symptoms, contact your doctor or seek medical help right away:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Signs of liver problems, including:
+ Dark urine
+ Tiredness
+ Decreased appetite
+ Upset stomach or stomach pain
+ Light-colored stools
+ Vomiting
+ Yellow skin or eyes
Chest pain
Abnormal heartbeat
Flu-like symptoms
Severe diarrhea, which may lead to hospitalization
Signs of dehydration, such as:
+ Severe dizziness or fainting
+ Inability to urinate or changes in urine output
+ Extreme fatigue
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only mild ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects, contact your doctor or seek medical help if they bother you or persist:
Stomach cramps
Bloating
Dizziness, tiredness, or weakness
Gas
Constipation
Heartburn
Weight loss
Back pain
Nose or throat irritation
Headache
Diarrhea
Upset stomach
Vomiting
* Stomach pain
A low-fat diet may help reduce the likelihood of experiencing these side effects. If any of these side effects are severe, bothersome, or persistent, contact your doctor.
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns about side effects, contact your doctor. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Seek Immediate Medical Attention If You Experience:
- Yellowing of skin or eyes (jaundice)
- Dark urine
- Severe stomach pain
- Nausea
- Vomiting
- Unusual tiredness or fatigue
- Loss of appetite
- Easy bruising or bleeding
Before Using This Medicine
Before Taking This Medication: Important Information to Share with Your Doctor
It is essential to inform your doctor about the following conditions to ensure safe treatment:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Describe the allergic reaction you experienced, including the symptoms that occurred.
If you have liver disease or elevated liver enzymes, as this may affect the medication's safety and efficacy.
If you have been diagnosed with difficulties processing certain sugars, including lactose, glucose, or galactose, as this may impact the medication's suitability for you.
If you have difficulty swallowing the medication whole, as this may require alternative administration methods.
If you are taking any prescription or over-the-counter medications, natural products, or vitamins that may interact with this medication. Certain medications, such as those used to treat HIV, infections, or depression, should not be taken concurrently with this drug. Your doctor or pharmacist can advise you on potential interactions and help you manage your medications safely.
If you are pregnant or think you may be pregnant. Do not take this medication if you are pregnant, as it may pose risks to the fetus.
* If you are breastfeeding. It is recommended that you do not breastfeed while taking this medication, as its safety for nursing infants has not been established.
This list is not exhaustive, and it is crucial to discuss all your medications, including prescription and over-the-counter drugs, natural products, and vitamins, as well as any health problems, with your doctor and pharmacist. They will help you determine whether it is safe to take this medication in combination with your other treatments and health conditions. Never start, stop, or modify the dosage of any medication without consulting your doctor first.
It is essential to inform your doctor about the following conditions to ensure safe treatment:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Describe the allergic reaction you experienced, including the symptoms that occurred.
If you have liver disease or elevated liver enzymes, as this may affect the medication's safety and efficacy.
If you have been diagnosed with difficulties processing certain sugars, including lactose, glucose, or galactose, as this may impact the medication's suitability for you.
If you have difficulty swallowing the medication whole, as this may require alternative administration methods.
If you are taking any prescription or over-the-counter medications, natural products, or vitamins that may interact with this medication. Certain medications, such as those used to treat HIV, infections, or depression, should not be taken concurrently with this drug. Your doctor or pharmacist can advise you on potential interactions and help you manage your medications safely.
If you are pregnant or think you may be pregnant. Do not take this medication if you are pregnant, as it may pose risks to the fetus.
* If you are breastfeeding. It is recommended that you do not breastfeed while taking this medication, as its safety for nursing infants has not been established.
This list is not exhaustive, and it is crucial to discuss all your medications, including prescription and over-the-counter drugs, natural products, and vitamins, as well as any health problems, with your doctor and pharmacist. They will help you determine whether it is safe to take this medication in combination with your other treatments and health conditions. Never start, stop, or modify the dosage of any medication without consulting your doctor first.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication. Regular blood tests will be necessary, as directed by your doctor, to monitor your condition. Be sure to follow the diet and exercise plan recommended by your doctor.
To avoid potential interactions, do not consume grapefruit or grapefruit juice while taking this medication. If you plan to drink alcohol, discuss this with your doctor first. It is recommended to limit alcohol intake to no more than one drink per day.
When taking this medication, avoid taking colesevelam, colestipol, or cholestyramine within a 4-hour window before or after taking your dose. If you experience diarrhea or vomiting, please note that oral medications, including birth control pills, may not be absorbed properly. As a precaution, if you are taking birth control pills, use an additional form of contraception, such as a condom, for 7 days after the diarrhea or vomiting has stopped. If you have any questions or concerns, consult your doctor.
Your doctor may recommend taking supplements like vitamin E and fatty acids while on this medication. It is crucial to follow your doctor's instructions regarding these supplements.
If you are 65 years or older, use this medication with caution, as you may be more susceptible to side effects.
This medication can cause harm to an unborn baby. A pregnancy test will be conducted before starting the medication to confirm that you are not pregnant. If you may become pregnant, it is essential to use birth control while taking this medication and for a specified period after the last dose. Your doctor will advise you on the duration of birth control use. If you become pregnant while taking this medication, contact your doctor immediately.
To avoid potential interactions, do not consume grapefruit or grapefruit juice while taking this medication. If you plan to drink alcohol, discuss this with your doctor first. It is recommended to limit alcohol intake to no more than one drink per day.
When taking this medication, avoid taking colesevelam, colestipol, or cholestyramine within a 4-hour window before or after taking your dose. If you experience diarrhea or vomiting, please note that oral medications, including birth control pills, may not be absorbed properly. As a precaution, if you are taking birth control pills, use an additional form of contraception, such as a condom, for 7 days after the diarrhea or vomiting has stopped. If you have any questions or concerns, consult your doctor.
Your doctor may recommend taking supplements like vitamin E and fatty acids while on this medication. It is crucial to follow your doctor's instructions regarding these supplements.
If you are 65 years or older, use this medication with caution, as you may be more susceptible to side effects.
This medication can cause harm to an unborn baby. A pregnancy test will be conducted before starting the medication to confirm that you are not pregnant. If you may become pregnant, it is essential to use birth control while taking this medication and for a specified period after the last dose. Your doctor will advise you on the duration of birth control use. If you become pregnant while taking this medication, contact your doctor immediately.
Overdose Information
Overdose Symptoms:
- Exaggerated gastrointestinal side effects (severe nausea, vomiting, diarrhea)
- Potential for increased liver enzyme elevations
What to Do:
No specific antidote. Treatment should be symptomatic and supportive. Contact a poison control center immediately. Call 1-800-222-1222.
Drug Interactions
Contraindicated Interactions
- Moderate or strong CYP3A4 inhibitors (e.g., ketoconazole, itraconazole, clarithromycin, erythromycin, diltiazem, verapamil, grapefruit juice, ritonavir, indinavir, saquinavir, telithromycin, nefazodone)
- Moderate or severe hepatic impairment
- Pregnancy
Major Interactions
- Warfarin (increased INR, requires monitoring)
- Statins (increased risk of myopathy/rhabdomyolysis, dose adjustment needed)
- P-glycoprotein substrates (e.g., digoxin, dabigatran - increased exposure)
- CYP3A4 inducers (e.g., rifampin, phenytoin, carbamazepine, St. John's Wort - decreased lomitapide efficacy)
Moderate Interactions
- Bile acid sequestrants (administer lomitapide at least 4 hours after sequestrant)
- Ezetimibe (no specific interaction, but often co-administered)
Monitoring
Baseline Monitoring
Liver function tests (ALT, AST, alkaline phosphatase, total bilirubin)
Rationale: To assess baseline hepatic function and identify pre-existing liver disease.
Timing: Prior to initiation of therapy.
Lipid panel (LDL-C, HDL-C, TG, Total Cholesterol)
Rationale: To establish baseline lipid levels and confirm diagnosis of HoFH.
Timing: Prior to initiation of therapy.
Routine Monitoring
Liver function tests (ALT, AST, alkaline phosphatase, total bilirubin)
Frequency: Monthly for the first year, then at least every 3 months thereafter, or as clinically indicated.
Target: Within normal limits.
Action Threshold: Discontinue or reduce dose if ALT or AST âĨ 3x ULN and confirmed on repeat testing. Discontinue if clinically significant liver injury or persistent elevations.
Lipid panel (LDL-C, HDL-C, TG, Total Cholesterol)
Frequency: Periodically to assess treatment response and guide dose titration.
Target: Individualized based on treatment goals for HoFH.
Action Threshold: Not applicable for dose adjustment based on lipid levels alone, but guides titration.
Symptom Monitoring
- Abdominal pain
- Nausea
- Vomiting
- Diarrhea
- Dyspepsia
- Fatigue
- Jaundice (yellowing of skin or eyes)
- Dark urine
- Pruritus (itching)
- Loss of appetite
Special Patient Groups
Pregnancy
Contraindicated. Lomitapide may cause fetal harm when administered to a pregnant woman. Women of reproductive potential must use effective contraception during treatment and for at least 2 weeks after the last dose.
Trimester-Specific Risks:
First Trimester:
Potential for teratogenicity, though human data are limited.
Second Trimester:
Not recommended due to potential for fetal harm.
Third Trimester:
Not recommended due to potential for fetal harm.
Lactation
Contraindicated. It is not known if lomitapide is excreted in human milk. Due to the potential for serious adverse reactions in the breastfed infant, breastfeeding is not recommended during treatment with Juxtapid.
Infant Risk:
High (potential for serious adverse effects).
Pediatric Use
Approved for patients 12 years and older with homozygous familial hypercholesterolemia (HoFH). Dosing starts at 5 mg and titrates up to a maximum of 40 mg/day for patients 12-17 years old. Safety and effectiveness in patients younger than 12 years have not been established.
Geriatric Use
No specific dose adjustment needed based on age alone. However, older patients may have age-related decline in renal or hepatic function, which should be considered.
Clinical Information
Clinical Pearls
- Lomitapide is part of a restricted distribution program (REMS) due to the risk of hepatotoxicity. Prescribers and patients must enroll in the JUXTAPID REMS Program.
- Strict adherence to a low-fat diet (less than 20% of energy from fat) is critical to minimize gastrointestinal side effects and the risk of hepatic steatosis.
- Patients must take daily supplements of fat-soluble vitamins and essential fatty acids due to the drug's mechanism of action affecting fat absorption.
- Significant drug-drug interaction potential, especially with CYP3A4 inhibitors and inducers. Concomitant use with moderate or strong CYP3A4 inhibitors is contraindicated.
- Not indicated for heterozygous familial hypercholesterolemia (HeFH) or other causes of hypercholesterolemia.
- Regular and frequent monitoring of liver function tests is mandatory.
Alternative Therapies
- LDL apheresis
- PCSK9 inhibitors (e.g., evolocumab, alirocumab)
- Evinacumab (Evkeeza) - ANGPTL3 inhibitor, specifically for HoFH
- Inclisiran (Leqvio) - siRNA, for primary hyperlipidemia including HoFH
- Other lipid-lowering agents (statins, ezetimibe, fibrates, niacin, bile acid sequestrants) - often used in combination, but not as monotherapy alternatives for HoFH.
Cost & Coverage
Average Cost:
$10,000 - $20,000+ per 30 capsules (monthly supply)
Insurance Coverage:
Specialty Tier, requires prior authorization and often step therapy due to high cost and specific indication.
General Drug Facts
If your symptoms or health issues persist or worsen, it's essential to contact your doctor promptly. To ensure your safety, never share your medication with others or take someone else's medication. This medication is accompanied by a Medication Guide, which is a valuable resource that provides important information. Please read this guide carefully and review it again whenever you receive a refill of this medication. If you have any questions or concerns about this medication, don't hesitate to discuss them with your doctor, pharmacist, or other healthcare provider. In the event of a suspected overdose, immediately call your local poison control center or seek emergency medical attention. When seeking help, be prepared to provide details about the overdose, including the amount taken and the time it occurred.