What is Juxtapid?

Lomitapide mesylate can cause elevations in transaminases. Measure ALT, AST, alkaline phosphatase, and total bilirubin before initiating treatment and then ALT and AST regularly as recommended. During treatment, adjust the dose of lomitapide mesylate if the ALT or AST is 3 times the ULN or greater. Discontinue lomitapide mesylate for clinically significant liver toxicity. Lomitapide mesylate increases hepatic fat (hepatic steatosis) with or without concomitant increases in transaminases. Hepatic steatosis associated with lomitapide mesylate may be a risk factor for progressive liver disease, including steatohepatitis and cirrhosis. Because of the risk of hepatotoxicity, lomitapide mesylate is available only through a restricted program called the Juxtapid (R) REMS Program. Prescribe lomitapide mesylate only to patients with a clinical or laboratory diagnosis consistent with homozygous familial hypercholesterolemia (HoFH). The safety and effectiveness of lomitapide mesylate have not been established in patients with hypercholesterolemia who do not have HoFH .

Lomitapide is used together with a low-fat diet and other treatments (including low density lipoprotein (LDL) apheresis) to lower bad cholesterol levels in the blood.

This medicine is available only under a special restricted distribution program called Juxtapid® REMS program.

Take this medicine only as directed by your doctor. Do not use more of it, do not use it more often, and do not use it for a longer time than your doctor ordered.

This medicine should come with a Medication Guide. It is very important that you read and understand this information. Be sure to ask your doctor about anything you do not understand.

Take this medicine without food, at least 2 hours after the evening meal.

Swallow the capsule whole with a glass of water. Do not break, crush, chew, dissolve, or open it. If you cannot swallow the capsules, tell your doctor right away.

Carefully follow your doctor's instructions about low-fat diet. This medicine is part of a complete plan for lowering bad cholesterol in your blood.

Your doctor may also want you to take supplements (eg, vitamin E, fatty acids) each day while you are using this medicine. This will help prevent stomach problems, such as trouble getting some nutrients into your body.

If you are also using cholestyramine (Questran®) or colesevelam (Welchol®), take it at least 4 hours before or after you take this medicine.

Do not drink more than 1 alcoholic drink per day with lomitapide. This could cause unwanted effects on the liver.

Grapefruit juice should be avoided while taking this medicine.

The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

• For oral dosage form (capsules):
  • For lowering of bad cholesterol:
    • Adults—At first, 5 milligrams (mg) once a day. Your doctor may adjust your dose as needed and tolerated. However, the dose is usually not more than 60 mg per day.
    • Children—Use and dose must be determined by your doctor.

If you miss a dose of this medicine, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

If you stop taking this medicine for more than a week, call your doctor right away before restarting treatment.

Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Keep from freezing.

Keep out of the reach of children.

Do not keep outdated medicine or medicine no longer needed.

Ask your healthcare professional how you should dispose of any medicine you do not use.

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Appropriate studies have not been performed on the relationship of age to the effects of lomitapide in the pediatric population. Safety and efficacy have not been established.

Appropriate studies performed to date have not demonstrated geriatric-specific problems that would limit the usefulness of lomitapide in the elderly. However, elderly patients are more likely to have age-related kidney or liver problems, which may require caution and an adjustment in the dose for patients receiving lomitapide.

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. When you are taking this medicine, it is especially important that your healthcare professional know if you are taking any of the medicines listed below. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following medicines is not recommended. Your doctor may decide not to treat you with this medication or change some of the other medicines you take.

• Aprepitant
• Atazanavir
• Boceprevir
• Clarithromycin
• Cobicistat
• Conivaptan
• Diltiazem
• Dronedarone
• Erythromycin
• Fluconazole
• Fosaprepitant
• Idelalisib
• Imatinib
• Indinavir
• Itraconazole
• Ketoconazole
• Letermovir
• Lopinavir
• Nefazodone
• Nelfinavir
• Posaconazole
• Ritonavir
• Saquinavir
• Telaprevir
• Telithromycin
• Verapamil
• Voriconazole

Using this medicine with any of the following medicines is usually not recommended, but may be required in some cases. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.

• Alfentanil
• Benzhydrocodone
• Buprenorphine
• Carbamazepine
• Ceritinib
• Codeine
• Dihydrocodeine
• Eliglustat
• Fentanyl
• Fosnetupitant
• Hydrocodone
• Ivosidenib
• Lovastatin
• Meperidine
• Methadone
• Netupitant
• Oxycodone
• Pentazocine
• Simvastatin
• Sufentanil
• Tacrolimus
• Tramadol
• Warfarin

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following is usually not recommended, but may be unavoidable in some cases. If used together, your doctor may change the dose or how often you use this medicine, or give you special instructions about the use of food, alcohol, or tobacco.

• Ethanol
• Grapefruit Juice

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

• Alcohol abuse, history of—Use with caution. May cause side effects to become worse.

• Bowel disease, chronic or
• Pancreatitis (inflammation or swelling of the pancreas)—Use with caution. May increase risk for nutrient deficiencies.

• Galactose intolerance (a rare genetic disorder) or
• Glucose-galactose malabsorption (a rare genetic disorder) or
• Lapp lactase deficiency (a rare genetic disorder) or
• Liver disease, active or
• Liver enzymes, elevated or
• Liver impairment, moderate or severe—Should not be used in patients with these conditions.

• Kidney disease or
• Liver disease, mild—Use with caution. The effects may be increased because of slower removal of the medicine from the body.

It is very important that your doctor check your progress at regular visits. This will allow your doctor to see if the medicine is working properly to lower your bad cholesterol levels and to decide if you should continue to take it. Blood tests may be needed to check for unwanted effects.

Using this medicine while you are pregnant can harm your unborn baby. Use an effective form of birth control to keep from getting pregnant. If you think you have become pregnant while using the medicine, tell your doctor right away.

You must have a negative pregnancy test before you will be allowed to take this medicine. If you miss a period while you are using this medicine, tell your doctor right away.

Do not use this medicine if you also use any of the following: aprepitant (Emend®), boceprevir (Victrelis®), conivaptan (Vaprisol®), crizotinib (Xalkori®), diltiazem (Cardizem®), imatinib (Gleevec®), mibefradil (Posicor®), nefazodone (Serzone®), telaprevir (Incivek®), verapamil (Calan®), medicine to treat infections (such as ciprofloxacin, clarithromycin, erythromycin, fluconazole, itraconazole, ketoconazole, posaconazole, telithromycin, voriconazole, Nizoral®), or medicine to treat HIV/AIDS (such as amprenavir, atazanavir, darunavir/ritonavir, fosamprenavir, indinavir, lopinavir/ritonavir, nelfinavir, ritonavir, saquinavir, tipranavir/ritonavir, Crixivan®, Kaletra®, Norvir®, Reyataz®). Using these medicines together can cause serious side effects.

Call your doctor right away if you have severe diarrhea, decrease in how much or how often you urinate, lightheadedness, or tiredness after using this medicine.

Using this medicine together with lovastatin or simvastatin may increase risk of a serious muscle problem called myopathy. Check with your doctor right away if you have unexplained muscle pain, tenderness, or weakness, especially if you also have unusual tiredness or a fever.

Check with your doctor right away if you have pain or tenderness in the upper stomach, pale stools, dark urine, loss of appetite, nausea, vomiting, or yellow eyes or skin. These could be symptoms of a serious liver problem (including fatty liver). Do not use other medicines to lower cholesterol that can increase liver fat. Ask your doctor about these medicines.

Do not change your dose or stop taking this medicine without first talking with your doctor.

Do not take other medicines unless they have been discussed with your doctor. This includes prescription or nonprescription (over-the-counter [OTC]) medicines and herbal or vitamin supplements.