Juxtapid

Juxtapid is used to reduce LDL-C levels in adults and children 2 years and older with homozygous familial hypercholesterolemia (HoFH) alongside a low-fat diet, exercise, and other low-density lipoprotein cholesterol therapies.

Juxtapid works by slowing the production of cholesterol in the body to decrease the amount of cholesterol that may build up on the walls of the arteries and block blood flow to the heart, brain, and other parts of the body. Juxtapid belongs to the drug class called cholesterol-lowering medications.

Accumulation of cholesterol and fats along the walls of your arteries (a process known as atherosclerosis) decreases blood flow and, therefore, the oxygen supply to your heart, brain, and other parts of your body. Lowering your blood level of cholesterol and fats may help prevent heart disease, angina (chest pain), strokes, and heart attacks.

It is not known whether Juxtapid will lower your risk of heart problems.

Juxtapid is available only from a certified pharmacy under a special program. You must be registered in the program and understand the risks and benefits of this medicine.

Take Juxtapid exactly as prescribed by your doctor. Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose.

Your doctor will perform blood tests to make sure Juxtapid is safe for you to take.

Take Juxtapid on an empty stomach, at least 2 hours after your evening meal.

Swallow the capsule whole and do not crush, chew, break, or dissolve it. Take this medicine with a full glass of water.

Your doctor may have you take vitamin E or essential fatty acid supplements while you are taking Juxtapid. Take only the type and amount of vitamins or supplements your doctor has prescribed.

Juxtapid is only part of a treatment program that may also include exercise and weight control. Follow your doctor's dosing instructions very carefully.

Store at room temperature away from moisture and heat. Keep the bottle tightly closed when not in use.

You should not use Juxtapid if you have severe liver disease or a history of abnormal liver function tests.

Do not use if you are pregnant. Birth control pills may be less effective if you vomit or have diarrhea. Ask your doctor about the best way to prevent pregnancy while you are taking Juxtapid.

Tell your doctor about all your current medicines and any you start or stop using. Many drugs can affect lomitapide, and some drugs should not be used at the same time.

Juxtapid can cause liver problems. Call your doctor at once if you have nausea, vomiting, upper stomach pain, tiredness, flu-like symptoms, dark urine, or jaundice (yellowing of the skin or eyes).

You should not use Juxtapid if you are allergic to lomitapide, or if you:

• are pregnant;
• have moderate or severe liver disease, or active liver disease; or
• have abnormal liver function tests.

Some drugs should not be used with Juxtapid. Your treatment plan may change if you also use:

• aprepitant;
• conivaptan;
• crizotinib, imatinib;
• nefazodone;
• an antibiotic - ciprofloxacin, clarithromycin, erythromycin, telithromycin;
• antifungal medication - fluconazole, itraconazole, ketoconazole, posaconazole, voriconazole;
• heart or blood pressure medicine - diltiazem, verapamil;
• hepatitis C medication - boceprevir or telaprevir; or
• HIV or AIDS medicine - atazanavir, darunavir when given with ritonavir, fosamprenavir, indinavir, lopinavir, nelfinavir, ritonavir, saquinavir, tipranavir.

To make sure Juxtapid is safe for you, tell your doctor if you have ever had:

• liver problems, even if caused by taking certain medicines;
• kidney disease (or if you are on dialysis);
• bowel problems, or an intestinal disorder;
• hereditary galactose intolerance, severe lactase deficiency, or glucose galactose malabsorption; or
• if you drink large amounts of alcohol.

You may need to have a negative pregnancy test before starting this treatment.

May harm an unborn baby. Do not use if you are pregnant. Use effective birth control while using Juxtapid. Tell your doctor if you become pregnant.

If you are pregnant, your name may be listed on a pregnancy registry to track the effects of lomitapide on the baby.

Birth control pills may be less effective if you vomit or have diarrhea. Ask your doctor about the best way to prevent pregnancy while taking Juxtapid.

Ask a doctor if it is safe to breastfeed while using this medicine.

Get emergency medical help if you have signs of an allergic reaction to Juxtapid: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Stop taking Juxtapid and tell your doctor if you have:

• severe diarrhea;
• a light-headed feeling, like you might pass out;
• decreased urination; or
• tiredness.

Call your doctor at once if you have signs of liver problems, such as:

• nausea, vomiting, upper stomach pain;
• loss of appetite;
• swelling around your midsection;
• flu-like symptoms, tiredness;
• dark urine, clay-colored stools; or
• jaundice (yellowing of the skin or eyes).

The most common Juxtapid side effects in adults may include:

• stomach cramps;
• nausea, vomiting;
• indigestion; or
• diarrhea.

The most common side effects of Juxtapid in children aged 5 to 17 years are:

• Abdominal pain
• Increases in liver enzymes, such as alanine aminotransferase and aspartate aminotransferase
• Diarrhea
• Vomiting

This is not a complete list of side effects, and others may occur. Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Usual Adult Dose for Homozygous Familial Hypercholesterolemia

Initial dose: 5 mg orally once a dayMaintenance dose: 5 to 60 mg orally once a day (should be individualized, taking into account the goal of therapy and the response to treatment)Maximum dose: 60 mg orally once a dayComments:-Dose should be escalated gradually based on response, safety, and tolerability. The dose may be escalated using the following incremental schedule: 5 mg orally once a day for a minimum of 2 weeks, then 10 mg, 20 mg, 40 mg, 60 mg orally once a day after a minimum of 4 weekly intervals between each dose increase.-Juxtapid should be taken with a full glass of water on an empty stomach at least 2 hours after the evening meal.

Usual Child Dose of Juxtapid for Homozygous Familial Hypercholesterolemia

• The dose varies depending on age.
• See the Juxtapid Prescribing Information for full dosage information.

Skip the missed dose and use your next dose at the regular time. Do not use two doses at one time. Talk to your doctor if you stop taking Juxtapid for more than a week.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

Avoid eating foods high in fat or cholesterol, or Juxtapid will not be as effective and may cause nausea, vomiting, and stomach pain.

Grapefruit may interact with lomitapide and cause side effects. Avoid consuming grapefruit products.

Do not drink more than 1 alcoholic drink per day. Drinking alcohol may increase your risk of liver damage.

Some medicines can make lomitapide much less effective when taken at the same time. If you take cholestyramine or colesevelam, take your Juxtapid dose 4 hours before or 4 hours after you take the other medicine.

Tell your doctor about all your current medicines. Many drugs can interact with lomitapide, especially:

• acetaminophen, isotretinoin, methotrexate, or tamoxifen;
• an antidepressant;
• antiviral medicine to treat hepatitis C or HIV;
• heart or blood pressure medicine;
• birth control pills;
• an antibiotic - demeclocycline, doxycycline, minocycline, eravacycline, omadacycline, sarecycline, tetracycline;
• a blood thinner - warfarin, Coumadin, Jantoven; or
• cholesterol medication - atorvastatin or simvastatin.

This list is not complete, and many other drugs may interact with lomitapide. This includes prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible drug interactions are listed here.

Store Juxtapid at room temperature, between 68°F and 77°F (20°C and 25°C).

• Keep in a tightly closed container.
• Keep the capsules dry.
• Safely throw away medicine that is out of date or no longer needed.

Keep out of the reach of children.

Active ingredient: lomitapide mesylateInactive ingredients: pregelatinized starch, sodium starch glycolate, microcrystalline cellulose, lactose monohydrate, silicon dioxide, magnesium stearateCapsule shell: The capsule shells of all strengths contain gelatin and titanium dioxide; the 2 mg capsules containblack iron oxide; the 5 mg, 10 mg, and 30 mg capsules also contain red iron oxide; and the 30 mg capsules also contain yellow iron oxide. The imprinting ink contains shellac, black iron oxide, and propylene glycol.

Juxtapid is manufactured for Chiesi Farmaceutici S.p.A., Parma, Italy.