Lomitapide

IMPORTANT WARNING:

Lomitapide may cause serious damage to the liver. Tell your doctor if you have or have ever had liver disease or if you have ever had liver problems while taking other medications. Your doctor may tell you not to take lomitapide. Tell your doctor if you drink alcohol. Drinking alcohol may increase the risk that you will develop liver problems. Do not drink more than one alcoholic beverage per day while you are taking lomitapide. Tell your doctor and pharmacist if you are taking acetaminophen (Tylenol, others), amiodarone (Cordarone), doxycycline (Doryx, Vibramycin, others), isotretinoin (Accutane), methotrexate (Rheumatrex), minocycline (Dynacin, Minocin), tamoxifen (Nolvadex), or tetracycline (Sumycin). If you experience any of the following symptoms at any time during your treatment, stop taking lomitapide and call your doctor immediately: extreme tiredness, lack of energy, weakness, nausea or vomiting that gets worse or does not go away, pain in the upper right part of the stomach, loss of appetite, yellowing of the skin or eyes, fever, or flu-like symptoms. Your doctor may need to change the dose or stop or delay your treatment if you experience liver problems.

A program called Juxtapid REMS^® has been approved by the Food and Drug Administration (FDA) because of the possibility of liver damage associated with lomitapide use. All people who are prescribed lomitapide must have a lomitapide prescription from a doctor who is registered with Juxtapid REMS^®, and have the prescription filled at a pharmacy that is registered with Juxtapid REMS^® in order to receive this medication.

Keep all appointments with your doctor and the laboratory. Your doctor will order certain tests before and during your treatment to check your body's response to lomitapide.

Your doctor or pharmacist will give you the manufacturer's patient information sheet (Medication Guide) when you begin treatment with lomitapide and each time you refill your prescription. Read the information carefully and ask your doctor or pharmacist if you have any questions. You can also visit the Food and Drug Administration (FDA) website (http://www.fda.gov/Drugs/DrugSafety/ucm085729.htm) or the manufacturer's website to obtain the Medication Guide.

WHY is this medicine prescribed?

Lomitapide is used along with diet changes (restriction of cholesterol and fat intake) and other treatments to reduce the amount of low-density lipoprotein (LDL) cholesterol ('bad cholesterol'), total cholesterol, and other fatty substances in the blood in people that have homozygous familial hypercholesterolemia (HoFH; an inherited condition in which cholesterol cannot be removed from the body normally). Lomitapide should not be used to decrease cholesterol levels in people who do not have HoFH. Lomitapide is in a class of medications called cholesterol-lowering medications. It 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.

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.

Are there OTHER USES for this medicine?

This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

HOW should this medicine be used?

Lomitapide comes as a capsule to take by mouth. It is usually taken once a day. Lomitapide should be taken without food on an empty stomach, at least 2 hours after your evening meal. Drink a full glass of water with each dose of lomitapide.

Take lomitapide at around the same time every day. Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Take lomitapide exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.

Swallow the capsules whole; do not split, chew, dissolve, or crush them.

You will need to take a vitamin supplement during your treatment with lomitapide Be sure to follow the recommendations made by your doctor carefully.

Continue to take lomitapide even if you feel well. Do not stop taking lomitapide without talking to your doctor.

What SPECIAL PRECAUTIONS should I follow?

Before taking lomitapide,

• tell your doctor and pharmacist if you are allergic to lomitapide, any other medications, or any of the ingredients in lomitapide capsules. Ask your pharmacist or check the Medication Guide for a list of the ingredients.

• tell your doctor if you are taking antifungal medications such as fluconazole (Diflucan), itraconazole (Sporanox), ketoconazole (Nizoral), posaconazole (Noxafil), and voriconazole (Vfend); boceprevir (Victrelis); aprepitant (Emend); ciprofloxacin (Cipro); clarithromycin (Biaxin); crizotinib (Xalkori); diltiazem (Cardizem, Dilacor, Tiazac); erythromycin (E.E.S., E-Mycin, Erythrocin); HIV protease inhibitors such as amprenavir (Agenerase), atazanavir (Reyataz), darunavir (Prezista), fosamprenavir (Lexiva), indinavir (Crixivan), lopinavir (in Kaletra), nelfinavir (Viracept), saquinavir (Invirase), ritonavir (Norvir, in Kaletra), ritonavir and tipranavir (Aptivus), and teleprevir (Incivek); imatinib (Gleevec); nefazodone; telithromycin (Ketek); and verapamil (Calan, Covera, Isoptin, Verelan). Your doctor will probably tell you not to take lomitapide if you are taking one or more of these medications.

• tell your doctor and pharmacist what other prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take. Be sure to mention the medications listed in the IMPORTANT WARNING section and any of the following: aliskiren (Tekturna); alprazolam (Xanax); ambrisentan (Letairis); amiodarone (Cordarone, Pacerone); amlodipine (Norvasc, in Caduet); bicalutamide (Casodex); cilostazol (Pletal); certain oral medications for diabetes such as saxagliptin (Onglyza in Kombiglyze) and sitagliptin (Januvia, in Janumet); cimetidine (Tagament); colchicine (Colcrys); dabigatran (Pradaxa); digoxin (Lanoxin); everolimus (Afinitor, Zortress); fexofenadine (Allegra); fluoxetine (Prozac); fluvoxamine (Luvox); isoniazid (INH, Nydrazid); lapatinib (Tykerb); maraviroc (Selzentry); medications that suppress the immune system such as cyclosporine (Gengraf, Neoral, Sandimmune), sirolimus (Rapamune) and tacrolimus (Prograf); nilotinib (Tasigna); oral contraceptives (birth control pills); other cholesterol lowering medications such as atorvastatin (Lipitor, in Caduet, in Liptruzet), lovastatin (Mevacor), and simvastatin (Zocor, in Simcor, in Vytorin); pazopanib (Votrient); ranitidine (Zantac); ranolazine (Ranexa); ticagrelor (Brilinta); tolvaptan (Samsca); topotecan (Hycamtin); warfarin (Coumadin); and zileuton (Zyflo). Your doctor may need to change the doses of your medications or monitor you carefully for side effects. Many other medications may also interact with lomitapide, so be sure to tell your doctor about all the medications you are taking, even those that do not appear on this list.

• tell your doctor what herbal products you are taking, especially ginkgo or goldenseal.

• if you are taking cholestyramine (Questran), colesevelam (WellChol), or colestipol (Colestid), take it 4 hours before or 4 hours after lomitapide.

• tell your doctor if you have or have ever had galactose intolerance or glucose-galactose malabsorption (inherited conditions where the body is not able to tolerate lactose), ongoing stomach or intestinal problems, or pancreas or kidney disease.

• Tell your doctor if you are pregnant or plan to become pregnant. If you are female who can become pregnant, you will need to take a pregnancy test before you begin taking lomitapide. If you can become pregnant, you should use effective birth control while taking lomitapide. Talk to your doctor about birth control methods that will work for you. If you become pregnant while taking lomitapide, stop taking the medication and call your doctor immediately. Lomitapide can harm the fetus.

• tell your doctor if you are breast-feeding. You should not breast-feed while you are taking lomitapide.

What SPECIAL DIETARY instructions should I follow?

Do not eat grapefruit or drink grapefruit juice while taking this medication.

Your doctor will tell you to eat a low-fat diet. Eating a low-fat diet may lower the chance that you will have stomach problems including nausea, vomiting, upset stomach, and diarrhea while you are taking lomitapide. Follow all dietary recommendations made by your doctor or dietitian carefully.

What should I do IF I FORGET to take a dose?

Skip the missed dose and continue your regular dosing schedule the next day. Do not take a double dose to make up for a missed one.

What SIDE EFFECTS can this medicine cause?

Lomitapide may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:

• diarrhea
• nausea
• vomiting
• stomach pain
• constipation
• bloating
• gas
• upset stomach
• weight loss
• headache
• dizziness
• sore throat
• runny nose
• back pain

Some side effects can be serious. If you experience any of these symptoms or those listed in the IMPORTANT WARNING section, stop taking lomitapide and call your doctor immediately or get emergency medical treatment:

• severe diarrhea
• lightheadedness
• decreased urine output

Lomitapide may cause other side effects. Call your doctor if you have any unusual problems while taking this medication.

If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online (http://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

What should I know about STORAGE and DISPOSAL of this medication?

Keep this medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom).

Unneeded medications should be disposed of in special ways to ensure that pets, children, and other people cannot consume them. However, you should not flush this medication down the toilet. Instead, the best way to dispose of your medication is through a medicine take-back program. Talk to your pharmacist or contact your local garbage/recycling department to learn about take-back programs in your community. See the FDA's Safe Disposal of Medicines website (http://goo.gl/c4Rm4p) for more information if you do not have access to a take-back program.

It is important to keep all medication out of sight and reach of children as many containers (such as weekly pill minders and those for eye drops, creams, patches, and inhalers) are not child-resistant and young children can open them easily. To protect young children from poisoning, always lock safety caps and immediately place the medication in a safe location – one that is up and away and out of their sight and reach. http://www.upandaway.org

What should I do in case of OVERDOSE?

In case of overdose, call the poison control helpline at 1-800-222-1222. Information is also available online at https://www.poisonhelp.org/help. If the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services at 911.

What OTHER INFORMATION should I know?

Do not let anyone else take your medication. Ask your pharmacist any questions you have about refilling your prescription.

It is important for you to keep a written list of all of the prescription and nonprescription (over-the-counter) medicines you are taking, as well as any products such as vitamins, minerals, or other dietary supplements. You should bring this list with you each time you visit a doctor or if you are admitted to a hospital. It is also important information to carry with you in case of emergencies.