About HoFH

HoFH, or homozygous familial hypercholesterolemia, is a form of high cholesterol that is rare and inherited. It can be difficult to treat using only other methods of lowering cholesterol, including diet and other therapies.

Why? To better understand, it’s helpful to know a little about how the body makes cholesterol and why this process is different in people with HoFH.

The Answer is in the Liver

While we get some cholesterol from the foods we eat, the liver is responsible for producing most of the cholesterol found in our body.

The liver is responsible for making cholesterol and releasing it into the bloodstream for the body to use, as well as bringing back unused bad cholesterol for breakdown.

But if you have HoFH, your liver is different.

In People with HoFH

  • The liver makes cholesterol and releases it into the bloodstream.
  • The body uses some cholesterol for essential functions.
  • The HoFH liver’s mechanism for removing bad (LDL) cholesterol from the bloodstream does not work properly.

In People without HoFH

  • The liver makes cholesterol and releases it into the bloodstream.
  • The body uses some cholesterol for essential functions.
  • The liver removes unused bad (LDL) cholesterol from the bloodstream for breakdown and to prevent buildup.

Cholesterol and HoFH

For adults with HoFH, there are 2 important types of cholesterol to keep track of:
  • “Bad,” or low-density lipoprotein (LDL), cholesterol: guidelines recommend that this number be at or below 100 mg/dL and less than 70 mg/dL for people who have signs of heart disease.
  • Total cholesterol: calculated using the main types of cholesterol found in your body.

Consistently elevated cholesterol can build up in your arteries and can lead to atherosclerosis. Your cholesterol goals may be different. If you do not have a goal, ask your doctor to set one with you.

Why Lower Your Cholesterol?

  • family treeHomozygous familial hypercholesterolemia (HoFH) is an uncommon condition, inherited from both sides of the family, that causes high levels of bad cholesterol to build up in your bloodstream starting from an early age.
  • ldlHigh levels of bad cholesterol can build up in your arteries. This buildup can lead to the narrowing and hardening of your arteries, known as atherosclerosis.
  • range Keeping cholesterol within a healthy range is important for everyone, but especially for someone with HoFH. Often, people with HoFH have trouble reaching their cholesterol goals with traditional methods alone, such as diet and cholesterol-lowering medications.

Learn Why Juxtapid May Be Right for You Go

quote person
We follow a low-fat eating plan as a family now. We have been avoiding oils and fats, and we don’t really miss them.” 

— Andrea, Diagnosed with HoFH

Important Safety Information

Juxtapid may cause serious side effects, including:

Liver Problems    

  • Juxtapid can cause liver problems such as increased liver enzymes or increased fat in the liver. For this reason, your doctor should do blood tests to check your liver before you start Juxtapid and while you are taking Juxtapid (especially if your dose is increased). If your tests show signs of liver problems, your doctor may lower your dose of Juxtapid or stop it altogether.
  • You should tell your doctor if you have had liver problems in the past, including liver problems while taking other medicines.
  • Stomach problems can also be a symptom of liver problems. Tell your doctor right away if you have nausea; vomiting or stomach pain that gets worse, does not go away, or changes; fever; yellowing of your eyes or skin; feeling more tired than usual; or having flu-like symptoms while taking Juxtapid because these may be signs of liver problems.
  • Do not drink more than 1 alcoholic drink per day while taking Juxtapid.

Harm to your unborn baby:

  • Do not take Juxtapid if you are pregnant, think you may be pregnant, or are planning to become pregnant.
  • You should have a negative pregnancy test result before you can start on Juxtapid. Use effective birth control while taking Juxtapid. If you become pregnant while taking Juxtapid, stop taking Juxtapid and call your doctor right away.

You should not take Juxtapid if you:

  • Are taking medications known as moderate o r strong CYP3A4 inhibitors (for example, certain medications used to treat bacterial, fungal, or viral infections, as well as certain medications used to treat depression, high blood pressure, or angina). These medications may affect how your body breaks down Juxtapid.
  • Have moderate to severe liver problems or active liver disease, including abnormal liver function tests.

Other possible side effects of Juxtapid:

  • The most common side effects of Juxtapid are stomach problems including diarrhea, nausea, vomiting, cramps/pain, indigestion, and/or gas. You may be able to reduce your chance of stomach problems by following an eating plan consisting of less than 20% of calories from fat.
  • Juxtapid makes it harder for some fat-soluble nutrients, such as vitamin E and fatty acids, to get into your body. Take supplements that contain fat-soluble vitamins each day while you take Juxtapid. Ask your doctor, nurse, or dietitian how to take them.

Tell your doctor if you have any side effect that bothers you or that does not go away. Stop taking Juxtapid and tell your doctor if you have severe diarrhea, especially if you also have lightheadedness, decreased urine output, or tiredness. These are not all the possible side effects of Juxtapid. For more information, speak with your doctor or pharmacist.

Tell your doctor about all the medicines you take, including prescription and non-prescription medicines, vitamins, and herbal supplements. Juxtapid may affect the way other medicines work, and other medicines may affect how Juxtapid works.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.

This is the most important information about Juxtapid. For more detailed information, Please see the Medication Guide and full Prescribing Information including Box Warning.