What is HoFH?

HoFH (homozygous familial hypercholesterolemia) is an uncommon genetic condition that is inherited from both sides of your family and leads to high levels of cholesterol in your blood.
 

Homozygous (Ho) means you inherited the condition from both sides of your family.

 

Familial (F) means the condition runs in your family.

 

Hypercholesterolemia (H) means high cholesterol.

Genetic high cholesterol—what does it really mean?

High cholesterol that runs in families is also called familial hypercholesterolemia, or FH. HoFH is the least common and most severe form of FH.

While high cholesterol is common, high cholesterol caused by HoFH is not. In fact, family members on both sides of your family may have a less severe form of FH, but you may be the only one with HoFH.

For illustrative purposes only
HoFH Familial hypercholesterolemia (FH) FH

Why are family and personal health history important?

In HoFH, both parents pass on the genes that cause it, which is why it is so uncommon. It is likely that some of your other relatives will also have high cholesterol and heart disease at an early age (but may not necessarily have HoFH). Knowing your family health history can be very helpful to diagnose inherited conditions such as HoFH.

Also, keeping careful records of your own personal health history can help your doctor determine if you have HoFH.

High cholesterol and HoFH: what's the liver got to do with it?

The liver plays a key role in the amount of cholesterol in the body. While we get some cholesterol from the foods we eat, the liver is responsible for making most of the cholesterol found in our body. In addition, the liver is also responsible for removing unused bad (LDL) cholesterol from the bloodstream.

How is the HoFH liver different?

The problem with HoFH is that the liver can't remove bad (LDL) cholesterol from the bloodstream as well as someone without HoFH. Over time, cholesterol can build up in the arteries and may lead to atherosclerosis.

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.

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.

 

 

Has it been difficult to reach your cholesterol goals?

You're not alone. People with HoFH who are taking other cholesterol-lowering therapies and following their doctor's advice on eating and exercise often still have trouble lowering their cholesterol to a healthy level.

When other cholesterol-lowering medicines alone may not be enough for people with HoFH

Medications such as statins treat high cholesterol, including more common types of genetic high cholesterol, but are not specifically developed to treat high cholesterol caused by HoFH.

These medications typically rely on the liver's mechanism for removing unused bad (LDL) cholesterol from the bloodstream in order to work. Since this part of your liver doesn't work properly, these medications may not be enough when used alone to help you reach the cholesterol goals set with your doctor.

Adding a medication such as Juxtapid® that is specifically developed to treat high cholesterol caused by HoFH may help further reduce your cholesterol numbers to get closer to the goal set with your doctor.

What happens when bad (LDL) cholesterol is high in HoFH

 

Why is it important to set a cholesterol goal?

Everyone should strive to keep his or her cholesterol numbers at healthy levels. But as a person living with HoFH, it’s critical for you to keep track of your cholesterol numbers and the goals your doctor has set with you.

Not everyone’s cholesterol goals are the same. Talk to your doctor about setting a cholesterol goal that’s right for you.

 

 

What is Juxtapid?

Juxtapid® (lomitapide) capsules is a prescription medicine used along with diet and other lipid-lowering treatments, including low-density lipoprotein (LDL) apheresis where available, in adults with homozygous familial hypercholesterolemia (HoFH) to reduce LDL (“bad”) cholesterol, total cholesterol, a protein that carries bad cholesterol in the blood (apolipoprotein B), and non-high-density lipoprotein cholesterol (non-HDL-C).

Studies have not been conducted to tell us whether Juxtapid can help prevent problems from high cholesterol, such as heart attack, stroke, death, or other health problems. Studies have also not been conducted to tell us whether Juxtapid is safe for use in people with high cholesterol who do not have HoFH, including those with heterozygous familial hypercholesterolemia (HeFH).

IMPORTANT SAFETY INFORMATION

Juxtapid is available only through certified pharmacies that are enrolled in the Juxtapid REMS Program. Your doctor must be enrolled and certified in the program in order to prescribe Juxtapid.

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). 
  • 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 or 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 your doctor. You may also report side effects to the FDA at 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 Prescribing Information.

All people featured on this site are real patients living with HoFH and were taking Juxtapid at the time of the photo shoot.

 

This information is intended for US healthcare providers

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