Familial Hypercholesterolemia (FH) is an inherited disorder that leads to aggressive and early heart disease, including heart attacks, strokes, and narrowing of the blood vessels. Patients with FH have a genetic condition which renders the liver unable to metabolize LDL (“bad”) cholesterol, resulting in dangerously high levels that can lead to arterial blockage and premature cardiovascular disease.

Even though patients who have FH can’t prevent it, an early diagnosis and making the right lifestyle choices at any age can significantly reduce its impact. FH is a serious condition and always requires medical treatment, but a commitment to prescribed medications, good nutrition, regular exercise, and not smoking may prevent or dramatically delay early heart disease.

Take medication

Because FH causes high cholesterol levels that are genetic and not lifestyle-induced, most patients will need medications that lower cholesterol levels to help ward off early heart disease. FH is most often treated by a combination of medicines, each of which serves a different function and complements the others. Statins are a commonly recognized category of medicines that inhibit the production of cholesterol in the liver. They are often prescribed in combination with a cholesterol absorber and a bile acid sequestrant, which prevents cholesterol from being absorbed by the intestine. Other cholesterol-lowering medications are fibrates, niacin, phytosterols, fish oils/omega 3 fatty acids, and PCSK9 inhibitors.

Eat a healthy diet low in saturated and trans fats

According to the National Institute of Health, making changes in your diet to lower the amount of fat to less than 30% of your total calories is one of the first steps FH patients should take. The most harmful types of fat are saturated fat and trans fat, which are found in foods from animal sources, such as meat and dairy products, as well as packed snacks, fast food, and baked goods. It is especially important to read food labels and beware of “hidden” trans fats, which are often found in ingredient lists under the phrase “partially hydrogenated oil.”

Nutritional changes don’t have to mean giving up entire food groups. Simple changes can make a big difference. Some easy ways to reduce fat intake without sacrificing flavor include:

• Choosing leaner cuts of beef and pork, such as eye of round and pork tenderloin, and trimming visible fat before cooking.
• Reducing meat portions to 3 ounces (about the size of a deck of cards) and eating more fruits and vegetables.
• Removing skin from poultry before eating.
• Eating more fish. The American Heart Association recommends eating 3.5 ounces of fish (especially oily, omega-3 rich fish) at least twice a week.
• Dining on a meatless meal once a week.
• Replacing butter with liquid or spray versions of canola, extra virgin olive, peanut, or sesame oil.
• Switching to fat free or 1% milk, or soy or almond milk.
• Replacing high fat ice cream and desserts with naturally sweet and colorful fruits or sherbet.
• In casseroles, soups and stew, cut the amount of animal protein in half and substitute cooked beans.
• Eat more plant-based fiber, such as fruits, vegetables, whole grains, nuts, and seeds. Research shows that eating more than 25 grams of fiber per day can reduce risk of heart disease.

FH patients often find it helpful to consult a registered dietician for help in developing a manageable healthy eating plan.

Exercise regularly

Aggressive treatment of FH takes a combined approach, and exercise is an important component. The American Heart Association recommends getting at least 30 to 60 minutes of moderately intense physical activity most days of the week to improve heart health. If that seems daunting, it may be encouraging to know that even short amounts of exercise offer heart benefits, and that you can reap the same health benefits if you break up your workout time into three to six 10-minute sessions each day.

Don’t smoke

The importance of not smoking can’t be overemphasized for people with FH. Smoking is widely recognized as a leading cause of heart disease in the general population, and those with FH are already at a 20 times higher risk for cardiovascular problems. It’s clear that not smoking (as well as not being exposed to secondhand smoke) is critical to the success of FH patients trying to avoid early heart disease.

Consider LDL-apheresis

For some people with FH, drug therapy and lifestyle changes aren’t enough to lower their LDL cholesterol to a safer level. These individuals may need a simple, regularly scheduled treatment called LDL-apheresis, during which blood or plasma is taken out of the body, the LDL cholesterol is filtered out, and the filtered blood or plasma is returned to the body.