Treatment:

Proper diet, exercise, and certain medications can bring lipids (fats in the blood) down to safer levels and reduce your risk of heart disease and heart attacks. Those who inherit only one copy of the defective gene may respond well to diet changes combined with statin drugs.

The first step is to change what you eat. You typically follow a modified diet for several months before your doctor adds on medications. You'll be told to decrease fat intake to less than 30% of the total calories you get each day.

You can reduce fat intake by:

• Eating less beef, pork, and lamb
• Choosing low-fat dairy products
• Avoiding coconut and palm oil

Cholesterol intake is reduced by avoiding:

• Egg yolks
• Organ meats
• Sources of animal-derived saturated fat

Further reductions in the percentage of fat in the diet may be recommended. For more information, see: Heart disease and diet.

Exercise, especially to induce weight loss, may also aid in lowering cholesterol levels.

Drug therapy may be started if diet, exercise, and weight-loss efforts have not lowered your cholesterol levels over time. Several cholesterol-lowering drugs are available, including:

• Bile acid sequestrant resins (cholestyramine and colestipol)
• Fenofibrate
• Gemfibrozil
• Nicotinic acid (niacin)
• Statin drugs

In rare cases, persons who inherited two copies of the defective gene may need surgery such as a liver transplant.

Expectations (prognosis):

How well you do greatly depends on whether or not you follow your doctor's treatment recommendations. Diet changes, exercise, and medications can lower cholesterol levels, and may significantly delay a heart attack.

Men and women with familial hypercholesterolemia typically are at increased risk of early heart attacks.

Risk of death varies among patients with familial hypercholesterolemia. Persons who inherit two copies of the defective gene have a poorer outcome. That type of familial hypercholesterolemia causes early heart attacks and is resistant to treatment.

Complications:

• Heart attack at an early age
• Heart disease

Calling your health care provider:

Seek immediate medical care if you have crushing chest pain or other warning signs of heart attacks.

Call for an appointment with your health care provider if you have a family history of high cholesterol levels.