Familial hypercholesterolemia (FH) is a genetic disorder affecting approximately 1 in 311 individuals, characterized by elevated levels of low-density lipoprotein (LDL) cholesterol, often referred to as "bad cholesterol." This condition significantly increases the risk of developing coronary artery disease at a younger age. While medications are often necessary to manage cholesterol levels in individuals with FH, dietary modifications play a crucial role in overall health and can potentially reduce the need for higher medication doses.
Understanding Familial Hypercholesterolemia
FH is primarily caused by inherited genetic mutations that disrupt the body's ability to regulate and remove cholesterol from the blood. Early detection and treatment of high cholesterol levels are key to lowering the risk of cardiovascular disease. A high LDL cholesterol level at a young age is a particular red flag that you may have FH. If your doctor suspects you have the condition, you can undergo genetic testing to confirm the diagnosis.
Recognizing the Signs
One of the main signs of FH is LDL cholesterol levels over 190 mg/dL in adults and over 160 mg/dL in children. Most people with FH also have a family health history of early coronary artery disease or heart attacks. In some cases, elevated LDL levels are found through routine blood cholesterol screening. Healthcare providers may detect physical signs of FH during a clinical exam, such as xanthomas (fatty deposits) around tendons in the hands, knees, Achilles tendons, elbows, and under the skin around the eyes.
The Role of Diet in Managing FH
Although FH is a genetic disorder that requires medication to manage elevated LDL-C levels, diet and food choices can still make a significant difference. A low saturated fat diet has been shown to lower LDL-C by approximately 10% in individuals with FH. This may allow for a smaller dose of cholesterol-lowering medication(s) to manage LDL-C.
How Diet Works
Diet lowers blood cholesterol in a similar way to statins, ezetimibe, and PCSK9 inhibitors - by increasing the number of LDL receptors on the liver cell surface. The liver seeks cholesterol to produce hormones, bile, and other key molecules. When the diet is low in cholesterol and saturated fat, the liver must look elsewhere for cholesterol, leading to the production of more LDL receptors, which lowers blood cholesterol.
Read also: Lifestyle Tips for Lipomatosis
The Best Dietary Approach
If your cholesterol is high, you need to lower your intake of saturated fat and cholesterol. Current diet recommendations for FH include:
- Limiting saturated fat intake to less than 7% of total fat (the typical American diet is 12%).
- Replacing saturated fats with polyunsaturated fats, protein, or carbohydrates.
Since many foods with saturated fat also contain cholesterol, a low saturated fat diet will also lower cholesterol intake. Exceptions include shellfish and eggs, which have cholesterol but do not have a lot of saturated fat and have a lot of healthy nutrients. Therefore, moderate consumption of shellfish or eggs is acceptable.
Specific Dietary Recommendations
Here are some cholesterol-lowering health tips:
Limit cheese intake: Just one ounce of cheese has roughly 6 grams of saturated fat. Try low-fat cheese or use a cheese slicer to slice a couple of slivers of a flavorful cheese to top apple slices or a few whole grain crackers.
Eat leaner cuts of beef and pork: Choose cuts like eye of round and pork tenderloin, and trim as much visible fat as possible before cooking. Reduce portions to 3 ounces, about the size of a deck of cards, and supplement your plate with a variety of colorful vegetables and some whole grains.
Read also: The Hoxsey Diet
Replace butter with liquid oil: Use liquid oils from the Healthier Fats chart, cooking oil sprays, or a small amount of tub margarine.
Switch to low-fat milk: Use fat-free or 1% milk instead of whole milk, or try soy or almond milk.
Replace high-fat desserts: Substitute high-fat ice cream and desserts with naturally sweet and colorful fruits or sherbet.
Use low-fat milk in coffee: Instead of cream, use low-fat milk in your coffee.
Reduce animal protein in casseroles and soups: Cut the amount of animal protein in half and substitute beans (drain and rinse first, if using canned beans) for the remaining amount of meat.
Read also: Walnut Keto Guide
Remove skin from chicken: Remove skin from chicken and other poultry before eating.
Eat fish: Fish is a great source of healthy omega-3 fats. The American Heart Association recommends eating 3.5 ounces of fish (especially oily, omega-3 rich fish) at least twice a week. Note: Tilefish, shark, swordfish, and king mackerel have high mercury content and should be eaten only occasionally.
Include a meatless meal: Have a meatless meal at least once a week.
Choose whole grain breads: Choose breads that list the word “whole” as part of the first ingredient, such as “whole wheat flour.” Look for 2 to 3 grams of fiber per slice.
Switch to whole grains: Use whole grains such as brown rice, quinoa, hulled barley, whole wheat couscous, whole wheat pasta, or bulgur cracked wheat for side dishes.
Keep fruit visible: Keep a clear bowl of whole fruit on the counter or table. Research shows that keeping fruits close-by and visible helps us eat more of these disease-fighting super-foods.
Understanding Fats
Saturated fats tend to cause your LDL receptor sites to "become lazy" at clearing LDL cholesterol out of the system. If your diet is high in saturated fats, it is counter-productive to the medications you take to lower your cholesterol, such as statins, which work hard to increase LDL cholesterol clearance.
Here's a breakdown of cholesterol-raising and healthier fats:
CHOLESTEROL-RAISING FATS
Saturated Fats: Animal sources like butter, cheese, cream, fatty cuts of meat and processed meats (hot dog, bacon, bologna, salami, sausage), ice cream, lard, poultry skin, sour cream, whole milk. Plant sources like coconut, palm, palm kernel.
Trans Fats: Foods with a high probability of containing partially hydrogenated oils: Baked Goods biscuits, cakes, cookies, doughnuts, muffins, pancake mix, pastries, pie crust, pizza dough. Fried Foods French fries, breaded chicken or breaded fish. Snack Foods - crackers, microwave popcorn. Stick margarine, shortening and non- dairy creamer
HEALTHIER FATS
Monounsaturated Fats: Nuts, seeds and natural nut butters: almonds, hazelnuts, pecans, peanuts, pine nuts, pistachios, pumpkin and sesame seeds. Avocados, olives. Oils: canola, extra virgin olive, peanut, sesame
Polyunsaturated Fats: High omega-3 seafood: Arctic char, Atlantic mackerel, black cod (sablefish), herring, mussels, wild salmon, sardines, trout. Nuts and seeds: chia, ground flaxseeds, hemp seeds, soy nuts, sunflower seeds, walnuts. Oils: soybean, safflower, corn
Additional Dietary Considerations
Oat Fiber: Oat fiber lowers cholesterol, so keep eating oatmeal!
Plant Sterols and Stanols: Add plant sterols and plant stanols to your diet. They actively reduce LDL-cholesterol and can be found in certain food supplements and substitutes (look for margarine or cheese that states “With added plant sterols/stanols”). An enhanced daily consumption of 2000 to 2500 mg of plant sterols/stanols per day may lower LDL-cholesterol by up to 15%!
Cooking Tips: The AHA recommends a diet that emphasizes fish, poultry and plant-based proteins and limits red and processed meats. Choose cuts of meat that have the least amount of visible fat. Use a rack to drain off fat when broiling, roasting or baking. Cook a day ahead of time. Stews, boiled meat, soup stock or other dishes in which fat cooks into the liquid can be refrigerated.
Special FH Populations
There are three groups of FH patients for whom diet might be especially helpful:
Children under 10: Learning to like a healthy diet might have lifelong benefit. The initial approach to these youthful patients is to use the NCEP step I diet. This diet provides calories and nutrients that support normal growth and development, but limits saturated fat and total fat intake to no more than 10 and 30 percent of total calories, respectively, and cholesterol intake to no more than 100 mg per 1,000 kcal per day, to a maximum of 300 mg. If the goal of reducing the LDL cholesterol level to below 130 mg per dL (3.35 mmol per L) is not achieved, the more restrictive step II diet should be initiated. However, the step II diet may not provide sufficient calories and nutrients to support normal growth and development; therefore, trained nutritionists may be required to effectively manage a child on this diet.
Pregnant women: Often cholesterol-lowering medications are stopped during pregnancy and breastfeeding, so a healthy diet will lower cholesterol levels in that setting.
Individuals with an FH gene but LDL cholesterol below treatment thresholds: These individuals are at higher risk but are currently not candidates for lifelong medication. Diet in this setting will have an important impact on heart disease prevention.
Lifestyle Modifications
In addition to dietary changes, other lifestyle modifications are important for managing FH and reducing the risk of cardiovascular disease. These include:
- Regular physical activity: Aim for at least 30 minutes of moderate-intensity exercise most days of the week.
- Maintaining a healthy weight: If you are overweight or obese, losing even a small amount of weight can improve your cholesterol levels and overall health.
- Avoiding smoking: Smoking damages blood vessels and increases the risk of heart disease.
The Importance of Early Detection and Treatment
Early detection and treatment of FH are crucial for reducing the risk of coronary artery disease. If you have a family history of early heart disease or high cholesterol, talk to your doctor about getting screened for FH. With a combination of medication and lifestyle modifications, individuals with FH can live long and healthy lives.
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