Familial Chylomicronemia Syndrome

1: Adopt a very-low-fat diet by either:

• consuming less than 15 to 20 g of fat per day or
• restricting total dietary fat to less than 10% to 15% of total daily caloric intake or
• individualizing fat restrictions to meet the daily essential fatty acid (EFA) needs of 2% to 4% daily caloric intake of a-linolenic acid (ALA) and linoleic acid (LA).

2: Meet EFA requirements of 2% to 4% of daily calories in the very-low-fat diet.

3: Medium-chain triglyceride (MCT) oil may be used as a source of fatty acids not transported via chylomicrons and therefore allow for adjustment of caloric intake and macronutrient composition of the diet.

4: Include fat-free or low-fat proteinrich foods with each meal and snack.

5: Choose complex carbohydrate foods, limit simple and refined carbohydrate foods, and avoid foods with added sugars.

Monitor levels of fat-soluble vitamins (A, D, E, K) and minerals regularly, supplementing as needed.

7: Encourage nutrient-dense foods as a component of the very-low-fat diet.

8: Distribute calorie and fat intake throughout the day while avoiding excess caloric intake.

9: Abstain from consuming alcohol.

10: Engage in at least 150 minutes of physical activity per week to improve overall health outcomes.

11: Drink sufficient water and stay adequately hydrated.

12: Develop an individualized, multidisciplinary, and collaborative management plan to address all aspects of FCS disease management.