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.

Assess the nutritional adequacy of the diet by monitoring developmental and cognitive milestones in pediatric patients with FCS.

Infants diagnosed with FCS require a special low-fat, low-LCT, high-MCT formula or skimmed and fortified expressed breast milk.

Transition children aged 12 to 24 months to skim milk and age-appropriate, low-fat solid foods.

Provide families and caregivers with specific meals and/or guidelines for grams of fat per meal and snack. The recommended age-appropriate protein consumption should be encouraged.

The goal for pregnant patients is to maintain fasting TG levels less than 500 mg/dL by following a very-low-fat diet, consume adequate calories to support pregnancy, and maintain electrolyte and fluid balance. Regular monitoring of TGs and diet adjustment during pregnancy is important.

Establish a multidisciplinary approach to develop an individualized management plan for FCS patients with diabetes.

Patients should be adequately hydrated after episodes of acute pancreatitis and should continue to follow the restricted very-low-fat diet.