Rare Disease Report

Dietary Guidelines for Familial Chylomicronemia Syndrome (FCS)

MAY 21, 2017
James Radke
Familial chylomicronemia syndrome (FCS) is a rare genetic disease characterized by extremely high triglyceride levels that puts patients at great risk for pancreatitis.
 
There are several treatments for FCS in development, but there aren’t currently any approved drugs for this condition. As such, patients must adhere to a very strict, low-fat diet to reduce the risk of pancreatitis.
 
At the National Lipid Association (NLA) Scientific Session meeting in Philadelphia this weekend, guidelines were presented to help both patients and healthcare professionals understand and adhere to a diet for FCS.
 
The diet is a lifelong commitment to adhere to less than 20 grams of fat or 15% of total calories daily. While fat is restricted, the diet should still, somehow, find a way to meet the daily nutrient requirements for essential fatty acids – omega 6 and omega 3.
 
Medium-chain triglycerides are recommended, while coconut oil is not. Medium-chain triglycerides can be sprinkled on food, but are not recommended for cooking. 
 
The guidelines stress that the diet should limit the carbohydrates eaten and to try to choose carbohydrates with fiber, and stress that alcohol cannot be consumed. While the diet is very restrictive, it is stressed that dieticians should help patients find recipes and cooking tips for preparing foods without using fats.
 
Additionally, the guidelines include a special section for the recommended diet for children with FCS. The children’s diet is essentially the same as the diet for adults, but supplemental long-chain triglycerides and fat soluble vitamins may be provided as needed.
 
Given the concern for adherence, the use of food diaries (and their regular review), as well as smart phone apps may be useful.
 
Foods to Increase
  • Vegetables (half your plate should be vegetables)
  • Whole grains and legumes
  • Fat-free dairy products
 
Foods to Avoid
  • Fat-containing foods
  • Refined Starches
  • Sugars
  • Sugary drinks
  • Alcohol
 
The authors of the guidelines note that patients with FCS are often in pain or discomfort that make adhering to a healthy, low fat diet very difficult. It is important that patients meet their daily dietary requirements and should regularly meet with a dietician or other health professional to discuss ways to best adhere to the strict diet.

Below, Wahida Karmally, co-author of the guidelines and a registered dietician at Columbia University, talks about the diet recommended for FCS patients.

Reference

Alexander L, Williams L, Welstead L, et al. Familial chylomicronemia syndrome (FCS): medical nutrition therapy guidelines for patients and healthcare providers. Presented at NLA Scientific Sessions; Philadelphia, PA; May 17-20, 2017.

Stay informed on the latest rare disease news and developments by signing up for our newsletter.
Copyright © RareDR 2013-2018 Rare Disease Communications. All Rights Reserved.