DWQA QuestionsCategory: QuestionsHeart Healthy Diets / Should there be a universal nutritional guideline for kids with FODs ?
Katie asked 5 years ago
Before my twins were born and diagnosed with MCAD a few days after birth, I had been really interested in the powerful healing effects of a plant-based diet. I understand that everyone is unique and will be helped/hurt by different types of diets – but it seems that plant-based can be good for just about EVERYONE, even if you have to customize the diet to weed out certain foods for sensitivity reasons, and add in some animal-based foods in order to ensure optimal health/nutritional intake for each unique person.
I realized that a high-carb, low-fat, plant-based diet (and when I say carbs I mean non-to-minimally processed whole grains) that I was reading about before their birth might actually be the best diet to put them on. When I brought this up to their medical team, they said they don’t restrict any foods and encouraged me to do what I wanted – but to just limit foods with medium-chain fats.
In reading the boards on Facebook for parents of FOD kids, I see a trend that a lot of physicians are recommending “heart healthy” diets for MCAD kids. There are not many specifics around that for people, except that it means try to eat as healthy as possible. However, in my research of plant-based whole foods, one of the things many advocates say to cut out is any type of oil. It is all fat and offers minimal-to-no nutritional benefit. People don’t realize that most foods (even seemingly healthy ones) have added oils. I didn’t realize this, and I’m a nutrition-info junkie. So people may think they are eating healthfully, but they are consuming a lot of extra oil (fat) throughout the day.
So, my question is: SHOULD there be a universal nutritional guideline for kids with FODs? Has this even been studied? Are there any thoughts at all on a specific type of diet, like plant-based?
Also: I am concerned about what my kids bodies do with fats they cannot process. Does that get stuck in their liver? (Another reason why I’m trying to keep them on low fat)

1 Answers
INFORM Physician answered 5 years ago
Katie, 
This is quite a complicated with an equally complicated answer. The simple answer is that we would all like to have more standard protocols for dietary management of all of the FAODs. However, there is not a lot of data (though a lot of opinions) on what is best, and it takes a long time to do a formal guideline. For example the metabolic dietitians group has been working for a couple of years on a VLCAD dietary guideline that should be finished soon. That said, there are some general responses that I can make regarding your comments. While there is some variability between a vegetable and animal diet, the similarities are greater. Fat in excess for anyone is bad. Thus the heart healthy recommendation of 30-35% of calories from fat. Oil is not inherently bad, but food with excess added oil tend to be higher in calories than needed. Do remember that we need to have ~10% of our calories from long chain fats to keep from becoming essential fatty acid depleted. Adding in the complication that patients with FAODs cannot  metabolize specific fats, the dietary issues are such that it really is necessary to work with an experienced metabolic physician to keep things balanced. For example in long chain defects, we like to use slight higher protein than is standard in a general diet, but need to restrict long chain fats to allow room fo the medium chain fats that these patients can metabolize, In MCAD deficiency, long chain fats are better tolerated than medium chain ones, but we still try to keep them at the heart healthy level. As to general diet resources, I tend to stay away from the fad literature and try to stick to something that I know is good with the basics. I like the American Heart Association Heart Healthy diet in this regard. It’s especially useful for MCAD deficiency, though long chain deficient patients need to modify the fat intake according to their metabolic diet. If I can take a moment to add a bit of a commercial for INFORM, this is exactly the type of topic that we hope the consortium can address going forward so that we can advance our clinical knowledge along with our scientific. Hope this helps. Dr. Vockley