DWQA QuestionsCategory: QuestionsPrescriptive Diet Recommendations for GA2/MADD/ETFA
Lori asked 1 year ago
What are the macro nutrient percentage recommendations for GA2 in a symptomatic adult? Or, to put it another way, what percentage of calories should come from carbohydrates, proteins and fats? Are there any additional recommendations relative to tryptophan?

1 Answers
Keith McIntire answered 1 year ago

There are no solid researched dietary guidelines, so the following is based on the pathway and experience in other fatty acid oxidation disorders.

I would say yes high carbohydrate (70% of energy), with adequate protein (15% of energy) and low fat (15% of energy) are good targets strive for.

That is not an easy diet. Needs to include really low-fat protein options like beans, tofu, nonfat dairy, egg whites. High protein options like beef

and pork or cheese will increase the total fat intake.

Small frequent meals are critical. Not more total energy than someone without GA2. Just spread strategically across the day.

There are few ETF mutations that are riboflavin responsive so I would recommend high dose riboflavin. May not help but it could.

About 10 mg per day. (RDA for women is 1.1 mg/day). Carnitine has been prescribed when free carnitine is low, but the benefit.

of carnitine supplementation has not been demonstrated. You can have your doctor measure a carnitine panel (free/acyl) to determine if free carnitine is low.

I’m not aware of recommendations relative to tryptophan.  Thanks, Melanie Gillingham, PhD, RD