DWQA QuestionsCategory: QuestionsCPT II deficiency – diet recommendations (essential fatty acids, LCT, dojolvi) and complications
Jane asked 2 years ago
Hi,
I have a few questions on diet recommendations, and complications, for patients with CPT II deficiency

What is the minimum daily recommended intake of long-chain fats? 10% or 8%?

Essential fatty acids: If we meet the DRI for omega-6, for omega-3 supplementation, can we supplement with DHA solely and NOT EPA? Let’s say that the DRI for omega-3 is 1.1 grams for my age, can I take 1.1 grams of DHA solely?

Is there a type of oil (or food) that contains more omega-6s than safflower oil with the same quantity of long-chain fats?

Is there a benefit in distributing long-chain fats evenly throughout the day, just like protein, or can we consume them all in one shot? Also, is there a moment during the day when it is best to take them?

Sick days: Is there a benefit in increasing Dojolvi intake (and adding some sugary drinks), or keeping the same dosage and adding sugary drinks, for optimal recovery?

Complications related to cpt2 deficiency (kidney injury, cardiomyopathy, etc): do they usually occur during acute decompensation, such as rhabdomyolysis, or can they develop slowly over time (for example, due to frequent elevations of ck levels, without necessarily reaching rhabdo)? Any resource you would recommend where I can read more on it, specifically for cpt2?

Thank you very much for taking the time to answer my questions!

1 Answers
Keith McIntire answered 2 years ago

What is the minimum daily recommended intake of long-chain fats? 10% or 8%?

I would recommend a minimum of 10% to prevent essential fatty acid deficiency. That being said, most people would be not be able to really accurately measure 10% vs 8% total calories from long-chain fats. Somewhere in that ballpark is as low as you would want to restrict fat intake. (This is fat from food and supplements)

Essential fatty acids: If we meet the DRI for omega-6, for omega-3 supplementation, can we supplement with DHA solely and NOT EPA? Let’s say that the DRI for omega-3 is 1.1 grams for my age, can I take 1.1 grams of DHA solely?

Yes. However, I don’t think you need 1.1 gm DHA. I think 250-500 mg would be plenty to keep your blood DHA within the normal range. You could consume some of the omega-3 in the form of Linolenic acid from flaxseed, walnut or canola oil.

Is there a type of oil (or food) that contains more omega-6s than safflower oil with the same quantity of long-chain fats?

Corn and safflower are the highest in omega-6 Linoleic acid.

Is there a benefit in distributing long-chain fats evenly throughout the day, just like protein, or can we consume them all in one shot? Also, is there a moment during the day when it is best to take them?

Almost all meals are a mix of protein, carbohydrates and some fat. It seems nearly impossible to me to eat completely fat free meals most of the day and just consume fat at one shot? So yes, I think long-chain fat should be spread throughout the day. When analyzing the diet records of a number of subjects with LCHADD deficiency, I observed breakfast is often fat-free (fat-free cereal with fat free milk for instance) and the more long-chain fat is consumed with lunch, dinner and minimal with snacks. Fat-free meals will limit the absorption of fat-soluble vitamins. (DON’T take vitamin supplements with a fat-free meal). Fat contributes to satiety and slows gastric emptying and absorption. Thus, spread out across meals helps with digestion and absorption of nutrients, and improves feelings of fullness.

Sick days: Is there a benefit in increasing Dojolvi intake (and adding some sugary drinks), or keeping the same dosage and adding sugary drinks, for optimal recovery?

We do not have good data to support an answer to this question. It is all based on opinion. I would recommend adding some sugary drinks first and leaving the Dojolvi intake the same but that might depend on the dose of Dojolvi and the circumstances of the metabolic crisis.

Complications related to cpt2 deficiency (kidney injury, cardiomyopathy, etc): do they usually occur during acute decompensation, such as rhabdomyolysis, or can they develop slowly over time (for example, due to frequent elevations of ck levels, without necessarily reaching rhabdo)? Any resource you would recommend where I can read more on it, specifically for cpt2?

Kidney injury typically occurs with acute decompensation. I’m not aware of cardiomyopathy in an adult-onset CPT2 patient. I think that risk is very low. Rhabdo is the biggest complication. Repeated rhabdo could lead to some muscle weakness. I can’t think of one good resource to read specific to CPT2. I’m attaching a book chapter on FAODs that is the most comprehensive available. ( Mitochondrial Fatty Acid Oxidation Disorders /  Jerry Vockley; Michael J. Bennett; Melanie B. Gillingham )

I would encourage you to work with a metabolic dietitian to help optimize your nutrition, balance your long-chain fat intake and develop a good sick day plan.

Melanie Gillingham, PhD, RD