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Hypoglycemia and Ketosis - your thoughts?

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21
While doing a search of PR for fatty acids I ran into this thread, and I was wondering if any of you already knew about fatty acid oxidation disorders?

I did Genos whole exome genetic testing earlier this year, and my results show a homozygous ACADS mutation at 625G>A. This defect is related to short-chain acyl-CoA dehydrogenase deficiency (SCAD deficiency) 1. This is a fatty acid oxidation disorder (FOD), and these disorders are associated with hypoglycemia and the inability to use fats as energy 2. If I'm understanding this articles correctly, short-chain acyl-CoA dehydrogenase is an enzyme which breaks down short chain fatty acids into the form of energy used by mitochondria 3. Additionally, when short chain fatty acids build up, they can create ethylmalonic acid (EMA) 4.

My elevated EMA was 11.4 mcg/mg, normal is <3.6, upper limit 95% 6.3 (per the Genova ION test I took).

I believe my youngest (8) has been having hypoglycemic episodes, although we don't have a glucose meter to verify. When fasting for any longer than about 12 hours (nighttime), she gets shaky, pale, sweaty, nauseous, and has thrown up once. She also feels anxious during these episodes. We have been avoiding this by feeding her frequent high carb meals, and that has eliminated these episodes. Additionally, she has always had an aversion to foods with texture, and especially doesn't like fiber. I haven't gotten her whole exome testing yet, but I did order the Great Plains Organic Acids test, so I should know in 3 weeks or so what her EMA levels are.

This article is interesting, and discusses the effects of ethylmalonic aciduria on energy levels 5.

Also, most of the articles I read about SCAD deficiency are related to infants and newborns. The homozygous mutation is not rare, but considered symptomatic if ethylmalonic acid levels are elevated. One of the articles I referenced earlier discusses adult onset symptoms being related to myopathy.

I personally do not have hypoglycemia as far as I know. When I was on the ketogenic diet last year, my A1C was 4.8, and my fasting glucose was 87. Insulin at that same time was 7.9 (which I don't know if that's pertinent or not). I didn't notice increased energy while I was on the ketogenic diet, and now am unable to tolerate most sugars (other than fruit and sugar alcohols), so I can't test if I feel more energetic with glucose. I do think I messed with my gut bacteria when I was on keto though, as I'm having problems tolerating more foods than before.

When I was younger, I do think I had hypoglycemia occasionally, but it was usually the morning after a softball tournament and a night of drinking ;)

I have CFS/ME, POTS, EDS, and Chiari. I'm not a medical professional or scientist.