“I had my daughter’s tested last year after reading about it in the Cherab group. It was low, although not super low. I just had her tested again and it was lower. 🙁 The reason I am really glad we had hers tested is because we found out around the same time, that she has an enlarged left ventricle in her heart.”
Risk of Not Enough Carnitine
While some may look to limit carnitine in light of the recent study, carnitine deficiency testing is recommended for children with autism and apraxia. The information below was written before the recent research, but the advice remains the same.
If your child has autism or apraxia it’s worth getting the carnitine levels checked due to the much higher than average amount in our group who have tested borderline or low. Do not supplement with carnitine unless your pediatrician orders the blood draws first. You want to know prior to supplementation if your child is deficient or the supplementation can skew the results.
From our apraxia.org support group one mother wrote: “I had my daughter’s tested last year after reading about it in the Cherab group. It was low, although not super low. I just had her tested again and it was lower. 🙁 The reason I am really glad we had hers tested is because we found out around the same time, that she has an enlarged left ventricle in her heart. (this was totally by chance – the geneticist referred us to the cardiologist to have a look at her even though we never suspected any heart issues). Low carnitine plus dilated left ventricle can be signs of cardiomyopathy. So she is getting echocardiograms every 6 months to see if her left ventricle is actually dilating or if it is just an odd shape to begin with. I’m just very, very glad she was tested, because it meant the cardiologist has taken this all much more seriously than he would have if there were no other signs. Her’s is a secondary deficiency. Now I encourage all my friends of kids with speech disorders to have their child’s carnitine levels tested too – you just never know!”
A carnitine deficiency if one is found in testing should be evaluated by a medical doctor who specializes in the metabolic system even when one believes or is told that it’s due to a vegetarian diet
According to the NIH
“Healthy children and adults do not need to consume carnitine from food or supplements, as the liver and kidneys produce sufficient amounts from the amino acids lysine and methionine to meet daily needs [1-3]. The Food and Nutrition Board (FNB) of the National Academy of Sciences reviewed studies on the functions of carnitine in 1989 and concluded it was not an essential nutrient” [1-3]
- Rebouche CJ. Carnitine. In: Modern Nutrition in Health and Disease, 9th Edition (edited by Shils ME, Olson JA, Shike M, Ross, AC). Lippincott Williams and Wilkins, New York, 1999, pp. 505-12.
- The editors. Carnitine: lessons from one hundred years of research. Ann NY Acad Sci 2004;1033:ix-xi.
- National Research Council. Food and Nutrition Board. Recommended Dietary Allowances, 10th Edition. National Academy Press, Washington, DC, 1989.
According to University of Oregon
“Nutritional carnitine deficiencies have not been identified in healthy people without metabolic disorders…Healthy individuals, including strict vegetarians, generally synthesize enough L-carnitine to prevent deficiency.”
According to the study Primary carnitine deficiency and pivalic acid exposure causing encephalopathy and fatal cardiac events published in The Journal of Inherited Metabolic Disease 2012
“Vegetarians have lower plasma carnitine levels than non-vegetarians, but still within normal range indicating an effective biosynthesis and renal conservation”
Carnitine, unlike and not to be confused with the amino acid carnosine, is something to take serious. You may not see changes on or off supplementation but if tested low you’ll want your child moderated by a physician and on prescription carnitine like Carnitor for the reasons covered in the study above as well as the reasons covered below.
Testing low in carnitine is typically a rare disorder affecting one in 20 to 40,000. Through the Cherab Foundation support group out of those that did blood draws the odds were much higher that a child with apraxia or autism tested borderline or low in carnitine. This was also the reason I took my son Tanner for all the blood testing -to test for this one deficiency since it can be so severe if left untreated.
Unlike other supplements -a carnitine deficiency could come with the most severe consequence over time since carnitine deficiency is associated with sudden death. The good news is that at least you’ll know early, and it’s very simple to treat. Most parents find out their child was deficient in carnitine upon autopsy.
“Impact of carnitine supplements may not be clinically obvious – but could be helping strengthen heart muscle. Low levels are associated with a floppy dilated weak heart, and when it approaches 10-20% of normal, sudden death can occur. Carnitine is essential for normal cell/mitochondria function. It allows fatty acids to get transported into the mitochondria (or the battery of the cell). Major problems if this is not working properly. So you may be getting benefits that you don’t obviously see. One does not want to run around with a severe carnitine deficiency and not treat it.”
If you don’t know if there is a deficiency through testing, how else are you going to guarantee that not only are you going to give and keep giving your child this supplement which they ‘have’ to take -but that your child will know to monitor with his doctor and continue to supplement if needed perhaps the rest of his or her life? This is why you want to investigate this aggressively with a doctor who specialize in metabolic or mitochondrial disorders.
Carnitine is an amino acid manufactured in the body from the essential amino acids lysine and methionine. Essential means your body can’t produce them -so you would need to consume foods that contain them. One thing to keep in mind however is that it’s not just what you consume, but what your body digests and utilizes. It is possible that nutritional support can make a difference in improving carnitine levels as reported by some of the parents. Carnitine plays a key role in transferring long-chain fatty acids (such as those found in fish oils) into the mitochondria so that they can convert these acids to energy.
Without sufficient CarniTine…the mitochondria (or cell “batteries”) don’t run very well. There can be symptoms from muscle weakness, heart problems to severe brain and metabolic abnormalities and in some (rare) cases… to again the extreme of sudden death from carnitine deficiency…depending on how severe and the exact cause.
Also just wanted to point out that me and many others here had the blood draws ordered through our regular pediatrician and the labs done through medical labs -and if tested low the child is put onto prescription carnitine like Carnitor with a referral to a doctor that specializes in metabolic syndromes. If it’s not prescription you never know what you are getting. While carnitor is regulated. Plus it gets covered by insurance…an added benefit, as all these supplements start to add up and are fairly costly.”
Prescription carnitine goes by the following names L-Carnitine is available as a prescription drug under the names: Carnitine, Carnitor, L-Carnitine and Vitacarn. Carnitor, manufactured by Sigma Tau, seems to be the most widely stocked brand. The generic name is levocarnitine. There are no known interaction effects between L-carnitine and other drugs or foods. There are no known diseases for which L-carnitine is known to be harmful. Note: If your L-carnitine comes wrapped in plastic containers, leave them wrapped up until you use them. Out in the air they absorb moisture and can crumple up
For those who have children that were tested low in carnitine if you haven’t already I suggest you take your child to a medical doctor; preferably a geneticist who specializes in metabolic disorders. Not much if anything may change in the tone or speech -but that’s not the reason to supplement with prescription carnotine.
Differences between primary and secondary carnitine deficiency from the Role of carnitine in disease
Two distinct carnitine deficiency states have been reported although a rigid distinction between “primary” and “secondary” carnitine deficiency is difficult to establish in some cases [10]. Primary carnitine deficiency (PCD) is a rare autosomal recessive disorder of fatty acid oxidation caused by deficiency of plasma membrane carnitine transport resulting from impairment in the plasma membrane OCTN2 carnitine transporter.
Secondary deficiency is characterized by increased carnitine excretion in urine in the form of acyl-carnitine due to an accumulation of organic acids [36,37]. Secondary carnitine deficiency can be caused by increased losses, pharmacological therapy, a number of inherited metabolic disorders [38], poor diet or malabsorption of carnitine, from increased renal tubular loss of free carnitine (Fanconi syndrome), haemodialysis, peritoneal dialysis, or the increased excretion of acylcarnitines[39] with certain drugs. There have been reported at least 15 syndromes in which carnitine deficiency seems to be secondary to genetic defects of intermediary metabolism or to other conditions [40]. Patients with secondary carnitine deficiency accumulate organic acids which causes enhanced urinary excretion of carnitine in the form of acyl-carnitines. Read more.
Blood work to consider
I got all the following ordered through my son’s pediatrician and all was covered by our health insurance.
- Celiac panel
- Fat soluble vitamins (A, D, E, K) (May have fat malabsorption)
- Metabolic studies: Lead level, CBC, complete metabolic panel, plasma quant amino acids
- carnitine (total, free, esters), acyl-carnitine panel (plasma),
- urine organic acids, lactic acid, comprehensive fatty acid profile
- (C8-26)
A sendout to Mayo clinic – but other labs do it too. (rule out metabolic disorder as cause of severe neurodevelopmental disorder -apraxia/dyspraxia).
Celiac pane includes serum for:
- human tissue transglutaminase antibodies (TTG),
- antiendomysial antibodies (EMA),
- anti-gliadin IgA antibody,
- anti-gliadin IgG antibody, and
- total immunoglobulin A.
From Tina, a parent of an apraxic son who has tested low in carnitine:
“Going to a geneticist that specializes in metabolic disorders would be very helpful. My understanding from what I have read a biopsy helps confirm mito disorders. Lab work is first though. The link below might help you find more answers.
United Mitochondrial Disease Foundation
Landon’s carnitine level was 23 normal was 25-70 in July. In Dec. it was ran again once he was on prescription carnitine and it was 76 again normal range 25-70. The geneticist that specializes in metabolic disorders isn’t concerned with his Carnitine level being high. He did run a lab to see if it is coming out in urine which is what would happen if it isn’t being used up.
And he did run Carnitine again in Feb so it probably will be higher than it was in Dec. We will probably have to adjust his dose. Landon could come off Carnitine right now, but I thought it was helping him some so the doctor said to continue. Regular blood test should be done to check levels so dosing can be changed either increased or lowered.
Here is an article that discusses Carnitine and CoQ10. It is a good explanation, hope you all will take the time to read it from the top to the bottom. HTH, Tina”
(note: Tina’s apraxic son was doing really well, but she like I did the blood draws for carnitine just to check. While my son Tanner tested normal, her son Landon tested low in carnitine)
Some use OTC carnitine brands, doctors against OTC for carntine if a child tests low for quality and standardization reasons.
Are you using prescription carnitine?
“Certain levocarnitine products like Carnitor have been specifically approved by the U.S. Food and Drug Administration for medical use and are available only with your doctor’s prescription. Other levocarnitine products are sold without a prescription as food supplements and should not be used to treat serious levocarnitine deficiency.” (original source)
Not much if anything may change in the tone or speech -but that’s not the reason to supplement with prescription carnotine
Even though I do recommend testing for carnitine, in general I’m a huge believer in trying the basics first which mean appropriate therapies for your child and trying essential nutrients. This mean essential fatty acids from fish oils and the essential amino acids and nutrients from foods like whey isolate protein, and ayurvedic botanicals like Turmeric, Amalaki, Haritaki, Gymnema, Green Tea, Guggul, Cocoa, Cinnamon, and Cayenne Pepper. And again carnitine is an amino acid manufactured in the body from the essential amino acids lysine and methionine found naturally in foods such as whey isolate protein. And carnitine plays a key role in transferring long-chain fatty acids (such as those found in fish oils) into the mitochondria. It’s all pretty simple.
Sometimes things can be simple, and then you can save your time and money for karate and swimming lessons -maybe some hippo therapy or a much needed vacation for the family.
Risk of Too Much Carnitine
If supplementing carnitine without a doctor’s recommendation and blood testing, which is not advised for all the reasons stated above, please know there can be too much of a good thing. A study published online April 2013, found that carnitine, a compound abundant in red meat that also is sold as a dietary supplement and found in some energy drinks, when digested by bacteria in the intestines, may increase the likelihood of heart disease and stroke. As this Forbes article wrote “The research also provides another quite surprising example of the previously unsuspected health effects of bacteria in the intestine. Published online in Nature Medicine, the new studies suggest a possible major role in atherosclerosis for carnitine, which is commonly added to energy drinks and is found naturally in high concentrations in red meat. The new theory combines several lines of evidence from studies in both animals and humans.”
Thank you for this article. My son is having labs done to see if he’s low in carnitine. He is apraxic and has severely low tone. This article is super dense in information, thank you.