Protein Supplements and Urea Cycle Disorder

Protein Supplements and Urea Cycle Disorder

So with the media circulating the craze about a 25-year old bodybuilding mom who died of over-use of protein supplementation, let's break down the facts about the underlying health condition that actually lead to her demise.

The media is pointing the blame at protein supplements. In actuality, it was a genetically linked metabolic condition called urea cycle disorder (UCD) that ultimately caused her death. [1]

Related - Deadly Protein Shakes? The Story of Meegan Hefford & Urea Cycle Disorder

Urea cycle disorder. What exactly is it? Glad you asked.

According to Nettesheim, et al, (2017), urea cycle disorders (UCDs) are a group of rare inherited metabolic disorders. Individuals affected often present with hyperammonemic encephalopathy (HE) or high levels of ammonia in the blood that damage the brain. Due to this, these individuals have an increased risk of severe neurologic disease and early death.

The disorder is caused by inherited deficiencies of the six enzymes and two naturally occurring transporters in the body that are involved in the irreversible metabolic process of detoxification or breakdown of ammonium to urea for excretion from the body. Ammonium and urea are essentially the byproducts of the breakdown or digestion of protein from the food we consume. [2][3]

This process in general points to a pathophysiological pathway that explains why if one has this condition, they should use caution in consuming a high protein diet regardless of if the protein source is from food or the use of protein supplements.

Three key factors that should have been examined in this incidence explain why you should always "think before you drink" and listen to your body and professional advice.

Protein Powder Causes Death? Meegan Hefford and Urea Cycle Disorder

Think: The first factor is to think about your health. Know the facts about your current health condition before you embark on any competitive sports endeavors or decide to ingest ergogenic aids, such as bodybuilding or protein supplements. This means talk to a professional health care provider prior to if you are unsure of your current personal health history or genetic predisposition. [2]

Act: Secondly, do what you are told! Once you know your body's personal health and genetic limitations adhere to the professionally prescribed regimen and recommendations.

If you are told by your doctor or health care provider that your condition warrants a protein-restricted diet, then by golly eat less protein. Find out what the safe limits are and stick to that regardless of what everyone else is doing or telling you to do.

If you have a medically diagnosed condition that can be aggravated by a high protein diet, then don't consume a high protein diet, Period! [2]

Read: Lastly, read your labels! The articles circulating social media and other media platforms stated that more regulations need to be placed on protein supplements. [1]

However, this incident could have easily been caused by a high protein diet. So where is the stab at meat regulations and other high protein foods and food products, when the consumption of any protein-based product, food or supplement, is really a matter of personal choice.

The Food and Drug Administration (FDA) defines dietary ingredients as vitamins, minerals, herbs or other botanicals, amino acids, or dietary substances used by an individual to supplement their diet, thus increasing the total dietary intake. This can also include a concentration, metabolite, constituent, extract, or combination thereof of the preceding substances mentioned in the body.

Also, unlike drugs, supplements are not intended to treat, diagnose, prevent, or cure diseases. That means supplements should not make medical claims of curing a disease or relieving symptoms of a disease, as these claims can only legitimately be made for drugs, not dietary supplements. [4]

In fact, most all legitimate bodybuilding and dietary supplements on the market explicitly state warnings for consumers to check with a physician or healthcare provider before consuming the product. These products will also indicate that whatever health affects or benefit claims listed on the label have not been directly evaluated by the FDA.

Both reasons to read and take action to ask a health care professional and do further research before ingesting large quantities, especially if you have a pre-existing health condition. [1]

Furthermore, in regards to label reading and the case for existing regulations, according to the U.S. Department of Health and Human Services (USDHHS), the FDA already regulates both finished dietary supplements and dietary ingredients. The regulations for dietary supplements are found under a different set of regulations than those covering conventional food and drug products.

These regulations can be found under the Dietary Supplement Health and Education Act of 1994 (DSHEA) which states that "manufacturers and distributors of dietary supplements and dietary ingredients are prohibited from marketing products that are adulterated or misbranded." [4] This is a fancy way of saying unsafe and incorrectly labeled.

That means that these manufacturers of bodybuilding, dietary, and protein supplements are responsible for evaluating the safety and labeling of their products before marketing them to consumers to ensure that they meet all the requirements of DSHEA and FDA regulations. [4]

Sadly, this case took place outside of the United States, for which their regulations may be completely different. [1] However, neither here nor there, if you choose to consume any dietary supplements or ergogenic aids you should always THINK, ACT, and READ before you DRINK!

1) Mazziotta J. Bodybuilding Mom of 2 Reportedly Dies of Protein Overdose. Published August 15, 2017. Accessed August 16, 2017.
2) Nettesheim S, Kölker S, Karall D, et al. Incidence, disease onset and short-term outcome in urea cycle disorders ?cross-border surveillance in Germany, Austria and Switzerland. Orphanet Journal of Rare Diseases. Published June 15, 2017. Accessed August 16, 2017.
3) Excretion and the liver. Chemistry for Biologists. Accessed August 16, 2017.
4) U.S. Department of Health and Human Services. Dietary Supplement Products & Ingredients. U S Food and Drug Administration Home Page. Published August 1, 2017. Accessed August 16, 2017.
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