Deadly Protein Shakes The Storyline of Meegan Hefford Urea Cycle Disorder

 

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Deadly Protein Shakes? The Story of Meegan Hefford & Urea Cycle Disorder Protein is essential in any individual?s diet. it’s all the more important (and critical) for bodybuilders as it provides the key amino acids to grow, repair, and rebuild muscle tissue. Meegan Hefford, a 25-year-old bodybuilder who was found unconscious in her Australian apartment, died due to urea cycle disorder, a rare genetic disorder. However, the 25-year-old had not known that she had urea cycle disorder, a genetic condition that makes it difficult for the body to break down protein. Urea Cycle Disorder causes ammonia to build up in the blood if you have too much protein leaving harmful waste products behind.

Meegan suffered permanent brain. Meegan Hefford of Mandurah, Australia started bodybuilding in 2014 and she began a high-protein diet consisting of lean meats, egg whites and protein shake. For Some People, Too Much Protein Can Be Deadly—Here’s What You Need to Know About Urea Cycle Disorders Meegan Hefford had an undiagnosed genetic condition called urea cycle disorder. Learn how. Hefford had been eating protein-rich foods over the last few months of her life in addition to drinking protein shakes and taking vitamin supplements.

Hefford was. Meegan Hefford was found unconscious in her West Australia apartment and was declared brain-dead, passing two days later. Unbeknownst to her, she suffered from Urea Cycle Disorder (UCD), such that when she consumed protein shakes and supplements she overproduced a toxic byproduct. What is Urea Cycle Disorder?After Hefford’s death, the doctors discovered that she had a rare condition called a urea cycle disorder, which affects how the body breaks down protein.

Experts at the local hospital said she was suffering from urea cycle disorder. This disorder is a genetic condition that affects the way your body digests protein, impacting the way your body removes ammonia from your bloodstream. When too much ammonia builds up, it results in permanent brain damage, and in Hefford’s situation, death.

List of related literature:

Children with urea cycle defects will develop dangerously high levels of ammonia after the ingestion of a high protein containing meal, or during times of increased muscle catabolism as seen during starvation states, severe systemic illnesses, or prolonged exercise.

“Pediatric Critical Care Study Guide: Text and Review” by Steven E. Lucking, Frank A. Maffei, Robert F. Tamburro, Neal J. Thomas
from Pediatric Critical Care Study Guide: Text and Review
by Steven E. Lucking, Frank A. Maffei, et. al.
Springer London, 2012

Patients with this disorder suffer from protein intolerance and episodes of hyperammonemia that result from having insufficient arginine and ornithine for proper functioning of the urea cycle.

“Encyclopedia of the Neurological Sciences” by Robert B. Daroff, Michael J. Aminoff
from Encyclopedia of the Neurological Sciences
by Robert B. Daroff, Michael J. Aminoff
Elsevier Science, 2014

Protein should be held for 12 to 24 hours if there is a possible protein metabolism disorder (aminoacidopathy, organic acidemia, or urea cycle defect) but should not be withheld for a prolonged period or the individual will break down endogenous protein, worsening the clinical course.

“Berman's Pediatric Decision Making E-Book” by Lalit Bajaj, Simon Hambidge, Ann-Christine Nyquist, Gwendolyn Kerby
from Berman’s Pediatric Decision Making E-Book
by Lalit Bajaj, Simon Hambidge, et. al.
Elsevier Health Sciences, 2011

The therapy of inborn errors of the urea cycle is based on as severe a restriction of protein intake as possible, with frequent small meals so as to reduce still further the amount of ammonia that must be metabolized at any time.

“Chemistry and Biochemistry of the Amino Acids” by Graham Barrett
from Chemistry and Biochemistry of the Amino Acids
by Graham Barrett
Springer Netherlands, 2012

The urea cycle disorders result from defects in the metabolism of nitrogen, which is predominantly produced during the breakdown of proteins and other nitrogen-containing molecules and transferred through ammonia into urea.

“Molecular Neurology” by Stephen Waxman
from Molecular Neurology
by Stephen Waxman
Elsevier Science, 2010

For infants and children with urea cycle disorders, catabolism from excess protein, weight loss, illness, or infection are dangers.

“Nutrition in the Prevention and Treatment of Disease” by Carol J. Boushey, Ann M. Coulston, Cheryl L. Rock, Elaine Monsen
from Nutrition in the Prevention and Treatment of Disease
by Carol J. Boushey, Ann M. Coulston, et. al.
Elsevier Science, 2001

This is important because patients being treated with glucocorticoids, or those with severe catabolic conditions will break down body protein stores to increase nitrogen excretion more than that derived from dietary protein.

“Seldin and Giebisch's The Kidney: Physiology and Pathophysiology” by Robert J. Alpern, Michael J. Caplan, Orson W. Moe
from Seldin and Giebisch’s The Kidney: Physiology and Pathophysiology
by Robert J. Alpern, Michael J. Caplan, Orson W. Moe
Elsevier Science, 2012

Advancing dietary protein intake and normal protein catabolism during the first few days of life lead to severe hyperammonemia in infants with urea cycle and allied disorders (subgroup C).

“Pediatric Critical Care E-Book” by Bradley P. Fuhrman, Jerry J. Zimmerman
from Pediatric Critical Care E-Book
by Bradley P. Fuhrman, Jerry J. Zimmerman
Elsevier Health Sciences, 2011

The diet for the patient with AKI is usually high in carbohydrates to prevent protein breakdown and low in protein to provide essential amino acids but to limit increases in azotemia (increased urea in the body).

“Davis's Diseases & Disorders: A Nursing Therapeutics Manual” by Marilyn Sawyer Sommers
from Davis’s Diseases & Disorders: A Nursing Therapeutics Manual
by Marilyn Sawyer Sommers
F. A. Davis Company, 2018

Treatment for urea cycle disorders requires restriction of dietary protein, conjugation of ammonia (in the form of the amino acids glycine or glutamine) to excretable forms, and prevention of catabolism.

“Emery and Rimoin's Essential Medical Genetics” by David L. Rimoin, Reed E. Pyeritz, Bruce Korf
from Emery and Rimoin’s Essential Medical Genetics
by David L. Rimoin, Reed E. Pyeritz, Bruce Korf
Elsevier Science, 2013

Alexia Lewis RD

Registered Dietitian Nutritionist and Certified Heath Coach who believes life is better with science, humor, and beautiful, delicious, healthy food.

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46 comments

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  • I know that in catabolic reaction, NAD/NADH is the enzyme that is involved in Glutamate Dehydrogenesis, whereas NADP/NADPH is more involved in anabolic reactions.

  • A thousands of thanks to you i was stuck for hours in transamination and deamination of amino acids. You didn’t seem to be lecturer at first but that was perfect lecture with impressive presentation. I am going to use your method im my presentation.
    Your new subscriber

  • I was a Fibonacci until i woke up then since i knew the truth when i did otherwise i would suffer because i had the awareness.
    Talk about a Blessing & a Curse.

  • Personally, I think the protein powder company should assist with the funeral. Yet, being sued for millions of dollars and more regulations on the protein shake market is foolish. I wonder if it’s G6PD!

  • This is absolutely fantastic, i’m in dedicated and this is taking my understanding of the interactions of all of these metabolic cycles to the next level. Phenomenal Job

  • Most people have very different biology based on their genetics and other things. I for example will be losing muscle mass on 60 grams of protein.

  • With that particular disorder, wouldnt powder form be the way to go anyways diet wise because it is way more digestible then meat?

  • You have the same strange look in your eyes as the pastor in our church. Some kind of “I mastered the way my intellect directs my self reflecting thinking about my own ideas” look in the eyes.

  • Only poor ppl should have this problems they can’t get good doctors to care a little more ask more questions and send you to take more regular test and go see specialists every few years but if your rich and that happens idk what to say

  • Thanks Ninja. That was very very helpful in understanding the urea cycle. Can you help me by answering this question? I have a periodontal bacteria in my system, that’s well colonized. It’s called Porphyromonas Gingivalis, a periodontal bacteria. It produces an enzyme that breaks off arginine-based peptides from surrounding protein (possibly from peptides and protein bound to serum albumin) and this enzyme converts arginine to citrulline. What is the effect of this on the urea cycle? Would this result in less urea being produced? Would I be able to detect less urea being excreted in the urine? What can I do to turn the reaction in the right direction, short of killing this bacteria?

  • Its a very sad situation but a freak accident if the mother wants to blame anything she should blame herself or her father for unknowingly passing on this genetic disorder

  • Thank u for Your Video
    Im Looking Forward2 Your Reply i Sent u Reply-Message +
    For i need Factual+Evidence Such,
    I Really Like Protien bats and protien Shakes.

  • Its also very important to get all the rich micronutrients from vegetables to prevent health detriments alongside proteins and carbs. For natural non competing bbs, carbs/protein/veges should be a staple part of each diet.

  • I was fascinated by the “anabolic vs. catabolic” slide at 3:45. Dumb question: Does this mean that if you are metabolizing protein anabolicly you can discount the calories in the protein for purposes of energy balance?

  • This is one video that I actually agree with you because I have been doing my research and you are correct about most of what you’re saying. Animal protein has been giving me some high acidic feelings.

  • Thanks for the video, I am a new subscriber. Unfortunately, the message about the “best” diets are very confusing to the average person. For example: almost all health / fitness thought leaders will agree on a few things: fruits and vegetables are good for us, exercise is good for us. Of course, smoking, drugs, alcohol, improper amounts of sleep are bad. After that, things get confusing. Is exercise marginally good unless it is HIIT? Is eating animals products at all bad for us? I don’t have all, or even some of the answers that’s why I listen in. Thanks again.

  • What was the “wearing my windscreen indoors”? Sorry, didn’t get that. PS Your looks are better than you’re sayin but still just on the okay side not to be too jealous I guess, lol. x

  • a small correction arginosuccinate should be argininosuccinate (arginine -> arginino)
    but the video is really amazing, thank you so much for giving a mini-lecture of this high quality

  • I’m an ACCP in Scotland and have recently started watching your lectures; initially because your COVID lectures were excellent. I’m now transfixed by the series of lectures you have compiled. I love your teaching style, helped me tenfold to consolidate my background reading and knowledge. You are a super cool guy ��. Thank you from Glasgow!

  • In mammals, glutamate dehydrogenase and the whole deamination reaction happens in the liver mitochondria not in the cytosol as you’ve drawn it. Glutamate dehydrogenase can also use both NAD+ and NADP+ as the reducing agent.

  • What they should be doing is focusing their efforts on awareness for the rare disease she had not the fact she drank protein shakes

  • After watching your lectures, biochemistry seems so easy now,that i can now easily correlate every think. Thank you sir for this great lectures, it helps me a lot. ����

  • The protein shake is the scapegoat here. Michelle demonised the trace elements in the shakes as if that is what killed Meegan when it was actually the excessive protein intake that killed her. And actually, excessive protein intake can kill anyone, not just people with a urea cycle disorder. Bottom line is, the protein shakes would not have killed Meegan if she hadn’t been eating so much meat. It was protein in general that caused her death. So why blame the trace elements in protein shakes? I don’t understand her reasoning.

    All in all this was a completely preventable tragedy that was caused by irresponsible behaviour that was probably triggered by an eating or body dysmorphic disorder and the general neglegence and ignorance of those surrounding the sufferer. It was not caused by unregulated fitness supplements coming from someone who hates the damn things.

  • Is there is any way to put hundred thousand million billion likes��������❤️❤️thank u so much for such an awesome illustration, you are my role model in teaching u made me think of teaching after being graduated from med school

  • She actually had a disorder unknown to her unfortunately in that her body wasn’t able to break down proteins. Not that she was taking too much.

  • Your videos are realy so helpful thank you so mush, but you forget to say that in the step of arginosuccinate synthase there is consumption of 1 ATP to form ADP. Any way your videos are super helpful����

  • Wanted to point out two major details in the beginning of the video that are wrong.

    First of all, Glutamate isn’t directly transported from the muscle to the blood. In the muscle, Ammonia is first added to Glutamate in the presence of the enzyme Glutamine synthetase to form Glutamine. This Glutamine is then transported to the Liver via the blood and is acted upon by the enzyme Glutaminase and is converted back to Glutamate and Ammonia.

    The second thing is that, while Glutamate undergoes oxidative deamination to alpha-ketoglutatarate in the liver, NAD+ is used as the co-enzyme and not NADP+. In fact, NADPH (and not NADP+) is used as a coenzyme during reductive amination of alpha ketoglutarate to Glutamine.

    And other tiny details,
    -> The Glutamate dehydrogenase reaction occurs in the liver mitochondria and not in the cytoplasm as you have drawn it.
    -> The Carbamoyl phosphate synthetase-1 reaction requires 2 ATP and only one bicarbonate ion, not 2 Bicarbonate and one ATP.
    -> The Carbamoyl phosphate synthetase-1 reaction also uses Ammonia (NH3) and not Ammonium (NH4+) ion. Ammonium ions are excreted by the kidneys whereas Ammonia can be actually transported to the liver cells.
    -> The name of the substance formed after incorporation of the nitrogen atom of Aspartate to citrulline is called Argininosuccinate and NOT arginosuccinate.

    Other than these details, Keep up the good work though ��

  • It’s not protein that killed her and rich it was the fukn steroids. Also fat is no good either from animals, clogs the arteries, thickens the blood. High carbs, sugar is the only cure, out body absolute loves it.

  • bro I like you but you have the build of a 15 year old girl. you got stronger from better muscle neuron recruitment not because hypertrophy. take nitrogen to balance the ammonia.

  • You know i have to say, your video production and effects like speaking to your reflection in the mirror stuff is amazing.. Stuff all this talk about food, you need to do a teaching series on video making stuff…

  • Great explanation. Reading biochemistry is not as effective as watching someone explain it to you, but some explanations are not clear anyways. This wasn’t the case with this video. How are you able to memorize all of this? I know a lot of your memory has to do with you having a genuine grasp of the concept but, please give us some tips on how you’re able to conceptualize information. Thanks!

  • Very interesting piece….however I will not drink a protein shake made with orange soda as Rip recommends! My favorite breakfast is a protein pancake made with two eggs mixed with 1/3 cup instant oatmeal.

  • First tym visit to this channel.
    Didnt skip, didnt get bored, but felt sad when the lecture got overi never had like this experience ��
    But ur video and way of teaching did it…��
    May God bless u and let him give u more and more ideas and many more wonderful skills for ur talent in teaching…
    Really a big fan of the way u teach✌….
    A big salute to u☺☺☺☺☺☺☺

  • In reality if you really want to know what to eat it’s very simple but most people are to stupid to really think about it, evolution dictates your diet, over thousands of years evolution has dictated what your ancestors ate and if you work out your ancestry you will know what diet suits you, listening to your body will let you know what DOSNT agree with you and phasing that food from your diet will help, in a nutshell a and b blood types require a plant based diet while o blood types require animal protein, if you eat the correct diet hydrate well even your sweat has no horrible smell, listen to your body and it will tell you what works for some won’t work for all.

  • I’m very glad that you encourage more protein. Before I started lifting I found myself eating less and less protein. Now I eat quite a bit. I’m even starting to like whey, sort of.

  • You should always eat the egg yolk, it has half the protein and all the fat is in the exact proportions it should be so it’s a great place to get your fat.

  • Another wonderful video, Dr. Sully. Now I’ll go and eat my “can of salmon” breakfast, as usual, but this time with a lot more peace of mind.

  • Hi, I love you <3

    ps now I understand why my boyfriend told me I’m hot when I was trying to explain to him the Krebs cycle to revise, lol

  • Great Video… and if it was really due to Protein Over Use, we’d see gym-rats coming out of the gym and supplement stores dying right there.

  • I actually lived on low carb,high fat and high protein (Incl. meat and fish) for over 2 years. Lost quite a bit of weight, but then started feeling sick over 8 month without any signs in the blood. It got worse every week…felt like running on 30 percent… in the end i started believing i had Cancer Then doctors asked me about nutrition and asked me to stop this diet. After just one week i felt better…now stopped animal consumption.

  • Yo I got talk to you about your Patreon…it’s only a dollar and you can’t put it for more? bro your fans dont even know it’s only a $1… are you aware there’s no where to easily make it more than a dollar I been putting it of ffigured I’d do $20 a month since im low on funds right now… BUT I LOOKED It’s $1 you should have more choices 1,5, 15, 25, 50, and 100 month sponsor… you onlu got like 100 people you need more. Do a whole entire video on Patreon! sooner the better $$$$$

  • She did not die from the protein, she died from a kidney cycle disorder that is extremely rare that was exacerbated by the protein.

    Whey protein is not very bio-avaliable in the body, most if not all of it is cycled back through your kidneys into your urine. So if your kidneys are not healthy this puts a huge load on them filtering so much unused crap, this includes plant based proteins. The only protein that our body seems to be able to use properly is animal protein. Sorry vegans but thats just how it is. Whey and plant proteins are fine for short term uses or to supplement animal protein but use too much and your kidneys will die and then you will die.

  • human evolution slowed down when we started farming. we are actually designed to eat protein and fat rich diet. You are looking greta though. discipline is treating you well…did you move?

  • The Doctors said that she had a rare disorder, what if she had no disorder and the doctors lied about it so they can cover up the fact that supplements can kill? Supplement/Protein companies or whoever is responsible for this.
    If people all around the world found out that protein can actually kill due to her death, every supplement company would ruin apart.
    I’m not saying that protein could kill, but that’s just a theory

  • Marc…. thank you very much… I was just having a argument with my wife regarding the protein headlines…. I knew how they were attempting to throw the protein industry to the wolves… I played his video for her to settle the discussion and made her realize that I’m not bias and you worded the discussion perfectly…��

  • That’s really sad. Not to minimize it, but Australia seems to be purposely salacious and misinformed about fitness/nutrition, especially nutrition. The family I’m sure is upset and wants to blame things, but just in general it seems they’re very misinformed. Even when it comes to other things like vaccines or etc, seems to be a lot of misinformation spread there.