Grayson McGill's story - Living with Maple Syrup Urine Disease (MSUD)

Toddler Grayson McGill has a rare metabolic disorder where regular food can make him ill. Chad Farquharson and his husband Wayne McGill must take extreme care in monitoring their son's protein consumption. The couple must measure the amino acids consumed by Grayson down to the exact milligrams; if they are off, the results could easily be catastrophic.
Grayson has Maple Syrup Urine Disease (MSUD), a rare and potentially deadly metabolic disorder that causes amino acids from proteins to accumulate in the body. Grayson is unable to process three amino acids -- leucine, isoleucine and valine. The disease's saccharine name, which comes from the sweet smell of the patient's urine, belies the seriousness of the condition. The toxicity it causes can lead to brain swelling, mental retardation, coma and death. Grayson's life will hinge forever on his ability to delicately balance the amount of amino acids he consumes each day.
Grayson's diagnosis was made just after he'd undergone open-heart surgery to repair life-threatening defects. The operation was successful, but a few hours later, his new parents were told that he had the disease. A neurologist had noticed subtle swelling in his brain and became concerned about a metabolic condition. Because BC Children's Hospital began screening for MSUD as part of an extended screening program that now includes 22 conditions, the neurologist's question was readily answered.
The quick result of the testing meant doctors could instantly respond to Grayson's dietary needs. Grayson was also born with a heart defect, and required open heart surgery as an infant. Through the testing prior to his surgery, his MSUD condition was discovered.

[@333pinkitty]

He's precious and so brave. You guys are amazing parents being so attentive to his diet and care. Bless him

[@AMCKEJB3]

Such a beautiful family that sweet little guy couldn't have better parents...

[@anastaziajade4604]

It would be nice if he had a mother… 🙄

[@winifredherman4214]

@@anastaziajade4604give it a rest! The world has advanced…

[@bartpelle3460]

@@anastaziajade4604 typical comment from a dual-mother child.

[@anastaziajade4604]

@@bartpelle3460there is nothing like a mothers love.

[@senpaisun7234]

cutest baby i’ve ever seen, with wonderful, WONDERFUL dads.

[@gwenmerchant5091]

What a fabulous video. Grayson is utterly amazing and his Dad's spend so much time with his feeding ritual and also teaching him to read. 'Good Going, Dads' and Grayson!

[@TrenchKoatKilla]

I think you meant his Dads

[@Norexed]

I literally thought this kid pees maple syrup

[@Centipedesnack]

Me too

[@KrosanBeast315]

This is the problem with its common name, "Maple Syrup Urine Disease." It is easier to say that its REAL name: Branch Chain alpha-Ketoacid Dehydrogenase Deficiency or B.C.K.D.D. for short. I have this disease and it took me 3 months to learn how to say correctly. The common name caused me nothing but torment and torture from the time I was 8 when I was diagnosed, until I was 24. Even today, people still laugh at me when they see the name of the disease. They have no idea how it killed me, I was brought back to life, and how for 16 years after, M.S.U.D. turned much of my life into a 24/7/365 nightmare.

[@chantrvll]

I was thinking it was gonna mean that his urine was really thick..

[@duckqueso3689]

Same

[@Shadow_7685]

same 😂

[@cookiemonster93fly]

you guys are awesome parents,he’s lucky to have you to take care of him

[@frankm.2850]

Imagine being a parent trying to care for Grayson or a child like him without modern medicine. The body goes bad in some weird ways sometimes, but its pretty amazing that we have the knowledge to care for people with diseases like this and people trained to diagnose and treat them. I have so much respect for doctors, and especially pediatrics specialists. Its depressing how little respect we have for caregivers that work with kids in this culture.

[@jpgr4ever553]

Great Parents

[@cometzzmp4]

He's got gay dads who treat him greatly! He's got great dads!

[@jacquelinescourageousworld3848]

His dad isn’t gay. Greyson has a mother. Otherwise Greyson wouldn’t be here. The sperm and the egg 🥚 go together.

[@emilytoma1733]

My sister has MSUD. Recently she has a operation that allowed her to eat what ever she likes. She says she feels normal now. Just always remember that there’s always hope!

[@KrosanBeast315]

Depends on the Variant and genetic mutation.

[@faizanmahmood2658]

Wait it depends when they were born, if they born when screening was available for MSUD then that can prevent it however when i was born in 2002 they didn't have then.

[@KrosanBeast315]

@@faizanmahmood2658 No parents can prevent M.S.U.D. from transferring unless they have their genetics fully tested before having a child. If both parents are discovered to be Carriers, they might not want to have a child at all because the potential level of severity of M.S.U.D. in their child. I was diagnosed at 8 years old. It was only in 2019 and long after my bio dad died, that I learned my Mother is a Carrier and my Father was too. Only certain variants of M.S.U.D. may be corrected through an operation. Many cannot.

[@faizanmahmood2658]

My bad i know sorry i had false info. It turns out you can't prevent but you can supply them with medicine earlier so kids don't have to go through what me and my family went through.

[@KrosanBeast315]

@@faizanmahmood2658 It still depends on the Variant. Sometimes medicine and supplementation can help to an extent, and sometimes it doesn't or is not necessary. Some are more restricted in protein than others. My case blew the roof off of all known M.S.U.D. cases. I am able to have 30g - 40g of normal protein. The exact reason for my absurdly high tolerance is still not fully understood.

[@miranda5638]

He’s a lucky boy with loving parents

[@joshchristianson2995]

As a fellow parent with a child with msud good luck to you guys. It’s hard n scary but as long as it’s managed n caught early everything should be alright.

[@alien_queenn412]

I have a question, my son started smelling like maple syrup all the time ab a couple weeks ago. At first I though he had just eaten pancakes with his daddy, and now he always smells like that. He doesn't have any of the other symptoms though, so is this still a possibility? And can he really start showing signs at 10 1/2 months old? Sorry for all the questions, but Google kind of scared me with this.

[@KrosanBeast315]

​@@alien_queenn412 I have M.S.U.D and am an unintentional pioneer of it. I will answer some questions for you. Only a comprehensive Genetics Test can conclusively prove if he has M.S.U.D. or not. If he does have it, contact Mount Sinai Hospital in NYC and they can tell you the closest hospital to you in case of an emergency regarding M.S.U.D. related Ketosis and help you reach the On Call Dr. for that hospital. If his urine smells like Maple Syrup so often, you should perform a Ketone Test with a Ketostick. These are used to measure levels of Ketones and for M.S.U,D., its presence is a warning sign and if not dealt with quickly, things can deteriorate and body systems can start to shut down at a frightening speed. Bayer is the only brand I trust and have used for 28 years so far. If his Ketones are Trace, he is stepping into the Danger Zone. If his Ketones are Small, he is in the first stage of the Danger Zone. However, only a comprehensive Genetics Test can conclusively prove if he has M.S.U.D. or not. If he does have it, contact Mount Sinai Hospital in NYC and they can tell you the closest hospital to you in case of an emergency regarding M.S.U.D. related Ketosis and help you reach the On Call Dr. for that hospital. Depending on the severity of M.S.U.D, the window for emergency intervention can be only a few hours or less.

[@DadHacksCentral]

@@alien_queenn412 please give me an update. my son just started smelling like it

[@ziggylaurie2268]

Genetic counselling is essential too. If one of the father’s is a carrier trying to find a surrogate or egg donor that isn’t. It’s so rare, I feel bad that this happened

[@believeinyourself7511]

You are both great Fathers. Thank God for you in adopting this little boy.

[@ziggylaurie2268]

He may not be adopted

[@dejesanaysmith6993]

My daughter also has MSUD so I can totally relate.

[@KrosanBeast315]

One thing you need to warn her about. In time, if she considers wanting to have a child one day, she needs to keep her carbohydrate count very high when she goes into labor. Extremely High Ketosis a real danger during delivery. She would need to be monitored around the clock during the whole process.

[@KrosanBeast315]

0:26 M.S.U.D involves the lack of the production of THREE Amino Acids: Leucine, Iso-Leucine, and Valine... not one. It is not due to missing an Enzyeme. Enzyemes are made of Amino Acids, which the lack of 3 Branch-Chain Amino Acids: Leucine, Iso-Leucine, and Valine, is the cause of B.C.K.D.D., or M.S.U.D. for short. Another mistake he made is that M.S.U.D is only based on the digestion of protein. Protein is divided into two catagories: Branch-Chain and Non Branch-Chain. A person with M.S.U.D. can likely have all of the Non Branch-Chain Amino Acid protein they want. It is the digestion of Branch-Chain type of protein that is the problem for someone with M.S.U.D.

[@tanyaceballos7753]

Im doing a research paper on it but it is so hard to find information..even people who are able to regulate it

[@KrosanBeast315]

@@tanyaceballos7753 what do you want to know about it? you can email me at [e-mail] just include subject matter in the Re:

[@KrosanBeast315]

I have been regulating my diet since I was diagnosed at 8 years old. However, I am an Intermittent Variant and my protein tolerance is so high that many of the rules for MSUD had to be rewritten because of my case. It is weird to be a Medical Celebrity, but at least what was pioneered on me saved many lives and children got tested and diagnosed faster because of me.@@tanyaceballos7753

[@KrosanBeast315]

​@@tanyaceballos7753I have had MSUD for 41 years, first diagnosed at 8 years old

[@faizanmahmood2658]

When ill it so difficult for the carer and the person with MSUD. Normal people, when having flu recover in 1-2 days however with MSUD people it takes 1-3 weeks. Also the amount separate food and stuff you have to make for them from the family is a tough job. So i just wanna thank my parents, it's been a long 17 years for them too

[@jacquelinescourageousworld3848]

I was born in 1994 and I was actually born with Maple 🍁 Syrup Urine Disease and maybe eight or nine other conditions. Because I was born in 1994 the doctors didn’t have a lot of information on anything.

[@sitalimbu3679]

@@jacquelinescourageousworld3848 I hope you are doing good.

[@kachoo2135]

I remember seeing a girl from North Carolina named Rachel Webb who also has MSUD along with her younger brother. Seth is healthy; Rachel came close to death on three different occasions. She is a lovely girl in spite of her MSUD; she and her younger brother, Seth, have to watch what they eat

[@peatrude4872]

Cheryl Keil I saw her on Mystery Diagnosis, but for some reason that episode is nowhere to be found now.

[@faizanmahmood2658]

MSUD can also be very dangerous if doctors don't know about it as I almost died when i was young due to my doctors not knowing what was wrong with me but thanks to god im alive and healthy.

[@KrosanBeast315]

I know the feeling. M.S.U.D. killed me when I was 8, and the aftermath after my heart was restarted was a nightmare.

[@saqibmahmood3175]

Faizan... I have 6 month daughter with msud. Can we discuss please in detail, how you are managing?

[@jacquelinescourageousworld3848]

For everyone who is wandering MSUD was supposed to be diagnosed after my birth. Because I was born in 1994 there was no literature on the disease. I’m scared 😱 out of my mind right now because there are less than 3 thousand cases reported. Let’s hope there is going to be major advancements made in medical 🏥 science that can improve a severely disabled persons life. This is so embarrassing 🙈 but I was in church ⛪️ when the head pastor came in and said isn’t it obvious it’s Maple 🍁 Syrup Urine Disease meaning she’s missing an enzyme that deals with access protein.

[@KrosanBeast315]

​​​@@jacquelinescourageousworld3848it's not about a missing enyzeme. There is an error in the genetic code on two Codons. Those Codons are pathogenic. MSUD is about the body having an error concerning the lack of the production of Leucine, Iso-Leucine and Valine. As I am probably the most extraordinary case of MSUD documented in the United States, it's important for me to let others know what MSUD is and isn't.

[@KrosanBeast315]

For one thing , in the old days, it wasn't always diagnosed at birth. Secondly, the pastor was WRONG about her missing an enzyme. It is NOT that simple. There are 2 Pathogenic errors in the genetic code, and those errors either limit or inhibit the production of Leucine, Iso-Leucine and Valine. My case led to many advancements and new protocols for treating MSUD and diagnosing it sooner. @@jacquelinescourageousworld3848

[@ashnkksmom2264]

With him having to measure out how much protein he needs to needs each day not to go over or under what do you do if he is more active or ill?

[@KrosanBeast315]

Depends on the Variant. If he got more ill, it could involve I.V. treatment, to also using Nasal-Gastric Tubes, or more.

[@brennadunnigan3868]

Praying!

[@janececelia7448]

What happens when this Grayson gets older and starts exerting his wants and desires that his strict diet won't allow. All the best for the future to this amazing and loving family.

[@KrosanBeast315]

It depends on the Variant he has, and his protein restrictions. As someone with MSUD, I know how difficult it can be to regulate your diet. However, I can eat 30g of regular protein because of my Variant... the highest amount ever documented in a MSUD patient. The disease can cause other problems in adulthood that children aren't warned about young enough.

[@imjustabakagirl]

I had three open heart surgeries as a baby, but maple syrup diesease is worse I think! :( I hope he stays ok.

[@KrosanBeast315]

it depends on the variant

[@LifeOfAMom38]

Yeah u really mean it

[@Gods1cowgurl]

My nephew has MSUD. You can follow his journey in the Facebook page I created. Is called Jaxons Journey

[@ibrahimurwa2896]

My daughter has this disease I cannot affort milk please help me

[@shwebbid8778]

Amjad Amjad but you can afford a phone and WiFi?

[@KrosanBeast315]

@@shwebbid8778 Stop talking negatively, and be more thoughtful.

[@KrosanBeast315]

@@ibrahimurwa2896 There are other alternatives to Milk. If she needs the Calcium from Milk, she can just take Calcium,. Mammalian milk is too high in protein for too low Carbohydrates. You need to find out what is her Maximum Grams of Protein Daily Limit is as well. Only a Dietician who specializes in P.K.U or M.S.U.D can further assist you.

[@VideoLogDotCom]

@@shwebbid8778 yeah dude. It can get pretty pricey if there is no help. My gf has it

[@m3wt518]

Is there a chance the initial test for a newborn is false positive? The reason I'm asking is because my 10 days old baby is positive on MSUD on her newborn screening test. Me and my wife are sure no one in our relatives have this disease and we don't see any symptoms of the disease on our baby except for the occasional vomiting which I believe is just normal for newborn babies. - no urine that smells like a maple syrup or anything. I really do hope that the re-test is going to be negative - which should be available this week- because I fear for my wife's depression.

[@caseyholmes6330]

How did the second test turn out? My daughter was born with msud as well and showed no signs but as time went on I did notice she was more fussy then usual she started eating less and at one point vomiting allot. Her urine never smelled like maple syrup. She's 4 weeks now and I smelled it for the first time yesterday. She's been in the hospital for five days now.

[@KrosanBeast315]

The smell is often associated very high Ketosis. A person with MSUD in trace Ketosis is the first stage of being sick. Urine may have no or barely any scent to it. Heavy Ketosis will likely have the urine emit its signature scent.

[@alien_queenn412]

My son is 10 1/2 months and a couple weeks ago started smelling like maple syrup all the time. I assumed it was bc his daddy gave him pancakes for breakfast, but now it's almost all the time. That's the only symptom he has though.

[@KrosanBeast315]

@@alien_queenn412 you should get him tested for M.S.U.D. to be safe

[@complexsonicz9239]

What a nice family

[@davidkurniansyahsinulingga1143]

Susu nya beli dimana ya pak? Di Indonesia tidak ada yang jual. Mohon bantuannya

[@tatsugamisaburo8394]

Is it Canadian maple syrup or that yucky storebought one that tastes like old caramel?

[@captnsalty9274]

I don't know man, 2 gay dads with a kid with maple syrup urine disease sounds like one hell of a gay porno.

[@aidanw7268]

Captn Salty screw you my uncles are cool as hell

[@captnsalty9274]

oh nigga you gay.

[@captnsalty9274]

I know what maple syrup urine disease is, I know what kind of mutation it is, I know the other uncommon types of MSUD, don't deny or argue about my knowledge on this subject. People die, people are lucky and people are unlucky. Shit happens for unexcused reasons and since I'm too busy to walk in some hopeless kids shoes, I bet any of you suckers will to make a point. Thanks for the essay.

[@paytonl3485]

And this is where KZbin arguments start. "But of course you wouldn't know that" or "You're just a dumb kid from america". If you're going to debate over stupid, mildly humorous comments, you may as well not start making assumptions.

[@MIC_Enjoyer]

We gotta get this kid to request Geo Guessr on the NLSS

[@anasalsaif5996]

Where is his mother ? to take care for him,

[@heidi8648]

He has gay parents

[@anasalsaif5996]

@@heidi8648they are adductors

[@heidi8648]

@@anasalsaif5996 No they’re just gay and there’s nothing wrong with them, they’re amazing parents

[@anasalsaif5996]

@@heidi8648 the child need his true mother

[@amishhackerlul3731]

My daughter's getting tested for this today

[@KrosanBeast315]

Does she have MSUD after the test?

[@LifeOfAMom38]

I pray that result must b negative. I don't want any child/parent getting affected by this

[@bananacake059]

GAY PARENTS YASSSSSSSS!!!!!! (Gay parents are the absolute BEST)

[@cookiemonster93fly]

wow poor baby

[@ibrahimurwa2896]

I want MSUD infant milk I cannot affort my little princess has same diseses please help me.

[@KrosanBeast315]

You need to find an On-Call Doctor and Dietician for M.S.U.D. I would suggest calling Mount Sinai Hospital in NYC. They can help you find someone you can go to for dietary needs and emergency treatment

[@user-ue2lx6py9j]

omg I remember when I was around 4 and my pee smelled just like maple syrup but I didn't know it was a disease and I'm 13 now but ive been living my life completeley normal should I be concerned

[@amrayabaptiste2933]

Luke Nakamura well I suggest you get it checked out

[@KrosanBeast315]

only a geneticist and experts in pediatrics and pediatric metabolics would be able to possibly to do the tests to determine it.

[@paytonl3485]

lu ke you would probably have more symptoms at this point

[@KrosanBeast315]

@@captnsalty9274 You need to stop saying things like this, or else I will have action taken against your account.

[@elisedion3185]

he is cute as ever

[@Sauullllllllll]

Well... Does it taste like maple syrup?

[@KrosanBeast315]

Of course not, you disturbed sicko!! That is a really messed up thing to say.

[@faizanmahmood2658]

I have MSUD im 17 years old

[@kristinahutapea992]

faizan Mahmood really? How did you survive? Can I have your phone number/Whatsapp/email please. I have a 2 months old nephew diagnosed MSUD.

[@KrosanBeast315]

@@kristinahutapea992 I have M.S.U.D too. I am 37 at the time of this comment. I was first diagnosed at 8 years old after cardiac and respiratory arrest then flatlining. The following 9 years after my diagnosis was really brutal but thankfully I had support to overcome a lot of odds stacked against me.

[@tanyaceballos7753]

@@KrosanBeast315 Im doing a research paper on it but it is so hard to find information..even people who are able to regulate it

[@KrosanBeast315]

@@tanyaceballos7753 i can eat or drink 30 - 40g of 'normal' Protein. I am an oddity of MSUD, and the only one known in the United States to have such a high Protein tolerance.

[@Rainbowcreamsoda]

@@KrosanBeast315 What were your symptoms before your cardiac event, if you don’t mind me asking? Have you ever heard of anyone else being diagnosed at such a late age? Thanks.

[@arshaarshasharon5711]

എന്റെ മൂന്ന് മക്കളും msud ആയിരുന്നു

[@etiafmorse2892]

is that 2 fucking dudes??? wtf this is some sick shit

[@KrosanBeast315]

You need to grow up. Parenthood takes on many forms. Stop living in the 1700s.

[@sam-im3os]

Is his dad gays

[@mrboobbarker]

not sure about that, but he has 2 of them, lucky kid. i never even had one.

[@lordortega]

Clickbait... I thought he actually peed maple syrup, I’d open my own syrup company aha

[@KrosanBeast315]

It's not clickbait, you uneducated and heartless monster! The urine smells like Maple Syrup due to extremely high levels of Ketones in the Urine, which means by the time the Urine smells like Maple Syrup, the body's systems are shutting down due to too much Amino Acids in the body and without help, the person will go into a Coma. This disease KILLS people, mostly Children. The disease killed me when I was 8, but I was revived and the disease put me into a 21 day Coma AFTER my heart was defibrillated. Think it is clickbait, now?!

[@deadchannel8740]

add some protein

[@KrosanBeast315]

you want to risk poisoning a kid? Each form is different in severity. Some people can have little protein, many can have none. Not even a potato chip worth of protein, that much can be fatal in some variants.

[@thetechnostate316]

Just spent 12 hours writing a report on this disease because I thought it sounded funny ok

[@KrosanBeast315]

Branch Chain alpha-Ketoacid Dehydrogenase Deficiency is the REAL name of it. Does it still sound funny?

[@thetechnostate316]

KrosanBeast315 it’s not funny it was a bad mistake picking it lol

[@KrosanBeast315]

@@thetechnostate316 It is not a mistake picking it. It is actually a very fascinating disease. I have it, but I also don't fit the normal paradigm. In fact, my case changed the paradigm for MSUD in 1990.

[@krisjutras2028]

No comment

[@Happy_HIbiscus]

dude, this kid has 2 gay fathers

[@KrosanBeast315]

He has loving parents who are caring for him. So, what exactly is wrong with that?

[@sirwoodzalot3174]

🙏🏻🙏🏻🙏🏻🙏🏻