# My Son Smells of Maple Syrup?!?!



## Dadoftwoboys (Nov 20, 2008)

First of all.....thank you ladies for letting a dad post on your forum!

I have conducted a bunch of web-searching and found a thread here that was over a year and a half old that addressed toddler-aged children that had maple syrup smell, but no other symptoms of Maple Syrup Disease.

My 16 month old son smells strongly of maple syrup in the mornings.

However, his urine does not have that smell.

It's like maple body odor. His pajamas really smell of it and hold the smell for days after one wearing.

His blood and urine test showed some sort of abnormality and the peditrician is sending us to a genetic doctor??!

Anyone else experience something like this??

Thank you very much!


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## yogachick79 (Apr 4, 2006)

No advice, but didn't want your thread to go unanswered either! I really hope that you guys figure out what is going on, and please do post whatever the doctor finds.








hugs for your little guy!


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## sbgrace (Sep 22, 2004)

My son has a metabolic disorder. Most of these are treatable. The one that causes a maple syrup smell is very treatable. It is good you're seeing genetics--are you seeing them very soon?

If you can find out what abnormalities your pediatrician found in the blood and the urine I might be able to help you more. Ask for a copy of the labs. They have to give them to you. I learned (eventually) that it is always good to have a copy of the labs myself. I assume they found something on his urine organic acids that indicated a metabolic condition.

Does your son seem healthy? Is he meeting his milestones? Growing well? Eating well? I think that he is 14 months before you see this is a very good sign. It would indicate, I believe without seeing the labs and realizing this is different than what my son has, that your son must have some metabolic ability in the area that is affected that has protected him. Or that he has a milder condition.

I'm not sure what they might be thinking since it is not his urine that smelled. But if you can get the labs I might be able to tell you more. By my first thoughts were MSUD (a varient form with onset later) or diabetes. MSUD would usually impact urine but it could come through sweat too. If you smell a diaper after the urine has dried do you smell the maple syrup smell? Diabetes would add a sweet smell to the breath and even the skin (does his skin taste sweet?). I'm assuming more in the metabolics area because your doctor should have been able to handle diabetes or would have at least started treatment I would think and I suspect they did a urine organic acids screen and found something. It is possible that a child in metabolic decompensation of another sort could smell sweet as they would have acidosis but your son would have been sick and your ped. would have hospitalized him.

I hope you're not waiting for genetics; your son needs to be seen immediately. The danger with this stuff is not treating it.
In the meantime don't let him fast (go overnight without eating or too long during the day) and protect him from illness. Both those can trigger decompensation if he should have a metabolic disorder. Has he been sick recently? Started sleeping through the night? Has his diet changed recently--did he for example start eating more solids?
Did the doctor tell you to watch for anything or to change anything?


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## Dadoftwoboys (Nov 20, 2008)

Quote:


Originally Posted by *sbgrace* 
My son has a metabolic disorder. Most of these are treatable. The one that causes a maple syrup smell is very treatable. It is good you're seeing genetics--are you seeing them very soon?

If you can find out what abnormalities your pediatrician found in the blood and the urine I might be able to help you more. Ask for a copy of the labs. They have to give them to you. I learned (eventually) that it is always good to have a copy of the labs myself. I assume they found something on his urine organic acids that indicated a metabolic condition.

Does your son seem healthy? Is he meeting his milestones? Growing well? Eating well? I think that he is 14 months before you see this is a very good sign. It would indicate, I believe without seeing the labs and realizing this is different than what my son has, that your son must have some metabolic ability in the area that is affected that has protected him. Or that he has a milder condition.

I'm not sure what they might be thinking since it is not his urine that smelled. But if you can get the labs I might be able to tell you more. By my first thoughts were MSUD (a varient form with onset later) or diabetes. MSUD would usually impact urine but it could come through sweat too. If you smell a diaper after the urine has dried do you smell the maple syrup smell? Diabetes would add a sweet smell to the breath and even the skin (does his skin taste sweet?). I'm assuming more in the metabolics area because your doctor should have been able to handle diabetes or would have at least started treatment I would think and I suspect they did a urine organic acids screen and found something. It is possible that a child in metabolic decompensation of another sort could smell sweet as they would have acidosis but your son would have been sick and your ped. would have hospitalized him.

I hope you're not waiting for genetics; your son needs to be seen immediately. The danger with this stuff is not treating it.
In the meantime don't let him fast (go overnight without eating or too long during the day) and protect him from illness. Both those can trigger decompensation if he should have a metabolic disorder. Has he been sick recently? Started sleeping through the night? Has his diet changed recently--did he for example start eating more solids?
Did the doctor tell you to watch for anything or to change anything?

Thank you for your post!

Yes. We're seeing the genetic doc in a few days. Our son is growing very well. Eating very well. Seems happy and very active. Strong and very social. Good balance. No changes in diet. Sleeps almost 10 hours a night and has for months.

I'll let a diaper dry and check that out..

His breath does not have the "juicy fruit" scent that you hear of from kids with diabetes.

I will say that his diet consists of about 80% fresh fruit. Seems like I am hearing some feedback from multiple places that people that have experienced this symptom with their toddlers also had them on a bigtime fruit diet. However, I'm not sure about the value of that assumption since I see all toddlers around mine being fed just about the same thing..?


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## sbgrace (Sep 22, 2004)

I don't think its the fruit. The fact that your doctor ran (I assume metabolic) labs that came back abnormal means something is likely up.

But he's doing well and most of the metabolic conditions are very treatable. I'm glad you're seeing someone fast. It will be ok.









If you call the pediatrician and get the things that came back abnormal I'll try to give you some information about what they might be thinking and/or if it seems like something to be concerned about.

And if after your appointment you have questions let me know. There are several of us on here with kids with metabolic conditions.


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## Dadoftwoboys (Nov 20, 2008)

Quote:


Originally Posted by *sbgrace* 
I don't think its the fruit. The fact that your doctor ran (I assume metabolic) labs that came back abnormal means something is likely up.

But he's doing well and most of the metabolic conditions are very treatable. I'm glad you're seeing someone fast. It will be ok.









If you call the pediatrician and get the things that came back abnormal I'll try to give you some information about what they might be thinking and/or if it seems like something to be concerned about.

And if after your appointment you have questions let me know. There are several of us on here with kids with metabolic conditions.

Thank you!

Our appt is Tuesday and I'll post what they say...


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## sbgrace (Sep 22, 2004)

I just wanted you to know I'll be watching tomorrow and hope your appointment clears up any questions you have! Some of those genetics/metabolics people are personable but most aren't!


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## Dadoftwoboys (Nov 20, 2008)

Blood and urine work came back negative for maple syrup urine disease.

Additional blood work sent to Emory for further testing. Doc said they would grow more cells from the blood and test it for other disorders...

Sound familiar to anyone?


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## sbgrace (Sep 22, 2004)

Sending out for further testing sounds familiar (though ours was skin cells).

Do you have copies of the labs? If you're comfortable letting me know what things came up abnormal I might be able to give you more help. Do you know what they sent it to Emory to test for? Did they mention doing a skin biopsy?


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## Authentic_Mother (Feb 25, 2007)

Hey there! My daughter's half brothers both smelled of Maple Syrup as well and were both checked for Maple Syrup Urine Disease (google it). One of the signs is that smell, and then seizures which they both had. But as it turns out - they do not have it.
Eitherway - goot thing you are getting it checked out and I hope you have some answers soon!


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## ellasmama2007 (Jun 9, 2008)

my dd has smelled like maple syrup a couple times in the past when i was eating too much honey or maple syrup and nursing her. now she gets too irritable for me to eat sugars so i dont know if it would still happen, but she has not smelled like maple syrup since i stopped with the sugars. i know that's not much of an answer, but it is my understanding that there really isnt a disease categorized by maple syrup body smell. the urine is a different subject, but my old allopathic ped said that maple syrup body smell is inocuous.
try cutting down the fruits and adding more vegs/pros/fats just for giggles and see what happens. a nice balanced diet could possibly help whether or not it is anything metabolic. imo, if his body is emitting a strong sweet smell, maybe he is getting too many sweets and his body is trying to tell you something. hth!


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## ambermorr (Jan 20, 2009)

My son is almost 15 months old and has been perfectly healthy since birth. He has had croup once for 2 days and that is it.

I noticed a few times over the last 2 months that he smelled of maple syrup when he woke up in the morning or after a nap. I would blame it one having syrup on his pajamas or just a combo smell of other things. But then my husband ask me last night after Jack woke up from his nap did Jack smell of maple syrup? I said I just put lotion on him and we thought nothing of it.

Then today when he awoke from his nap I couldn't deny the smell. They were brand new pajamas so it wasn't them. I called the doctor and they didn't act like I was crazy so I have an appt tommorrow.

My son also eats a lot of fruit, I always thought that was better than ice cream!

Any insight you could give would help. Thanks for posting. I don't feel like I am losing my mind anymore.


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## sbgrace (Sep 22, 2004)

ambermorr,
If you need support or have questions please come back and post or send me a message. Your doctor should run some metabolic labs (such as urine organic acids). Did your son have a newborn screening? If so, what state was the screening? Most states I believe do screen for MSUD. Hopefully you also live in a state that does expanded newborn screening. That your son is healthy at 15 months is quite reassuring even if they do find something.


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## ambermorr (Jan 20, 2009)

He blood and urine are being sent off today and we have an appt in 2 weeks to go over the results. We have asked all corners of our families to see if anyone has had any metabolic issues and none can be found.

Thank you for the support. I will post more when we get the results


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## ambermorr (Jan 20, 2009)

The test came back normal. They have nooo explanation as to why he smells of maple syrup but if I feel that he is still smelling like it I can have him retested in a few months.

I guess I feel relieved but on the other hand, I still don't know why he smells like that.


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## Siera (Nov 11, 2008)

Is he still receiving breastmilk? Does your wife take Fenugreek? This can cause her milk to taste like maple syrup and can pass onto the child as well. However, I don't know if it would be that strong of smell, but just trying to help.


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## daddad (Apr 5, 2014)

My kid smells like maple syrup too in the mornings...our kids sound identical. My kid is 18 mos. do you use a 12 hour diaper at night?


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## daddad (Apr 5, 2014)

We are going to ask our pediatric doc about it on Monday. Keep you posted


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