Urine - odd odor?

[Maggie]

My 9 month old is being worked up for possibly having HFI. She was switched to an elemental formula (it's not supposed to contain fructose, or other offending compounds, but I question this - I do know that the 'flavored' version contains sucralose).

She now has a very odd, strong smelling urine. It is not concentrated urine (like in dehydration), and is clear, but it does not smell like urine at all. I've never smelled anything like this before.

Any thoughts?

[CJSculpts]

It might be helpful if you can list the ingredients and describe the smell?

[Maggie]

Here are the ingredients of the formula:

55.9% Corn Syrup Solids*, 9.0% High Oleic Safflower Oil*, 7.6% Medium-Chain Triglycerides*, 6.5% Soy Oil*, 2.0% L-Glutamine*. Less than 2% of the Following: L-Asparagine, L-Leucine, DATEM†, L-Lysine Acetate, Calcium Phosphate, L-Valine, Potassium Phosphate, L-Isoleucine, L-Arginine, L-Phenylalanine, L-Tyrosine, Sodium Citrate, L-Threonine, Potassium Citrate, L-Proline, L-Serine, L-Alanine, Glycine, L-Histidine, L-Methionine, Ascorbic Acid, Magnesium Chloride, L-Cystine Dihydrochloride, L-Tryptophan, Calcium Carbonate, Choline Chloride, m-Inositol, Salt (Sodium Chloride), Ferrous Sulfate, Taurine, Ascorbyl Palmitate, Zinc Sulfate, Niacinamide, dl-Alpha-Tocopheryl Acetate, L-Carnitine, Calcium Pantothenate, Thiamine Chloride Hydrochloride, Cupric Sulfate, Vitamin A Palmitate, Manganese Sulfate, Riboflavin, Pyridoxine Hydrochloride, Beta-Carotene, Folic Acid, Biotin, Phylloquinone, Chromium Chloride, Potassium Iodide, Sodium Selenate, Sodium Molybdate, Vitamin D3, and Cyanocobalamin.
* Percentages refer to the weight of the ingredient per total product weight.
† An emulsifier

As far as the urine smell.. my husband described it as smelling like 'sweet poop' That description sounds too pleasant smelling to me - I think it smells gross. Can't really describe it beyond that - it does not smell like maple syrup though - I know there is some disorder that causes that.

[meaniejean]

Maggie,
I SO know what you are talking about with the urine smell. My boys get some really crazy smelling urine - kind of like a sweet, nasty smelling butter smell. My husband thinks it almost has a cat pee component to it. We have also had a lot of pee that smelled like poop. So weird.

I wish I could be of some help, but we are still up in the air as far as a solid diagnosis. But, my youngest had a really hard time with the "safe" formulas. The ONLY thing that ever worked for him was plain goat milk. I don't know why. I can't say he was completely normal on the goat milk, but it was the only thing he didn't vomit.

I hope you get some answers soon. My oldest breastfed almost exclusively until almost 2 years old. He did really well on breastmilk. There was such a complicated domino effect going on with my youngest that I have no idea what was making him so miserable. I'm pretty sure he was doing fine on breastmilk until juices were introduced to try to fix his constipation. But, I don't know ...

Thinking of you,
Sandra

[billiejh]

HI Maggie, I too breastfed my daughter until 12 months, and it is probably the best thing for baby, as far as Hfi is concerned, but if the doctors suspect it could possibly be any other inborn error, then breastmilk could be unsafe, depending on which one it is. If they are leaning towards Hfi, they should let you get nursing again. Which formula is it? My daughter has had to use Neocate Jr. since weaning. It has similar ingredients to what you listed, and the company is certain that it is safe for Hfi and that the corn syrup solids are pure. She is now almost 5, and thriving as far as growth. (we couldn't try milks, as she also has anaphylaxis dairy allergy)

[Maggie]

thank you everyone...

billiejh - you've posted some very helpful information in particular. yes, the doctors are leaning toward HFI, but we are still awaiting the genetic tests. I am nervous to possibly begin breastfeeding again for the reason you mentioned, but they didn't really say anything about suspecting any other metabolic disorders, so unless they haven't told me, they're not suspecting it. Are there other disorders with similar symptoms to HFI?

I may ask my doctor if we could try the neocate. I just want anything but this formula.