Sugar in Formula

[colormist]

My google alerts gave me this article this morning: medicalxpress.com/news/2018-05-sugars-infant-formulas-pose-babies.html

So at the very least there is now awareness that fructose/sucrose in infant formula can possibly kill an HFI baby. This doctor is recommending fructose/sucrose be labeled on formula, which is great for parents who are just starting out and don't quite know the ropes yet but do know that their baby has HFI and fructose = bad.

There's still parents that have an undiagnosed HFI baby, but if they notice their baby is not doing so great and maybe see a warning about HFI on the label, then thy might be able to put two-and-two together more quickly so they can adjust their baby's diet before their next doctor visit. Heaven knows you can't keep feeding a HFIer fructose while you go about waiting for a doctor appointment and waiting for a diagnosis to come through.

[Stefanie (Ziba)]

Thanks, Colormist! Wow, what a great article!! I am making it my mission to also get the word out to NICUs that feeding babies "sweeties" in the NICU can be deadly if they have an underlying fructose intolerance. That is why my son starting showing symptoms so early in life. Getting out of the NICU probably saved him as much as being in it did...

[colormist]

That horrified me when I heard nurses were giving babies sugar.

Any idea how you are going to approach getting the word out to NICUs? I mean, aside from berating them all personally (which would be my personal approach). I'm not sure what the news circuit would be for hospitals. It would be nice to get an article out similar to this one!

[antonia]

Does it seem that HFI is being recognized because it's becoming more common? I'm no geneticist, but if you think about diseases where you can be a carrier without expressing the disease, it seems like over time those carriers are silently passing on the gene, whereby eventually the population of carriers increases and therefore the likelihood of both parents being carriers increases and more are having children with expressed HFI.

Granted I'm pretty bitter about my own situation, so I'm slightly annoyed by the simplicity of this article, but this can only be seen as a good sign that there is more awareness of HFI. Bravo!

However, I can tell you the signs of possible HFI or other metabolic disease, even as infants, are likely to be passed off as common, newborn complications. Palmera was born 4 weeks early and only spending 12 hours in the NICU resulted in jaundice by the time we left the hospital, 36 hours later. I have no idea if she was given "sweeties" but was supplemented with plenty of formula because she was not breastfeeding well. After getting her home it was rocky to say the least. She kept loosing weight, screamed for hours (nothing consoled her) and never slept for very long or very comfortably (constantly scrunching up her face and making noises that sounded like she was uncomfortable or in pain). The nurses threatened to put her on formula if she did not gain weight on my milk. At every follow up appointment they would give her more formula. She was quickly "diagnosed" with colic and reflux because she started throwing up as well. I was told the jaundice was quite common, even for full term babies, and that all these things were probably just due to the fact that she was not quite done developing yet. She would grow out of it. The nurses were also quite adamant that she was fed every two hours, even if she was sleeping I was to wake her and feed her. I've wondered if her blood tests showed she was hypoglycemic and they didn't mention it to me because they just assumed she wasn't getting enough to eat. Eventually we were left alone long enough that I was able to solely feed her breast milk. She started gaining weight and the jaundice resolved with light therapy at home. I often wonder how different our story might be had she continued on formula. So, while it's great to get manufacturers to start labeling for added fructose or fructose containing ingredients, I agree that the education should start with NICU and pediatric medical staff.

[Stefanie (Ziba)]

Colormist- As soon as I saw your post, I sent an email to Dr. Li (the Emory researcher in the article you posted) to explain the NICU/sweeties protocol and to see if she could spread this information in a similar manner :-)
* I am still taking the individual approach as well, sending emails to Nurse Managers at the NICUs nearby.

I agree, Antonia, that doctors and nurses need to be educated. I had a dietitian tell me that a formula with brown rice syrup was fine for Remy even if he had a problem with fructose. She only knew about GSD as it turns out, so she just assumed it would be ok for HFI as well.
I feel your frustration. I often wish the HFI test had come back positive so that we would have an answer (esp since the HFI diet is working!). The carrier issue is interesting. Remy is a carrier for GSD 1b (as am I), and while docs swear that carriers cannot have symptoms, I did have one doc at Pitt say "never say never in Genetics".

[colormist]

Man, both of my parents get hypoglycemia but are not diabetic. They are both carriers (obviously and I have the ancestry results to prove it). My mom is not partial to sweets and has noticed other symptoms when she does eat sweet foods. I would not be surprised if carriers of HFI have occasional symptoms.

Keep us posted about Dr. Li! I'm sure I'll hear something if an article is posted, but it would be great to hear Dr. Li's response!

I wonder if HFI is being recognized more now because of the internet? I mean, we're all out here finally getting some answers because we can self diagnose and then we go on to tell everyone who might try to feed us food/medicine about our condition. Our combined efforts of communication (with the increase population of carriers), support groups, blogs, articles, etc. will eventually have to amount to something!

Now we have medications posting HFI warnings on their drug fact sheets and doctors publicly recommending formula manufacturers warn parents about fructose/sucrose in their baby formulas.

I feel like we're getting some momentum!

[rysmom14]

Ziba,

At Childrens hospital of Pittsburgh, Where we go, they also use the sweeties. Ryan didn't go into the NICU after being born, but we was hospitalized at 2 months old for a hernia surgery and that's when we met sweeties. When I think back to the tubs and tubs we must have given him, it makes me sick. I know that they use it to get the kids to use the paci so they aren't screaming, but it just seems so odd now looking back. that time we were in the hospital for 2 days and there was always an open tub by his bed. then when he went back into the hospital at 4 months for his month long stay with the metabolic distress, he was still getting sweeties.

and colormist as for the Formula, I actually argued with the director of genetics ( who was from Germany, and new in her role as director and new to the hospital) because she told me that there was no sugar in the formula and that food much had caused all of my sons issues early on. mind you he was 4 months old, developmentally behind and only ate formula. She tried to talk to me like I was a child and explained that" fructose comes from fruit" at that point I think my husband had to hold me back because I just went off. I also reported her because she refused to listen to me and I know that there was another child on this board that goes to the same hospital and I couldn't live with myself if they saw her and changed their diet because she told them that the child can eat anything except fruit.

I am going to look into see if we use sweeties at the hospital I work and I will also contact some friends at some of the other hospitals to see whats happening

[raluca]

When we diagnosed our daughter we immediately looked what her formula contained. On the box it says that the only sugar in it is lactose, and nothing else. In order to be sure, we also wrote an email to the distributor of this formula, and they confirmed it. I must say that even if she is 5,5 y.o., we are still giving her this milk, on daily basis. We are also using it for making her cereals in the morning. The logic in my mind was that this formula is enriched with many vitamins and minerals, which she is missing so much. So that is an easier way to substitute a part of them. I hope I am not wrong by doing this... did any of you experienced the situation that on the box everything seems to be safe, but then get a bad reaction to that product?

[rysmom14]

Hi Raluca!

Im not sure what formula you are using, but that's great that you heard back from the company and they confirmed that it is safe. I was using Similac sensitive stomach. The ingrediants are:

Corn Syrup, Milk Protein Isolate, High Oleic Safflower Oil, Sugar, Soy Oil, Coconut Oil, Galactooligosaccharides. Less than 2% of: C. Cohnii Oil, M. Alpina Oil, Beta-Carotene, Lutein, Lycopene, Calcium Phosphate, Potassium Chloride, Potassium Citrate, Sodium Citrate, Magnesium Phosphate, Ascorbic Acid, Choline Chloride, Calcium Carbonate, Magnesium Chloride, Ascorbyl Palmitate, Ferrous Sulfate, Choline Bitartrate, Taurine, m-Inositol, Zinc Sulfate, L-Carnitine, Mixed Tocopherols, Niacinamide, d-Alpha-Tocopheryl Acetate, Calcium Pantothenate, Vitamin A Palmitate, Cupric Sulfate, Thiamine Chloride Hydrochloride, Riboflavin, Pyridoxine Hydrochloride, Folic Acid, Manganese Sulfate, Potassium Iodide, Phylloquinone, Biotin, Sodium Selenate, Vitamin D3, Cyanocobalamin, Potassium Hydroxide, and Nucleotides (Adenosine 5’-Monophosphate, Cytidine 5’-Monophosphate, Disodium Guanosine 5’-Monophosphate, Disodium Uridine 5’-Monophosphate).

So the corn syrup, sugar, coconut oil, all were no good. After my sone was in the hospital they ended up changing him to an Enfalil brand product called progestimil with MCT oil. They said it was what they recommended for babieswith Liver issues. He was undiagnosed at the time, but because he was only 5 months when he left the hospital, we had many more months of formula until he would be eating all regular food. I know that this is partially what saved his life.

The ingredients of the Progestimil are:
Corn syrup solids (42%), casein hydrolysate* (from milk) (16%), medium chain triglycerides (MCT oil) (15%), modified corn starch (7%), soy oil (7%), corn oil (2%), high oleic vegetable oil (safflower or sunflower) (2%) and less than 2%: Mortierella alpina oil†, Crypthecodinium cohnii oil‡, vitamin A palmitate, vitamin D3, vitamin E acetate, vitamin K1, thiamin hydrochloride, riboflavin, vitamin B6 hydrochloride, vitamin B12, niacinamide, folic acid, calcium pantothenate, biotin, ascorbic acid, choline chloride, inositol, calcium citrate, calcium phosphate, magnesium oxide, ferrous sulfate, zinc sulfate, manganese sulfate, cupric sulfate, sodium iodide, sodium citrate, potassium citrate, potassium chloride, potassium hydroxide, sodium selenite, L-cystine, L-tyrosine, L-tryptophan, taurine, L-carnitine.

I think that those with HFI ( rather than being a parent of someone with HFI) have come across food that is supposed to be safe, but tastes off. in those instances maybe the safe product is made in a plant that didn't clean the machines before packaging the safe food. Im sure someone who has better experience will be able to answer you better. and if you are still using the formula, I don't see anything wrong with it. Its impossible to have a well rounded diet because of all of the food restrictions. we use powered multivitamin but I would think the formula isn't hurting anything.

[antonia]

I think it's safe to say doctors understand food and nutrition about as well as they understand HFI. Granted there is a whole lot they have to learn and I don't expect that they should know everything. I just wish "I don't know" was part of their vocabulary when appropriate.

[raluca]

May 20, 2018 7:48:20 GMT -5 rysmom14 said:

Hi Raluca!

Im not sure what formula you are using, but that's great that you heard back from the company and they confirmed that it is safe. I was using Similac sensitive stomach. The ingrediants are:

Corn Syrup, Milk Protein Isolate, High Oleic Safflower Oil, Sugar, Soy Oil, Coconut Oil, Galactooligosaccharides. Less than 2% of: C. Cohnii Oil, M. Alpina Oil, Beta-Carotene, Lutein, Lycopene, Calcium Phosphate, Potassium Chloride, Potassium Citrate, Sodium Citrate, Magnesium Phosphate, Ascorbic Acid, Choline Chloride, Calcium Carbonate, Magnesium Chloride, Ascorbyl Palmitate, Ferrous Sulfate, Choline Bitartrate, Taurine, m-Inositol, Zinc Sulfate, L-Carnitine, Mixed Tocopherols, Niacinamide, d-Alpha-Tocopheryl Acetate, Calcium Pantothenate, Vitamin A Palmitate, Cupric Sulfate, Thiamine Chloride Hydrochloride, Riboflavin, Pyridoxine Hydrochloride, Folic Acid, Manganese Sulfate, Potassium Iodide, Phylloquinone, Biotin, Sodium Selenate, Vitamin D3, Cyanocobalamin, Potassium Hydroxide, and Nucleotides (Adenosine 5’-Monophosphate, Cytidine 5’-Monophosphate, Disodium Guanosine 5’-Monophosphate, Disodium Uridine 5’-Monophosphate).

So the corn syrup, sugar, coconut oil, all were no good. After my sone was in the hospital they ended up changing him to an Enfalil brand product called progestimil with MCT oil. They said it was what they recommended for babieswith Liver issues. He was undiagnosed at the time, but because he was only 5 months when he left the hospital, we had many more months of formula until he would be eating all regular food. I know that this is partially what saved his life.

The ingredients of the Progestimil are:
Corn syrup solids (42%), casein hydrolysate* (from milk) (16%), medium chain triglycerides (MCT oil) (15%), modified corn starch (7%), soy oil (7%), corn oil (2%), high oleic vegetable oil (safflower or sunflower) (2%) and less than 2%: Mortierella alpina oil†, Crypthecodinium cohnii oil‡, vitamin A palmitate, vitamin D3, vitamin E acetate, vitamin K1, thiamin hydrochloride, riboflavin, vitamin B6 hydrochloride, vitamin B12, niacinamide, folic acid, calcium pantothenate, biotin, ascorbic acid, choline chloride, inositol, calcium citrate, calcium phosphate, magnesium oxide, ferrous sulfate, zinc sulfate, manganese sulfate, cupric sulfate, sodium iodide, sodium citrate, potassium citrate, potassium chloride, potassium hydroxide, sodium selenite, L-cystine, L-tyrosine, L-tryptophan, taurine, L-carnitine.

I think that those with HFI ( rather than being a parent of someone with HFI) have come across food that is supposed to be safe, but tastes off. in those instances maybe the safe product is made in a plant that didn't clean the machines before packaging the safe food. Im sure someone who has better experience will be able to answer you better. and if you are still using the formula, I don't see anything wrong with it. Its impossible to have a well rounded diet because of all of the food restrictions. we use powered multivitamin but I would think the formula isn't hurting anything.

Hi rysmom! I also give to my daughter some multivitamins, but unfortunately I don’t succeed in doing that on daily basis, even if I “hide” them in her cereals. She still feels their sweet taste of glucose and rejects the food sometimes. I have to search for some other kind, tasteless – so I will investigate this topic more, on the related threads.

As for formula, I give her a German brand, called Hipp. The ingredients are: Skimmed milk, vegetable oils (palm oil, rapeseed oil, sunflower oil), lactose, calcium carbonate, vitamin C, bourbon vanilla extract, iron lactate, magnesium carbonate, vitamin E, vitamin A, niacin, zinc oxide, panthotenic acid, copper sulphate, vitamin K, vitamin B1, vitamin B6, potassium iodate, vitamin B2, folic acid, manganese sulphate, sodium selenate, vitamin D, biotin, vitamin B12.

So the only sweetner in it is the lactose. I checke online, it seems to be available also in the States, unfortunately with a more than triple price compared with how much I pay here, probably because of some import taxes…
:-(
www.formuland.com/product/hipp-organic-formula-toddler-milk-2-years/

But even so, I listed the link above, in case that somebody reading this thread is still using formula and is interested in different options – Hipp also is safe for HFI, fact confirmed also by the producer.

[rysmom14]

it looks like you have a good formula option! and maybe others can benefit as well.

I use a powdered vitamin called NANO VM. I mix it in his yogurt every day. It is made by Solace Nutrition and I order it from Amazon.

[jenn123]

We use kirkman children’s multi vitamin in capsules. It does not have sugars and can get the recommended dosage in pretty easy, especially when you open the capsule and sprinkle into food. May daughter opted to swallow capsules around 7. I order on Amazon

[colormist]

Here's another article that just popped up in my feed about this very same topic--this time in India: www.uniindia.com/~/sugar-in-baby-food-dangerous-to-liver/World/news/1236716.html

[Stefanie (Ziba)]

Rysmom,

We went to Pitt and had Whole Exome done through their rare disease program. I loved the genetic counselor, but was unimpressed with the docs and the dietician.


I am working on a way to get the "sweeties" habit out of the NICUs. Dr. Li has not responded yet. The irony of him being essentially poisoned with the very thing that was causing him to be ill in the first place in an attempt to diagnose him is mind-blowing!



I may have to send an OpEd piece to the NY Times.

[hfimomof3]

HFI is quite rare (the estimates I've seen are 1 in 25,000 or fewer people) and, when taken all together, there are many many many rare diseases, each of which has different requirements for how doctors and nurses should handle them. A method to prevent HFI babies from being fed sugar needs to take this into account. I think what is needed is a targeted approach that goes through medical specialists who are already focused on our field of interest. Essentially, we need HFI specialists to know what logistical problems we have faced, and what a solution would look like from our end. Then we need to get them to create solutions (possibly with our input) that can be spread through the medical world by medical people. Perhaps we should have another thread (I think we did one a couple years ago) talking specifically about things we'd like the medical world to address regarding HFI.

Reading what you've written, I'm not sure how to systematically address the problem of NICU nurses giving sweet things to infants who might have HFI. A GI doc a couple of years ago told me that HFI is now part of the standard panel of genetic tests given to newborn babies in our state of the USA. So nurses may feel that they don't need to worry about whether a baby has HFI, because the genetic screening should pick that up. Of course we know that not all HFI mutations might be picked up by this screening, and also that genetic screening methodology does not help HFI babies in non-wealthy countries who don't have the luxury of the newborn genetic screening.

The issue of unsafe formula to me seems like that should be easily addressed. Formula makers should know the answer to how much fructose is in their formula, because their job is to feed babies who cannot be fed by breastfeeding. So they know that many of the babies who use their formula will be premature, and might have any number of genetic or developmental differences that will affect their ability to digest that formula. Knowing and giving out the fructose content should be part of their license as makers of infant formula. Why can't the grams of fructose per serving (or per day) be part of their label just like vitamin A and folate?

Likewise, doctors should be able to get that information from formula makers with ease, and should know how to interpret that information so they can give an answer to parents' questions "Is it safe to feed my child who has HFI this formula? And if it is not safe to use this formula, what is a safe formula for my child?" To me it seems the easiest thing to do would be to have a fact sheet for parents of HFI babies and probably a separate (more medical) one for doctors. The fact sheet should be designed by a doctor who knows about HFI, presumably with input from people who suffer HFI or their parents.

[Stefanie (Ziba)]

Has anyone had positive/regular communication with Dr. Tolan? I emailed him a few years ago, and he was quite kind but made it very clear that he is a researcher and not a clinician. But, even though he does not want to give medical advice to individual patients, perhaps if someone who knows him would communicate our growing need for medical information to be dispersed within the medical community (e.g. NICU "sweeties", etc.). he may be amenable to being the catalyst.

P.S. I live in FL and they did the newborn screening on both my kids. HFI is not part of the panel here, and even if it had been, it would not have helped because Remy has a new mutation.

[colormist]

I wrote Dr. Tolan once. I don't recall what I was writing about, but I shared a link to my blog and all the info-gathering I was doing. He responded within a week and it was positive. I think I was just saying thank you for his website and the work he was doing. I know he's working on that consumable for treating HFI. That might be taking up a majority of his time right now.

[Stefanie (Ziba)]

Maybe this will help. Please sign:

www.change.org/p/american-hospital-association-get-sugar-water-out-of-the-nicu?recruiter=65790259&utm_source=share_for_starters&utm_medium=copyLink

[rachael]

I signed and shared.