Cold sweats DELUX!

[kollynsmom]

Well, the last few days my lil man (4months old) has had MAJOR cold sweats and is very fussy... And by very I mean crying all the time unless bottle or jug is inserted. He has no temp and besides the fussiness, minimal naps & major pee pee diapers there are no other symptoms. I have tried to cut down the soy milk bottles to just 2 a day (one around noon and the other at midnight). I am on a no gluten diet and am low blood sugar as well. Any ideas? Is this just a normal symptom of HFI? I know when they are about to go through a big growth spurt the cluster feed. He is drooling a lot and I suspect we will start to see a tooth in the coming weeks as he will be 5 months on the 21st.


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[lucky]

Hi Kollynsmom,

If seeing anything different from the norm in your daily lives, I would always err on the side of caution and touch base with your son's specialists. Listen to your "mommy radar". That being said, at first glance, it looks like your son may be experiencing low blood sugar symptoms. But have a look at the below to see if anything else looks suspicious...


In a previous thread you mentioned your son was put on formula until tests could verify that Galactosemia was not an issue. And after his HFI diagnosis, you reintroduced breast milk. To work alongside your specialists, consider these:

If you just started noticing these symptoms a few days ago, something has changed:

When you formula fed only:
* Was you son completely content and thriving?

When you breast fed only:
* Before he was in metabolic distress, was he content?

Now that you are doing both:
* Did you just recently start this?

If neither milk type is an issue:
* Did you recently change his feeding schedule?
* Are you feeding him on demand? Try to.
* Are you trying to reduce formula to increase breast milk feeds?
* Is your son happy/full or fussier after formula feeding?
* Is your son happy/full or fussier after breast milk feeding?

If happy on both, consider:
* Does one leave him full longer?

Formula takes longer to digest = fuller longer.
Breast milk digests faster = *hungrier* sooner.

If either makes him grumpy:
* Ask if soy formula is an issue.
* Ask if your diet (for nursing) is an issue.

Ask how to fix YOUR low blood sugar.
* Should you just only eat more often to compensate?
* Are you getting dehydrated?
* Are you balanced / taking safe vitamins?

At 4mo. old you also may be seeing:

Growth spurt:
* Few days of needing far more frequent feeds.

Increased awake time / activity level:

If no solids yet introduced:
* May permanently need more milk feeds
* Especially if breast fed vs formula

Increased saliva:
* Nature's prep for soon eating solid food.
* Sign of teething (inflamed gums).
* Tip: Vaseline on clean area prevents rash.

So...
If it's a growth spurt, especially feed your son "on demand" till the spurt ends. It usually lasts a few days. But also compensate for yourself. Extra fluids, food and rest -as all three (but especially rest & good sleep) dramatically increase breast milk production.

However, overall, if you generally notice formula lasts longer than breast feeding, then you may learn that you'll just need to breast feed more often to keep your son as full and comfortable. Once solids are advised and safely introduced, this timeline will again change... it will get easier as milk frequency and quantity will decrease as solids increase.

If you notice that your son tolerates both breast milk and formula -but you consistently (long term) seem to need to feed him sooner than you used to even a few weeks ago- then, you may be generally seeing that *overall* more food is now needed. Either more breast milk, or formula. Or soon... a little 1st stage solid baby food.

At 4.5mo old and 14lbs
Your son might just need the extra food energy:

Ask your doctor:
* Is it safe (or suitable) to introduce a bit of solid 1st stage food?
* If so, what safe amounts to test / start.
* If not... is cornstarch supplements advised?
(to help maintain blood sugar between feeds)

* With your son's medical background and recent serious hospitalization, you'll need to confirm everything. EVERYTHING. At least until he is much older, stable long-term and you know what a healthy "norm" is for your son.

Crankiness / not sleeping well:
* Is it because of hunger or hypoglycemia?
* Or discomfort from either type of milk?
* Is other food a factor?

Peeing:
If normal = good
If far more...
* Due to minor fructose ingestion?
* Is soy formula or other food an issue?
* Are kidneys working harder to filter / expel?

If peeing more than usual:
* Careful for potential dehydration.

Stool:
* Keep track of colour, frequency & consistency.
* Formula stool differs from breast fed
* Dehydration = harder stool

Dehydration / Electrolyte Imbalance...
* Balance is needed to help keep blood sugar stable.
* Infants & small children are more susceptible.
* Also lost through sweating, and excess urination.
* Tip: look for dry white salt stains on bedding, clothing.

HYPOGLYCAEMIA :: low blood sugar
Some signs include:
* Sweating, irritability, sleepiness, hunger.....
* Infants can also have a weak cry.
* Ask the doctor to fully inform you *



* I would recommend you discuss hypoglycaemic symptoms and proper correction protocol for your little man. At 4mo old, and with other unanswered issues still under investigation, you'll want to be as informed as possible to keep your son safe, healthy, comfortable and happy.

A happy, comfortable child is a thriving one.
And a thriving child makes for a happy, relieved mom.
Who then can finally rest, and eventually sleep well...

To cover all metabolic safety bases, we were always told to feed on demand. So if you think you are seeing possible hypoglycaemic issues, this will help. But definitely follow up with your son's specialists for proper medical advisement on all things "HFI :: baby". The signs you are recently seeing might be transient -or- clues to potential energy and storage / retrieval issues as in FBPase deficiency (which is genetically very close to HFI). Always better to be cautious...


Hypoglycemia thread:

*Category: HFI Lifestyle: Children (5-12yrs)
*Thread :: Hypoglycemia
hfiinfo.proboards.com/thread/1561/hypoglycemia
(-includes cartoon print-out pictures of symptoms-)

[kollynsmom]

We have a genetic * nondiagnosis (pending liver biopsy) for the HFI ... Could there be more then one issue? HFI and FBPase? We ARE going to the Pediatrician today and possibly the Metabolic Geneticist tomorrow.


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[colormist]

Kollynsmom - I had a really bad reaction to soy milk before I had my diagnosis. Soy products are tricky. Sometimes they're okay and sometimes they can give you a severe reaction. I'm not saying that's the issue, but from what you have described, that would be my first suspect. Those symptoms sound exactly like a hypoglycemic reaction to ingesting fructose or sucrose.

Can you post the ingredients of the soy formula? Have you found that formula listed in the nutrition database?

Nutrition Database: nutritiondata.self.com/

[charlie]

Hi Kollynsmom

I didn't answer this straightaway as wanted to leave it to those with HFI infants. I back everything said so far, especially Lucky's check list.

It does sound as tho hypoglycemia is an issue, why are you using soya formula, is there a lactose question for him. Alot of those with fructose issues do struggle with soya.

By the sounds of things you are off for medical advice today which is the best thing, get him thoroughly checked out, and double check his formula. Are you starting to wean him yet, is anything slipping in there?

[kollynsmom]

Our metabolic geneticist said it was the only formula she was comfortable with giving him. Lactose was an issue until they ruled out galactosemia. They (pediatrition) still think he has an issue with cow's milk. The pediatrician ran a metabolic panel and a complete blood count. Only thing wrong on the metabolic panel was his BUN and his red blood cell count is pretty high on the complete blood count. Some other things are going on in his labs as well but his liver enzymes and creatinine are normal. Our MG was off today so I will call her first thing in the morning.


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[kollynsmom]

He uses Enfamil ProSobee ready to feed. We are trying to only feed his 2 bottles per day. The rest of his feeds are breast milk. This has been his diet for almost 2 months.

[kollynsmom]

Infant formula, MEAD JOHNSON, PROSOBEE, with iron, ready-to-feed
Read More nutritiondata.self.com/facts/baby-foods/441/2#ixzz34rnx13Nb

[charlie]

Ok, just had a look at the ingredients on a page about prosobee and there are two things that jump out. Corn syrup solids is the biggest ingredients, could corn be a problem, also coconut oil. I know there are those on the csid facebook page whose children have not been able to manage this formula. I would query the ingredients with your physician as a potential problem.
Corn Syrup Solids (55%), Vegetable Oil (Palm Olein, Soy, Coconut, and High Oleic Sunflower Oils) (26%), Soy Protein Isolate (14%), 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 Phosphate, Magnesium Phosphate, Ferrous Sulfate, Zinc Sulfate, Cupric Sulfate, Potassium Iodine, Sodium Selenite, Sodium Citrate, Potassium Chloride, Potassium Hydroxide, Potassium Citrate, L-Methionine, Taurine, L-Carnitine.

[colormist]

So I took a glance at the nutrition database to see if it listed the types of sugars (of course it didn't). It did list that there were 2g of sugar per serving. But we don't know if those are good or bad sugars.

Looking at the ingredients that charlie has posted, I'm not seeing anything that would register as a sugar--but inositol sounds suspicious as it has the same ending as sorbitol and malitol (both of which are not safe for people with HFI). I looked inositol up on wikipedia and discovered:

Inositol is a sugar. en.wikipedia.org/wiki/Inositol

I've never seen this ingredient before, so I'm not sure if it's safe or not. It's not listed on Dr. Tolan's site.

This excerpt makes me suspicious: "Inositol or its phosphates and associated lipids are found in many foods, in particular fruit, especially cantaloupe and oranges.[6] In plants, the hexaphosphate of inositol, phytic acid or its salts, the phytates, are found. These serve as phosphate stores in the seed. Phytic acid occurs also in cereals with high bran content and also nuts and beans, but inositol when present as phytate is not directly bioavailable to humans in the diet, since it is not digestible (s)ome food preparation techniques partly break down phytates to change this."

All those foods listed in the that excerpt are not safe for people with HFI.

On further in the wiki article, it say that inositol is synthesized from glucose-6-phosphate--which makes me think it might be a safe sugar (glucose is a-okay for people with HFI). However, if you're not seeing any improvement in your child, you might want to ask your doctor about that ingredient.

I'm not sure where else the sugars could be coming from. If they're not coming from inositol, then it must be coming from soy or corn syrup--both of those sources would mean that it definitely has unsafe sugars in the formula and you should not be feeding it to a child with HFI.

[lucky]

Should it be of interest to exclude....


FBPase Deficiency :: testing / info

*Catagory: Odds and Ends: Miscellaneous
*Thread :: FBPase testing
hfiinfo.proboards.com/thread/1209
(-includes Hospital & lab info / Metagene: HFI & FBPase deficiency breakdown, definitions-)



Additional note ::

Metagene :: synonyms for HFI, FBPase deficiency
(-notice the gene locus # of each... very close-)


HFI:

* Hereditary Fructose Intolerance
* Fructose 1 Phosphate Adolase deficiency
* ALDB
* Adolase B deficiency

-OMIM # 229600
(Online Medalian Inheritance of Men)

-Enzyme : Fructose 1,6 Biphosphate Adolase B

-Gene locus : 9q 22.3



FBPase deficiency:

* Fructose 1,6 Bisphosphatase deficiency
* Fructose 1,6 Diphosphatase deficiency
* FBPase deficiency
* FDPase deficiency

-OMIM # 229700
(Online Medalian Inheritance of Men)

-Enzyme : Fructose 1,6 Bisphosphatase

-Gene locus : 9q 22.2 - 9q 22.3

[lucky]

I'm not sure how helpful...

Old threads that reference HFI vs FBPase deficiency ::


1./
*Catagory: Odds and Ends: Miscellaneous
*Thread :: FBPase testing
hfiinfo.proboards.com/thread/1209
(-includes lab info / Metagene: HFI & FBPase deficiency breakdown, definitions-)

2./
*Catagory: HFI Lifestyle: Younger Children (Newborn-5yrs)
*Thread :: In Desperate Need of Answers. PLEASE HELP!
hfiinfo.proboards.com/thread/1444/desperate-answers-help
(-includes links to 'FBPase testing' thread / 'New Diagnosis... not many symptoms' thread-)

3./
*Catagory: HFI Lifestyle: Younger Children (Newborn-5yrs)
*Thread :: New Diagnosis... not many symptoms
hfiinfo.proboards.com/thread/1337/new-diagnosis-symptoms
(-includes personal understanding of FBPase deficiency & examples of our testing protocol-)

4./
*Catagory: HFI Lifestyle: Children (5-12yrs)
*Thread :: HFI, FBPase deficiency, or??
hfiinfo.proboards.com/thread/1151/hfi-fbpase-deficiency
(-includes links to 'FBPase deficiency testing' thread-)

[kollynsmom]

Okay got off the phone with genetics and if you have an issue with Adolase B you have HFI and not FBPase. Also, sine we have not done a liver biopsy we are not "confirmed" HFI. We have the genetic labs for a "heterozygous mutation, g.-132G>A... Like most of you, there is no copy mutation. I am trying to avoid the liver biopsy while he is so small. From what I understand you generally do the biopsy first, then the genetics to confirm.

[colormist]

I only had to do the genetic test to confirm that I had HFI. I'm not sure why you would do a liver biopsy first as a genetic swab is way easier for the patient than a biopsy. If they find two genes (of their known pool of HFI genes), then you have HFI. However, if they can't find a pair of genes with the swab, then they might go on to do a liver biopsy to confirm HFI--then later add those genes to the list of known HFI genes. This is just my understanding--I totally do not blame you for not wanting to do a liver biopsy on a young child. I don't want to do a liver biopsy as an adult. I'd rather just presume I had HFI my whole life rather that have them chop me open to confirm it.

I could, however, see putting a fructose challenge AFTER a liver biopsy, as performing a fructose challenge on a young child who has HFI could end up hospitalizing the child. The fructose challenge will test for Fructose Malabsorption--which is a less restricted diet than HFI and (from what I understand) is more similar to lactose intolerance. From what you've wrote so far, it doesn't sound like Fructose Malabsorption is even on the table, so I have no idea why I even mentioned it.

[lucky]

Yes...

HFI = Adolase B issue
FBPase deficiency does not



EDIT ::
For those w/o a definitive HFI diagnosis:
If FBPase deficency is mis-managed and a patient metabolically unbalanced, it can look like HFI. So, this is why HFI is always first suspected until an HFI DNA test comes back negative, other tests show inconclusive (or show additional unexplained symptoms), or biopsy testing confusingly shows no problem with Adolase B.

There are similarities and difference between the two genetic issues, but the largest is that HFI has a problem with Adolase B. FBPase deficiency does not. But it's an ugly cousin to HFI, and great at mimicking it if improperly managed. And especially serious in infancy and early childhood (until under control).

HFI's issue is Adolase B deficiency.
FBPase deficiency's primary issue is energy storage/retrieval... and then fructose... (which is what eventually starts the imbalance later toward full HFI symptoms -if- primary issue is not managed).

You asked if you can have both issues? I don't know. I'll leave that to the specialists. But if there are still pending long term unresolved issues, your specialists will probably have to exclude this, glycogen storage disorders, and possible mitochondrial disorders also. Ours did.

[kollynsmom]

They said he would only have a low blood sugar if exposed to Fructose and believe the soy formula is safe. I keep questioning the formula as he spits it up quite a bit and barely burps anything up when I nurse. The pediatrician thinks its a virus.


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[colormist]

Could you try not feeding him the formula for a day to see if his condition improves?

I mean, I've seen dietitians and nutritionist suggest vegetables that were LOADED in fructose and say they were safe for people with HFI. It's really hard even for people with HFI to be able to parse an ingredients list and tell if the food is safe. Food manufacturers don't exactly make this easy on us.

[lucky]

Kollynsmom,

So he only spits up the formula?

Did he also when he was SOLELY formula fed?
If so, could this be a reason for needing the wedge / sling to reduce reflux issues?

However, if this is only now recent...
Then maybe the regurgitation is from a virus. But illness or growth spurt -even though taxing his system temporarily- shouldn't give hypo signs in a regularly fed / hydrated baby. That should warrant investigation, no?

And even if every ingredient is "safe" in the formula, did either specialist address what to do in the event of seeing possible hypoglycemic symptoms at some point in the future? Or give you safety parameters to identify / properly handle / document?

If your son is healthy on breast milk, is it possible to just switch to that at this point?

[lucky]

Just thought of something...

Years ago, we were told by our Metabolic/Genetics specialist (when under investigation for HFI) that the act of converting stored energy back to useable energy creates fructose... This happens when one is too hungry and initial accessible energy storage held by the liver is depleted.

Even today, if our son (12) is ill (and doesn't eat), or fasts way too long, or doesn't snack after more than usual increased activity (metabolism increases and activity burns off food energy faster), he'll often say he feels a "fructose stomach ache". And the nausea and stomach pain will increase till he vomits (mostly stomach liquid/acid) if he doesn't eat. However, if he regulates his food intake and doesn't get empty to that point of reconversion (on that very empty stomach), he has no issue. And if we catch it early, just feeding him (even a little) will nullify it. So, this stomach ache is the beginning, but also just one of the signs of hypoglycemia potentially starting. Does the reconversion 'poison' him with a dose of fructose? Yes, most likely.

Our son is not HFI, but he still has to avoid that fructose reconversion. His liver is much larger now (and can hold far more stored, useable energy than in early childhood/infancy), so not as necessary to reconvert from deep body storage. He just eats to accommodate. And not to excess as he eats more often.

However, at 4mo old, if your son is HFI and is not getting dosed by anything "food" (milk or formula), but is showing signs like he is, could it be he's too empty and being naturally dosed with fructose in reconversion? A lot of these infants/children will go into a cycle where they eat to feel better, but eat too much so they throw up excess. No one thinks much of it, because generally they are healthy weight. However, if the cycle continues, weight increases because the eating cycle becomes a coping mechanism... and not well understood or managed properly.

Or... it's none of this, and your son is just under the weather, or in a growth spurt and needing more feeds to alleviate the symptoms that appear hypoglycemic in nature.

[lucky]

Colormist,
You are right.

1./
A Hydrogen Breath test is for testing Fructose Malabsorption ONLY. A fructose solution is consumed, breath samples analyzed, but NO blood or urine is tested. There is also NO specialist monitoring or extra safety measures (other than being conducted in a hospital). It is NOT advised for those suspected of HFI.

2./
A Fructose Challenge is essentially a Hydrogen Breath test -BUT- now also under careful supervision of a Metabolic/Genetic specialist and metabolic nursing team. On this, the patient is hooked up to an iv (for safety) and blood and urine collected for a few hours (and later fully analyzed by lab). Blood sugar is very carefully monitored and any dip (if seen) is swiftly handled with corrective solutions through iv. If this happens, testing ends.

3./
A Fasting test is also helpful to determine metabolic issues connected to fructose. Similar to the above (#2) but without drinking the fructose test solution. Same metabolic team supervising, same iv, same blood sugar monitoring and blood / urine analysis. Just basically hooked up to protective iv and fasting. Then labs are analyzed to see how body responds to extended fasting.

However, none of these are necessary if one has a positive HFI genetic diagnosis, though.

And...
Like yourselves, we would not subject our son to an invasive liver biopsy unless it is a last resort (and very ill). If either one of our son's DNA blood tests would have come back positive, it would have been be a positive diagnosis. No need to biopsy just to confirm a confirmation. A DNA match to an already identified mutation is enough. We'd know the mutation's coding.

Alternatively, if DNA tests were negative, but labs from other metabolic tests (Fasting, Fructose Challenge or very compelling blood work during metabolic distress) show positive, then we were told further confirmation might still, unfortunately, be necessary. Unless labs were very obvious for diagnosis.

Likewise, if a mutation is identified through DNA (positvely diagnosed) but amount of functioning enzyme activity needs to be known... a needle biopsy (liver) is a less invasive potential option (to a liver biopsy). But it does have limitations. The needle can't extract as much material for full testing (to eliminate a larger range of possible issues). But to test a very few, or confirm one (and it's coding), or analyze enzyme capacity... it can. Still, who wants that. Unless necessary.

If you have genetic DNA confirmation (and nothing else needs to be looked into or excluded), it should be enough. It would be for our specialist and our family.