Hereditary Fructose Intolerance (HFI) and metabolic disorders

[nurturebaby]

x-posted in health and healing...

anyone familiar? my 9 month old is suspected of having this. the doctors made her stop breastfeeding and she is on an ng tube being fed Elecare, which she is not responding well to. Lots of diarrhea and vomiting with most feeds.

Found out the first ingredient, corn syrup solids, may not be tolerated in HFI patients, according to the BU HFI lab site, which lists sugars and their safety. I believe this is the lab that the hospital sent her bloodwork to.

what about breastfeeding? everything I've read has actually said that breastfeeding is safe and it's only during weaning that issues come up. But I do know that with some other metabolic disorders, breastfeed may not be safe.

We're ust trying to figure out the safest way to feed her. She was tolerating my milk well, with the exception of some loose stools and slow growth, but the doctors think this is attributed to milk protein or gluten, both of which have been eliminated from my diet for quite some time.

She has had 2 episodes now after eating pureed veggies - violent vomiting, passing out/possible seizure according to the doc who saw her, diarrhea. This is was preceeded the most recent hospitalization and when we were to to discontinue bf'ing.

Thoughts? I'm at a loss and freaking out. I want to safely feed her and I feel like my milk is the safest way to go, but will just about die if we end up switching her and she suddenly reacts to it when it was fine before....

[purplepaisleymama]

[QUOTE
We're ust trying to figure out the safest way to feed her. She was tolerating my milk well, with the exception of some loose stools and slow growth, but the doctors think this is attributed to milk protein or gluten, both of which have been eliminated from my diet for quite some time.

She has had 2 episodes now after eating pureed veggies - violent vomiting, passing out/possible seizure according to the doc who saw her, diarrhea. This is was preceeded the most recent hospitalization and when we were to to discontinue bf'ing.

Thoughts? I'm at a loss and freaking out. I want to safely feed her and I feel like my milk is the safest way to go, but will just about die if we end up switching her and she suddenly reacts to it when it was fine before....[/QUOTE]


It sounds with what you wrote that she was doing immensely better when she was breastfeeding, she may have a metabolic condition that means that you will have to limit your diet while bf'ing but it seems that based on what you are saying she would be better off if she was exclusively nursed. My youngest was 10 months old before he tried food and he nursed until a few days before his 4th birthday, he has severe food allergies and I had to avoid the foods (pages of them) from when he was diagnosed until he weaned completely. I will say that it was not always easy but it certainly was better than trying him on formula, with his allergies I don't believe he would have survived on formula. I was advised by doctors to quit nursing and put him elemental formula, thankfully I didn't and when we finally had a diagnosis and met with the allergist he mentioned that Finn would have suffered if not died if we had persisted and kept him nursing. I know that you are feeling alone and stressed right now but YOU KNOW YOUR CHILD BEST, you know when she is feeling better and if you think that breastfeeding is better for her, you have the right to do that. You can tell the doctor that you have decided that she will be breastfeeding, if they have a problem with that you can find a new doctor. I know that sounds scary, but you must do what you feel is best for your daughter and your family. When we first started going to doctors for Finn it was so hard to stand up and tell them my opinion, it took many difficult appointments where I was scared to talk to the doctors before I learned to take control of my son's health. I still have times when I feel that I am taking a risk, that they will stop trusting me if I tell them what I am thinking, thankfully we have gotten to a place where we are on the same page as all of his doctors, it took a long time!

If you need someone to chat with pm me I hope that you get answers soon!
Take Care,
Laura

[mrsfru]

I would breastfeed her, b/c you said that she was doing better before. DS1 does not have HFI, but he does have the less-problematic issue w/fructose (don't remember what it's called--it is a fructose intolerance, but not severe like what HFI is). He has many issues, each one seemingly small, but all together adding up to something. We just don't know what exactly yet.

At any rate--stand up to them. Tell them you have decided to get her back on the breast, b/c she was better. You do have that right, even if they try to fight you on it. My boys were both in the NICU and I had to fight for them to only have bm at times. It was tough, but you can do it! hugs!!!

I don't personally ever remember reading that breastfeeding was contraindicated w/HFI. I read a lot. We refused any further testing past the bloodwork and the sweat chloride test for CF. We figured out ds's fructose intolerance thru trial and error, but like I said, his is not HFI.

hths and good luck!

mrsfru

[nurturebaby]

thank you both for responding... it is good to know that there are strong parents out there who have fought similar battles - you are an inspiration to me, really...

The only thing that makes me nervous about bf'ing again is in the event that she has some other metabolic disorder where Bf'ing is unsafe. Anybody know if it's possible for breast milk to be tolerated well (although accompanied with slow growth and loose stools - but this is a possible milk protein intolerance), and then become dangerous after several (9, to be exact) months?

At the very least, I am going to ask to be switched to a different formula.

I feel confident that bf'ing is safe for a child with HFI though - I'e seen enough evidence to make me comfortable with that. It's just that now I am nervous it could be something else...!!!

[2boyzmama]

I'm confused...why did they make you stop breastfeeding her? Was it only because of slow growth? Why the NG tube then?

My son had very slow growth, is still officially failure to thrive at 3 years old (is 24 pounds and 34 inches, will be 3 years old in a few weeks, birth weight was 8lbs 4oz, so he hasn't even tripled birth weight yet) He also had a host of other medical issues. I was pressured repeatedly to stop breastfeeding him, or to at least supplement him. I never did, and it turned out to be a good thing because he had a dairy and soy intolerance, so we probably would have had one very sick little boy until we figured out which elemental formula worked for him (if any).

We later found out he has a genetic syndrome involving one of his chromosomes, and now everything about him makes sense, including his very small size. Turns out that it's NORMAL that he be this small, and tube feeding him will not make him any bigger (some kids with his syndrome are tube fed for other reasons, and they are still very small)

SO...follow your gut. If she was better before, then I'd switch her back to breastmilk. Keep pushing for the right testing and right diagnosis to explain anything that is abnormal.

[mrsfru]

i can tell you that my little nursling right now had food intolerances so bad, that he was failure to thrive along w/severe reflux, and i had nightmares that something was seriously wrong and we would lose him. he's now 27 lbs and 32" tall at 17 mos! he turned around w/i 2 wks of eliminating a bunch of foods. dairy, eggs, and soy were his big intolerances.

it's possible they are missing an intolerance. also, there was a post a week or two ago about special formulas...and it appears that enfamil and similac make other special ones w/o corn syrup. you can look them up on their websites.

hths

mrsfru

[nurturebaby]

Originally Posted by 2boyzmama

I'm confused...why did they make you stop breastfeeding her? Was it only because of slow growth? Why the NG tube then?

I was/am confused, too - originally it was because they suspected milk protein intolerance. Apparently now it's because they want to confirm that my milk could not possibly contain fructose or any substance that could be harmful to a child with HFI. The only thing is - breastfeeding looks like it's the safest thing for HFI, according to most, if not all articles I have come across, so I'm not sure how seriously they're taking their research, otherwise I feel they would have come up with something by now.

I also think they recommended formula simply because they were not sure what was going on - they wanted to start wth a clean slate of being on formula for a couple of weeks, and then reintroduce foods later. I'm just afraid that by the time they give me the go-ahead, that my supply will be diminished (I don't respond well to the pump) and/or her sucking/latch reflex will disappear since she's off the tube.

she's on an ng tube because she's been exclusively bf'd and will not take a sippy/bottle.

[sbgrace]

Did you actually talk to a metabolics doctor? What tests did the run to figure out if she does have a metabolic condition?

[TzippityDoulah]

i have fructose intollerance as does my oldest boy. not HCI though... i've never known anyone else with it! interesting thread!

[nurturebaby]

Originally Posted by HennyPenny

i have fructose intollerance as does my oldest boy. not HCI though... i've never known anyone else with it! interesting thread!

do you have fructose malabsorption or hereditary fructose intolerance? they're a bit different, but the HFI is far more rare... If you do have HFI - how were you diagnosed? This diagnosis phase is lasting to long - hopefully we get the results from the genetic test soon... if it's negative, I think the doctors are concerned enough about it that they would want to do a liver biopsy since the genetic test can have false negatives.

[mrsfru]

do you have fructose malabsorption or hereditary fructose intolerance?

"Fructose Malasorbption"---that's the term I couldn't remember!

mrsfru

[ericswifey27]

I think best bet would be to follow a strict diet and use breastmilk, if your own instincts are leading that way, our gut has a funny way of usually being on the right track. Have you kept a food diary to help you track the big offenders?

I am glad someone mentioned Fructose Malabsorption- I will look into that for my son and my oldest son. I had been seeing some success with a gluten free diet, but now am wondering if fructose is an issue.