New to HFI- looking for support/answers!

[rysmom14]

Hi All, I have been reading through a lot of posts and wanted to throw my story out there and ask for some feedback/ suggestions as well. I am so happy to have stumbled on this discussion board. I have learned so much, and to hear things from others dealing with the same this is a HUGE help.

My son, who is now 13 months old, was just diagnosed with HFI through genetic testing. at 4 months old he went into the hospital with fluid in his abdomen and they also found liver cirrhosis. He was hospitalized for a month. before he went in, he was a happy baby, and very laid back. He pooped more often and had projectile throw up, but the peds office tha he needed a different formula We also notice that he was slower to develop than his fraternal twin sister, but thought he would grow at his own pace. All in all, there was nothing al that alarming going on He was on Similac sensitive formula since he was born. He did seem to poop a lot and he would have terrible diaper rash. I always said that poop must be really acidic or something. In the hospital, the ran every test under the sun, but came up with nothing. They sent the xsome sequencing test to Baylor, and we were waiting for results. they switched his formula to Enfilmil- progestimil when he was in the hospital and it seems the actually ended up treating him without even knowing it because that formula doesn’t have any sugar in it. From age 6 month until he was diagnosed at 1 months he had been eating baby food and then real people food including all the things that we now know are bad for him ( carrots, sweet potatoes, fruits at least 2 times a day) and we never really noticed a difference. He started to catch up developmentally and didn’t have the acid poops. The throwing up stopped and there has only been an occasional spit up.

we have been following up with a liver specialist and getting labs drawn quite often. His numbers have been going down steadily towards normal, and he was taken off all of the diuretic medication he was put on the help keep the fluid out of his stomach. The only other issue we had was that his liver was still enlarged, but everything is functioning normal. A couple month ago genetics called and said that there was NOTHING that came back on his sequencing test. We count be happier and chalked the whole thing up to a freak illness. We were very surprised to hear back from genetics a week ago telling us that they re-ran his test with different parameters and it showed that he had HFI. He is currently the only patient in the Childrens hospital with theis diagnosis, and they have only ever seen 3 cases. Needless to say, they are trying to offer help and support, but I think we are all trying to learn together which leaves us feeling very alone in this whole process.

So I guess some of the questions I had are:

are there different degrees of severity for HFI and how can you tell where you fall on the spectrum?

How do I know what he can really eat, I find so many conflicting things. Some sits say a food is ok and other sites say the food should be avoided at all costs. It seems it all depends on the person, but I am worried that even if he doesn’t get sick from a food, it still may be causing damage.

Any other suggestions or help/ thoughts would be great. We have only known for a week and my husband and I have been researching as best we could and just don’t want to do the wrong thing.

[heatherp]

Hi,

I replied to your message, so look for that! If you email I will send a list of Wyatt's safe foods. We were told by Wyatt's metabolic specialists (who also have rarely seen HFI, but have been extraordinarily helpful....he has a metabolic dietitian in addition to a Genetics and Metabolics team of doctors, and I have to say we have been extremely pleased with all of them) to trial foods one new food per week, using the BU HFI diets and the sugars list, plus what we learn on here, as a baseline guide and starting with the safest foods first and then expanding from there. Wyatt now has a very solid and decently easy to work with diet (in fact we have HFI safe family meals, which we never imagined initially). He was diagnosed around 16 months. Also as an FYI, Wyatt is on the clinical nutrition NanoVM from Solace. They run bloodwork for vitamins and minerals every 6 months. The NanoVM has worked well for us. He also takes extra Vitamin D (drops made for little ones from health food store) and Vitamin C (powder from health food store). There is not a whole lot of Vitamin C in the NanoVM.

We are constantly on the lookout for symptoms and we still trial only one food per week. Wyatt's first symptom is usually bad breath and sweet smell to urine, followed by diarrhea which is very acidic. He refuses sweet foods, which is nice, so we haven't had too many problems. The most he will do with an unsafe food is put it on his tongue and immediately spit it out (so far anyway! We hope that sticks around!!!).

I don't know anything about how you can determine degrees of severity, although theoretically speaking you would see some differences between homozygous vs compound heterozygous. I'd need to have a conversation with a researcher to feel confident in knowing more about that, though. My hunch is that there just really aren't even that many people out there who know a whole lot about HFI, save for those people who have it.

I think, also, we have learned these things 1. It is hard when the littles are sick. There are few safe things to give them. We have ibuprofen compounded for Wyatt by a very fine compounding pharmacist. We have had her compound antibiotics for him, also. When she retires I will cry. I trust her, and that is huge. 2. We still spend a good chunk of time researching, but what we research has shifted from foods to medicines, vaccinations, section 504 plans for school aged kids with metabolic disorders, etc to help us prepare for the future. 3. You will make some mistakes and you will learn from them. HFI or not, these things happen. 4. If your kiddo doesn't reject sweets then to dextrose or not to dextrose seems to be highly debated and you will need to decide what is best for your child in your circumstances, but you don't need to do that yet. 5. A kid with HFI is just that...a child first, who has a manageable condition. Yes, it's a pain to make and take food everywhere, and I haven't stopped doing dishes since he was diagnosed. But it can be done really quite easily these days, what with dishwashers and all. The HFI part, we have found, is only as big a deal as we allow it to be, and we just aren't in the business of allowing it to be a big deal, and as a result, Wyatt is like any other almost two year old out there, except for eating from his own little cooler lunchbox. 6. While no one in your community has HFI (likely, anyway) there are lots of anaphylaxis allergy moms living near you, and while it is a different situation, they do understand the care with which you have to approach food, birthday parties, etc, and they are great resources, also.

I hope some of that is helpful!

Keep posting!

Heather

[Tammy]

Hi and welcome here. Heather has given some great advice to start with.
I just want to add that my daughter was the same way. She ate all foods before we knew what was the problem with no reactions at all except cirrhosis of the liver. After the liver biopsy and the the DX we started on the diet. At that point we couldn't tell if she ate something wrong or not. After she got "cleaned out" and healthy again on the diet, then she developed her reactions when she eats something wrong. YOu may find that will happen with your little guy too. It's not a bad thing to wish him to feel ill as then you'll be able to tell when something isn't right.

As for spitting out sweets, that is a good thing and don't push them on him if he doesn't want them. As Heather has pointed out, it's a hot topic on here, but even I - as a supporter of using dextrose - would rather see the kids develop a taste AGAINST sweets.

And the biggest thing to do is BREATHE. It will get easier and reading labels will just become a part of normal life for you.

[rysmom14]

Thank You Heather! This is such great info. I never thought that far ahead as medicines, and vaccines. o geeze. so far the doc's didnt think he needed a vitamin, but I think when we go to the next folow up I will bring the names of what you are using and get their opinion. I guess i should also have some other meds on hand like the pain meds.

Tammy, you said your daughter only had the liver cirrhosis too. it seems from researching that that is such an serious complication and the doctors kept asking and asking if there were other symptoms that I wasnt seeing. I wish he had other things that we could have caught it earlier. he was a very sick baby, and they were not sure if he was going to make it at one point. because of the liver cirrhois and the fluid he had a lot of procedures and between that and just being in the hospital and so compromised he turned septic and was put under complete sedation/ paralysis in the PICU for a week. I hope that Ryan does develop begin to dislike foods that are harmful. we have been trying to stick to the allowed foods since we found out, so i am hopeful that he will be cleaned to be able to start fresh soon. if it is ok to ask, how is your daughter's liver now? has it healed at all. I know they say that the cirrhois is permanant, but did she also have an enlarged liver and did that ever go back to normal?

Thanks so much

Emily

[Tammy]

Hi Emily
How horrible for you to have a baby that sick. I do understand as a parent what that is like. Regina wasn't quite that sick but when she was 7 months old we went for an hour apt, and stayed a week. That was when they first discovered the enlarged liver. After a week, they at least left us go home and did everything from there as an outpatient.

The liver is the only organ of the body that will heal itself over time. It's not overnight but if you stick to the diet the liver will begin to function just fine. Her tests come back fine now and it is normal size again. The part that still "haunts" her is that while her liver wasn't working correctly, the body forced itself to create a web network of small blood vessels around the liver. These will be there all her life. The only time it really matters is if she'd need any more abdominal surgeries - for example if she needed her gall bladder out. We actually only know this because she did require surgery 10 years ago and it turned into a much more major surgery than was expected.

Now don't panic. This was 30 years ago and she had much more advanced cirrhosis. She was almost 3 until they figured out her HFI and by that time she was also eating all normal adult foods. Fruit, candy, cookies, everything. They estimated that if we wouldn't have found the HFI she would have had liver failure within 6 months. She is now 33.

I hesitated to even write this as I don't want you to be concerned about it. But I decided to stress just how important it is to stay on the diet even though you don't see any reactions from him yet. It was probably a couple of years until we "goofed" something she ate and reacted to it.

Again, this was so long ago that there wasn't any kind of wonderful board like this so I didn't know to watch for things like stinky pee as an early sign. Did she maybe at least have that as a baby? I have no idea. My memory isn't that good looking back.

The important thing is that now you know what the issue is and now you can handle it. It seems daunting at first, but it just gets easier as you go. And there are several wonderful people on here that are always glad to help if they can.

[colormist]

Hi Rysmom! I'm glad you stumbled upon us. We do tend to bicker over some finer details about HFI, but we're all generally on pretty much the same page in regards to what foods are generally safe/not safe.

As for the HFI spectrum, so far it appears there are two camps: those that think sweet foods taste bad and those that like the taste of sweets. Clearly the latter camp is more difficult to deal with. I have a theory that this spectrum might partially develop due to a child being a breast milk vs. formula (from birth). Then there are HFI children with added complications like lactose, wheat, and soy allergies that make finding food even that much more difficult.

As for diet, the best thing to do is start with what you know. Fructose is naturally occurring in fruits and vegetables. Add in sweeteners to that list (honey, molasses, high fructose corn syrup, sugar, etc.). That's pretty much the gist of it, but memorizing the list of sugars/bad stuff will make grocery shopping easier. You'll have to read everything every time you shop because one day the manufacturer will change the packaging ever so slightly and suddenly there's an added sweetener on your previously safe food. The other words you need to look out for are broth or natural flavoring--those two words are almost guaranteed to have onions, carrots, or sugar in them.

Keeping a food diary will help. I think maybe even clipping the ingredients and adding them to the diary (or making note of the ingredients) would be beneficial in the beginning. Note what he ate, how much, and how he was doing afterward (breath smell, lethargy, shaking, nausea, urine color/smell, how much water he drinks).

I've put together a couple resources for people with HFI:
List of sugars to avoid and how to read labels - fructose-free.tumblr.com/post/15777874026/which-sugars-to-avoid-with-hfi
Pinterest board of HFI Foods - www.pinterest.com/colormist/fructose-free-diet/ (please note my comments for recipe modifications/notes. Since your child is young, you're going to want to avoid anything that I mention has tiny amounts of sugars.)

Other things that are helpful:
Cronometer: allows you to see what vitamins are missing from a diet - www.cronometer.com/
SELF Nutrition Data: sometimes breaks down the sugars in food for you (in the carbohydrate section) and is pretty awesome resource for troubleshooting - nutritiondata.self.com/facts/dairy-and-egg-products/116/2

Lastly, I just had my gallbladder out (three weeks ago tomorrow). I've had crazy elevated liver levels leading up to the removal (no jaundice though, which seemed to be confusing every medical person I talked to). My parents weren't big on doctors or hospitals or regular checkups as a kid (due to a bad case of the poors), so I don't have much of a medical history. I didn't even know I had HFI until I was in my mid-20's and I was eating all kinds of food that I shouldn't have been eating up until then (including the occasional piece of chocolate, muffin, and bite of cookie). I had my gallbladder removed laparoscopically without any issue or concern, but they also didn't mention any buildup around my liver.

[rysmom14]

Thanks Tammy. I wonder if he will become more sensitive over time as he learns that certian foods make him feel sick. also, I think I may have been writing off some of the other things as "baby stuff" if he was cranky, or spit up. and its good to hear that the liver has such a good change of going down to normal again. I was surprised when the dr. told us it was 5x the normal size. Now i can notice that it does stick out on that side ( he is very lean) and I am paying more attention to the spit ups and anything else that could be signs.

Thank you Colormist! I'm glad to hear you are doing well from your surgery. Thank you for sending me those links, I printed out a copy of the " which sugars to avoid" list and keep in in my purse fo when i go shopping. I am hoping to use that to develop a list of food to try and then i can add then to a good safe list for him. I started a journal, so hopefully that will help. when you said to keep track of how he is after he eats, and how much water he drinks, should i also count milk? I find that he likes to drink alot of milk after he eats. he usually dosent drink with his meal, but he likes to make a game of it and carry it around for a while and drink it.

Another question. Since there are so many contradicting lists for safe foods, does anyone have a list of veggies that should be safe, and also the ones to experiment with? Maybe its just me, but I find it hard to only give him carbs and meat and milk. It just dosent make sense to not have a rounded diet and am looking to see which veggies to have on hand. and does fresh, frozen or canned make a difference if I make sure that no surar is listed on the ingredients. the non processed foods are hard to know whats ok to give. for instance, canned green beans ingredients are greenbeans, salt and water, but I dont really know if greanbeans have sugar in them or not.

Thanks for all of the help and for getting back to me so quick.

[charlie]

Hi rysmom,

email me [e-mail] and I will forward you the food list handed out to us Brits, it is fairly comprehensive.

As for rounded diet...... Forget the principle of balanced, nutritious, varied, tasty, colourful and go for sustenance, same safe old as much as you can, especially in the early days.
a very hard thing for us mums to get our head around is the food is boring for them, very limited, is it tasty, ahhh. they aren't having their supposed essential 5 a day..... I eventually realised and my daughter can't have starch either that they don't need it. She doesn't mind the same sorts of foods because she feels good on them, a couple of things on the plate are enough to keep her going because her body can metabolize them safely. Forget plate piled high, she used to get bloated and lethargic, a small plate of protein and a safe veg and she fuels up beautifully. It just took me a long time to accept it. when the dietitian analysed her diet she was getting everything she needed, ok a bit high protein and fat but not dangerous. the only thing deficient was Vit C and D.

[rysmom14]

Thanks Charlie! I will email you this morning

[Tammy]

Throw that "rounded diet" thought right out the window. It's probably one of the hardest things to do as a parent but it's what has to be done. I used to let Regina have 1 green vegetable a week. Now she probably doesn't even get one every month. She does better without any. Potatoes, and cucumbers are the only veges she eats without restrictions.

Regina lives a very healthy life on meat, pasta and dairy. She very seldom gets sick. Everyone around us this year was sick, and she never got any of it. So stick to what keeps HER healthiest and she stays healthy other wise also. I think she gets more colds and flu when she gets run down from trying to expand her diet.

[colormist]

This has been on my mind for a while now, so I finally pieced one together. I'm still not certain on the amount of space I gave the veggies. If anything it should be LESS space, but I really like mushrooms, spinach, and olives. Actually, I probably shouldn't have created this while I was hungry. I kept trying to add feta to the dairy section.

I also put it on my blog: fructose-free.tumblr.com/

[charlie]

That's a great way of showing it, well done. Only thing I would add somewhere is glucose maybe?

[colormist]

Charlie, I can't tell if you're joking or not. -_-;

Edit: if you're not joking, I could add it to the fats/seasoning side maybe?

[charlie]

Ha ha.... No, not joking..... not this time anyway, I think it is important to know it is possible as a sweetener.....

[colormist]

It is important, but as Fred always warns me "you don't want to get sued". Since it's a hot button topic and since I refer people wanting to learn more to come here, I think it might be okay. Maybe? Anybody else want to chime in?

[Tammy]

Or maybe add it to the very tippy top as a very small space. Or putting it in with the fats would also be a place.
Regina's "go-to" food is always potatoes.

[colormist]

Oh snap! I forgot potatoes! I'd eat those for every meal if I could.

Maybe I switch the bottom category from grains to starches and add potatoes there?

[Tammy]

That would be a good place for them.

[rysmom14]

Colormist i love the food chart! I printed it out for my fridge and I am thinking about also giving it to daycare and our families.

and speaking of daycare... my kids go to daycare 2-3 times a week and after Ryan was diagnosed, I spoke to them about the condition and started packing his food and told them he can only eat what is in the lunch box i send. I am learning that they are signs to look for to see if he is able to tolerate food or not. Is there a standard list of things to look for and when they would occur after eating them? I would like to put together some basic infomation for daycare aand also to have on hand for when we get around to trusting a babysitter

[colormist]

I would hesitate to give it to others. They might see the chart and say "it says he can have bread, so I gave him honey-glazed rolls. Honey isn't fructose." There are a lot of conditions to control for still with that food pyramid. Added sweeteners being a large portion of the problem. If it's food that's in your house, that's one thing, but food in another setting could be dangerous.

As for symptoms to look out for: shaking hands, lethargy, inattentiveness/glazed over look, drinking lots of water, very hungry/binge eating, nausea/vomiting/upset stomach, sweating/clamminess. Those are the symptoms that I can think of that happen in the beginning.

There's another symptom that I'm not sure if easy to tell. Last week I was having an attack, but I didn't know it was happening because I wasn't hungry. I was in the midst of making dinner. I became extremely dull witted. For example, the timer went off for the food and I just though "yes, that's the timer for the food the food is done" and did not remove the food from the oven. I burnt my meal horribly and continued burning it because I couldn't think coherently to realize I needed to remove the food from the burner and turn the burner off. I also ended up putting my hand on a burner I had just turned off and grabbing a pan out of the oven with my bare hand. Thankfully I didn't burn myself, but a minute or two earlier and I would have had a nasty burn. Little alerts were going off in my head, like "something is wrong! You need to pay attention!" but I couldn't think beyond that.

In the end, I think my blood sugar was just really low and I didn't know it. After I ate food, I was fine, but it scared me pretty bad that I almost caught the house on fire and burnt myself because I was using the stove while suffering from low blood sugar. It was like I was sedated.

I'm not sure if Ryan is at the age that you can tell he's acting off. The other symptoms are a bit easier to notice.