Controlling as many variables as possible is not easy...

[Stefanie (Ziba)]

Hi everyone,

First, I wanted to share the great news! Not only were Remy's labs excellent (perfect liver panel for the first time ever), but his ultrasound Friday showed "the size of the liver is now in the normal range" and "the lesions have shrunk".
We are beyond thrilled, but there are also so many looming questions. Our geneticist had said if the labs and sonogram look good, he wants us to try introducing low fructose foods to see what his threshold is....I said "so, low fructose veggies?". He is not a nutrition expert, though he is brilliant, so he asked me which veggies are lowest in fructose. I told him that kale, spinach (no stems and stalks of course), mushrooms and olives would be good ones to test. 
I am so nervous because I just managed to get some family members to understand how serious this condition is and already one of them said "great, he will be having ice cream in no time".

Interestingly, our doc said there has been research that individuals such as those with galactosemia have been found to have problems down the road if they are never exposed to any galactose because the pathways become "blocked" and some other important pathways don't get activated as a result. So, he said it may be important for Remy to get some limited/controlled exposure to small amounts of fructose as he grows...but, I am scared because he is only 4 and genetic testing has revealed nothing (he had Whole Exome which is the best available). I did apply for the Rare Genomes Project and we just shipped them our entire family's DNA for Whole Genome Sequencing. But, we still don't and may not ever have a clear answer.  We know over the past 4 years since the day he was born and rushed to the NICU, he has been diagnosed with severe IUGR, fructose intolerance, anemia, low carnitine (normal with supplementation), history of high liver enzymes, liver lesions, cholestasis, hypoglycemia, lactic acid elevation, hepatomegaly, high triglycerides, and his amino acid profile has been abnormal at times. In the NICU, his liver almost failed as his ammonia rose and his blood sugar dropped to 36, and I never want to go back to that time of terror. He is healthy and happy right now and while his diet is limited, he seems quite content with the ten or so meals I make for him and never feels deprived. I almost feel superstitious messing with such a good thing...

As I introduce foods, I will continue to take the copious notes as I have for the past 4 years (10 composition notebooks full of daily journal entires). But, there is so much fuzziness. For example, last night he had ( a lot) of unsweet black iced tea (home brewed), he ate some homemade meatballs with oregano and parsley (doesn't eat herbs often but loves them) and he had a tortilla (looks safe but has potassium sorbate in it which he has never had before, so I only let him have a little). Earlier in the week, he was stuck by the thorn on a rose (which I have been a tad paranoid about since rose thorns can contain a serious fungus). This morning he felt fine, we went swimming, he swallowed a bit of pool water. Later, he got very irritable and cried and puked. He felt fine right after and I checked his blood sugar which was 92. So, in my mind, I try to control all the variables but there are too many to control sometimes. Was it the pool water? Too much iced tea? The parsley? The oregano? The potassium sorbate? A fungus from the thorn? I used to be able to control more variables, but now he is more active, getting injured, exposed to germs, etc. so when we try a new food, it becomes even muddier trying to see if there is a direct effect. 

Anyway, I am thrilled with his health right now and I guess I am just scared of going in the wrong direction now that everything is so good. 

[rysmom14]

Hey Ziba! Awesome news about Remy's Labs and liver updates! That is very interesting about the pathways being blocked and the Docs reasoning behind adding low fructose foods. I would be like you as well, and not want to rock the boat! Does the Dr. have a timeframe of the food introduction? one way to limit the variables would be to try 1 new food at a time so it would be easier to pin point if he was having a reaction.

Keep up the good work Mama

[Stefanie (Ziba)]

Hi Rysmom,

Thanks for the encouragement!

Doc left the planning to me. I had told him I would only feel comfortable if I could introduce one food per week (like with an infant). I still feel like there are so many other variables out there though (for example, yesterday he was so tired and cranky. If he had kale the day before, I would have been thinking "aha, he must be irritable because of the kale".) I would hate to check his blood sugar every time he is cranky since he is an almost 4 year old boy after all...
Anyway, I would love to know if you (and Antonia and the other mamas since I don't want to compare him to an adult) think this is a good introduction order (one per week):

Kale
Spinach
Mushrooms
Olives
Lettuce (are all OK to try or just certain ones?)
Green Beans (is canned the only option?)
Sesame Seeds
Chick Peas
Black Beans
Avocado

I came up with this list on my own from my readings, so I welcome any input.

Thanks!

[jenn123]

I understand not wanting to rock the boat by introducing too many things. We move very slowly with anything new as we have a busy life and it throws all us off. Lately, We have had great success here with our version of spring rolls. It’s basically chicken, waterchestnut, rice noodles wrapped in spring roll rice wrapper. We dip in a Soy sauce / sesame oil.

I am curious, when was your last ultrasound from which he showed progress ? We are awaiting ours and hope to see some improvements.

Great job ! Congratulations.

[hfimomof3]

Glad to hear his labs looked so good. I have a kid who had multiple food allergies so I can relate to the situation of a mysterious vomiting incident that makes you frantically look back at everything that happened in the past few days to figure out if this is a red alert situation or just a general stomach virus or what. I have actually become more medically conservative over time. My kids' doctors agree that it's with good reason. Most of my mom friends became more relaxed over time but their kids don't have medical conditions, so I think it's just different for every kid.

In terms of the specific difficulty of interpreting symptoms and signs when your child is out and about and having more varied experiences -- a danger that others have flagged to me is that I might not allow my child to have normal child experiences because I am managing my own fear. I recognise this as a valid concern. My initial solution was to break this down into small steps and approach each step in a systematic way. That way, I'm only introducing one variable at a time. This might seem restrictive to others, but your child is only four years old. He has plenty of time to drink iced tea, go to the pool (catch the usual stomach viruses at the pool.....), go to the amusement park, etcetera. If taking only one of those new things at a time makes it easier for you to (eventually) incorporate them into his life on a regular basis, then in the end that works to his good.

I did a similar schedule as you are suggesting: one new food per week. That gives you plenty of time to try a tiny bit of the food, a little more the next day, maybe a full serving the day after that, or whatever method your doctor has approved. For my child, if I saw any worrying symptoms or he got sick, then I delayed the schedule until he got better, and didn't consider that food "OK" until I had retested it or done bloodwork in the allergist's office (not a part of your pathway, it sounds like, but that was my situation), etcetera. It might be a bit different for you because you are dealing with metabolism rather than allergy. If he got sick with a fever or anything, then I waited until he was completely better to continue introducing new foods.

In terms of the specific foods you are trying, all look fine to me except I'm not sure about the lettuce and green beans. Personally I find green beans a bit sweet. Iceberg lettuce I have heard is not really ok for us, but you can probably look that up to see if I'm correct. I eat small amounts of Romaine (the green part; I feel the whiter, least iron-rich parts of lettuce are just a waste of my fructose tolerance) but I don't particularly like it. I can't work out if that's because I taste sugar or if it's just not to my taste.

[Stefanie (Ziba)]

Hi Jenn,

His ultrasound was last Friday.

I would love to know which brands/where you purchase the rice paper and water chestnuts. I am on an autoimmune diet, and that actually sounds like something our whole family can eat :-)

So, we tried one piece of Kale the other night. He was not very interested in it (not shocking!). I will try again, but I am taking all of this verrrry slowly I think texture is going to be a big deterrent for him with a lot of these low-fructose foods. My poor husband made him mashed white potatoes and Remy took one lick and threw up. He won't eat eggs or anything mushy really.

HFImomof3, thanks for the advice. My son is starting pre-school 2 days a week in the Fall, so I think I am a bit nervous about that even though I know he will love it. I have gone over everything with the director and the teacher and he will only eat what I bring in (he will only be there in the mornings). I thought about homeschooling, but he is such a sociable, athletic and curious little guy, I don't want to hold him back because of y fears (as you stated), so I just have to take it one day and one step at a time.

[hfimomof3]

Yeah, kale is a tough flavor! How are you preparing it? It is probably easiest to eat boiled (since it's very tough when raw) but I find that brings out the sulfur flavor. What I do with mine is, remove stalks, cut into approx one inch fragments, then I massage it for about 5 min with lemon juice (which I can tolerate) and salt and then let it marinate overnight. That softens it enough to eat raw, though it still requires competent chewing. That might not work for a small child, since little kids don't always chew things properly. If you cut into smaller pieces that might help. Or you could try pureeing it, to get the bits small enough. If your child can't tolerate lemon juice (or lime juice, which is less sweet), then you can try vinegar. I assume what's happening is the acid and the massaging break up some of the bonds that make it so tough. The tartness also makes it a bit more palatable, I think. It also tastes much less sulfur-ish this way. Be aware that raw kale may make him a bit gassy. He might have to build up to it a bit.

I remember being a little kid with HFI (though we didn't know what it was at the time), and I'll tell you the bright spot was that the other kids were not put off by it at all. I still went to lots of birthday parties and everything. I just ate cheese curls instead of cake! I might have eaten more than my fair share of the cheese curls, but on the other hand it meant there was more cake for everyone else. Your son will have a great time in preschool.

[rysmom14]

Hey Ziba

I took a look at your list.

I think the kale and spinach are ok with no stocks, and only the dark leafy greens. I don't think iceberg is ok. Just romain maybe? White mushrooms are good. I know a lot of people eat olives, but Ry had a red butt from what I am going to call acid poop for lack of a better term on the few occasions he tried them. not sure if the reaction was HFI related, or not. Greenbeens, I believe in the can are the only ones. Ryan used to like the canned white beans and chick peas. Im not sure if black beans would have the same amount of fructose? Avacados make me nervous.

I would try the spinach and kale first like you did and then the mushrooms, then greenbeans, then the other beans, then olives. I don't think I would trial the avocado. But it is so good for you health wise that if the goal is to introduce small amounts, I would do just like ou are doing and try to pick the best nutional value first and avocado would be a good one for that. I don't know how it ranks in fructose content though.

Kale
Spinach
Mushrooms
Olives
Lettuce (are all OK to try or just certain ones?)
Green Beans (is canned the only option?)
Sesame Seeds
Chick Peas
Black Beans
Avocado

[jenn123]

I am not committed to any one brand of Spring Roll rice wraps. They all pretty much have just rice and water as ingredients. I pick them up at our local Safeway in the Asian section. Right now we have Banh Trang.
Same with the water chestnuts, not committed to any one brand. . Most brands have citric acid, water and chestnuts. I usually just let her have one chestnut per day. My child is about 70lbs.
From Amazon, i subscribe to Thai Kitchen and Annie Chun rice noodles. Thai Kitchen noodles are thicker than Annie Chun. We also subscribe to Ka-Me plain rice crackers. We get a case of noodles every 3-4 months and case of crackers every other month.
Snoop thru the Asian section at your grocery or go to an Asian market. There is a lot of things that could work. I also got some canned straw mushrooms that i wanted to give a try.
Trader Joe has some black kalamon olives that have no vinegar or red wine or garlic in them. We buy those by the case as well.
Her favorite treat right now is Barilla GF noodles. They have some corn so it is only a once a week thing.
She also likes Erewhon corn flakes for cereal. Once again, corn products make me nervous so keep the frequency about once a week.

[jenn123]

A few more things ...
For dessert, my child loves homemade ice cream. Two cups heavy whipping cream, one cup milk, mix in flavors and then put in ice cream maker. For flavors, we do a drop or two of peppermint oil by DoTerra, another flavor is warm the milk and make earl grey or green tea milk, then chill with heavy whipping cream before putting in machine. We are going to try rose tea for rose ice cream. (Just heat milk with tea in it, strain and chill)
We bought a case of unsweetened Koolaid packages a while back. The strawberry and cherry flavors looked safe. We make the koolaid with no sweetener and then freeze into popsicles, mix a hint of koolaid with seltzer water for flavored water and we were going to put some of the powder in the milk/heavy whipping cream mixture for strawberry or cherry ice cream.
I always buy items by the case so that i have enough if there is an emergency or if the manufacturer changes recipe.

[antonia]

I also think your doctor’s theory on keeping those fructose metabolizing pathways (outside of the liver) open is really interesting. My immediate thought though is how much and how often is fructose needed to keep them open? I would think as long as you’re eating any plant based food at all, including starches and grains, you are getting trace amounts of fructose and would that be enough to keep those pathways open? The only way to achieve a totally fructose free diet would be to eat only animal products (minus eggs).

Palmera doesn’t eat very many vegetables. Occasionally she eats leafy greens, green lettuces of varying types, spinach, mint and basil. I would put tea in the leafy category as well and when dried and then steeped into tea, I would be concerned about the concentration. I’ve only tried mint tea with Palmera and she doesn’t like it, although she likes fresh leaves torn and sprinkled onto the top of savory foods. The tea is too concentrated for her I guess.

Other than that occasionally she eats olives and rhubarb. The only seeds we’ve tried are celery seeds as a spice or flavor enhancer. I’m guessing the small seeds, like sesame and poppy, are probably tolerated because they are so small you don’t need much, dilution being the solution 😉. I’ve not been brave enough to try anything else. But then we don’t have the benefit of regular liver enzyme testing, so I rely on symptoms alone to determine her tolerance to new foods. For this reason I’ve been really conservative with her diet. She also eats eggs regularly, and knowing they have a little fructose I feel vegetables on top of the eggs may be too much. Also, I feel the nutrition value she gets from eggs outways what she would get from the small amounts of vegetables she could eat, so I guess I’ve chosen eggs over veggies.

If you’re going to use blood glucose levels to monitor his tolerance I would take fasting readings in the morning. Unless he’s having a severe reaction you’re not likely to get any meaningful readings during the day when food and activity levels will have glucose levels all over the place. Or if you’re able to take them while he’s sleeping he may not even wake up for them. When Palmera’s in deep sleep it takes a ton of bricks to wake her. Just take your readings at the same time whenever you do take them.

It’s time to start enjoying his new found health that you’ve worked so hard for! ❤️☺️ So I wouldn’t feel pressured to introduce new foods too quickly. Take it slowly and enjoy your time with him. I’m trying to do this with Palmera as well, and admittedly it’s very difficult to loosen the reins and let her get involved in activities where she’s exposed to germs and other health dangers out there in the big, wild world. But we’ve a couple years under our belt without a trip to the emergency room (for hypoglycemia), so it’s time. It’s impossible to keep their diet controlled enough or their environment sterile enough to make true, scientific conclusions. So I’ve really had no choice but to rely on that gut, mom feeling over any sound, scientific findings to live our lives from.

[Stefanie (Ziba)]

It is amazing that we spent the first two years of my son's life cleaning up acidic, watery, foul poops, and then once we got him off fructose, he became contipated with hemorrhoids...
When one looks at the HFI diet - dairy, meat, animal fats, some carbs....it is not hard to imagine a child not being able to meet the 11g daily fiber intake recommendation.
I added magnesium and vitamin C powders to our routine last year. He eats oats daily. But, none of that has been able to combat the iron in his supplements and the lack of fiber in the foods he can eat.
This weekend, he became so backed up, I had to give him a saline enema for the first time. He was so cooperative and felt relief afterward, but I knew then and there that I needed a better, more permanent solution. So, I started him on Benefiber yesterday and plan to continue it to stay ahead of things (I think he is also just prone to constipation being my son, poor kid).
As for the "veggie test", I tried kale, spinach and mushrooms and he is not a fan of any of them (only took one bite each time offered). Canned green beans were also not successful (though they have little nutritional value anyway, so no great loss). He ate some green lettuce leaves, but I don't think he will eat enough to get the fiber necessary to overcome the rest of his diet.
Chickpeas are next but I am a bit concerned that he actually will like them and that they may make him ill :-( Has anyone tried these with success? They do have fiber and I could make him hummus which would be amazing, but they do not appear on all the HFI-safe lists...

[colormist]

Chickpeas (Garbanzo beans) have 3.048 grams of sucrose per serving. It's quite a significant amount for a child considering sucrose is made of 1/2 Fructose and 1/2 Glucose.

Shredded Wheat big biscuits have 0.259 grams of sucrose per serving.
Steel Cut Oatmeal has 0.396 grams of sucrose per serving.

I would suggest trying either of these two options before chickpeas. I eat Shredded Wheat a few times a week to help keep things moving.

[lucky]

ziba,

We know this issue well.

2 options that I know of:

1./
Ask your son’s paediatric gastro specialist if PEG 3350 (Polyethylene Glycol 3350) is suitable, safe and if it will help.

It’s known under the names:
- MIRALAX
- RESTORA-LAX
- LAX-A-DAY

I won’t give daily amounts...

But, if it’s deemed suitable, your dr will start your son off with extremely little amounts of it -and then if he seems to tolerate it well- work up to a daily amount that regulates him.

The only thing I think is *extremely* important to remember is to make sure there is *NO chance of dehydration or electrolyte imbalance before or while using it*.

This is because altho our Gastro team deemed it safe when my son was suspected as HFI, it is still a chemical that acts to draw water into the colon to keep things moving. So, if not well hydrated or balanced, it could be dangerous. More info regarding safety measures can be provided by your son’s drs and/or packaging.

Otherwise, we were told if nothing is keeping things moving, something has to. In this case, for those that tolerate it well.. it passes through the body without affecting the liver or anything else.

We were told that it is used for months to stabilize the colon and give it time to go back to regular shape. Constipation stretches muscles, so to heal, the body needs time and softer stool for everything to strengthen and resume performing properly.

Years ago we tried it. My son didn’t seem to tolerate it well, but honestly, he was also at his sickest and weakest. Just this spring, we got the same info again. We may try it once more to just permanently regulate his digestion better.


* What I know about constipation is that the system has to be cleared out before adding more fibre. Without doing so, it just backs up that “traffic jam” even more.

Saying that, and after a digestive system is not backed up, an alternative to using the above PEG 3350 method is... Psyllium.


2./
PSYLLIUM flakes

Ukbill raves about it so I mentioned it to one of our specialists. We were told if it’s well tolerated it’s of course more natural than PEG 3350 products, but to slowly increase the amount over time to the amount that regulates him. Again make sure a person using it is well hydrated (read packaging info).

I won’t give amounts as parents should consult drs for recommendations, but we sprinkled it in foods we made or you can use, I guess, also as a thickener in creamy soups & sauces (roast/gravy).

It starts off as flakes but then with moisture it gels, so it’s easy. *Just keep track of how much is given daily.*

Suggestions:
- mix in scrambled eggs
- mix in eggs and make french toast
- mix in waffle or pancake batter
- mix in yogurt or yogurt drink
- mix in homemade pasta soup noodles
- mix in chicken noodle casserole
- mix in macaroni & cheese


Maybe try:
- sprinkle onto a pizza base with pesto(?) sauce and bake with cheese & pepperoni on top.

- sprinkled into grill cheese. cook.

- sprinkle into oatmeal or cream of wheat (with cinnamon or artificial vanilla)

- mixed into home made soup pasta noodles.

(I have 2 recipes for a “drop” type noodles for soup. If you like, I’ll send you their simple recipes. They take maybe 10 min total from mixing to cooking them in liquid. I usually put them into chicken/turkey bone soup (I add meat after to soup).

Oh... that reminds me:


3./
SOUP... BONE SOUP.

It’s a phenomenal way to increase fluid amount for a child and even better at soothing/healing a gut. It should also really help to keep your son’s digestion moving.

Bone soup is naturally infused with many vitamins and minerals and tastes great.

And, other than consuming it as a soup or “tea” type drink, it can be used for making thicker sauces or as a thin liquid for pasta/meat or.. cooking rice in it. For very young children, it’s also a good liquifier and taste enhancer for “first stage feeding” of minced meat (which is often pasty for children).

For those that are unaware, the wonderful taste we associate with soup broth actually comes from the bones, not the meat.

Go online and research it / recipes, or.. I can always send you info on how I make broth if you like.

*****

ziba,
If you check your messages later tonight, I’ll upload a copy of info given to us on constipation, tips, how to treat it and general info on how it can affect the bladder / urination and potentially lead to UTI infections. It’s for children 1yr and older.


DISCAIMER: (for all)
* But as always, ALWAYS follow up with a child’s specialist before attempting anything new. Any advice given here on this site (or other) should always just be taken as clues or suggestions to first explore with a health care specialist. I can’t emphasize this enough with little ones. Safety first.

[ukbill]

A quick warning (not warning but cannot think of the word to use tonight) about Psyllium Husks .. yes I rave about them and rightly so.. they changed my life for the better in so many ways!

However when 1st given.. they act as a cleanser.. a "detox" if you like.. anyway the 1st few times you take them you might get an unpleasant surprise.. i.e green slimy poo!

Do not be alarmed this only usually happens once!

Thereafter never again even if not taking any detox type compound for many years..

Well it happened to me and at least one other person I recommend them too and forgot to warn him about that initial side effect..

Keep smiling and your stocks of loo paper high.

[ukbill]

I have made a version of Lucky's Bone soup using a whole chicken, obviously for chicken soup, and stewing beef to make a beef "tea". A Slow cooker or similar method of cooking slowly and for many hours is ideal.

I have some Oxtail in the freezer to make some oxtail soup for myself when the weather turns colder.

[ukbill]

Ziba " This morning he felt fine, we went swimming, he swallowed a bit of pool water. Later, he got very irritable and cried and puked. He felt fine right after and I checked his blood sugar which was 92. So, in my mind, I try to control all the variables but there are too many to control sometimes. Was it the pool water? Too much iced tea? The parsley? The oregano? The potassium sorbate? A fungus from the thorn? I used to be able to control more variables, but now he is more active, getting injured, exposed to germs, etc. so when we try a new food, it becomes even muddier trying to see if there is a direct effect. "

Do not forget that Fructosemic children are just like normal kids the world over they get odd unexplained tummy bugs and viruses as much as any child.

At his age he is effectively a bug factory as his immune system gets trained up to know what is bad and what is not. So do not panic

He will be fine and grow into a big strapping lad long before you are ready for him too.

As always keep smiling

[Stefanie (Ziba)]

Thank you for the breakdown of sugars, Colormist. I think this is why I was having doubts about trying chickpeas right now (I must have remembered seeing some troubling stats once upon a time). We will hold off on the chickpeas. He eats oats daily. I remember a thread once about Shredded Wheat. There was some concern about cross- contamination (was that just the minis). Are you having success with this right now? Anyone with little ones able to have shredded wheat big biscuits?

I thought about trying oat milk, too? Rice milk is made with brown rice and it is constipating just like...well, rice.

Lucky, thank you for the advice. We drink bone broth regularly and you are right, it is wonderful for the gut. I read about psyllium and will try it if the benefiber does not work (I felt a bit more comfy with benefiber because there is a risk of choking with psyllium if the child doesn't drink plenty of water with the husks).

Many people have mentioned Miralax, but I am such a purist and do not use meds unless I have tried every other avenue. I just cannot get over the fact that the chemical in Miralax is used in anti-freeze. I know many people say it works well with no ill effects, but I am going to try dietary changes/supplements first.

The enema seemed to help as he has had a good two days :-)

I, on the other hand, am off to an ENT consult to see if they can fix my nose which was broken on Sunday by my sweet daughter while we were playing disc golf. I am pretty sure I am the first person to have her nose broken by a disc traveling 30 mph from 30 yards away!

[Stefanie (Ziba)]

Oh...quick word about corn starch. It is excellent for bedtime and before strenuous exercise, but it can also be very constipating if used too much. I have had to back off of it and only use at night for that reason.

[lucky]

Hi ziba,

Oh dear... take care.

If your son’s system is now cleaned out and Benifiber works longterm then that’s really great!!

But on the subject of psyllium flakes/powder:

If you mix it into wet foods, it swells up into a jelly type substance -so once swollen and mixed/cooked in- it should not have a higher choking risk than regular food. But yes, drinking it in liquid could potentially be an issue if not careful or if it’s not very well mixed within a liquid.

My son is a teenager and lately puts it into a yogurt drink, shakes it vigorously, and then drinks. He says he’s not had any clumping issue. Not even remotely. But then again, its well mixed and he drinks it immediately. However, this method is relatively recent, so I’ll ask him to be mindful.

Normally though we just usually cook it into food so by the time it’s eaten it has long swelled into the rest of the ingredients. Putting it into scrambled eggs or waffles are the easiest for us. Literally no one can tell it’s in there. My husband (who does not have a fructose issue) also puts it into smoothies or yougurt. He raves how convenient & effective psyllium is as 1 tsp., I believe, equals 4-6 grams of fibre.

Thank you for mentioning Benifiber.

It looks like it’s consumed in a drink format much like Metamucil (which we have not used -but also contains psyllium powder). We seem to be having success with using psyllium for now, so we’ll continue, but knowing of alternatives to PEG 3350 containing products is always appreciated.


* Other issues contributing to constipation other than a lack of fibre:

- Thyroid
- Dehydration / electrolyte imbalance