Newly dx and still waiting to hear from Dr's

[Worried Mama]

Hello, 

I've had a horrible time finding an active support group for HFI. Two weeks ago my 11 year old had some genetic testing come back for HFI. Dr Tolson doesn't believe we need a liver biopsy to confirm, but I'm not so sure. Its terribly hard to manage this, he also is allergic to (anaphylaxis) milk, eggs, and peanuts. He lost 10 lbs over the past 3 months. A year ago he was in the hospital all the time vomiting and in ketosis, with metabolic labs all over the place while on a formula because he was a failure to thrive on his own. Now he is off the formula, was gaining well and then stopped again and back in ketosis losing weight. Our Dr's haven't even told us how to handle this since we found out 2 weeks ago, is it ok that he is not being "treated" right now until our Geneticist decides what to do? He looks sick. He also has motility issues, do you have that?

THanks in advance!

[colormist]

I consider treatment to be "eating a fructose- and sucrose-free diet", so if he isn't on the diet, then that is not okay. He could end up in a coma if he continues to eat foods that are unsafe.

Motility issues are not a part of HFI, thought a person with HFI eating unsafe foods can be lethargic, dull, and shaky (from the hypoglycemia).

[Worried Mama]

I'm definetely doing my best through research online to take all fructose out of his diet. Sometimes they wont list sugar or fructose/sucrose or sorbitol in the ingredients but it will still say 1 gm sugar in the nutrients section. Sometimes it says brown rice syrup and yet states 0 gms sugar in the nutrient section. Its all so confusing. One page will say certain veggies are safe and another will say they are not. Some say stay away from high fat foods others say to eat them. Is corn flour/popcorn ok? Are almonds ok?

thanks in advance!

[colormist]

Nuts (of all varieties) are not safe. They all have fructose of varying degrees in them--same goes for peanuts.

Corn is a complicated ingredient:
Corn is NOT safe.
High fructose Corn Syrup is NOT safe.
Corn Syrup might or might NOT be safe.
Corn meal is safe.
Popcorn might or might NOT be safe.

If you can avoid corn and other vegetables right now until he starts feeling better, that's your safest avenue. When he's feeling better (in a few weeks--it really takes that long), he'll be a better judge of what's making him feel ill when he eats certain foods.

For HFI, fatty foods are safe. I have never read anywhere that says fatty foods are not safe for HFIers.

And yeah, ingredients are impossible. Lactose, Glucose, and Dextrose (in their pure, uncontaminated forms) are all safe for people with HFI, but they will show up on a nutrition label as adding to the "sugars".

The word Syrup is tricky. Sometimes it's just a thickening agent (and sugar free) and other times it's just like the syrup people put on pancakes. I tend to avoid things that have any form of syrup listed on the label.

It's worth noting that HFIers cannot process any fructose or sucrose. Even amounts of 400mg (which would not show up on the nutrient section because it's below .5 grams) can have an adverse effect on an HFIer's health. It's really a good idea to ignore that sugar section on the nutrient label and stick to deciphering the ingredients.

You're also going to want to look out for the always tricky ingredients: natural flavor, broth, or vegetable broth. Those will almost always have some vegetable in them that is not safe (carrots, onions, etc).

[Worried Mama]

Yikes this is getting more and more complicated. Before I ask a few more questions. I appreciate your answers by the way! My son has not had a liver bx. He has the diagnosis as opinion from Dr Tolan in Boston given his variant mutations, one has never been tested before. Would you bx based on this information? I know its invasive but this diet is so hard and he is allergic to milk & eggs. Would you want to be 100% sure with a bx?

That said, I didn't realize no onions, no garlic, this is getting more and more difficult. I'm making him french bread. Is that ok to eat in large portions? Its just flour, salt, yeast and water. He is also eating sugar free no celery bacon, sugar free hotdogs (they do have celery)...we haven't stopped the ritz crackers or cheerios yet and plan to tomorrow when we are home from work with him.

Thanks again!

[colormist]

Not ritz crackers or cheerios! I think you emailed me on my blog on tumblr! Purley O's by Cascadian Farms is a sugar-free version of Cheerios (actually made by General Mills and they taste near identical). Saltines are okay (check the ingredients--always, some varieties have added sweeteners) and a safe alternative to Ritz crackers.

French bread is okay--particularly if you make it at home. If you buy it in stores, check the ingredients.

Celery does have fructose. Hot Dogs MIGHT be okay. Hebrew National Hot Dogs are typically safe for people with HFI. If you give him bacon, make sure it isn't cured with sugar, molasses or honey. Usually the low-sodium bacon is sugar-free (for some odd reason).

If Doctor Tolan suspects he has HFI, then I'd go with what Doctor Tolan says--particularly if you notice an improvement of his health while he's on the diet. Doubly so if your son begins to notice not feeling well when eating foods he previously ate (like Ritz crackers) after being on the diet for some weeks.

If you can somehow get the diagnosis on his medical record WITHOUT the biopsy (and his health improves while on the diet), then I don't think you would need the biopsy (unless other issues arrive and you'd want to rule out HFI as a diagnosis). I am not a doctor, though. If a doctor tells you otherwise, then I'd follow what the doctor says.

I'm not sure if I've pointed this out to you already, but I made a pinterest board with safe foods:

Please be sure to read my notes about sensitivity, ingredients, and what to look for when buying food.

Also, please check back with us and let us know how he's doing!

[rysmom14]

Hey Worried Mama!

Welcome to the board! This is a great place for questions and support as we all trudge along through having HFI or having family members with HFI. Colormist has given you so much good information. I wanted to let you know that one of the members, heatherarp, has a son with HFI and he can also not have milk. She may have some good food ideas for you. I know when her son was first diagnosed he just ate chicken, broth( just chicken and water, and I think white rice. I am hoping she will comment on here too. maybe she can give you some specifics.

I also just wanted to comment on the liver biopsy comment you made. My son ( now 2 year old) had a liver biopsy because they couldn’t figure out what was wrong with him, and it showed nothing. Now at the time, I don’t think HFI was a thought. The Dr's were thinking a metabolic disorder, but i have never heard them talk about HFI. the biopsy showed nothing and in the end was no help in his diagnosis. Could this be because they didn’t have a specific disorder they were trying to confirm? Maybe. The only way we were able to find out what was wrong was after the genetic test came back. It showed HFI and since then there have been no talks of doing a liver biopsy. He has been diagnosed for over 9 months. His liver Dr and genetic Dr say that as long has he follows his diet and is growing ect. then there would be no need for him to have a repeat biopsy. As an invasive procedure I would think they would want to avoid it. We had no trouble getting HFI listed on his medical record.


a side note, when my son was in the hospital, the case management staff applied for medical assistance for him and this secondary health insurance covers all costs not covered by my regular insurance. so any meds or Dr visits, and even hospital stays are all covered. I know the top priority right now is getting your son feeling well and getting a handle on the diet, but that is just a thought to keep in mind.

As far as food, here is a list of some of my go to foods. I’m not sure what area you are from, so some of this may not be available… also just pretend you don’t see the items with milk in it or that don’t work with your sons other allergies.
Menu:
milk
Cheese ( we eat alot of cheese!)
plain yogurt (Danon brand)
Eggs
Spinach
Mushrooms
Greenbeans
white beans
Gluten free chicken nuggets ( I have only found 1 brand that is safe, from Aldi- glutenfree chicken nuggets)
organic hot cereal with cinnamon
Pasta with butter
Turkey Lunchmeat (Applewood Farms Brand)
Sausage ( not sure of the brand, but its made with dextrose)
Ground turkey or chicken ( mostly from whole food store so no brines)
Purely O’s ( good substitute for Cheerios. Cascadian Farms- organic brand)
Cheeze it’s
Oyster crackers/ Saltine crackers
Cheese puffs ( pirates booty brand)
Cheddar Pierogies (Mrs. T’s brand)
Pancakes ( Walmart generic mix)
Kraft Mac and Cheese
Store bought crumpets ( sold by the milk and eggs)

It has been hard to change my thinking about his diet since you want to feed your kids a healthy, rounded diet with variety, but he is happy eating the same foods and has truly thrived right before my eyes. Keep up the good work, and I will help any way I can.

I don’t have as much experience as most of the other members since my son was only recently diagnosed, but I will try to help any way I can.

[Worried Mama]

I feel like the biopsy is more for my own peace of mind. You see my son's genetic test came back with two varients. One is a well known and the most common A150P. The other variant is E207Q and is a variant that has not yet been tested but according to Dr Tolan plays a critical part in the role if intact so a mutation he feels combined with the other mutation we are most likely looking at HFI and he doesn't see a need to biopsy, but said to consult his physician to run liver test. Its just so hard to make this change with a second variant that is untested.