New to HFI. Is there any new research?

[sherry]

Hi, I'm in US, and glad to find this forum. Is there any new research on HFI? The few sites, areas I have found are confusing/contradictory etc.

I'm Adopted and recently did the "medical" portion of the 23andme.com genetic testing, which became legal 4/17 to provide genetic results directly to the client vs having to going to a doctor to read it to you. Their labs are considered reputable for Genome testing for markers etc.

I have a genetic mutation of Chromosome 9, which results in HDI.

I was born a premie, sick all my life with all the noted symptoms. This makes total sense as I've never been able to eat most forms of sugar, (IBS), Severe Anxiety, Depression.

Doing elimination of foods in diet now.

Are there different levels (gms) of fructose, sucrose & sorbitol that each person can tolerate? Is this an elimination of foods in diet & try to put a little back in lifestyle? I'm not seeing standard guidelines.

I just happen to be going to GP doctor this week to review annual blood work. I don't feel my doctor will have any idea what HFI is, as it appears to be specialized. I will be interested to see liver activity as it's been on the cusp before.

Any pointers, advice would be appreciated on learning about HFI.

Thanks!

[rysmom14]

Hi Sherry!

Welcome to the board. I have heard about the 23 and me tests being helpful with diagnosing HFI. There is not much research out there on HFI since it is such a race condition, and you are correct in saying that a lot of the information is contradictory. To answer your question about the different levels or degrees of HFI and the amount of sugar each person can tolerate, it really is an unknown because there is no real way to tell how much of the adalose B enzyme each person produces. The enzyme is what non HFIers have to metabolize the sugars, and so HFIers may have varying degrees and amounts but it would still bevery very small amounts. ingesting sucrose, fructose and sorbitol with HFI causes damage because the enzyme is missing to breakdown the sugars and I think the kidneys end up filtering it out, but this is a longer process and since its not the correct pathway and isn’t the ideal situation for the body, it can cause damage. So because there isn’t a way to really tell how much an HFIer can tolerate, you should aim to have none ( or as little as possible)

Doing the elimination diet is great. I would cut back to a very basic diet that is considered HFI safe, and let your body heal and then add 1 food at a time that is also safe. There are not any standards out there, but I have found the advice I get on this board has been the most helpful. I don’t personally have HFI, but my3 year old son does.

You said you are going to the Dr. this week. Was the blood work you had drawn before the liver metabolic? This is a good one to monitor how your liver is doing and how well your diet is. My son also goes for a yearly elastography liver ultrasound. He has cirrhosis and this is just another test to look over many years to make sure that his livers condition isn’t getting any worse.

As far as pointers on learning about HFI. I have found that the help of this board has been the best. Ask whatever questions you have and someone will try to help you, or offer suggestions. The best advice I was given was to throw out everything you have learned about what eating healthy is because “healthy” for a person with HFI is completely different. There isn’t a ton of variety, but being creative can help in transforming the same ingredients into something new. Also, it is much easier to cook yourself rather than trying to find pre-made convenience foods. Most of those items are unsafe.   check through some of the posts because there have been many sample diets given.  and depending on where you live someone may even be able to help with the brand names of the foods to purchase at the grocery store.

There are a couple of documents that I have found very helpful.  the HFI food pyramid is a good visual, and the list of sugars has been a life safer when trying to shop. Those 2 were given to me by someone on this board.  The HFI modified handout is something that my sons dietician gave to me and I changed it a little to be more correct.

Hope this helps!

Rysmom14
Food Pyramid.docx (714.6 KB)Which Sugars to Avoid with HFI.docx (21.35 KB)Food Pyramid.docx (714.6 KB)HFI Modified Handout.docx (22.53 KB)

[jenn123]

This has been super helpful to me in looking up various sugars contents in foods
csidcares.org/treatment/food-composition-database/

[colormist]

Seconding what Rysmom just said! Zero fructose, sucrose and sorbitol is the safe amount of sugar. From what I understand (watching many youtube videos by liver-specialists/educators), HFIers are missing the enzyme that breaks down fructose. So any amount of fructose an HFIer ingests will cause damage. The "tolerating" is how many milligrams of fructose we can consume before we start to feel the side effects.

For example, I might eat 1/8th a portion of unsweetened black beans. I do not feel sick immediately from eating those black beans, but the next day I am hypoglycemic and my urine is very dark (because my kidneys are flushing the fructose out of my system). I say I tolerate this amount of black beans, because I value their taste enough to put up with the consequences. But, truly, my liver is not tolerating what I'm doing to it because I still get sick.

Honestly, as soon as you go on the elimination diet, (if you do have HFI) you will find that you feel best when you eat 0 fructose, 0 sucrose, and 0 sorbitol. The more you remove it out of your diet, the better you'll feel and the less you tolerate.

If you try to add things back into your diet, you'll get VERY ill. I equate it to salmonella poisoning. Like, how much salmonella will you tolerate on your food before you get sick? Most people would answer that question, "none. Zero salmonella. I do not want any salmonella in my food, thanks. Could you please bring me some food that has no salmonella in it and hasn't been prepared on the same surfaces as your salmonella-laced foods?" And, in truth, the early stages of HFI fructose poisoning feels much like salmonella poisoning.

Fructose Malabsorption, however, is a different condition with a similar diet. Their one benefit is that they can process small amounts of fructose normally and once they start to feel better, they can add back in some foods to their diet. Their condition is not life threatening like HFI, though, and they are not missing an enzyme in their liver. If you find you have Fructose Malabsorption and test negative for HFI, then you might be able to add back in some foods.