Health problems HFI Question

[chrisnyc]

Hi!
So as I mentioned in another post, my sister heather and I have HFI- vomiting from birth at anything sweet, only can eat pixi stix and fun dip, birthday parties where everyone's eating ice cream and I'm eating plain yoghurt. I'll get the genetic test at some point point but I don't need it to know.

I'm writing because even though heather (my sister) and I have not eaten much of anything "sweet" to the taste our whole lives, we are still having A LOT of health problems.
I eat the following: cream of wheat, grits, oatmeal, fresh organic red meats, fish, chicken, lettuce, greens, whole grains, "white" grains. Some dairy (I get VERY mucousy from dairy).
Until I was about 24 (I'm 36 now), my health was excellent. However, between 24 and 36 things have started to fall apart. I've got everything from arthritis, tendonitis, to prostate infections, to unexplained pains in my hip joint, testicular pain, low thyroid, endocrine imbalances etc.

I did not actually put together any of these problems with HFI until a couple of weeks ago. I have known I had hfi for 3 years, but figured that since I ate "nothing sweet" my whole life, that I was "treating" it correctly.
Still, my health problems seem to accelerate. MD doctors have no answer except generic catch-alls like IBS (code for, we don't know what the problem is) and symptom chasing. Alternative doctors provide some relief, but never really solve things. About 2 years ago I figured out that all of my symptoms can be attached to acidosis. It was only about 10 days ago that I realized that acidosis occurs as a result of HFI. Is everyone aware of this? In my opinion this is a major issue. When HFI people eat fructose it is transformed into *fructose 1-phosphate*, this is normal. Fructose 1-phosphate is normally broken down by aldolase B but since we don't have aldolase B, the fructose 1-phosphate accumulates. This accumulation does at least 2 important things
1) It stops your body from forming glucose properly (I usually feel hungry even when I've eaten), anyone else feeling ADD?? Sounds like continuous low grade hypoglycemia to me.
2) causes lactic acid buildup (acidosis)
Acidosis causes all kinds of problems ie: inflammation, kidney stones, thyroid and endocrine imbalance etc..
Chances are unless you are really hitting the sugar the acidosis will be mild to moderate. I monitor mine with a urinary ph paper. Over the last five years I have monitored MANY peoples pee (yes, I'm nuts) trying to figure out what normal is. Basically, assuming you are in overall good health rising morning urine ph should be around 6.5. For years mine was 4.5 which is 100 times more acidic than 6.5. Now it's around 5.5.

MY QUESTION! (sorry it took so long to get here

So, if we're not supposed to eat anything with fructose what do you guys eat? Almost every healthy food has fructose in it. I have been eating things like brown rice, green leaf lettuce, broccoli rabe, whole wheat, pasta, asparagas etc. I don't eat a lot of dairy because I'm allergic (major mucus). Even potatoes have fructose depending on variety and how theyre stored. I've been eating only foods that "aren't sweet to the taste" my whole life in order to avoid puking, but it would appear that even the rather small amounts of sugar that i'm getting from my diet are too much?
HFI ers, what do you eat?
All I can think of for absolute minimum levels of sugar would be white rice, cream of wheat, commercially prepared white corn grits (no germ/bran), eggs (i'm allergic), dairy (I'm allergic). WHere's the roughage! How do you poop!?? Where's the nutrients!!?? Where's the minerals!?? I really don't think you can just supplement all that stuff. How are you all? Are you healthy? what's working for you? Does any of this make sense or sound familiar?

Any help greatly appreciated and wow, I'm so happy to say hello to you all.
Chris

[CJSculpts]

Chris,

I have a similar history in terms of time-frame of crisis, etc.

First thing, you REALLY need to revisit your diet!!
Here are some examples:
Brown Rice - NO!!
Whole wheat - NO!!
Processed wheat, broccoli, asparagus, artichokes, etc. - these are only tolerated by some of us.
Lettuces - some varieties of spinach are okay but iceberg and watercress are out. I am still trying to get a handle on all of the other varieties.
Onion, garlic, things with inulin added, etc. - these are only tolerated by some of us.
Legumes, etc. - these are only tolerated by some of us.
Honey, sugar and most sweeteners - NO!! You might be eating foods (breads, crackers, cereals, sauces, etc.) with these that are not necessarily "sweet".
Vinegar - some are in, some are out.
Meats - be careful about how they are handled & processed. Even if they are organic, meats & poultry that are cured, marinated or brined with sugars are problematic. If you have a problem with garlic or onions, the marinades that might contain these would be a problem.

Do you have copies of recommended diets or are you trying to wing it? Even with the help of posted diets, I have found that this is VERY difficult to navigate. The diets all have conflicting information - partially because some of these things are subjective. They also only cover a very limited window of available food products especially if you live in a place like NYC or SF and have access to an amazing variety of produce & other ingredients. I will try to post links to some online diets later. I am also working on a spreadsheet that combines all of these diets but it will take me months to finalize.

Some added SAFE food ideas for carbs and roughage: risotto, choice of polenta, corn, quinoa or rice pasta, pearled barley, quinoa, kasha, french fries, baked potatoes with olive oil, fleur de sel & cracked pepper, celery, popcorn, corn nuts, nuts/nut butters (you have to see which work for you - for me all are okay except for possible pecans), seeds...

Ironically, before hopping on to this forum just now, I updated my FB status to: "UGH! Quick grits for breakfast. : / Gone from eating yummy fruits & veg to popcorn, french fries, baked potatoes & potato chips & now this!?!? Whichever evil presence has occupied my body is apparently trying to turn me into an Amurican! : P" hehehe

In terms of being able to poop, you would be mighty surprised. To put it simply, fructose caused a water imbalance issue for us. I gather that for most HFIers, diarrhea (ewww!) is the issue, for me it was constipation (ewwww too!). (I think that due to some germaphobic harassment from my father throughout my childhood, that my body regected diarrhea after awhile.) For decades, even though I had a traditionally healthy I was lucky to poop more that once a week and even then it was always an ordeal. Within a week after starting a very strict HFI diet, I went into the bathroom & shut the door. When I reemerged 30 seconds later, my fiance was standing in the hallway with his jaw dropped and eyes bugged out. He told me that he could not believe his ears!! If I stick to the diet, I poop like a normal person.

I was cracking up about your pee experiments. I realized a few years ago that I was always talking about poop even at dinner parties. (I have some weird friends.)
While I am familiar with the acidosis concept, I am curious about the PH testing & would love to learn more. I am also going to purchase a glucose monitor next week to see if, how, and why my blood sugar would vary.

While I am highly intelligent, have a memory like a steel trap and am highly motivated, I am also a total space cadet. My ADD has ADD. As my illness has gotten worse. I have had moments of forgetting how to make coffee, forgetting why I was walking towards the stereo - duh!, having trouble accessing common words like stapler (paper holder together thingy) and post office (mail station), etc.

UGH!UGH!UGH!

Because you and I have not yet been tested, It is possible that we only have Fructose Malabsorption. BTW, I will be sending my blood to Dr. Tolan's lab for testing ASAP. He hold samples until he has a large enough batch to process. He just ran a batch so it might take up to three months before he runs another unless he reaches critical mass sooner. So, give yours and your sister's to the cause now!

[chrisnyc]

hmm, lots of great info here CJ, but how am I supposed to tell what's tolerated? At this point almost everything upsets my stomach so that's not an indicator. I mean, I almost never puke at this point because I'd stop way before, and so I think the problem for me is one of subtle poisoning. I eat something and I don't feel any sicker than usual...but it may be doing me harm. Foods don't correctly list what's in them (i can show you plenty of brown rice that says zero sugar). I eat almost nothing processed but I will keep an eagle eye out if I do I have also gone long periods without eating any brown rice and little improvement. Additionally quinoa tastes as sweet to me as other sweet grains and on nutrtion data it says unknown. Pearled barley has some sugar as does polenta (cornmeal) but the data is all screwy listing whole grain as having less sugar than degermed. Me confused!
I am thrilled about your constipation story. WOuld LOVE for that to get fixed
With regard to diagnosis, did you throw up since weening from anything sweet and only eat pixi stix your whole life?
I will send to tolan unless I can get my insurance to pay for it from a geneticist.
Thank you!!!

[CJSculpts]

Hey Chris,

It's not quite as straight forward as just "no sugar". A lot of variables come in to play. Some of it is related to fructose to glucose ratios and other things like that.
Take a look at some of the following links if you have not already. Please be aware that some of them are related to FM and not HFI so, they may be slightly more relaxed.

Also, you will continue to feel that low-grade, generalized crappiness unless you "cleanse" by going on a really strict diet for AT LEAST two weeks. You will have the shock of your life when, all of a sudden, you stop feeling like crap. Unfortunately, the next time that you eat something "bad" there will be no question in your mind. I was feeling great until I had a salad on the 27th with either the wrong kind of spinach or the wrong kind of champagne vinegar (I had been eating variations of both almost daily). I knew that something was wrong before we got home. I then had several days of severe headaches, shaking, abdominal pain, queasiness, etc. I could not drive safely for a few days I was so ill and just plain out of it. After years of vomiting violently from migraines (hmmm...or was it fructose poisoning??) I seem to have lost my ability to throw up. I might spontaneously vomit a teeney weeney amount but, to really throw up, I have to stick a finger down my throat. I am sure that I will eventually get over it but, for now, I can't get past the mental block against forcing myself to throw up food even if it is poisoning me. It is now two weeks later and today is the first day that I am almost headache free.

Your insurance company might cover a nutritionist which could be very helpful especially in making sure that you are getting a balanced diet. Once you get a better understanding of the diet, and have cleared our system, you can start in on a full elimination diet and add in "suspect" foods one at a time. If you cannot tolerate them, you will know. If you can then, yay! You will need to wait until you recover from a reaction before trying another food.

www.bu.edu/aldolase/HFI/treatment/sugar_table.htm

www.bu.edu/aldolase/HFI/treatment/index.html

www.healthhype.com/nutrition-guide-for-fructose-malabsorption.html

www.healthhype.com/low-fructose-diet-in-fructose-malabsorption.html

www.healthhype.com/fructose-malabsorption-cause-symptoms-and-diagnosis.html

www.healthhype.com/rare-genetic-disorders-hereditary-fructose-intolerance.html

www.foodintol.com/sugar.asp

This book can be very helpful especially in trying to get your loved-ones to understand what you are experiencing. It is about FM not HFI but it does explain some of the concepts and the symptoms in layman's terms. :
www.amazon.com/Fructose-Malabsorption-Survival-Guide-1/dp/0984077707/ref=sr_1_1?ie=UTF8&s=books&qid=1263116652&sr=1-1

Most of my more severe symptoms came in my late teens/early twenties. Severe life disruptions since then. Crisis mode hit this year - I'm the same age as you, minus 36 days - something in the water? Anything earlier on was either dismissed as being an impossibility (reactions to iceberg lettuce, processed turkey, whole wheat, etc.) or was just completely ignored. I suffered from a classic case of "the cobbler's kid has no shoes." My father was an orthopaedic surgeon and so we were not allowed to be sick. If you could not see it in an x-ray, it did not exist. Suck it up and go to school. The only medication that I was allowed to take with any regularity was Drixoral. I think that this was because he was prone to colds more than any other illness. Even then, he concerned it a form of weakness because it was only to treat the symptoms. blahblahblah.

If you still have questions after poking through all of this let me know. ;D

[chrisnyc]

OI! that stuff with your Dad, that is rough. My mom was/is a health fanatic and was always trying to get us to eat fruit. She would put oranges in out Christmas stocking! Ha too funny an weird. When we were babies she took us to the hospital and the doctor simply said "well what happens when you don't give them anything sweet?" and my mom said, "they're fine" so the doctor said, "well just do that". So that's what my mom did but there was always an urging to *try* to eat those "healthy" fruits. many vegetables were not negotiable. mom decided that things like zuchini were "not sweet" and so we had to eat them. kinda hilarious thinking back- my mom to this day loves the line "that's not sweet". weird control issue I guess. I think this whole thing really messes with the "health food" mindset. they want to love us until they realize we can't eat fruit...or most veg...and yet we can go apesh*t on the fun dip. I think it really drives people crazy because it's so outside the box.

[colormist]

As I've recently noticed, it takes a very long time to work this sugar-poison out of one's system.

Be sure to check your poultry--I've noticed that many brands now "plump" their poultry with "natural flavorings" in order to make the poultry look fatter and to charge the consumer more per pound while providing less meat. The natural flavorings usually have some kind of fructose in them. I'm still trying to find a local butcher that doesn't plump their meats.

You might want to consider cutting out all forms of veggies--green or not--to see if that helps first. If you find this helps, you can slowly start adding them back in (one at a time a week--if you can manage it).

I don't have any issues with my digestion--I guess I'm lucky. Then again, I eat a large bowl of shredded wheat every morning. I don't seem to have a reaction to it--or, at least, not that I can tell.

Do you use a milk-substitute regularly? I had a really bad reaction to a soy creamer and a soy coffee. It didn't taste sweet at all, but my body thought otherwise.

I take a daily supplement. I don't think it includes other missing ingredients, so I'm thinking about shopping around for other supplements.

I was doing fabulously until the holidays, then I made a grave error and have been trying to recover since then. It's very slow progress. I would say I'm healthy--about 2lbs overweight, but healthy. One part of me wants to eat things I enjoy as I really don't like eating and the other part of me wants to eat healthier (I'm still trying to definite what that is to a HFIer) to lose a few pounds an not have to buy larger jeans. Being ill from fructose seriously prevents me from exercising. I have to feel at least 80% awesome in order to be motivated to exercise. Either that or I risk hypoglycemia.

[CJSculpts]

Hey Colormist,
You crack me up! I don't even know how one measures "2lbs overweight." I know your photo is from your wedding but you look like the Irish Spring Girl or some other icon of beauty & wholesomeness! ;D

I am dying to figure out the soy thing. I switched to only soy milk once my GI problems started and I think that it might have made me much much worse. I have since learned that, besides being potentially bad HFI-wise, soy is pretty toxic in large quantities period. Yes, it is healthy in moderation but apparently, the whole soy is perfect thing is another one of those large agri-business myths. Unfortunately, my palette is destroyed for anything else & I do miss edamame : (

You are absolutely correct about the poultry thing. This is why I amazed that so many people on this board can eat chicken nuggets & fingers. I am extra extra cautious about meats & poultry at restaurants. Most (including my hunny's) do at the very least brine a bird before roasting to help keep it moist. We had two turkeys for Thanksgiving. Both fried but one was not brined for me. It was extremely dry. : (

One of the issues with exercise & HFI, in addition to the who wants to exersize when they feel like crap thing is that exercise speeds up digestion. This means that the "bad" goes through us faster and then does it damage all the more quickly.

Chris, it's funny because my father was anything but a health nut. He hid his food tendencies for the most part when we were children (except for the Cookie Monster cookie jar on his office desk) but in high-school I noticed that he had cases of Twix and other candy bars in his home office. He also always ate TONS of pretzels. (I think that this was a Philly remnant.) One of our closest family friends is a dentist & his wife was a semi-hippie health nut. My friend, their daughter, was never allowed candy, etc. We got used to Trident gum, trail mix & Hansen's soda instead of candy & cola. It's funny, I still can't stand the taste of regular canned Coke w/HFCS but the Mexican coke that has cane sugar is okay. I wasn't totally restricted but didn't have much exposure to candy. My bro & I would get an occasional allowance & ride our bikes to the candy store. Top choices were always Pixie Stix, Fun Dip, Sweet Tarts (OMG the giant chewy ones especially - unfortunately I have checked and they are not HFI safe) and candy buttons.

Dad was truly a complicated guy. He refused to eat fruits and veg except for canned peaches and apple sauce or pineapple & watermelon when in season. Watching a surgeon de-seed a watermelon is pretty hilarious! On ethe other hand, My most favorite breakfast ever came about due to a raging fight between my parents. I still have no idea how it started but Dad started yelling at Mom that the cereal, etc. that she was feeding us was no more healthy than chocolate cake. (Funny thing is that we rarely had "sugar" cereals. At worst, we had Frosted Flakes or Apple Jacks.) I remember him listing the the ingredients - milk, eggs, flour, sugar. The result was that he made me breakfast for the first time ever - waffles & coffee ice cream! : d Yummy! Of course, this was followed up on another day by actual chocolate cake for breakfast. tHe turned out to be an amazing cook later in life.

[chrisnyc]

colormist, thanks for scoop on the chicken but would love to hear what you eat if you don't mind, start to finish on an average day- some favorite foods etc. that would be really helpful if it's not too much to ask.

CJ, cool story about yiur dad, reminds me of the bill Cosby chocolate cake bit. very funny. I do want to say that although I'm no doctor, are you sure your syptoms match HFI? Because it's a genetic mutation, an hfi-er will have never had the ability to digest a piece of chocolate cake, or a single coke. to do so would lead to a violent throwup festival. my sister and I got to test this theory on every sweet food that crossed our paths for the first 15 years of our lives and we've literally thrown up ON doubters many times (school nurse/camp counselor disbeliver types). I sure don't want to lead you astray and I would definitely get the HFI test to be sure or course but, colormist? Fred? have u ever been able to drink a coke?

[Tammy]

There's no way Regina could drink a coke. But she waits a couple of days before she throws up. Makes it much harder to find the culprit when a mistake is made.

As for her daily foods, she mostly lives on plain pasta with just butter and milk and sometimes cheese, potatoes of any kind - mashed, boiled, baked, french fries, and a plain meat - mostly beef or chicken. A LOT of HFI safe hot dogs.
For snacks she has HFI safe pretzels or Triscuits crackers with cream cheese "dip".
Her favorite dessert is SF chocolate pudding with SF Cool Whip.
Breakfasts are either Puffed Rice cereal, Safe Bacon and eggs, Bisquik pancakes, or cream of dried beef.

Of course we've been at this so long that there are other snacks etc that we know she can have, but these are her staples.

[colormist]

My typical daily meals involve:
breakfast:
shredded wheat & milk
cup of tea

lunch:
mac & cheese (or) peanut butter toast (or) baloney, mustard, mayo, cheese, & spinach sandwich
cheese nips
almonds (or other safe-ish nut)
tea
water
latte

dinner:
I hate dinner. I usually have some iteration of what's been previously posted above (if I'm feeling lazy) or a pasta salad. I think this week I'll make a big batch of turkey noodle soup. I tend to make big meals on the weekend so I can have leftovers through the week. If I'm feeling ambitious I'll try something out of a cookbook. Usually not feeling ambitious, though.

[chrisnyc]

cool, thanks a lot. that helps. I used to eat shredded wheat all the time but I stopped. gonna give it another go

[julienc]

Chris,

I'll try to add my 2 cents. First, no, I've NEVER been able to tolerate a coke, much less more than 2 sips of one.

Here's a sample of what I eat daily. Similar to Colormist (no surprise...we are twins separated from birth. ;D )

Breakfast: Shredded wheat, every day. On weekends I do waffles (with only butter, made from Jiffy Mix), or eggs and toast. I buy a loaf of Panera bread weekly - they have several no-sugar options. I use it for toast, sandwiches, and even grind it to make bread crumbs for meatloaf, etc.

Lunch: mac & cheese (Kraft brand with the "gooey" cheese, no powder), roast beef sandwiches by the truck load, plain pasta with butter or sometimes with Ragu alfredo sauce.

Dinner: Lots of plain meats (chicken, fish, steak, or pork) with a side of white rice, orzo, etc. For veggies I eat loads of spinach, some asparagus, some broccoli, and canned green beans.

Not much variety in my diet, but it really doesn't bother me. It is odd what some seem to tolerate, yet others can't. For me, I can eat edemame with no problem at all. It's the only soy product I eat, but I have had no reactions to it. I also do fine with beans. My husband makes a wonderful bean soup with ham (not sugar-cured), and it's soooo good. Again, no reactions.

As for my overall health, I'm (knock on wood), doing fantastic. Since I discovered HFI and kept to a strict fructose-free diet, my GI problems have completely disappeared. And believe me, I had lots of "issues" in the past and just told everyone I had IBS.

Anyway, try starting with the basics for a few weeks and see how you do. Hope this helps.

[CJSculpts]

HI Chris. As I have said before, I am not sure if I have HFI or FM. In addition to my to my other unexplainable illnesses, I have had some otherwise unexplainable kidney dysfunction. I had a positive Hydrogen Breath Test which also gave me a pretty severe hypoglycemic reaction which should not happen with FM. Nonetheless, I would love to learn that I had FM over HFI. I don't think that anyone can be absolutely sure withOUT testing DNA, Amalayse or liver biopsy unless, of course there is a known family history. Given the percentages, lack of understanding throughout much of the medical community, difficulty in deciphering symptoms, etc. the odds of a known family history are fairly slim. It is actually pretty amazing that there are multiple sibling groups on this site. Having a sibling or other family member with a confirmed diagnosis would certainly aid in diagnosing a new patient. From my readings, there are different levels of reactions and they do get worse as time goes on. This would account for the fact there are many people on this board that were diagnosed as adults despite the fact that if they had had HFI, they "would have died at birth" (as has often been incorrectly stated by misinformed doctors). As you have said for yourself, you have a constant, low grade yucky feeling. This is because you are consuming small and consistent enough amounts of "bad" without knowing it. They delayed reaction described by Tammy also explains a lot of the difficulties in telling what has caused some of my symptoms. Some HFIers are not pukers. Also, some can tolerate cane sugar {this may or may not be true, as I have seen conflicting information on consumption of cane sugar} just as some can tolerate wheat, asparagus, garlic, onions, etc.

[elilly]

Just a comment here, I don't know of anyone with genetically proven HFI that can tolerate cane sugar. Anyone out there that can? This was "news" to me.

[julienc]

Jan 12, 2010 2:16:45 GMT -5 CJSculpts said:

I don't think that anyone can be absolutely sure with testing DNA, Amalayse or liver biopsy unless, of course there is a known family history. Given the percentages, the odds of a known family history are fairly slim. It is actually pretty amazing that there are multiple sibling groups on this site. From my readings, there are different levels of reactions and they do get worse as time goes on. This would account for the fact there are many people on this board that were diagnosed as adults despite the fact that if they had had HFI, they would have died at birth.

This is just simply not true. It is absolutely possible to have a diagnosis through a DNA test. HFI is an autosomal recessive condition. The discovery of two mutated genes in genetic testing confirms HFI. Each parent must either have HFI (very rare) or be a carrier (roughly 1 in 100 according to my geneticist) in order to pass on the bad gene. If both parents are a carrier, then there is a 1 in 4 shot at the child receiving both "bad" genes and having HFI. 1 in 4 are actually decent odds, so it is very common to have multiple siblings with HFI. In fact, it was because my brother exhibited the same symptoms that they agreed to study me further. The only other "patients" at my same clinic with HFI is a sibling pair. Completely normal, and almost expected.

As for "dying at birth"...again, not true. Most of us who did make it through to adulthood w/out a diagnosis refused sweets literally from day one. My older brother refused baby foods that were sweet from the day my mom started feeding them to him. She didn't push them on him, fortunately, and followed suit with me when I came along. It was not a matter of our tolerances becoming more strict with age. We both had tons of GI problems while growing up, and upset stomachs were the norm. We always knew there was something wrong, and repeatedly talked to doctors about it, but they just shrugged their shoulders and had no idea. Even today (let alone 30+ years ago), most doctors have never heard of HFI.

The tolerance levels that are sometimes discussed on here are varied to some degree, but it's in relatively small amounts. For instance, the difference between eating 4 spears of asparagus or 1, or a handful of edamame or none. Nothing to the extreme of being able to drink a soda.

And to clarify the cane sugar...that is an absolute no-no. Cane sugar is chemically the same as "white" sugar, or sucrose. It just differs in the refining process. It's the new buzz word at stores like Whole Foods, since supposedly it is more "natural", but bottom line is it's sucrose.

[millan]

I agree with julienc.

As for siblings having HFI; when I was young, I was told of a family here in Sweden with 4 children. 3 of them had HFI.

Also, as a child I vomited every time I was fed anything with sugar. I was diagnosed after a couple of years in hospitals and a long line of different doctors not having any clue, but if my mom had not gotten any answers, she would probably had figured out a way to keep me alive anyway. Malnourished, probably, but alive.

From reading this board I have come to the conclusion that I am probably not quite as sensitive as others, but I would never drink a coke, or even half a coke.
I know my limits and sometimes push them, resulting in nausea after an hour or two that lasts from anything from an hour to half a day. Luckily I don't seem to have the problem of feeling bad for several days after.

I wonder if it's the simple fact that I'm less sensitive than others, or if Sweden has been blessed with slightly less additives, but I have no real problem finding food to eat. I have to go now, but will try to log on later and list what I eat from day to day.

[CJSculpts]

YIKES!!! Forgive me for typing while exhausted!!! I tend not to complete my thoughts into well understood ideas when I do. Won't do it again - except for right now so that y'all don't think that I am a raging idiot.

Julie in terms of "I don't think that anyone can be absolutely sure with testing DNA, Amalayse or liver biopsy unless, of course there is a known family history. Given the percentages, the odds of a known family history are fairly slim. It is actually pretty amazing that there are multiple sibling groups on this site. From my readings, there are different levels of reactions and they do get worse as time goes on. This would account for the fact there are many people on this board that were diagnosed as adults despite the fact that if they had had HFI, they would have died at birth. "

I LEFT OUT PART OF THE MOST IMPORTANT WORD!!! I MEANT TO SAY "withOUT testing..."!!!

OY! And I am sorry that you I didn't make clear my intentions when I was talking about "dying at birth." I was using this attitude as an example of how there are different outcomes given the combination of different tolerances and exposures. Basically agreeing with you.

In terms of family history, using the difficulty in diagnosing (with no Dx prior to the 50s, the potentially lethal outcome, and basic statistics, the odds of extended, non-sibling, (especially living) family members showing up with an actual diagnosis are quite slim. If you do have HFI this would mean that both parents are carriers and yes, would increase the odds of having a sibling with HFI, but by no means would guarantee it. I had not looked at the overall board statics but, based on active posters, it appeared to me that there was a pretty high number of potential sibling outcomes. What I was trying to say (which would have been more obvious if I had not left the "out" out of "without") was that having a diagnosed family history would be a key part in the potential diagnosis of a new patient.

I very well may be wrong about the cane sugar. It is definitely sucrose. I was not implying otherwise. Sucrose comes from two sources, sugar beets or sugar cane. My research (I cannot quote sources because I have been pouring through too much information) has shown that, because of the fructose:glucose ratio, sucrose from cane sugar [[[might]]] be tolerated by [[[SOME]]] HFIers. In terms of "marketing strategies", C&H brand has always been marketed as "pure cane sugar from Hawaii - that's the one." Unfortunately, if a product is listed as "sugar" or "sucrose" without a definition of "derived from (insert cane or beet here)", one cannot tell which is which and should stay away no matter their tolerance. The more recent marketing trend is "Raw Sugar" which is a DEFINITE no-no for HFIers because it still contains sugar molasses. Obviously, ask your own Dr., nutritionist, body, etc. about whether it is okay for you.

While there are some guidelines and some hard and fast rules, the diet in general is such a subjective thing. I have seen so many posts here about people who are absolutely diagnosed HFIers who can tolerate things that are on every list that I have read as a "no" not even as a "maybe."
******************************************************************
So to reinstate my original intent in answering: There is a triad of clues beyond just symptoms necessary for a true Dx.
1) DNA or Amalayse Testing
2) Liver Biopsy
3) Known family history - this would not be 100% but would absolutely be a high indicator

If one was to go purely by symptoms alone, one would run the risk of misdiagnosis. In my *own* opinion(stress on the own - not judging here), by resting on the laurels of self-diagnosis by symptoms alone, one could possibly miss diagnosing a different, potentially very important, problem.
******************************************************************

Sorry for creating the confusion! I will try to read my posts multiple times before hitting "send".

[CJSculpts]

Quick note in regards to Fred's post...

I could not agree more about this being a downfall in the medical community. Even though I live in a hub of genetic and bioscience research, it has been incredibly hard to find any true experts. The "doc on the street" certainly has had NO exposure. Even in speaking with Dr. Tolan, the last doc in California, or at least within miles of me, that he has worked with was nine years ago.

Over the past few weeks, I have finally been in touch with geneticists at Stanford and at UCSF who have had multiple HFI patients. Neither institution seems to think that puking is the be-all-end-all for defining HFIers. Additionally, their experience is that the severity of early symptoms, exposures, etc. can have a GREAT effect on the ability to tolerate the taste of "sweet" foods.

There are definitely two types of fructose problem: HIF vs FM (formally know as DFI). I am doing my best to keep the two apart but, even publishings, especially the diets, from the medical community have confused the two or at the very least, crossed boundaries. I have even found articles that were completely mislabeled.

I personally have had the breath test of both Frutose & Lactose. I am Lactose intolerant and at least one way or the other Fructose Intolerant. My physical reaction (hypoglycemia) to the Fructose test would partially indicate HFI, however I am still waiting to get the official Dx of HFI vs FM. My Fructose symptoms are NOTHING like my Lactose symptoms. If eating one salad can put me out for two weeks with major GI symptoms, head-splitting migraines, foggines, etc., then either I have a very extreme case of FM or what all of you might consider a mild case of HFI since I do vomit but my body seems to be morally opposed to doing so and tries to prevent this from happening no matter how violently ill I feel.

[CJSculpts]

;D YES!!! # 4! Thank you Fred!
I think that I blocked that one out of my psyche because it seems like cruel and unusual punishment.

[meaniejean]

And don't forget FDPase deficiency ...

Also, some systemic disorders can cause fructose malabsorption or liver dysfunction that can affect the bodies ability to handle large amounts of fructose.

Hey - we are better off - fructose in large amounts is toxic even for people who have no fructose intolerance ...

I just want to reiterate the STAY AWAY FROM SUCROSE IF YOU HAVE HFI!!! If you have FM, it MIGHT be okay, but why use up any absorption capacity on something that has no nutritional value.

-Sandra