My wife's symptoms, please help

[roman]

Dear all, I am sure that my wife has had HFI all her life, once hospitalized as a child after a night of vomiting with hypoglycemia and ketonuria (acetone in urine), when she was 5. Her doc gave her glucose, but did not diagnose her as anything (FM nor HFI). She struggled all her life with generalized GI pain, severe headaches and a few years ago nearly died in a hospital after gallbladder surgery when post-op morphine drip could not stop her pain (discontinuation of drip and oral vicodin did). I suspect, but can't prove, that there was sorbitol in the solution. Chewing Dentyne (sorbitol-based) gum makes her ill with cold extremities and severe headaches, same goes for dried apples. We have only recently made the connection between fructose and her condition. Interesting thing - she never did develop a distaste for apples, but she did develop a distaste for bananas. She recently had several episodes of falling into a stupor-like sleep after eating small amounts of fructose in low-carb protein bars (no glucose buffer, I presume). Needless to say, she is not eating any more fructose, sucrose or sorbitol now, but she still has reactions to unexpected sources of fructose such as almonds (I know, you all know about this, but we are learning fast too). What concerns me is that frequency of her reactions seems to be increasing (she always had "migranes" but now they seem to come on even after eating potatoes with meat). She is also mentioning more frequent urination than previously, which I do not understand. Her doctor is no help - they want to do a fructose tolerance test, which I adamantly refused. Can anyone please comment on her symptoms? Have you ladies experienced worsening of your symptoms while trying to eat low-carb foods (low starch) and/or when becoming pregnant? A appreciate any reaction at all. Thank you all.

[buddhasbelly]

Hi Roman, I understand that you are worried about your wife, these are scary things that are happening to her. Since I am not officially diagnosed yet, I don't think I should be giving advice about the HFI part of your question, but I can tell you this. I have been diagnosed several times with a shortage of vitamin B12, until it was decided that I have an absorption problem with some vitamines. The shortage of vitamin B12 gave me the urge to urinate very frequent, even when I hadn't drinken anything since the last time I went, like during the night, sometimes three times or something like that. I don't think it would explain all of her symptoms, obviously, but it something that doctors can check very easily with a bloodtest. I get monthly injections now with vitamine B12 and that problem is solved. Good luck, I am sure other people on the board have more indept information for you about HFI.

[Tammy]

Hi Roman
Unfortunately, no one on this board is a doctor or qualified to make a diagnoses. This isn't a medical site, just a nice group of people looking to discuss a common ailment. With that said, please get her back to her doctor, or another doctor if you aren't happy with hers. I really would not want to see someone help you jump to wrong conculsions and end up making her worse, or threatening her life in another way.

If the HFI diet makes her feel better, then by all means stay with it for awhile until you can find a doctor that's willing to help.

[esmee]

Hi Roman,

Where are you located?

As far as getting a diagnosis is concerned, it is always recommended that you have the genetic test first. It is the least invasive of all the tests, and it has a high degree of accuracy for people of Caucasian descent. If the test comes back negative, that does not mean your wife does not have it. You would then have to decide between a biopsy of liver or sm. intestine or a proper fructose tolerance test. The best thing to do is to print out the information on HFI from Dr. Tolan's website and take it to your wife's doctor: www.bu.edu/aldolase/HFI/hfiinfo/detail.html and request that they order the genetic test.

Since I have been on a very low fructose diet, I am urinating less frequently and I have even started to sleep through the night (8 hours) without having to relieve myself. Normally, I would have to urinate an average of 2 times every night. As a child, I wet my bed until I was 10 years old. Fructose is toxic to the liver and kidneys of HFI people, so it stands to reason that we would have to urinate more often if we ingest fructose.

I do not know yet if I actually have HFI (I am waiting for the results of my genetic test from Dr. Tolan's lab), but I am definitely fructose and sorbitol sensitive. I HATE bananas and every time I eat them they give me a migraine. I have a long history of migraine headaches which I now realize was a consequence of eating fructose. No fructose = no migraines.

Fructose does not cause nausea or vomiting immediately after ingestion for me, but I do experience debilitating episodes of hypoglycemia after eating anything that contains it and the hypoglycemia will then preciptate a migraine (which then causes nausea and vomiting). Consequently, it has been difficult for me to pinpoint exactly what the trigger was until very recently and, of course, fructose is in virtually everything I eat so it was very difficult to figure out.

I do think that many people with HFI may have nutrient deficiencies. Like buddhasbelly, I also have had chronically low B-12 levels and take injections on a semi-regular basis as needed.

[roman]

Thank you all for responding - we are of course working to get her properly diagnosed and she is going to see a geneticist at Kaiser in San Diego this Friday. Her symptoms are a classic HFI - hypoglycemia following fructose or sorbitol (from gum), "migrane" headaches, passing out, vomiting all night every once in a while, and many symptoms of metabolic acidosis - acetone in urine (as a child), fuzzy vision, pain in muscles and bones, and best of all: all these symptoms are relieved by glucose tablets. I think symptomatically she fits HFI, but she has got to be heterozygous for it - these symptoms only occur on one side of her family. Normally, heterozygous carriers of mutated HFI alleles are considered asymptomatic, but I have heard reports that there are symptomatic heterozygots too. There is also a related condition that is caused not by the ALDOB mutation, but by a heterozygous mutation in the FBP1 gene, see www.ncbi.nlm.nih.gov/pubmed/20096900 . Both of these genes cause accumulation of phosphate in the last step of fructose metabolism, which then becomes unavailable for neoglucogenesis in the liver, therefore hypoglycemia. So even if you don't have anything wrong with Aldolase B, which is Dr. Tolan's speciality, you can have a mutation in the FBP1 gene, (fructose-1,6-biphosphatase) and still have the same symptoms. I think HFI genetic testing should be expanded to cover this gene as well - oh well, it's expensive.

I realize that the only medically recognized diagnosis involves a positive fructose tolerance test, or a positive genetic test, and I am not looking for a diagnosis from this board. I was merely asking for your experience - for instance, frequent urination is not reported in medical literature, AFAIK, but one of you confirmed it for me. That's what I am looking for - sharing our experience. Has anyone also experienced meteorism after an "episode"? Have you found it difficult to lose those last 5 lb of weight? Do you feel bloated and retaining water when eating even small amounts of fructose? Thank you again!

BTW my wife also reacted to "dextrose" (corn-derived) from the brewing store. Can anyone corroborate? She does not have a bad reaction to pharmacy-bought glucose tablets for diabetics.

I will also have her checked for a B-12 deficiency.

[ukbill]

Hi and welcome to the board.

You must be very concerned for your wife.

I can answer the dextrose question simply.. most dextrose derived from corn will naturally have about 4% sugar because its made from sweet corn which has about 4% sugar (not identified which one but most likely Fructose) inside it. this is not removed or effected by the conversion of the starches in the maize to Dextrose. so any Dextrose made form "Corn" will contain at least 4% sugar (sucrose or Fructose).

It gets worse!

Dextrose / Glucose is also made in the same factory as HFCS (High Fructose Corn Syrup), in the same reacting vessels and is sent down the same pipes, packaged in the same machine etc.. as HFCS

There are few (if any) analytical laboratory instruments that can identify low %'s of one sugar in another.

Therefore even labels on foods that state a % sugar can be wildly misleading.

I have a tin of mackerel (fish) in tomato sauce which states 0% sugar.. really I wonder how they managed that??

In Tescos (like Walmart) in the UK the label for sweet potatoes has a lower sugar content of a variety that I can tolerate (if cooked correctly)

Therefore for us with HFI shop bought Glucose or Dextrose is just not safe.

Medical grade Glucose / Dextrose is made (in the UK) from Rice starch

So is missing this contamination.

I would if she were my wife, given the severity of the symptoms and the increasing frequency of attacks, treat her as (for some time) a double zero HFI.

I.E. NO Fructose or any sugars (except lactose from milk so long as she is not intolerant to it)

I am not a Doctor but I have HFI and certainly can recognise the symptoms. Although that of course is not a diagnosis.

The usual dis-calmer applies..

Its your wife's life! The advice I can give is only based on my own experience and information I have gleaned from research Documents etc.. in my quest to improve my own (and others with HFI) lives and allow us all to live fuller happier healthier lives.

However please check with a health professional any advice given and basically its your own risk..

Sorry but these days it has to be said.. I think its the fast buck everyone's to blame type society we live in.

OK back to her symptoms.

IF she has HFI then by what you are saying she is still getting far too much Fructose in her diet!

You really have to check EVERYTHING she eats for Fructose content.. The food manufactures are really sneaky and slip in Fructose in as many things as they can (because its addictive of course) .

Read the small print check up on the internet (companies these days often list ingredients on their web sites)

Products change and are different in different countries. Wheatabix in the UK contains sugar.. in Australia it is Sugar free, the opposite is true for Shredded Wheat!

If your wife is HFI then by the sounds of it she is having a really bad time of it and her liver and kidneys are really not happy!

The HFI food lists available contain both safe and "tolerated" foods.. Tolerated is not the same as HFI safe!

I remember after my "challenge test" being ultra sensitive to everything for at least 6 months before I started to recover.

Potatoes are really risky for HFI I can only "tolerate" a few varieties and then they have to be boiled in lots of water to reduce the amount of Fructose in them before I can eat any reasonable amount.

I hope this helps?

Above all else keep smiling

[ukbill]

PS

For some time I would avoid all prepared shop bought foods, pies, sausages, bread just everything has to be suspect.

Even fresh meat in a butchers can be "brined" to pump water into the meat (so they can sell you water at meat prices) this almost always has added sugar in it!

Buy white bread flour and make your own bread, use only butter etc..

Be suspicious of everything!

I know its a pain in the proverbial butt..

I have posted here many times (so will not repeat it here) how Fructose can be included to damaging levels into food and we with HFI can still eat the food and not react to it.

The damage is still done to the liver and kidneys however..

Its the starches that break down into Glucose and Glucose itself in foods that will support the blood sugar level and "hide" the Fructose .

Hence it is possible to eat bread with quite a high Fructose load (for example) and not know its damaging you!

[roman]

ukbill, OMG that is scary, and thank you for the info! The dextrose I bought will be for me only - it's a sure thing that "normal" people do so much better on it than on sucrose or fructose - fructose is metabolized like alcohol, as many of you know (liver cirrhosis, high triglycerides, insulin resistance, type-II diabetes, obesity, high blood pressure!!!) Glucose does not do this.

Also, this sentence was particularly useful to me: "I remember after my "challenge test" being ultra sensitive to everything for at least 6 months before I started to recover." - I thought she was getting more and more sensitive! Now I understand that this may, in fact, be the case. She will eat only meat, fish, eggs, cheese, cream (unsweetened of course) for a while now, until she recovers. It's been very hard on her the past few weeks.

[ukbill]

Yes my heart go's out to her I know how much a reaction hurts and just how terribly sick she must be feeling!

The ultra safe HFI diet is the exact opposite to what we are all told is a "healthy" diet.

It is simply as you said animal protein, animal fats and white rice.

Bread made without added sugar (or a tiny amount of your dextrose as a yeast starter) should be also safe as the sugars that are in the bread are turned into alcohol and CO2 gas .. hence the bread rising

Pasta is usually safe also providing you are boiling the pasta (not wholemeal pasta) in plenty of water any remaining sugars should move into the water leaving the pasta pretty safe.

According to a patent for the manufacture of Glucose (one of several I have read) the resulting Glucose is 60% glucose and 40% "other sugars" it is still sold as 100% pure Glucose!

[roman]

I have potentially access to pure lab-grade dextrose, so that is what I am going to get. No more brewer's dextrose.

We have been doing the Paleo diet for a few months now (plenty of meat, fish, eggs, animal fat -such as beef tallow- veggies, fruits, no dairy, no grains, no legumes), so the transition to a HFI diet will not be difficult at all. We just cut out fruits for good and veggies for now, with the exception of home-made fermented cabbage (yeasties are good friends, as you said!) and add pure dairy products, preferably from goat and sheep milk, some cow's milk such as cream and butter, and we'll be set!

I think, also in agreement with your description, that her previously high-starch diet (potatoes, bread, rice) allowed her to suffer quietly yet function in life, whereas the Paleo diet uncovered the true cause of her pain - fruits, unbuffered by the extra starch, manifested themselves as toxic, which they truly are. I am rateful to the Paleo diet for losing 35 lb in just 6 months and normalizing my blood lipids, now I have this to add. I am truly happy and relieved that my wife's suffering is over. I know it is.

We do not buy processed foods, but the juiced-up meats I have to be extra careful with. We buy grass-fed beef, even if it is more expensive, but that kind of meat is SAFE.

BTW I know how they managed a 0g sugar in a tin of mackerel. When the serving size is small enough, the sugar amount drops below certain level (1g? - not sure) and they are legally able to state "0g". Simple.

[ukbill]

I will try to eat the fish and see if I get a reaction .. but that will only tell me if there is more than a certain level of Fructose in the tin.

Recently been making some toast from bought white loaf of bread.. Hovis thick sliced.

The ingredients list has no added sugar.. but included the line "Fermented wheat flour" now that could cover a multitude of sins!

It tastes so sweet to me.. I doubt I would get a reaction because of the buffering from the starches in the bread but it made eating it quite unpleasant.

PS Yeast extract is a great source of vitamins particularly from the "B" group.

The stuff I get (Vegemite or Marmite) contain a tiny amount of sugar but the dosage is so low as not to cause me a problem.

PPS

You mention urination.. yes some days I could pee for the Olympics (if it was an Olympic sport) and win a GOLD!

Also some days I can be insatiable thirsty all day and drink litres of water (on top of my usual pots of tea), days later I will have a peeing day so to speak.

The only time this causes a problem is if this coincides with me visiting a pub or bar..

Then I get quite "messy" after the first 3 or 4 pints go down in a few min....

[esmee]

Roman, to answer one of your questions: tikitavi, another member of this site, has reported losing the puffiness since adopting a fructose-free diet. so, yes, i do think frucotse ingestion can cause water retention in people with HFI.

Please let us all know how your wife's appt. with the geneticist goes on Friday.

In order for your wife to have HFI, she must have a mutated gene from each parent. It could be that several others on one side of her family have HFI, while the other side only has carrier with one genetically mutated gene.

The link you provided on FBP1 gene is no good. Maybe you could try posting it again. I don't know anything about that and would like to learn more. thanks.

[roman]

The link again is
www.ncbi.nlm.nih.gov/pubmed/20096900

Name and abstract:
Metabolism. 2011 Jan;60(1):107-13. Epub 2010 Jan 25.
Novel compound heterozygous mutations in the fructose-1,6-bisphosphatase gene cause hypoglycemia and lactic acidosis.
Moon S, Kim JH, Han JH, Ko SH, Ahn YB, Kim JH, Yang SH, Song KH.
Source
Department of Internal Medicine, Incheon St. Mary's Hospital, The Catholic University of Korea, Incheon 403-720, South Korea.

Abstract
Fructose-1,6-bisphosphatase (FBPase) deficiency is an autosomal recessive disorder caused by a mutation of the fructose-1,6-bisphosphatase 1 (FBP1) gene and results in impaired gluconeogenesis. We describe a male patient with typical FBPase deficiency who presented with hypoglycemia and lactic acidosis. The FBPase activity in his peripheral leukocytes and liver was very low. We amplified and sequenced the entire FBP1 coding region of the patient and his family members. Direct and allele-specific sequence analysis of the FBP1 gene revealed that the proband had a compound heterozygote for the G164S and 838delT, which he inherited from his carrier parents. His father and mother had heterozygous 838delT and G164S mutations, respectively, without any symptoms of hypoglycemia. Gene tracking within the family revealed that his elder sister had a heterozygous G164S mutation without symptoms of hypoglycemia. A G164S mutation of FBP1 in a heterozygous pattern (G164S and InsG960_961) has been reported previously, but the heterozygous 838delT mutation is novel. Transient transfection studies using COS-7 cells demonstrated that FBPase proteins with G164S or 838delT mutations were enzymatically inactive. In conclusion, we report a new case of molecular diagnosis of FBPase deficiency and provide evidence that impaired FBPase activity may be caused by novel compound heterozygous mutations in the FBP1 gene. (emphases mine)

So it looks like there may be several liver enzyme deficiencies that present just like typical Aldolase B HFI, yet they may be heterozygous (ALDOB deficiency is believed to have to be homozygous to be symptomatic), so it is possible to have symptoms while heterozygous, but possibly because of a deficiency in a different enzyme, not necessarily ALDOB.

I will post about our appointment with a geneticist.

ukbill - Don't eat the tinned fish. There is no way there is no sugar in the tomato paste, so why try.

[roman]

ukbill - yeast extract has a lot of MSG, which is a neurotoxin. It positively gives me anxiety, very quickly. It takes days to pass. This is of course unrelated to my wife's fructose issues - this is purely my own "thing". I avoid glutamate-rich foods such as wheat, grains and legumes, and I am a very happy man, free of all drugs, after years and years of SSRI use.

[tikitavi]

Roman, I wanted to mention that I've definitely gotten a fructose reaction from dextrose that I bought at the health food store. So I totally avoid it now, along with sucrose and fructose.

You mention grassfed meats; I believe in that as well. I use locally raised, 100% grassfed raw dairy (butter, milk, cream, and cheese). I was using their meat as well, but I was getting a reaction to certain cuts (especially ground meats), so I believe that perhaps some "natural flavors" or brine are added by the processor to the ground meat. I also got a reaction to plain, locally raised and pastured pork. But once again, I don't think it's the farmers' fault. I don't believe the farmers have any control over this, it is contamination that happens at the processor. And of course, there is nothing disclosed on the label. Frustrating!!

I have gotten reactions from fish as well, I do think even wild-caught and organic isn't necessarily safe either. I think fish is often pumped with brines and colorings to prevent off-smells or colors. And I don't think they necessarily have to declare it on the label. So, since getting all these reactions from meat and fish, I've actually been avoiding them too. I'm doing a non-vegetable vegetarianism. My diet is mostly the raw grassfed dairy, local free-range eggs from my friend's chickens, with small amounts of homemade white flour products such as homemade bread, croissants, and dumplings. It's my "Heidi" diet.

[tikitavi]

P.S. You mention you are having homemade fermented cabbage. I would urge caution with ingesting any cabbage products, they are very high in fructose and sucrose. I have posted the data for cabbage on another thread, so if you do a search you should be able to find it.

I would also urge caution with fermented products. I have had very bad reactions from them. Fructose is produced in the fermentation process, so depending on where the item is in the fermentation process, there can be residual sugars including fructose. I have gotten very ill from homemade kefir, yogurt, and other fermented products.

[roman]

tikitavi
Thank you so much for the info. We are definitely going the way of grassfed meat. If necessary, we will grind it at home. I need to pay attention to ground meat and whether my wife gets a reaction to it. White flour is a definite no, because of gluten. As far as fish goes, I think the safest fish is wild caught, minimally processed, FROZEN. If it's frozen, it means it was frozen on the ship that caught it, therefore it wasn't marinated or otherwise adulterated to cover up smells. I will not buy fresh, non-frozen fish from now on. Frozen wild caught Alaskan salmon has been no problem for my wife. I also think that the safest milk and dairy is from goats and sheep. Grass-fed butter, ghee and cream are also good (high fat, low protein content - cow's milk proteins are problematic, immunoactive in humans). We are learning fast!

[tikitavi]

I have still had problems with frozen fish too (and shrimp), so please be careful and note any reactions.

Re: gluten; I have slight gluten intolerance, but I am not celiac. I use DPP-IV enzymes (I buy Enzymedica's Digest Spectrum) which helps me eat both gluten and casein without any of my previous problems. So if you/she are not celiac, that is an option. It certainly makes things easier and cheaper, to be able to eat some homemade wheat products!

[roman]

Again, titikavi, thank you for the info. It is amazing that people that helped me most are people who I have never met, seen or will likely see in my life. I am so grateful to the internet. There may be some DPP-IV in my wife's future - not mine, because I am not fructose sensitive, but for her, I will definitely think about it.

[colormist]

Hi Roman,
In regards to bathroom frequency, I'm pretty sure I visit the bathroom at work 10x more often than anyone else in my building. I'm in there every two hours (at least). If I don't drink anything (essentially dehydrate myself) then I only go a couple times a day but I get really, really thirsty.

I don't wake up (very frequently--maybe a couple times a year) to go to the bathroom.

I don't know if anyone mentioned rice, but she can have white rice, too. It's one of my go-to easy meals. Rice & butter, fried rice, or Rice & chicken.

And one last warning, if your doctors or geneticists suggest doing a fructose challenge or fructose loading (or basically any test mentioning fructose or sucrose), I would suggest you tell her to decline that test until after genetic testing is complete. It sounds like she's having a rough time as is and she doesn't need to endanger her liver or kidneys any further by ingesting or injecting fructose or sucrose in her system.

Good luck and keep us posted. We're like a family here. We fight, we argue, and we support one another when we need it most.