How were you diagnosed?

[colormist]

LOL@Canned Pear in Syrup

Why would they feed that to ANYONE? Bleah. That makes me nauseated just thinking about it.

[lukesmom]

I have known Luke was HFI since he was 3; he is now 7. Because my husband works for the Archdiocese, genetic testing was excluded. We finally went ahead and paid out of pocket and his diagnose was confirmed by blood at Baylor University. He has 2 homozygous mutations. We are the only current case of HFI at Cedars Sinai here in Los Angeles. I also have a 3 year old who is HFI-free.

[hfimomof3]

Genetic testing was excluded on moral reasons? Wierd! Good thing you figured it out.

I had a midwife tell me when I was pregnant and failing to gain weight that I should eat banana splits and fruit smoothies. Another nurse told me that I should be ok as long as I ate my fruits and veggies. Eventually I would just pause and say... "Ok, that sounds good." My allergist is good though, she actually thought about it and made smart suggestions.

It is frustrating because if you have diabetes they know exactly what to tell you. Even if you have phenylketonuria they know what to tell you. I don't expect every doctor to know about every rare condition but it seems to me that HFI is something that is easily managed by diet and good dietary management can make an enormous difference for us, so HFI is a condition that in my opinion should be a standard part of dietitian or nutritionist training, even though it is only present at 1 in 10 000.

[angelad]

Hi, Lukesmom,

We are looking at the testing through Baylor and may have to pay out of pocket, too. Would you be willing to say how much you paid for the testing?

Thanks,

Angela

[lukesmom]

Hi Angela,

Cedars is contracted with Baylor, so they say the prices are "reduced". Our visit and blood draw at Cedars in the metabolic clinic were both covered by insurance. Final cost is 1208. Now my husband and I have to go in, as well, and our tests will be 408 each. Hope that helps.

Amy

[angelad]

Thank you, Amy. That does help. I think we may move forward with the test and we will need to budget for it.

Angela

[angelad]

Hi, Amy (Again!),

I have one more question for you. Do you remember how long it took to get your results back from Baylor? We're probably going to go ahead with the test very soon and I'm getting antsy for an answer.

Thank you,

Angela

[lukesmom]

H,

They said 4-6 weeks... we called to check at 6 weeks and the results had just come back the day before!

Amy

[angelad]

Thank you! More waiting, I suppose.

Angela

[carolyn]

Hi. Having just signed in as a member I feel I can write about my experience of diagnosis tp people who understand! My family have always been great, except for my older brother when I was a child. He kept telling me it was all in my head. After diagnosis (& now both much older adults) he's apologised!
As a baby, my mother found that putting sugar in my formula milk feed always made me very sick. Leaving the sugar out stopped the problem. I self selected what was OK to eat, & am forever grateful to my mother for going with what my body was telling her. Every birthday & christmas I would invariably be sick, as although by then I had an aversion to sweet foods, I was always up for trying them.
By the age of 18 I'd worked out that fructose was the only common constituent in the foods that made my sick (& shaky, headachy, abdo pain & generally feel tired & ill). My GP at the time was not interested & merely said "You always get one..." when I asked for my polio booster to be dropped straight in my mouth rather than go on a sugar cube. For the school boosters I used to try to hold the sugar in my mouth & go straight to the toilet to spit it out.
I trained in nursing & midwifery, & it was whilst training to become a Health Visitor that we had a talk from a paediatrician about unusual food intolerances where he described HFI & it could've been me he was talking about. He recommended that I get it officially diagnosed, & get referred to a geneticist. My GP was great. Referred me to gastroenterologist & genetic counselling. The gastroenterologist was the most arrogant & dismissive man that I have ever come across. I still think about the consultations with anger. I was 25 then. I'm now 52 but feel no less angry.
The guy took a history. He diagnosed IBS, & told me to go away & eat all that I had never been able to & I'd be fine. He looked at results from liver function tests, (which showed liver damage) & asked how much I drank. I answered honestly. Maybe 1/2 can of cold lager in the summer if it was really hot (& even this amount would make me feel sick). So he then asked how much I really drank. He told me I didn't need the genetic counselling appointment that my GP had arranged, & to cancel it. Apart from being shocked that he thought I would lie to him, I duly went home & tried to eat those things that I always wished I could. Melon, apple, etc. I didn't do it for long. I was so ill!! I cancaelled the geneticist appt.
I went back to Dr Arrogant for a review. He said, and I quote "Fructose intolerance is so rare, of course you haven't got it. But as you're a nurse, we'll bring you in to do a fructose tolerance test to prove to you that you don't have it". The test was duly arranged. I was given I/V fructose, & blood was taken regularly, by a very nice, but nervous doctor on his first day as a house officer. I was sick at the end of the test.
When I returned for the results, Dr Arrogant told me that the house officer had forgotten to take blood for phosphate levels, so I'd have to have it repeated.
The second test was done with much more care. The registrar sat with me throughout the proceedings, I was visited by the senior pharmacy guy who had made up the fructose infusion, & by the consultant biochemist (with his bow tie) who would be doing the blood testing. All went well. As I write this I'm wondering why there was so much fuss made. Was it just because it was unusual, or they thought I'd complain about the first time, or now I'm cynically thinking, did they give me too big a dose the time before & wanted to double check everything? Never thought about it before.
When I went back for the results, Dr Arrogant just said "I think you'd better read it for yourself" & pushed the results paper over the desk. 'This patient has a moderate degree of fructose intolerance'. I asked what I should do next, & if he would refer me to a dietician. He said "You've had this all your life. You know what you can & can't eat. Just carry on as you always have". No apology. I never did get to a dietician or geneticist. I'm now inclined to go & have a look at my medical records.
Thanks for listening. I feel better for having told my story.
Kind regards to all

[carolyn]

Forgot to say. I'm in Devon, England. Glad to have some contact!

[colormist]

Oh Carolyn! That's awful! My physician referred me to a geneticist--who also didn't believe me, but humored me with bloodwork to check for the genes. I can't believe you had an IV of fructose not once, but twice. You had to be miserable for weeks and that does so much irreparable damage.

How many years ago was it that you had the test done?

[carolyn]

Thanks. It feels lovely to have understanding from someone who knows what they are talking about! The test was about 27 years ago now, before I had children. I certainly didn't feel well & ended up being treated for depression because I thought my liver was packing up & I would die! Liver function checked annually now & all OK.
Isn't it just so awful being humoured! Did the geneticist you saw admit to you that you were right all along?

[charlie]

Oh Carolyn, that does sound so very British.

Sadly it is a very common story, thank god for yours and your mums instincts really. Unfortunately the dietitians here do not really know anything about HFI and strangely don't seem to want to know about it either. I suppose the NHS is so overstretched they don't have the time? That is a lame excuse for them, I know with my work if I have an unusual case I rush straight home and start googling.

Welcome to the board and I hope the start of your sane life

Charlie

[colormist]

Isn't it just so awful being humoured! Did the geneticist you saw admit to you that you were right all along?

Yes, he gave me a very elaborate speech about how I was right and he was wrong and that he was very sorry to have assumed otherwise. It was all quite nice.

[julienc]

The similarity between stories amazes me. My GI doc didn't believe me but agreed to refer me to a geneticist. I never saw him again. The geneticists were quite helpful and supportive from my first visit, fortunately.

Kudos to your mom for being so observant!!!

[esmee]

I posted this in the HFI-Adults section, but thought I would post it here also since this thread seems to have more participation.

I have not been officially diagnosed and have only just discovered the existence of HFI but, as another member wrote, "my whole life now makes sense." I am 41 and have felt ill for most of the past 25 years. I suffered from severe hypoglycemic episodes and migraine headaches in response to sugar. If I ate anything sugary for breakfast as a child, I was sure to be sick and unable to concentrate before lunch time.

Ice cream almost always gave me a stomach ache (though that did not stop me from eating it!). Pixie sticks and sweet tarts were my faviorite candies as a child, and a purely "sweet" tasting dessert has never appealed to me (I always chose sweet & sour like lime sherbet or key lime pie). Whenever I ate fruit, the "sweet" taste (acid-forming for me) would become offensive quite quickly and I would look for something salty (alkaline) to balance it out.

I think I started to get more noticibly sicker in my late teens because I decided to become a vegetarian at that point, which naturally meant more fruits and vegetable and lots of carrot juice. Man, did I feel terrible on carrot juice! Quite honestly, I don't really want to take a fructose tolerance test, because I KNOW just how bad it will make me feel.

I was manifesting symptoms of celiac disease starting at age 17, but it was the more rare skin blister type (seen in only 10% of cases) and none of the docs recognised it. I self diagnosed it when I was in my late 20s by looking a photos on celiac web sites. None of the excellent doctors I have seen over the years have come even remotely close to implicating fructose as the the culprit.

Fruit causes severe acne on my face, neck, shoulders, and back, and the pH of my urine will turn quite acid (below 5.5) if I ingest any fruit. I have asked many other people to test the pH of their urine in response to fruit and only one other person said he was acid (he has many symptoms of HFI also).

My teeth are extremely sensitive and I think this is from the chronically acid pH caused by continual fructose consumption. If I eat a lot of fruit (which I was doing at one point because I thought it was healthy), my mouth gets a sour taste and my teeth absoultely kill me after only two days. I thought this might of been from having braces (adhesives are used), but others who have had braces do not seem to have this problem.

If I eat fruits with high levels of free fructose, like watermelon or pears, it will send me into a complete funk. My body and brain both stop working for at least 4 hours. If I eat fruit with equal amounts of glucose to fructose I don't respond quite as bad, but it is still pretty awful. If I do not eat something else (safe) to over-ride the reaction to the fructose, my blood sugar will begin to plummet within 30-60 minutes and I will become EXTREMELY irritable and highly uncompanionable to be around.

I have had 2 good periods. Once I ate only goat yogurt and raspberries (very low fructose for a fruit) for 2 entire years. But then my immune system started reacting to these foods and I had to come up with something else that worked. I switched to raw beef, olive oil, and leafy greens which I followed for two years. This is not an exaggeration. My low weight before stubling onto the yogurt/raspberry diet was 87 lbs (i'm 5'6" and normal weight is 115 lbs). Then I started having problems with that and I started fishing around again and decided to try a low-fat fruitarian diet which, as you can imagine, has been a complete disaster! I am in limbo now and trying to figure out what I can eat without feeling like total crap all the time and it has not been easy.

I seem to do reasonably well with white sweet potatoes and steamed leafy greens. I cannot get near orange sweet potatoes (called yams in the market) and I have come to the conclusion that they must have a lot more fructose than the white variety. I am currently experimenting with avocado, which also seems to work to a certain extent, but there is really nothing perfect. I cannot do whole grains or beans at all (though white rice is a possibility if I eat it with fat so slow down digestion--I may try it with avocado and see how that works), and I really do not want to go back to meat (for ethical reasons). I am contemplating giving the yogurt another try since it has been almost 5 years since I lived on it.

I also wanted to add that when I was at my all-time low weight-wise and I was admitted to the hospital for what I thought would be a gall bladder removal (since I could not figure out what else was causing all my food problems--fortunately the surgeon decided my symptoms did not fit and refused to operate because he was afraid I might die on the table), my GI doc asked a psychitrist to come talk to me and he decided I was suffering from anorexia nervosa (that appointment still makes me angry to think about--and I almost want the genetic testing just so I can march back into his office 7 years later and shove it in his face). After a week of no change (could not eat anything and was on an IV) my GI doc decided I had taken up enough room at the hospital and sent me home--basically to die. Fortunately, I discovered goat milk yogurt and it saved my life.

Charlie responded:

If you suspect you have HFI do as completely fructose free diet as you can for several months to clear your system out then start reintroducing safe foods first which can be found on the boston site. Sweet potatoes are definately out, alot also can't have have avocado (also FM can't have avocado). Raspberries are out too for HFI but ok for FM. So basically cut out all fruit, veg and sugars for a while, see how you feel and then go from there.

If you can't do grains and beans you are sounding more FM as these are on the no list. Look up www.fructosemalabsorb.proboards.com for more info on that. But do get proper medical advise too.

Then I replied:

Isn't severe low blood sugar more a symptom of HFI than FM? I thought that is what I read. I have been plagued with this all of my life, and it is definitely connected to fructose consumption.

It seems to me that if 3 different enzymes are potentially missing in people with HFI then there can be different degrees of severity. I may only be missing one enzyme, while another person may be missing all three. They would experience a negative reaction from only a small amount of fructose, while I may be able to eat that same small amount without completely falling apart. But I can tell you that if I eat just one cup of bluberries, it will make me insatiably hungry. And if I do not eat something else "safe" pretty quickly, I will end up with a migraine headache a few hours later. However, I will check out the other board too and compare notes.

I am also fascinated by the info someone posted somewhere on this forum about converting body fat into fructose and glucose. I have done a lot of fasting over the past 25 years because food (almost all was high in fructose) made me feel so awful all the time and fasting was the only thing I could figure out to give me a rest.

However, I always experience a migraine with vomiting during within the first 1-3 days of beginning the fast, then my body seems to adjust and I feel fine for the rest of it. This happens pretty much every time I fast.

I have asked a lot of other fasters that I have met over the years, and know one else goes through this. Now I am wondering if it has something to do with HFI, as others on this board have reported getting very sick if they skip meals (I will also get a severe migraine if I skip a meal). Anyways, a little off topic here, but it was on my mind.

and then:

I just looked at the FM site. Thank you. I really feel like my symptoms are much more of a fit to HFI. The only thing that ever gives me diarrhea is cherries because of their high sorbitol. But none of the other fruits or vegetables mentioned will cause this for me. I do however, suspect that I have some level of FM going on in addition to the HFI, just not that severe in comparison with others. I do get bloating from cruciferous vegetables and beans, for example. But a small amount of ANY fruit, including the low FODMAP fruits will preciptate me towards a low blood sugar episode. Bananas and grapes are perhaps the worst, next to watermelon and pears, and most of the vegetable listed to avoid are not that problematic for me. My worst symptom is low blood sugar and foods high in fructose will send me into a tailspin.

Any input would be appreciated.

Many thanks to all of you for the great information on this site.

[lynneangela]

Hi
My name is Lynne I am 57 and have HFI and live in West Sussex. I was 40 when I first asked to have a test. I had to digest a sugar solution and have blood taken over a 5hr period. I felt so ill afterwards i dont know how i got home, the hospital had no idea what they were doing to me. Then at 50 with the knowledge of the internet, I asked for a DNA test to prove what I knew I had, Heredetory Fructose Intolerence. But even with this confirmed all I was told was to get on with it. As a newborn baby. I was told by my parents that I had a adversion to anything sweet so they knew I had some thing just did'nt know what it was called. They said I could only keep water down as a new born and that I was lucky to suvive as the midwife was no help. I never had a birthday cake as a child or eaten sweets. I can smell the smallest amount of sugar and as I have such an adversion to it I think thats why im still alive today. I am becomming more intolerent too now. It is now on my medical notes at my Doctors now. I always worry that if im in an accident and cant communicate they will give it to me. I dont know anyone with this awful thing. Most doctors and people think im a fussy eater, only a small circle of friends I have met through the caravan club over the last 3 years are learning to understand this and what it means to have HFI.
Carolyn your story sounds just like mine as a child. Im one of six children and only I have it. I was always treated like a freek as a child at school and by doctors.
Lynne

[susan]

This may be the wrong place to post.

However, my allergist, whom at least knew about HFI, agreed to help me get tested. He, himself, is interested in genetics. He is interested in learning more about HFI.

HALEHLUYAH CHORUS !

Since he does not have a lab in his office, he is calling Dr. Tolan for specifics then he is going to call different labs in my region. He was very helpful and kind about the matter.

He confirmed, no geneticist in Northwest Arkansas.. only found in Little Rock.

Anyway, I am happy to have a pleasant experience with my allergist and happy to "get the show on the road".

[colormist]

Great news, Susan! It's really a wonderful feeling when you find a doctor that's willing (at the very least) to humor you. That's awesome that he's interested in genetics and willing to learn more about HFI.