Adults diagnosed with HFI?

[OklahomaMatthew]

I'm 28 years old and been having serious problems for 10 years with brain fog, extreme fatigue, light-headedness, feeling terrible and irritable, dry mouth, constipation, nausea, feeling full a ton of the time, and muscle weakness to where on a worst day, for a short 10 minute drive i literally will switch which foot i drive with because it is so exhausting to push the pedal down.  The most recent issue is weight loss.  I'm 6 foot 2 inches and i graduated high school 10 years ago at a thin figure of 135 pounds, but now am down to 120 pounds.  I've been suspicious that it was something that i was eating for a long time, so i have changed my diet, and tried this or that change for years, and a doctor mentioned the possibility of fructose at the start of this year.  I took a hydrogen breath test and that tested positive(it went up to 50 after an hour).  The doctor said i had fructose malabsorption, and to go on a low fructose diet, but the sucrose should be fine.  From what i read fructose malabsorption didn't really explain most of my symptoms, but HFI explained them all.  I asked the doctor to test for HFI just to see and he had never heard of it before.  He went off and read stuff for 15 minutes and said if i had HFI i either would have been diagnosed as a kid or clearly had an aversion to sweets and since it was going to be some extra struggle to pursue this rare test, he wouldn't do it.  I have not had an aversion to sweets.  I'll list what I've noticed in my diet in my next post if you are interested, has anyone heard of an adult being diagnosed without an aversion to sweets?  Should i push to pursue the test or do you think that the doctor's suggestion is best.  I understand that you all are not medical professionals, but you all know a lot more about this than me(and probably my gastroenterologist).  I just really want to figure out what's wrong. 

Thank you for your time and advice,
Matthew

[OklahomaMatthew]

Years ago my friend suggested a certain diet to try for a month and i was dead set on trying anything to possibly feel better. It was eating salad with basic oil and vinegar dressing, fruits, and nuts. That was it. And after 4 days of that diet i felt so awful, it didn't matter how determined i was to keep the diet for a month, there was no way.

Earlier this year when i was trying to go low fructose i decided that low fructose didn't seem to make much of a difference so i added back in Kind bars(which have honey, nuts, and dried fruit) I started having 2 of those bars everyday, and after a month of having literally feeling full and nauseous every day i noticed that that timeline corresponded with having those Kind bars for the last month. I got off the Kind bars and my stomach didn't feel perfect, but very noticeable improvement.

Just a couple of months ago i started trying to stay away from as much fructose as reasonable(not eating dessert foods with lots with sucrose, but still eating things like bacon with added sucrose, not eating any fruit, but still having some vegetable with some fructose) I still wasn't feeling perfect, got frustrated with the limited diet figuring it to not be worthwhile, so i added back in gluten free dessert food with lots of sucrose(i was still staying away from honey, fruits) So sucrose is the only thing i added back more of and over the next 3 weeks on that diet, felt awful. Just plain awful. Getting home from work, it was all i could do to muster together the energy for a shower. I would fall asleep on the couch after dinner. So very tired and out of it. Well, i noticed that the deterioration in how i felt matched when i added all the sweet food back in, so i got off all dessert food with sucrose again, and have definitely noticed an improvement.

The only thing my mom noticed when i was younger was in kindergarten class she wouldn't let me have chocolate milk with the other kids, because she noticed that that gave me headaches. So i had regular milk. In high school i remember feeling tired all the time(i heard that teenagers need more sleep than any age bracket after babies, so i attributed it to that and hoped it would get better in my 20s), but instead it got much worse

Thank you for any thoughts,
Matthew

[fred]

Hi Matthew

Welcome to the HFI Discussion Board. HFI is caused by a genetic mutation and not all of them have be defined yet. However, to treat it with diet you have to eliminate all sucrose and fructose. Sucrose (table sugar) is 50% Glucose and 50% fructose chained together. Lactose (milk sugar) Maltose and Glucose also called Dextrose should be Ok. However need to understand though, the manufacturing process for glucose or dextrose only renders those sugars at about 80% pure.

If you have HFI and you ingest sucrose or fructose, your blood sugar if tested will show normal and a blood glucose test will show low. You have to get off all foods that contain sucrose and/or fructose all together, or you won't feel any better. Having done this for a couple of months, if you still feel the same, then you probably do not have HFI.

Try and talk your doctor into arranging to get you the DNA test.

Bt the way, what country are you located in?

Fred

[OklahomaMatthew]

I live in Oklahoma in the USA. Thank you for your reply. As noted earlier I am finally noticing some pattern with my diet in the last month thus i have developed some "aversion to sweets," but has anyone heard of a person reaching adulthood(I'm 28 now) who did not have an aversion to sweets that was diagnosed with HFI? I've been eating fruits ans sweets all my life. Maybe not as serious form of HFI as some? Thank you all.

[colormist]

Hi Matthew,
There are a few of us on this board that do not have a distaste for sweet foods. I, personally, would love to eat berries of all kinds. I do not enjoy super-sweet drinks like soda pop and fruit juice or super-sweet foods like icing. I knew I didn't like sweets when I was in my mid-20's, but I didn't know why. My friends suggested it was a mental issue. With THAT advice, I started doing serious research. I found my diagnosis when I was 27 and had read lots of horrible things about people not being taken seriously by their doctor and getting bad medical advice. I made an appointment with my doctor, told her I was pretty sure I had HFI and asked for a referral to a doctor that could perform genetic testing. My doctor believed me, the geneticist I was referred to did not believe me, BUT he was willing to rule out HFI first. He said that I would have died as a baby if I had HFI and did not have a diagnosis. The geneticist apologized when the test came back positive.

There are quite a few people on this board that did not discover what their illness was until they were adults. I think I saw a couple that were over 40 years old. So, it is possible to live for a very long time without a diagnosis--though I think it helps ignorance if the person is particularly picky about what they eat.

I'm not a doctor or a medical professional, but if you think you might have HFI, I would recommend that you try an elimination diet for a least a week before adding in some additional foods. For an elimination diet, you're going to want to read the labels on everything (even meat). Don't eat anything that you haven't read the label--no matter if the person swears up & down that it's okay for you to eat. The easiest elimination diet would consist of plain block cheese (cheddar, mozzarella, feta), white pasta (not whole wheat), tuna tinned in oil (not water), oats (plain), milk (plain), plain yogurt, beef (no brine or broth), chicken (no brine or broth), white rice, cottage cheese, sour cream, cream cheese (dairy is pretty safe so long as you stay away from the added flavors), butter, oils, water, black unsweetened tea, coffee (no soy), salt, and pepper.

If you have HFI, you should be feeling WAY better after a couple days on this diet. A week would leave you feeling fantastic providing the foods you're eating are safe (I have found bad sugars in cheese, meats, rice, and salt).

I would also highly recommend you keep a food journal. List the foods you eat and how you feel afterward. Some foods can take a few days to cause an issue and some happen near-instantly. Things like brain fog, tiredness, irritability, kidney pain, nausea, vomiting, bad breath, sweatiness, shaking, dark urine, and sickly-sweet smelling urine can all be signs that someone with HFI has consumed something that has a bad sugar.

After your week, you might be antsy to try some new stuff. I've made a pinterest board of safe foods (please be sure to read my notes on ingredients to leave out or modify).

I also have a list of links that are useful here: fructose-free.tumblr.com/resources

Please check back with us and let us know how you're doing!

[charlie]

Hi Matthew,

another idea for you is that you have the same problem as my daughter, she has fructose malabsorption but also Congenital Sucrase-Isomaltase deficiency, and alot of how you feel is also what I notice with her. The only thing is usually they have diarrhea which I see you don't.

Some with FM can tolerate sucrose but others find that is still too much for them but with the CSID then that rules out the sucrose too. But with both conditions you don't tend to get the natural aversion in fact sometimes the body craves it.

Sometimes you only get answers by complete elimination.

The reason I wonder if you need to look at other options: You say you have had the problem for 10 years, do you know if you used to be able to eat other foods ok and it has come on later in life or you have always had a problem with fructose containing foods.

[OklahomaMatthew]

Thanks a lot Colormist. That's a very big help. I will check back in. My doctor wouldn't even refer me to a geneticist. He said he would retest me with a hydrogen breath test(which i declined since i already had that test and tested positive) and to see him again in 3 months. Um...I don't think i will be going back to him.

[OklahomaMatthew]

Oh, i just saw your post Charlie. I asked my mom about my younger years, and the only thing she noticed was back in Kindergarten they would give us chocolate milk. She had them give me regular milk because she noticed they gave me headaches. And i remember being really tired throughout high school, had some lightheadedness...but i never noticed any of the other symptoms until i was about 19 years old. At 19 years old my stomach started giving me problems that seem to have progressively gotten worse. The biggest problems being feeling very full, and constipation, hardly any diarrhea. Back at 19, the right side of my abdomen would feel tight usually at the same same my stomach felt full and was tender to the touch, so i though it must be part of the digestive tract, until the mirror side started feeling tight with a touch a pain. Now i figure that must be the kidneys, because it's gotten worse over the last year to where after a meal sometimes it will feel like my side would hurt if i ran too far. And I'm thinking the strain on my kidneys is causing me to be dehydrated, and that the dehydration is causing my dry mouth, constipation, and some migraine headaches. I've started drinking lots of water when i get the headaches, and it seems to noticeably help.

[OklahomaMatthew]

Charlie one thing you wrote in another post sparked my interest...

"However if she only has a little as in the other day when she sneaked a nibble of a sweet she is hyper and manic for the next few hours then OK for 2 days. On day 3 she starts to go downhill, disturbed sleep and cries and screams and throws tantrums, Over that day she goes pale and has less and less energy. This then lasts several days and gradually gets better. "

...because I've noticed a similar pattern, but have not been able to attribute it to something i ate yet. I would definitely like to know more about CSID. i had never heard of it before. I've been thinking about how potato chips give me problems(i had kind of thought it was the grease), and i know of multiple times that i ate steak with a bakes potatoes it gave me a problem. Maybe it's the starch. I was also realizing that over the last 4 weeks when i have tried to seriously limit fructose i have turned down rice every time that it was offered to me, even though i like rice and know that it is safe in regards to fructose. Possibly i have recognized rice to be a problem. I know that a couple of weeks ago i ate bread sticks made from tapioca starch(no sugar) as an appetizer and i was out cold on the couch before the dinner even arrived. I tried eating popcorn exclusively and after a day and a half i had to stop because i got a bad headache.

Charlie what other info do you have? Can you tell me more about what symptoms your daughter was experiencing? Or what would be a good CSID safe elimination diet to start with? Thank you so much for the help.

[charlie]

Hi matthew,
sorry I would have posted more but my computer kept crashing so I lost the will......

If you think you didn't have problems with fruit and veg and sugars until you were 19 then less likely to be HFI, your mother would probably have had concerns quite early. CSID is also congenital but there are people coming on the facebook page reporting they were fine until teens or later, so I think there is a secondary condition which is aquired either due to hormone changes, stress or a gastric assault of any sort. Or you used to produce enough enzymes but for whatever reason they slowed down. There it would be more likely a Disaccharidase Deficiency.

We have also voiced before the possibility of an aquired form of Fructose Intolerance too, less severe than HFI in that you don't get the liver complications but more severe than Fructose Malabsorption.

anyway, here are a few very good sites on CSID:
www.csidcares.org
www.csidinfo.com

There is also a very good facebook page, CSID Parents & Patients.

Have a look through, see what you think and come back with any questions.

I have posted an elimination diet process on a proboard for FM that I have built up but it isn't very active but may give you an idea how to plan your own.
fructosemalabsorb.proboards.com/thread/193/elimination-diet-indicate-fm

[teri a.]

hi matthew,

u are the first person in the 5 yrs that i have been researching my progressive condition, who so closely mirrors my own experience. im 53 yrs old. ive written down my personal experience so many times that when i read yur's it seemed as if i was reading mine. finally, another verification that im not crazy. thank u so much matthew for sharing.

i had migraines till about the 3rd grade, still have motion sickness, and had a pattern of queasy, sweating, & weakness relatively soon after eating, since before the 3rd grade. over the years those episodes got worst; (now i know that they were episodes of hypoglycemia) to the point of becoming debilitating, i couldn't walk. the main point is that i could tolerate sugar and didn't have an instinctive aversion to sweets. while a teen, i cut down on sweets (pop and candy) for general healthy eating. i didn't connect the early queasy and weakness and the later adverse progressive symptoms to sweets until my forties.

i had an traumatic accident in 1997 (at about 36 yrs of age) and after that it seems the multiple symptoms began and increased in severity over the years. my symptoms included severe night numbness, profuse sweating, intense colon pain, bloating, queasiness, i never vomited, diarrhea more so now and constipation earlier. before i understood which foods had fructose and whether or not i had fm or hfi, i would get these severe swelling like headaches. i developed hypertension and my blood pressure always goes up when i seemingly have foods with sugar/fructose/carbs. my knees got so bad that i had to do the handicap train stations with elevators/lifts and escalators.

as charlie mentioned tho, im apparently having more issues than the classic hfi symptoms and reactions. i also, as i mentioned did an elimination diet in 2009 and discovered that some of the symptoms were due to gluten sensitivity; which seem to manifest after the trauma. at this point i try to avoid anything with sugar (lactose, fructose, sucrose, possibly glucose???) or gluten or carbs; so no fruit, vegies, wheat, oats, rye, barley, corn, rice, potatoes...ive tried homemade, and nothing works. im learning that foods stated with no sugar an't necessarily so. appaently, 0 grams dn't mean no micrograms of sugar. my weight is below 100lbs and im not sure how much lower it can go without developing more complication just from being malnourished. i believe i tried black tea, but sometimes it's hard to keep account of everything. i have documented how certain foods make me feel and tried to differentiate the gluten symptoms from the lactose symptoms (the milk products seem to produce an allergic response in addition to bloating and such) from the fructose/sugar/carb symptoms.

i was a 3 sport athlete (division 3 school), a double major college student and very very active, like hyper energetic. I was this way seemingly until 1997 when i fell and was misdiagnosed with muscle spasms. it turned out i was bleeding internally (lung)for a month and a half. the most striking energy depletion occurred in 2006 and i just couldn't do anything, slept all day. thought it was just depression. i appeared to get better since late 2009 as i removed gluten and fructose from my diet. i would have spurts of moderate to very low energy. as time has past, i mostly have low to little energy and fewer & fewer moderate to high energy moments. currently, i have to negotiate what activities/chores i may do & i can feel muscle fatigue from just raising my arms.

im also looking into scid per charlie's suggestion; in addition to looking into mitochondrial disease because my grandmother died of a mysterious muscle deterioration condition.

mostly, i thank God that i found a person who as an adult, manifested these symptoms & who did not have an aversion to sweets growing up.
thanks matthew

to colormist, thank u for sharing this...
[Hi Matthew,
There are a few of us on this board that do not have a distaste for sweet foods. I, personally, would love to eat berries of all kinds. I do not enjoy super-sweet drinks like soda pop and fruit juice or super-sweet foods like icing. I knew I didn't like sweets when I was in my mid-20's, but I didn't know why. My friends suggested it was a mental issue. With THAT advice, I started doing serious research. I found my diagnosis when I was 27 and had read lots of horrible things about people not being taken seriously by their doctor and getting bad medical advice. I made an appointment with my doctor, told her I was pretty sure I had HFI and asked for a referral to a doctor that could perform genetic testing. My doctor believed me, the geneticist I was referred to did not believe me, BUT he was willing to rule out HFI first. He said that I would have died as a baby if I had HFI and did not have a diagnosis. The geneticist apologized when the test came back positive.]

[charlie]

The good news, if it is CSID is that in America you may have access to Sucraid, enzyme replacement through your health insurance, and buy starch enzymes which will open up your diet options massively.

[Logan Hudson]

Wanted to ask about exhaustion. I have hfi and lately I’ve been exhausted almost always, any dietary options to help bring back my energy?

[colormist]

Opt for slower burning foods. Protein and fats burn more slowly than carbs. I have that same issue. I just recently found a few meat sticks that are shelf stable for energy on the go. (Chomps are my current favorite) A meat stick and a snack-size serving of cheez-its is just the right amount of energy to keep me moving, but I have to remember to eat before the hypoglycemia kicks in. If I wait until after I'm already shaking, then I'll need to sit down and wait for the food to kick in. At that point, I might as well just eat a proper meal.

You might also be exhausted because of hypoglycemia. Double check you foods to make sure you're not eating something with "natural flavor" or "vegetable broth" or other things that might be contributing to you energy depletion.

[Doyle Smith]

I have sucrase-isomaltase deficiency. Similar to HFI but different. Anyone else out there have this?

[Rose]

I'm hoping some of you may be able to help because I am at a loss. My OB-GYN has HFI, and she suspects that I might also. We sent in my bloodwork (to Natera in Texas), which came back negative, but they also only test three genes, which covers only about 70% of HFI cases.

What's your non-medical opinion? These are my symptoms:
If I eat a small amount of fruit (one grape, one raspberry), within 10 minutes I have intense brain fog/confusion, abdominal pain, fatigue, and sometimes hypoglycemic symptoms (shaky hands, slow speech, chills, fatigue.) But I also get brain fog after eating lots of stuff, just fruit is the most obvious trigger.
These same hypo symptoms happen during or after I exercise (usually running a few miles.)
I've worn a blood sugar monitor, but my blood sugar is not actually low.
I am almost never nauseous and don't vomit when I eat fruit.
I've had four ER visits recently where sudden I feel super nauseous, cold, then I start shaking uncontrollably where I can't sit up and can barely talk. These usually last about 30-60 min, and then they pass.
Also, some of my liver numbers are high.

Most of these symptoms are adult onset. Although my Mom says I was very spacey as a kid, I don't remember feeling sick much. I would often eat a few apples and oranges a day. And in the summer, I would eat a container of blueberries in one sitting. Even recently, I would still eat a whole piece of fruit even though I reacted because I love it so much.

Is it possible to have HFI if I ate so much fruit as a kid?

Is there anyway to test for other types of HFI that are not covered by the main three-gene test? (I live in the US.)

Thanks so much for your help! I honestly am not sure what to do next.

[colormist]

"I've worn a blood sugar monitor, but my blood sugar is not actually low."

I was having some pretty bad hypo-shakes and my blood sugar also registered as normal. I think this is because the liver is hoarding the glucose but not actually doing much with it. All the hoarding causes the brain fog and shaking because your brain is not getting all the glucose it needs to perform normally.

I also ate fruit as a kid, but I'd prefer "sour" fruits--unripe apples, sour/wild cherries. I'd always try to eat fruit, but could never manage more than a slice or two of apple or half an orange before I'd feel like I didn't want anymore. I'd also eat sugary candies, but I'd prefer the glucose-based ones (sweettarts, smarties, pixy sticks, laffy taffy, gobstoppers). I'd still eat the sugary ones, but not as much or as many. I WOULD get sick if I drank soda pop, fruit juice, or ate a whole candy bar and I would actively refuse sugary drinks as a child.

Have you tried the elimination diet? I didn't become super-sensitive to fructose until I removed it completely from my diet.

My genetic test was sent to Baylor in Texas. My test was performed by a geneticist who assured me I didn't have HFI because I would have died as a kid.

By all appearances, we look healthy on the outside but we can tell something's wrong on the inside.

[ukbill]

Rose HFI is a condition from birth its not something that can come on at a later stage.
Its possible you have really bad FM (Fructose mal-adsorption) or even CSID (but I think that is also form birth)
Avoiding eating Fructose is not a problem.
There is a common misconception that you HAVE to eat fruit and Vegetables.. this is not accurate or even close to being in anyway true.
The Nutritional value of say and 8 oz Rump steak is far more than you could possibly eat in one sitting of any amount of vegetables!
If you do go veg /fruit free all you need to watch is
1/. vitamin C, you will need to take a supplement
2/. if you are of child bearing age Folic acid supplement is essential just in case you accidentally or plan to get pregnant.
3/. Our Diet is very low in Fibre so eating a high fibre supplement such as psyllium husks is a very good idea.
4/. A full range of meats (red and white) diary and fish needs to be eaten.

I hope this helps?

Keep smiling

[Rose]

Thanks Colormist and Ukbill. This is very helpful. I am actually expecting a baby currently, and also am not able to eat gluten or dairy, so I'm finding the elimination diet really hard to do.

I guess Ukbill mostly answered my most pressing question. But do you think there's any way that someone with HFI could eat a peach, an apple, or a couple slices of watermelon (all of which I've done recently on a regular basis) and have symptoms that are fatigue, brain fog, abdominal pain, and some hypoglycemic symptoms (cold sweats, confusion, shaky hands, blurry vision etc.)? Or do you think that if I had HFI my symptoms would be more severe (vomiting, for example?) Do you think that the fact that I ate fruit and sweets as a kid, and never really thought twice about it, rules out HFI?

I ask because since my HFI test came back negative (which covers 70% of cases), and since I am pregnant, I am reluctant to go on a full HFI diet (especially without gluten and dairy) unless I think there's a chance that I could actually be HFI. I mean, I'm pretty sure that I should stop eating fruit at the very least, given my symptoms. But if I thought that I really could be HFI, I would try my best to do the full diet...which I think for me would be meat, fish, eggs, and oatmeal...right?

Thanks so much for your help!

[colormist]

Sounds like your symptoms post-fruit-eating are hypoglycemia. Your liver is stealing all the glucose from your brain. This can cause HFIers to go into a coma.

I ate fruit as a child, but I always would prefer more sour fruits and unripe fruits. I didn't vomit very often (unless it was from motion sickness, then it was rather regular), but I'd often have a belly ache and would decline sweets but cave to peer pressure.

Have you had your liver function tested?

Your diet can also include white rice and rice products.

Do you have celiac's disease? Are you lactose intolerant or allergic to milk products?