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Post by Deb on Feb 16, 2012 18:00:07 GMT -5
Hi everyone
First of all, thank you for all of you who post- you are such a wealth of information and it is so appreciated in a world, where even with the internet at my fingertips, finding out what I want to know about fructose metabolism disorders has been so challenging.
I frequented this board a few years ago when my daughter began having problems with fructose. She had always been bloated and seemed intolerant to different foods, never slept through the night, etc - but it all came to a head after she got a very bad stomach virus at three and a half. After that she seemed unable to tolerate anything-- and eventually we started to figure out that it might be fructose. She was administered the hydrogen breath test by our local children's hospital (in Canada) and her results were off the charts- higher than anything they had ever recorded. Because of her results, together with very high levels of reducing substances in her urine and my reports to them that she seemed to be having frequent hypoglycemic episodes, they were strongly considering HFI, so she had the genetic test out of Boston. The results came back negative, so they told us that she had a bad case of dietary malabsorption and to just reduce her fructose moderately and she would probably outgrow it eventually. So we reduced her fructose (but didn't eliminate it) and for the last two years she has had periods of relative wellness interspersed with very difficult periods - the difficult periods are triggered by her getting sick with a cold/flu that involves fever, or with the stomach flu.
Which brings us to the present - she got a particularly nasty flu this past December and since then, she seems to be back to where we were a few years ago - completely unable to tolerate any fructose. When we are extremely vigilant, she is great-no stomach pain, rashes, hypoglycemia etc. But if for example, I mess up and make her salad dressing with balsamic vinegar instead of red wine vinegar, she has an immediate reaction and isn't well for the rest of the day. She hasn't previously had an aversion to sweets, but is possibly developing one as she is telling me more and more frequently that she can't eat something after tasting it and feeling that it tastes too sweet. From what I have read, the aversion to sweets is often missing in FBPase deficiency and is more characteristic of HFI, as is her pattern of having very bad flare-ups following illness- can anyone share their experiences with whether or not they experience more severe symptoms following episodes of illness? We have been re-referred to children's hospital but it could be months before we are seen - in the meantime I am trying to figure out as much as I can as I know that I will have to advocate for proper testing for her. Also, she seems to only be able to tolerate small amounts of dextrose-- she can have rockets (smarties) for example, but larger quantities (like in baked goods I have attempted to make) don't seem to go over that well...Any thoughts, advice, would be very appreciated!!
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esmee
Full Member
gluten, lactose, fructose, histamine, and salicylate intolerant
Posts: 236
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Post by esmee on Feb 16, 2012 19:14:53 GMT -5
I do think viruses can play havoc with our bodies. I had a terrible flu in 2000 and I have never been the same. I now know why people actually died from the flu. I think that localized fevers in the body can damage the tissue involved. For me it was my right sinus cavity, right nasal passage, blood vessels in my right eye. When I eat things that do not agree with me, all of the tissues in these areas become inflammed now.
Many people with Chronic Fatigue Syndrome (believed to be caused by an unknown virus) have terrible problems with there digestion and have many food sensitivites which never existed prior to developing CFS.
As far as your daughter's diagnosis is concerned, you must remember that the genetic test is NOT diagnostic. If it comes back positive, then it is definitely positive. But if it comes back negative, this does not mean that you do not have HFI. For 20% of the people with HFI, the test comes back negative because they have a mutation that has yet to be identified.
Fred, who is a long-time member of this board, has severe HFI, yet the genetic test only found one copy of a known mutation. You must have two copies to manifest HFI, so he obviously has a copy of another mutation that remains unknown.
Deb McQueen (a former participant of theis forum) and her children had severe health problems that were manifesting as Ehlers-Danlos Syndrome (a connective tissue disorder) among other problems. They all tested negative for HFI through genetic testing, but liver biopsies confirmed an absence of aldolase B enzymes. When they stopped eating fructose, the EDS went away.
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Post by charlie on Feb 17, 2012 4:49:58 GMT -5
Hi Deb, welcome back, join us as a member and become a full part of the community. As Esmee has said not all gene tests are a true negative which is very frustrating, I think they are starting to work this out more and more. Or is there an in-between disorder of HFI/FM which affects at a different level. Join us too on www.fructosemalabsorb.proboards.com too as there are several on there that are debating exactly that issue, do we have kids with very severe FM or undiagnosed HFI. In fact we were just discussing yesterday this cold/flu symptoms associated with fructose ingestion as Meg definately has that as it would appear does another child. How do we solve it, well we need to go with our instincts and restrict the diet as we as their parents feel is necessary for a safe and healthy life and hope one day medical science catches up with us. Megans breath test blew the graph too but they still say it is malabsorption. |
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Post by colormist on Feb 17, 2012 8:48:58 GMT -5
Hi Deb,
Have you ever thought of having a sample taken on her liver to see if she has the enzyme needed to process fructose? It's the only way to get a definite diagnosis for HFI. The genetic test only tests for known genes that contribute toward HFI. It's a fairly invasive test, so if you don't want to do it, that's completely understandable.
We're also bouncing around ideas here about heredity and what region your ancestors are from. A lot of members have reported in northern European ancestors, if you have ancestors in northern Europe, then that might be a clue toward HFI.
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Post by Deb on Feb 18, 2012 2:39:50 GMT -5
Thanks everyone for your posts. We are seriously considering the biopsy -- albeit with hesitations as she is only 6 years old and we worry about the effect of the procedure on her physically. It is a low-risk procedure, but just going through that would be very taxing I think. But we are concerned about going forward "in the dark" - knowing that she does much better with limited fructose but not knowing if we are doing her harm by letting her "test the limits".
Given the imperfect nature of the genetic tests, it does seem like the only surefire way to know what's going on is to do the the biopsy...
Thanks Charlie for that link to the other board--I had a look and there are some very interesting posts there. Makes me wonder how many out there (especially children) have been diagnosed with FM when it is really HFI or FBPase deficiency. I just don't see how else you can explain the hypoglycaemia connection- there is no biological mechanism by which FM would cause hypoglycemia, as far as I know...?
On another note, do any of you (or your children) experience any symptoms in the bum region after ingesting fructose? I know that may sound weird, but my daughter complains that her bum is irritated after she eats something that doesn't agree with her - and sometimes it is visibly red down there when she is complaining of it (even if she hasn't had a bowel movement-- it just seems to "turn" that way -- like a skin/mucosal reaction?)...I always associated that with more of an allergic type of reaction, so I was just wondering if anyone has ever heard of this being related to fructose ingestion? Thanks for the support and advice ;-)
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Post by charlie on Feb 18, 2012 5:26:45 GMT -5
Hi Deb,
To test or not to test- that is a good question, especially when they are young and vulnerable. As parents we like to know why our child is doing what they are doing so we can know how best to help them but how much do we put them through to get that answer, especially as some tests can be dangerous. But if we don't know then we never know whether something else is going on that can be helped with the right diagnosis.
I have held off more definitive testing with Meg but now we are back to square one as all her symptoms have got worse since increasing her fructose consumption with the FM diet so what do you do first. Cost and convenience has to be the biggest decider. The gene test is the easiest and least intrusive test so maybe if possible do that first, just incase. I don't know where in the world you are so I don't know if that would cost you much. Then if that doesn't come up with something maybe the liver biopsy is the most conclusive answer, then you can plan and move on with appropriate diet without guess work.
As for the bum symptoms, one of the first signs Meg is on a reaction is she goes very red and sore (as well as irritable temper+++) around her bottom, I guess the acid level is higher.
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Post by charlie on Feb 18, 2012 5:51:37 GMT -5
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Post by tikitavi on Feb 18, 2012 6:28:19 GMT -5
Deb, I do have those symptoms that you mention when I ingest fructose or sucrose. A literal "pain in the ^%$". It gets irritated and painful.
Charlie, I have the irritability/bad temper too! It's like Dr. Jekyll/Mr. Hyde. It's one way for sure that I know I've accidentally ingested fructose/sucrose!
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Post by Deb on Feb 19, 2012 1:30:33 GMT -5
Thank you for sharing....and for that link re the FBPase-I came across that very recently as well, and thought it was interesting that for FBPase it was mentioned that half of the cases are diagnosed in infancy and the other half quite a bit later, usually following an illness...
I really appreciate all of the support on this site -- I must admit, despite seeing improvements in her behaviour, stomach pain, blood sugar swings, and overall health, it is hard to wrap my head around how hard it is to follow the diet...She has been expressing her frustrations a lot more lately with questions like "mommy, why does EVERYTHING have to have fructose in it!!" and "what is wrong with me - why does this keep happening?" when she has reactions....I imagine that eventually, it gets easier....especially as I get better at identifying everything that she cannot tolerate....
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Post by charlie on Feb 19, 2012 6:37:26 GMT -5
It is a hard diet to follow, yes, but hopefully the longer your daughter feels better and the older she gets the more she will see how important it is to follow.
Its a case of rethinking about food and making safe foods fun and as varied as possible. I am very lucky Meg is not a fussy eater, and as there are just the two of us I tend to only have safe foods in the house now and just serve the food without debate. But it is hard, especially socially so sometimes it just has to come down to damage limitation and clear the system out later.
As to why and explaining that, do you have anyone else around you with an obvious problem. We have a little boy next door who was born without the femur bone shaft in one leg, he has the two joint ends but no main bone so his leg is considerably shorter. Its not genetic, it just happened in the womb that that group of cells didn't develop so I liken her problem to him, he has to wear a prosthesis to get about, she has to limit her diet to stay healthy. That does seem to help her at the moment to handle it, she isn't the only different one.
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susan
Full Member
CONFIRMED HFI
Posts: 114
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Post by susan on Feb 19, 2012 9:13:21 GMT -5
Explanations to children?
Perhaps something is wrong with altering food away from how it was originally designed.
Or...
At one time food could not be transported great distances...so our bodies adjusted to the food most available to us.
I also like the idea of compassionately showing and explaining that everyone is made differently by their Creator...such as the child with the missing bone.
Perhaps our mission is teach compassion for others.
Susan
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Post by tikitavi on Feb 19, 2012 10:17:08 GMT -5
Susan, I really like your comment about how we had to eat foods that were most available to us! That makes the most sense for me, and I often use that similar idea when explaining my intolerance to friends. But also your comment about how science has bioengineered food is very good too. Certainly we have bioengineered apples, for instance, to be far, far, bigger and sweeter than what our ancestors would recognize as an apple! But going farther back, our previous ancestors (in Northern climates) didn't even have apples. So that reasoning (us being adapted to eating what was available locally) is the one that really reverberates with me most! And it helps me not only keep on track, but feel a real biological connection with my ancestry!
The child with the missing bone is an interesting way to explain it too. I agree, it does teach compassion and understanding, which is always a good thing!
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esmee
Full Member
gluten, lactose, fructose, histamine, and salicylate intolerant
Posts: 236
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Post by esmee on Feb 19, 2012 15:00:02 GMT -5
Yes, to echo Susan and Tikitavi, none of the foods we eat today really resemble their wild counterparts. The meats are over fat and contain the wrong kinds of fats, the vegetables are less bitter, and the fruits are ALL way, way sweeter than anything found in Nature. Everything is been hybridised. Very few foods would have contained any apprciable amount of fructose and, the ones that did, would have only been available for an extremely short period of time.
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Post by ukbill on Feb 19, 2012 16:41:21 GMT -5
Also the amount of Fibre we all eat is far far lower now than any time in Human history. Fibre is the control for the digestion of Fructose and Carbohydrates I think as well. Too little Fibre = too fast breakdown of Fructose (for those who can of course  ) |
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Post by Tammy on Feb 19, 2012 18:08:28 GMT -5
Hi Deb.
Dealing with the kids questions is a hard part of being a parent with HFI. This is one of the reasons that I always support finding safe alternatives to make any food they want. I highly think it good for the childs well-being to use the HFI-safe sweeteners and create what will make the child feel like they are fitting in with their peers. They want to be the same. They don't want to stand out.
If you restrict them and give them no options, they will find a way to go off the diet and eat things that will cause them problems. It's much better - regardless of what Bill will try to tell you - to find a way to use substitutes for them.
They will be healthier, will feel better, and will learn to trust you to keep them from getting sick, while also learning at the same time to manage the diet on their own.
Tammy
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Post by ukbill on Feb 19, 2012 22:22:00 GMT -5
Taken from an article called Hereditary fructose intolerance, published by Manir Ali, Peter Rellos, Timothy M Cox. University of Cambridge, Department of Medicine, Level 5, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK M Ali* P Rellos T M Cox If the undiagnosed infant survives the difficult initial period of weaning, the child usually develops a self-protective aversion to foods which cause distress.6' Voluntary dietary exclusion, which is refined by trial and error over a life time, includes restriction of most sweet tasting foods. There is no alternative.. I really wish there was.  I rest my case. |
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Post by Tammy on Feb 19, 2012 22:37:08 GMT -5
Bill wrote: If the undiagnosed infant survives the difficult initial period of weaning, the child usually develops a self-protective aversion to foods which cause distress.6' Voluntary dietary exclusion, which is refined by trial and error over a life time, includes restriction of most sweet tasting foods. There is no alternative.. I really wish there was. I rest my case. BUT The difference is that once we know we have options. We can help that child learn and understand. We don't need to let them live all alone in their confusions. That was not an option in this report. Besides, this report is fact and cannot be disputed - why? Because it was written by the medical community? Is this the same medical community that knows soooooooo much about HFI? |
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Post by Deb on Feb 21, 2012 1:42:05 GMT -5
I agree of course that our Western diet has strayed so far from that which we were originally designed to consume- I will try explaining that in terms that my daughter can understand - thank you for the suggestions  On a day to day basis, though, she definitely wants to eat things that she can't. I think that perhaps because she weaned so late in toddlerhood and then we limited her fructose intake thereafter, she didn't develop an extreme aversion to sweets--or --if she has fdpase deficiency and hfi that might explain things as well, as the fructose tolerance in fdpase deficiency is supposedly higher than in hfi, at least in between "episodes of illness". In any case, I found medical grade dextrose and she has been doing well with small quantities of that - but it is going to send me to the poorhouse sooner rather than later! 20 dollars (Canadian for 300 grams--ouch!) Just wondering something on a different note-- with all of the "hidden" sources of fructose, how does one manage going on vacation? Now, just to leave the house, it is quite a feat just to pack enough food to last both kids for the afternoon! We have been contemplating a trip to Disney later this year, but I just can't even fathom how that would be possible....Any advice? |
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Post by colormist on Feb 21, 2012 9:16:42 GMT -5
Hi Deb,
I went to Disney World a few years back and did fine on their chicken strips and french fries--those are found just about everywhere. There's a delicious fish & chips stand in England in Epcot. And some places have pesto pasta. It is possible to find safe food, you just have to be vigilant. Also, lots of people have luck talking to the cook and saying "grill plain, unseasoned chicken and mashed potatoes" or whatever you think would be easiest to find at that restaurant. Plain hamburger should be pretty easy to find, too.
I have been reluctant to travel outside of the US--especially to non-english countries (since I'm not fluent enough to communicate about HFI), but inside the US I can almost always find something edible.
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Post by Tammy on Feb 21, 2012 9:45:19 GMT -5
Hi Deb
We've also been to Disney. We had a great time. I didn't try the fish and chips place, but there were burger joints everywhere that you could just get plain meat without the bun.
We also made reservations for the evenings. We would let them know when we booked the restaurant that we'd need to see the chef. They were wonderful. We didn't try to explain the whole thing, we just said that she had an allergy to sugar. Today, the word allergy gets everyone's full attention. I think it makes it easier for others to understand how important it is. They were more than willing to help and make her something special. Usually plain chicken and mashed potatoes worked for us.
Regina never developed an adversion to sweet tastes either. She doesn't like things as sweet as I do, but she still likes her sweets. This is another place where everyone is different. I have always used dextrose and artificial sweeteners. I always adapt everything I can to make her as much like everyone else that I can. It helps with the "why can't I have it?" quite a lot.
The funny thing about it is a lot of time, she doesn't really want it. I make her a birthday cake every year. Lately just cupcakes because they work better. They freeze well. When she goes to a birthday party, or a wedding, we just take one along for her. She's happy because she has one, but usually we end up throwing most of it away because she'll eat a bite when everyone else is eating it, and that's good enough. Christmas cookies is another one. We HAVE to make her some cookies every year. She'll eat a couple that day, we have to put them out for santa, the rest go in the freezer. She has access to them anytime she wants. She can just get them herself. And every year, we throw last years away when we put this years in....lol. But we HAVE to make them.
You may find the same will be true for your child. They just want to fit in and be the same. Regina doesn't like Iced Tea - unless she is playing tea party with her friends. Then it HAS to be tea. And of course we sweeten it with Equal because her friends will only drink it that way and they all want to share the same teapot.
When you find what foods do and don't work then you can start adapting things to make her life easier. I think that's just as important as the diet itself.
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