A girl with gluten, fructose, & lactose issues

[kim55]

But I have to add, I am already pleasantly surprised by the number of web sites that already mention HFI if you google "fructose intolerance." We just need to get that phrase on other web sites too. A good place to start would be with some of the articles that discuss high fructose corn syrup, since that is one of the current big food controversies and articles about it are appearing in mainstream media. We could make a point of writing letters to the editor and posting responses to online articles mentioning that for a small group of people, there is definitely a danger in adding HFCS to foods.

[ukbill]

Kim I like the idea.

I suggest we start a new thread where we can list the sites and articles we have posted comments on.

It might be best if several people post on each site or article to illustrate there are quite a few of us.

I know the BBC count each comment or letter (e-mail these days) as coming from 100,000 people because only 1 in 100,000 people can be bothered or have the time to write in or call if they feel passionately enough about a subject.

[ukbill]

I know of one case recently where the Doctor suspecting HFI put the baby onto a formula that contained 46% "corn Syrup solids" because the doctor did not know it was very high in Fructose!

We managed to get the child off the formula before he died although he has a lasting fatty liver problem we are hoping he will fully recover with time.

So even Doctors are not immune to being confused by the naming structure of sugars!

[hfimomof3]

I agree with you that clear labeling would help us a lot. Phenylketonureics get labeling (for phenylalanine) and presumably that is also a rare condition so it seems like HFI ought to get the same courtesy, especially as doctors are unlikely to have heard of HFI so people who have the condition are often relegated to having to figure out what to eat without being able to go to expert advice.

In particular I think there ought to be a sucrose-free formula available. This came up when my oldest was a baby and I was pretty shocked that standard baby formulas were not fructose-free. I will ask on the allergy board whether the specialised infant formulas (eg Neocate, Elecare) are fructose-free. Of course, those are extremely expensive so not an easy solution for babies with HFI who for whatever reason cannot be breastfed.

It is really not that easy to get human studies done. You have to have someone design a study, go before an Institutional Review Board to ensure the study is worth the effort, safe for participants, and ensure that peoples' privacy is maintained, and so on. This makes it all very expensive and time-consuming. If we want a study done then probably the best thing to do is to have someone (probably Fred?) get into contact with the fellow who studies HFI at Harvard and tell him we are the people who have HFI and this is what we feel like we need so can you consider incorporating this somehow into your next grant?

So then the question would be, what do we feel like we really need studied or what would benefit us? I agree that it seems like the true frequency of HFI coudl be more than the 1 in 10 000 that is reported, because maybe there are people who are never counted because they have sublinical symptoms or because they just self-help by selecting what they eat without ever seeing a doctor. But, if the true frequency were more than 1 in 10 000, then woudl that realy help us? If the true frequency were even as high as 1 in 1000, then manufacturers still woudln't have any reason to cater to our needs.

One thing to ask woudl be whether HFI shoudl be something that is tested for in newborns. In my state, a number of tests are done in newborns to identify various metabolic disorders. I am not sure if HFI is one of those but it seems like somethign that we could ask about (I'm not sure who we would ask), since it is easily fixed via diet but can be lethal if not identified early. But that makes me think they probably already do test for it.

If you could see a human research study done in our area of interest, what else would you want to see studied? What are the areas where you feel that not enough is known about HFI?

[hfimomof3]

Here is an article about a woman who went undiagnosed until age 50.

www.ncbi.nlm.nih.gov/pubmed/19452588

[hfimomof3]

www.ncbi.nlm.nih.gov/pmc/articles/PMC2954661/?tool=pubmed

Here is an article from Dr Tolan's lab at BU (sorry, I was incorrect, he is not at Harvard). My conclusion from this is that newborn screening is probably not done at this point because they rae still trying to figure out the range of possible mutations.

[julienc]

I think the biggest issue is increasing awareness. If parents, let alone doctors, were astute enough to know that HFI could be a reason for FTT, then that's a big piece of the puzzle. If an infant is having troubles, an astute doctor should be able to immediately identify HFI as a potential cause and call for proper testing, or at least eliminate it from the diet and see the response. This is where labeling would be hugely important. "Fructose Free" identification on all foods, especially infant formula, would be huge.

I went through a looooong conversation with my geneticist about testing and opted not to test my own newborn for HFI. It's a complicated test due to the different genes/mutations that cause HFI, and the results can be somewhat ambiguous. (Not all mutations have been identified.) The article linked above explains this quite well. But, the key for me is that I am about as clued in as anyone can be on HFI and the symptoms to look for, and obviously knew it was a possibility for my daughter. Virtually no one has that advantage since HFI tends to crop up unexpectedly w/out any family history.

So, I guess bottom line, awareness and food labeling would be my biggest push, over testing/screening or any further studies.

[hfimomof3]

Regarding food labeling, the FDA evidently has a minimum frequency of people who have to be affected, in order to require labeling for that condition. So, if you are allergic to milk, then yay, they label for that because lots of people are allergic to milk. But, if you are allergic to sesame seeds, then they don't require companies to label for that because it is not a documented allergy that is very frequent. The documentation is key. You have to do a study that shows that the frequency of your condition of interest is above the threshold for labeling. So, as i see it, an easy genetic test is probably going to be needed in order to determine how many people in the population are living with undiagnosed fructose intolerance. If there was an easy genetic test, then it seems likely that they would use it on newborns because, like other metabolic insufficiencies, it can be lethal if not identified early. If they coudl test newborns, then we woudl start to have a better idea of whether there are lots of people with undiagnosed HFI who would together meet the frequency criteria for food labeling. So, to my eyes, genetic testing seems like a high priority.

We know that they label for phenylketonurea and I don't know if the frequency of that is high so maybe we could figure out what law was used to get labeling for that.

[ukbill]

sounds exactly what we need to be doing to me.

[hfimomof3]

I also think that there ought to be a standard protocol out there for management of kids who are suspected of having HFI. It is one thing to say well you have to consult your physician but as we have observed here this is a rare disease and (a) physicians have often never heard of it and (b) even if they have heard of it, they may not know how to manage it. So let us make a list of the information resources that WE think ought to be out there for people with HFI. We cannot put these resources out there because we are not doctors. So we need someone who is qualified (perhaps Dr Tolan, maybe we could get together and send him a petition or something) who can put together some detailed information sheets that doctors could use.

First of all, there ought to be an infant formula that is labeled as safe for HFI. When my oldest was a baby, there was a brief concern that he might have HFI (he didn't) and the pediatrician in the hospital went looking for fructose-free infant formula and came back with a formula that contained sorbitol. We should be able to do better than this.

Second, I think we need a standard protocol sheet for school-aged children who have HFI. This should include things like an emergency health form that lets the staff know that the child is not to be fed or injected with anything containing fructose, sucrose, or sorbitol, if there is a need for emergency medical care. Also, shelter-in-place kits for that child's classroom should include food that is fructose-free like safe crackers or shelf-stable milk, or whatever is chosen by the parents.

What else do we want, in terms of documentation and standard practices that would make it easier for doctors to identify and manage their patients who may have HFI? This condition is fairly easy to manage with diet, unlike other conditions like diabetes that require expensive medications. So I feel that it is not a lot to ask taht the medical powers-that-be get together and agree on how to manage us so that chldren who coudl EASILY be helped are not instead harmed out of mere ignorance.

[ukbill]

I like it all!

I have been horrified by Doctors giving the Hydrogen breath test to children when they suspect HFI.. it will give a negative but stress the liver seriously with no positive outcome possible. This is very bad practice!

I like everything you are saying.. it is so much more precise and professional than my approach would be how about starting a new thread so other HFI's will possibly get involved?

Something along the lines of "Standardised advice for Professionals and Schools"?