New diagnosis Poland

[dorota]

Hello there
I'm a mother of a 7-year-old daughter, Maria, just diagnosed with HFI through genetic testing. I'm Polish, living in a small village in south-eastern Poland. At the same time my nephew, 16 years old, has also been diagnosed with HFI. I guess it was for a student of mine (I'm an English teacher) who said, after I described my daughter's aversion to anything sweet, that I must search for the underlying cause. I started digging the internet, found the description HFI and finally convinced my family doctor to send a letter to the Children's Memorial Hospital in Warsaw asking for genetic testing. My sister-in-law did the same about her son, Bartosz. So now after waiting nearly 2 years for the results (I wonder why so long?) the confirmation diagnosis is hereboth for Maria and Bartosz.
My daughter is doing well although in a recent scan the doctor said something about her liver being too pale. Well, I don't know, all her tests are OK.
Although 2 years ago I made the diagnosis myself, now I have the official results I feel scared! Can you comfort me?

[colormist]

Now that you have the official results, you should feel relieved! You now know what cause the adversion, you have a general idea of a diet (well if you've read through the site ), and you have all of us HFI veterans to help you with any questions you might have!

I can't believe it took two years to get the test results, though. That's crazy! What's also crazy is that your nephew also has it. I have no idea what those odds must be.

I can't give you any feedback on the liver color issue. I haven't had anyone check out the color of my liver, so I'm not certain what a typical color would be or even what the color of mine is. Maybe someone else around here can give some feedback on that.

[Tammy]

HI,
I also agree with that you should now be relieved. You have an answer. You know you were right all along, and you aren't crazy. Things should just get easier from here.

Regina's liver was enlarged but no one ever said anything about the color of it. How did they even see the color?

As you follow the diet for your daughter, the liver will heal itself. Just stay strict with diet and give the liver time to heal.

[charlie]

Hi there,
no don't feel scared, although I know you will, I did. It is mind boggling to start with as so many foods contain forbidden things, start at the beginning from scratch and re-introduce things slowly. Use the Boston Diet or similar, I have a copy of the english one if you go on members details and send me your email address I will try to send it once I have put it into readable format - sorry fred didn't realise my system was so modern, its got to be a first for me!!!!!
Start on really plain foods and forget all about food looking pretty and being varied, I've only just got my head round this thanks to these guys on here and use all the info availabel. The good news is if you stick to the diet your daughter and nephew will be fine but it will take a while to get their system settled so go for really easy foods, don't experiment and read every food label at least twice. It takes quite a few months to settle their systems so don't rush it.
Good luck , and don't be afraid to use the support on here - its the best advice you'll get.
Charlie

[dorota]

Thank you so much for all the kind words.
In fact, the doctors in Warsaw were amazed that cousins have got the same symptoms even before the results. They asked if my husband and my brother's wife were brother and sister. Well they are not even related and my husband is Ukrainian. I guess it's an amazing coincidence.
The doctor's remark about her liver was 'I've never seen such a pale liver', and the paper he gave us said something about increased echogenicity? but that was last autumn, the tests he suggested doing turned out to be OK. In December she had another scan because she was complaining about stomachache, but it turned out to be a virus and then he didn't say anything about the 'paleness' of the liver (at least my husband says he didn't because he was with her at that time).
As far as the diet is concerned I got a list of the food to avoid and the food she is allowed to eat and the table how much sugar is in different kinds of fruit and vegetables. I'll have to go to Warsaw for some tests and advice every year now.
Her diet won't change now because it's always been nearly fructose-free. She was breast-fed for a very long time (nearly 3 years!), and of course, after 6 months or so, I tried to introduce other food according to what they say a baby should eat. She immediately refused any kind of fruit, and then vegetables. My attitude was 'you don't want it, you don't eat it' so it was mostly rice, bread, potatoes, pasta, meat, cheese, Polish and Ukrainian pierogi, natural yoghurt, tea and water. Then she went to nursery school, once tried (or they forced her?) a dish of sweet rice with strawberries, became quite sick and weak, and later there were similar episodes every time she went out without me and was convinced to eat what she shouldn't eat. Even after I read about HFI two years ago there was this thought at the back of my mind 'it could be a passing phase, she may grow out of it'.
Now, my husband and I are quite keen on cooking but since she was born it's been very hard to introduce any variety to what we eat. It's all pretty boring, always the same, and I feel guilty every time I prepare something I know she won't even touch. On the other hand, my older daughter form a previous marriage (that's why no HFI) is addicted to sugar (not so good, either!)
Are there any recipes for home-made chocolate with glucose?
Chocolate is the only sweet thing Maria is interested in (the amount she eats - just as much as the dirt under fingernail).
How about nuts? She doesn't mind them, but the chart says no. There is nothing about garlic on the list- she quite likes it.
And one more thing - should I make a bracelet for her to wear (just in case) and what should be engraved there? My brother is thinking to have it made for my nephew.
And one more thing, she is very small. When she was born, she was over 4 kilos. Now she always looks 1-2 years younger than she is. Can it be due to HFI?

[charlie]

It sounds like your daughter diagnosed herself before the medics, Megan was the same when weaning her she refused all foods for weeks and weeks as they always advise pear and apple first with baby rice and she used to projectile vomit at nursery if they gave her vegetable cubes. the first thing she ate was potato and homemade ground rice. They somehow seem to know what suits their system.
As regards bracelets I did think about this too, Tammy had a great idea and made a T shirt for Regina, see an earlier post I made on psychological and social effects, that she wore in social occasions if not being supervised. The best thing though is to teach your daughter to be aware of what she can't eat and why and how to ask what the ingredients are. Megs is amazing and has always from as early as 3 or 4 when we first suspected this condition checked if it is OK for her and does it have sugar and has never taken any food without asking. I had a giggle today at a party, she is now 6, and she sat calmly explaining to her friends why she had to eat her own food and couldn't eat the cakes, she just told them her tummy didn't like sugar and it made her sick. We as parents get alot more stressed about this condition than they do. I have never made a deal about it to her so she just accepts it as the way things are. I was having a chat with a couple of parents at the party about my worries of offending someone if I pack her own food and they all immediately said they would never be offended as at least then they wouldn't have the worry of misunderstanding and giving her something that would make her ill. I sent her to a friends house with a long list of OK food once and forgot to specify breads, they made her a cheese sandwich with brown bread that had sugar in the ingredients but as I had said a sandwich was fine that is what they had done, she was ill for 2 weeks, it just isn't worth it, educate friends and family and you will find everyone is happy to work with you, some positively relish the challenge.

On the subject of size I think failure to thrive is one of the warning signs for HFI, in fact that is why they took so long to listen to me as Megan was growing well, once her system settles on good foods for her she may well catch up, Megs shot up once I cut out veg and fruit from her diet.

[colormist]

As for the bracelet, it depends on how you expect others to use it. I was advised against a medical alert bracelet/tags (in case I was knocked unconscious in a hospital). The doctor who advised me against it said that it might confuse doctors who weren't familiar with HFI and instead of risking doing something wrong, they wouldn't do anything at all. I've told enough family and friends that HOPEFULLY someone would come along and let them know about HFI before anything bad happened.

If Maria went around showing the bracelet to people that were trying to feed her, that might help.

As for chocolate--yes, I love it too but can't eat it. I think Tammy has some recipes. I think I also posted a recipe for no-bake cookies here: hfiinfo.proboards42.com/index.cgi?board=Recipe&action=display&thread=408 (chocolate-peanut butter).

[meaniejean]

Welcome!

We are awaiting diagnosis and are another of the very weird families where a number of us seem to be affected - me, my two sons and my little brother - although there is still a possibility that there is something even a little weirder going on with us - we'll see. We have an appointment with a geneticist in April and I am going to be having a liver biopsy at some point in the near future.

Mmmmm ... chocolate. I am with colormist - LOVE chocolate - well, DARK chocolate. I make hot chocolate sometimes with unsweetened cocoa and dextrose, but it seems to meet my fructose quota for the day - two cups and I am over the limit.

I found some sugar-free chocolates here in the US - Dove dark chocolates - that use maltitol. I seem to be able to handle a couple of them without a problem and they satisfy the chocolate cravings.

Congratulations on pursuing a diagnosis and helping your little girl to lead a healthy life. I really wish my mom had looked into things when I was younger. We are also Polish - my mom's mom was from Poland I believe. I wonder if we have the same mutation?!

All the best to you and your little girl!

[Tammy]

Regina loves chocolate. She eats it every day. She is fortunate in that she can handle maltitol just fine. Too much will act like a laxitive, but that is true with non-HFI people also.

I've never been able to make chocolate candy starting with cocoa. Just doesn't work for me. Thankfully there are several companies that make a sugar free chocolate without using sorbitol. We just stick with those.

[charlie]

Word of warning on maltitol, read medicine leaflet that advised it isn't good for HFI as it may convert to fructose, sorry can't be any more specific yet am in process of researching it. Got very excited as dietition rang on Friday to say he had found safe Ibuprofen in Nurofen for Children suspension but when my pharmacist read label it gave the above advice

[Tammy]

Yes, some do fine on maltitol and some don't.
I follow the sugar chart on BU website:
www.bu.edu/aldolase/HFI/treatment/sugar_table.htm
Regina does well by this list.

[irene]

Hi Regina

Hope you don';t mind me popping in, printed off the sugar table, thanks for the website. It will be a great help, with their being so many sugar and sweetners added to foods.