fasting & bloodsucker drop

[Marylene1]

Hello,

My daughter (1,5 years old) is suspected to have HFI or an Aldolase B defect. We are waiting for results of bloodtests. This disease is new to me, I have so many questions

My daughter refuses to eat sweet foodproducts. In fact, she only eats some rice, beans, salted cookies (tuc) and yoghurt. She has a stomach feeding tube. When we leave the feeding tube out, letting her eat on herself, the bloodsucker drops to a dangerous level.

My daughters motor development stays behind. She can't move on legs and hands, she only stands up with much help and there are times she can't stand at all.

We notice a very fluctuating energy level. Mostly she sleaps from 12-14:30 but when we ask more of her energy, she wants to sleep in bed at 10 in the morning. She can't get up by herself before the tube feeding and ca 1,5 hour after the feeding her energy level slowly drops.

My most important question at the moment is, do all HFI patients show a drop in bloodsucker after eating fructose? My daughter only shows a very low bloodsucker when she is fasting for over 12 hours.

Does the above sounds familiar? I would very much like to receive information about the state of expression of HFI and Aldolase B deficients by infants. You will help me very much by telling me your story.

I hope this message is readable, I'm from Holland and I haven't practised my English in years!

Thanks for reading, I hope to get answers,
Marylene

[Tammy]

Hi Marylene
Welcome to this site.
My daughter is now 25. She has HFI. We started this diet when she was about 2.5. She's never had the problems with her bloodsugars (I think this is the word you are looking for) but there have been other mothers on here that have talked about the same things you are talking about. Even some of the adults have some of the energy problems. They can tell you themselves.(Laura - I'm still giggling over how soft cement can look)

Although this isn't a medical site and we can't really help you with the diagnosis, there are a lot of very nice people here who can hopefully help by giving you ideas of how to deal with living with HFI. Once the Doctors know for sure what your daughter needs, then it's easier to start to deal with it.

My daughter is also handicapped. She does crawl on hands and knees, can stand if holding onto something, and has become independant in many ways, but can't walk on her own. She was always developmently delayed. She didn't even sit on her own, without support until she was 2. Once we started the HFI diet, she started catching up.

On a side note, I am familiar with your pretty country. The company that my son works for sent him to Amsterdam. He has now married a wonderful girl, and they are still living in Amsterdam. I have been over a few times to visit. My daughter was over once, and we had no problems with her diet. Of course it would have been much harder because of the language, but since my daughter-in-law is Dutch, she did all the explaining to waiters, etc, for us. She is also the one who read all the ingredient lists on crackers and such.

Also, I'm curious, how did you find this site? It's kind of nice to hear where this board is getting it's exposure.
Tammy

[colormist]

Hi Marylene!

I'm going to have to disagree with Tammy and think you meant blood pressure instead of bloodsucker. But you could mean blood sugar. Either way, the symptoms you describe sounds exactly like how I feel when I've not eaten in a while or I've accidentally had sugar.

As far as I know, I didn't have any motor development problems when I was a kid. However, even to this date I still feel like sleeping a LOT when I have sugar. Either I sleep or I puke--usually both. Other side effects are loss of energy, shakiness, complete inability to focus on anything other than sleep or food.

HFI people, after eating something with fructose, usually get uncontrollable cravings for potatoes. I usually want to eat a whole bag of potato chips. I usually DO eat a whole bag of potato chips, which solves my problem with the fructose, but makes me feel horribly fat.

Good luck with your daughter. My guess is that she is HFI, but I'd wait for the blood results to be certain. I definitely wouldn't let her eat any sweet foods until you have her results back.

[sarahk]

Marylene,

I’m sorry to hear about your daughter.

my name is Mike-my wife sarah has HFI-she is 29 yrs old now, and was diagnosed at age 14 with HFI-with is considerably younger than most people who get diagnosed in their late 20s.

To address your first question: a drop in blood sugar after eating fructose: That’s probably what is going on. Also, your daughter likely has hypoglycemia-as sarah and many others on this board do
de.wikipedia.org/wiki/Hypoglyk%C3%A4mie

Or

nl.wikipedia.org/wiki/Hypoglykemie

After sarah eats sugar she usually throws up within a few hours and feels tired for a long time. The next day she doesn’t feel “normal” I had a co-worker once whose wife did not have HFI, but would become almost “drunk” afer having fruits or other sugars-and would “pass out”.

If your daughter doesn’t eat for a long time, she may have a drop in blood sugar as well. In the US they sell Glucose tablets which raise blood sugar quickly. Glucose/Dextrose is an OK sugar with HFI

Sarah started talking very late, but has since made up for. Other people on this site have mentioned that once they got their diet in order, many of their symptoms went away.

She has had an aversion to sweet foods her whole life. There are different levels of HFI-to sarah, broccoli and asparagus are too sweet, while other people may tolerate them. Sarah loves avocados, but others have never had them.

Your daughters diet sounds similar to the HFI diet-I am not sure what beans your daughter is eating, but most beans are not too good for HFI–all beans have a sugar which humans cannot digest (hense the problem with beans) but it may be especially tough for with HFI. Black beans are OK and garbanzo beans are even better. Sarah REALLY likes lima beans–the greener a food is, the better-usually. Spinach has less sugar than lettuce

As you are in Holland, here are my recommendation-I was in Amsterdam a few years ago: Have her eat all that delicious smoked Gouda and other cheeses produced. Dairy products (lactose) do not have an effect on HFI-if you give her yogurt, make sure it is plain and not sweetened. We found crackers that she could eat there. Plain meat is OK, but most pork products are cured with sugar-chicken and beef are safer.

Watch out for hamburger buns, especially at places like Quick-the chicken nuggets make also have sugar in the coating/batter. No ketchup, but most mustards are OK-french mustards usually have sugar-try plain yellow.

As your daughter is 1.5 I’d feed her rice cereal in the mornings (cream of wheat, rice...) You can make her food by mixing plain chicken and rice in a food processor/blender for something more substantial. Stick with dark leafy green vegetables: spinach, broccoli-but she may only like the top part-the stem is too sweet for sarah, cucumbers, bok choy, mushrooms are OK

Most doctors have never heard of HFI and will give you bad advice-you have to be careful.

I would also look to see what type of sugar is used in hospitals in the saline/ fluid bags that get attached to a person. They are usually filled with Dextrose sugar which is OK because it is like glucose, but it may be filled with fructose.

It sounds like you are doing your best with her, it is important not to force her to eat something as too much sugar can damage her liver.

You’d probably find it very helpful to read many of the posts on this site as people have discussed how HFI has effected them
I'm going to disagree with Laura AND tammy and asume you meant bloodsucker I mean I saw "sweat breads" on several dutch menus

[Marylene1]

I'am really glad my husband found this forum! We were searching for about three months to find more information about HFI. Reading your stories really helps us to deal with the probability of HFI. Every difficulty we encounter with our daughter fits in the syndrome but we have to wait two more months before we will get the results of the blood tests.

Isn't bloodsucker a little animal that sucks blood? You will all remember me by this mistake Yes, I meant blood sugar! My husband and I had a good laugh about it yesterday That's worth something, especially in a difficult time like this.

Tammy, is your daughters handicap a result of HFI? Can she handle a wheelchair?

Mike, thank you for the information about food and doctor/treatment!

Marylene1

[Tammy]

Regina can handle her wheelchair just fine, although we only use it when we are out somewhere. At home, she crawls. She is more independant when she's not stuck in that chair. She can go up and down stairs, she can stand at the sink, get her own drinks, snacks, etc, and do many things that she can't do if she's stuck in the chair. Years ago they said it was probably HFI related, but now that we know more about HFI, and looking back at other things, I doubt if it is related. The Doctors can't answer that question. They don't know, and we probably will never know. It's something that I've answered for myself a long time ago.

Bloodsuckers could be little leech like things, or they could be vampires, or zombies (well, I guess they'd be more brain-eating, huh?) or other scary-movie things. I did have an un-fair advantage knowing you probably meant bloodsugar as I know from traveling to Holland that the Dutch word for sugar is suiker.

Glad it gave you a laugh. You are right in that it's very important to laugh.