HFI diagnosis of my 2Y-4M Son

[rasash2008]

Hello all,
First of all, I am very thankful to the moderator, staff & all members who founded this forum.
My son 2Y4M/M has been diagnosed as HFI before 5 months. I want to share here my experiences about his diagnosis & treatment: from birth he had very common symptoms like flatulence, diarrhea (which we thought because of teeth), spastic constipation, vomiting (in case of syrups and when we tried a little dose of any fruit juice) and abdominal pain (cried continuously). After a difficult period of before weaning, an aversion to sweet-tasting foods appeared. He consumed mostly milk formula. We had to visit doctor’s clinic repeatedly .doctors couldn’t diagnose the actual disease and always prescribed syrups (sweetend-sorbitol based) for each symptoms. [glow=red,2,300]SORBITOL IS RESTRICTED FOR HFI PERSON.IT CONVERTS INTO FRUCTOSE AND AN EASY & ECONOMICAL BASE FOR SYRUPS![/glow] I was totally unaware about it. when first time doctor diagnosed of HEPATOMEGALY(Enlargement of liver) and after some scanning test size of liver was found 5 times greater than normal about [glow=red,2,300]11cm![/glow].then we changed 2-3 doctors but all of them prescribed syrups & more syrups, also directed to repeat the dose in case of vomiting. We all were very tense & moved to big hospital and this was a start of HFI journey. Doctors started with OLD CHRONIC LIVER DISEASE(METABOLIC?) and prescribed some tablets(consist of ZINC-for healing liver damages, FOLIC ACID-helps in metabolism))
After 15 days a very risky (medically controlled & monitored conditions were not available) test performed as fructose challenge test- In which 10ml/kg about 100ml of liquid fructose orally ingested via a tube from mouth, 0 Hr (after fasting and before starting test), 1Hr blood samples collected and further, blood sugar level was monitored in the 0hr, 1hr, after 30min, every 15 min and found severe hypoglycemia(Blood sugar was falling very rapidly) & due to seriousness, ingested glucose(dextrose) through I.V. to control it. So the test was positive for HFI . LFT (Liver Function Tests) of 0hr-1hr samples reported increment of SGPT-158 to 217(range up to 50), SGOT-195 to 309(range up to 50), Serum Alk Phosphates-671 to 908(range 240-840),Total Serum Billirubin-0.5 to 0.8(range 0.1-1.0) ,Uric Acid-2.10 to 5.50(range 2.50-7.00),Calcium-7.40 to 9.60(range 8.1-10.4) and most importantly phosphate declined 2.60 to 1.80 (range 2.50-4.80),on this basis my child is confirmed as HFI.Alodolase-B activity test of liver enzyme is not available in India.
After 2 days, some more tests applied, endoscopic findings were normal, liver biopsy findings are possibility of metabolic disorder, thick band of fibrosis showing sparse chronic inflammatory infiltrate, the hepatocytes show mixed vesicular steatosis & histological features are those of micronodular cirrhosis with fatty change.
After 3 months, in SEP, 2008, doctors examined him physically & told size of liver has decreased up to 4-5 cm. Using HFI diet, No more Vomiting and Diarrhea noticed. Doctors were prescribing him sugar coated medicine (Zinc, folic acid) from last 5 months by mistake and refused when I get them noticed about it. His latest blood findings are not satisfactory and increased (as on 05/12/08: SGPT-140, SGOT-124, S.K. PHOS.-1112, Total Serum Billirubin-0.6, Uric Acid-2.80, Calcium-10.8, Phosphate-7.0(range 2.5-4.8).
Doctors told to continue the medicines.Now I stopped all the medicines and using “apo-HEPAT” liquid homeopathic remedy from PEKANA, Germany (from 05/12/08) and found noticeable improvement in my Son’s activities. I am from INDIA. HFI is very rare and except the big Hospital, No other hospital has any known case of HFI and NO nutritionist has HFI diet chart. Does anyone has any suggestion and discussion? Most Welcome.
---rasash2008

[rasash2008]

hi all,
i am still waiting for any reply of my posted thread.i request to moderator and all members to share blood findings reports with me if you have or any idea.however i am searching day-night about HFI? and HFI-diet.i came to know that some foods ingridents are questionable in some recipes posted or replied by some members like onoin or spring onion both have fructons(fructon is the molecule of fructose) and further research is going on this.so i think for a questionable food for HFI,either consult from a dietician or avoid the food.
i found some websites claiming alternative treatment for HFI & 100% moneyback guarntee ( herbal tablets and homeopathic medcine) during search,due to the lack of the time i am not discussing here more.i am going to post a thread soon about it.
pls reply
----rasash2008

[Tammy]

I agree with Fred on all his points here.

I just also want to add that playing with any "alternative" type treatments is just risking your health. There is NO tablet or herbs that can replace the HFI DIET. It may possibly ease up some of the symptoms but I really think you'd be risking your liver's health. Not something I'd ever try for my daughter.

[colormist]

Yeah, I got a printout of what my gene mutations were, but I don't remember where I put it or what they were called. Other than that I don't really know anything aside from my symptoms and that I have the HFI genes.

Going to echo Tammy's remarks about the alternative treatments. There are no alternative treatments. Your liver has to deal with the sugar and HFIers' livers can't deal with sugar. Not that we'd want to deal with the sugar--as it generally makes us nauseated just being near sugar.

The good news is that I like Indian food--in moderation. I usually stick with the jasmine rice and a cream-based sauce (with chicken!). And I LOVE plain naan. Tandoori chicken is also all kinds of awesomeness. As is Spicy Peanut chicken. Hmmm... I think I'm going to have to pick up some Indian food this week.

Like Fred said, sticking to a diet with less than 40mg of fructose a day (and plenty of water) should be fine, but you have to find your son's tolerance level (which might be less than that since he's so young). Right now, you're the expert on your son's condition, so if you think something the doctor suggests might not be a good idea, be sure to let them know. You're going to be teaching them more than they're going to be teaching you.

[rasash2008]

Hi Mr. Fred
I read your P M and have replied, furthermore co-operation is required. Although I am personally thankful to you.
I already have printouts of HFI-diet 1,2,3 from the Boston university & I am strictly following it for my son but I found some questionable foods like in Diet-1,”green beans & wax beans” w. ref. to diet www.food-info.net/uk/intol/hfi.htm .
”Asparagus, onion” are also questionable, check the topic ‘foods with high fructon content” w. ref. to link en.wikipedia.org/wiki/fructose_malabsorption.
I was unaware about the trend in CANADA, but now understand your right of privacy. But in INDIA; even doctors of big hospital (Govt.) don’t have much experience about HFI and its precautions. Even when I did a request from dietician to discuss about HFI-Diet, she refused and told me to discuss it with her trainees. I am a software professional having MCA with science(chemistry) stream at graduation level, so I understand the blood chemistry a little bit and its seriousness that’s why I want to find some information about it and enlarged liver from all the experienced members, if they have and wanted to be share.
Sorry to say, I am not a doctor or research scholar, only a [glow=red,2,300]conscious father with a HFI son[/glow].
About the recommended fructose 40mg/kg to body weight by B.U.,I think it is ok for some DFI person, because fructose tolerance & symptoms varies from person to person in case of DFI and study shows, DFI is not as rare as HFI.

rasash2008
(CAN DO WILL DO)

[olivias mom]

hello I wanted to shared the results of my 9 y.o. daughter's liver biopsy results. She was diagnosed by DNA test at 18mos old, but she needed to have her gallbladder out so went ahead and took a biopsy while they were in there. (gallbladder disease is common with HFI's but I think there was only one person on here who said they were having issues) Here they are: "Moderate to severe mixed microvesicular and macrovesicular steatosis with low grade steathohepatitis, mildly active...Low grader ductular reaction and focal mild portal/periportal fibrosis...." Her AST and ALT are usually in the 60's range. Her liver has always been larger than it should never did return to normal as they had expected it would. She also has hepatospleenomegaly(spleen involvement). I had her LFT"S checked about every 6 mos to one year, she was also having ultrasounds every 6 mos but since it has been stable for 7 years we now do them yearly. I have been to many GI doctors and I really don't think anyone understands why her findings are this. They have explored other conditions that could present in this way but so far all we have for sure is HFI. My daughter is active, well, and doing well in school(all A's). I say this for anyone who gets abnormal results and wonders what the future child will be like. Hope this helps

[olivias mom]

.....One more thing you might find interesting....I am a pediatric RN for a very large practice in Florida. I asked all of the Docs about any of their experiences with HFI, only one had a story for me and it was that the child came in to the office almost unconscious and they had to call 911, when the child got to the ER the blood sugar level was 30!!!!This child was also from India and had the diagnosis of HFI.

[rasash2008]

Hi all,
This is the time of relief. My Son’s latest blood findings are satisfactory and decreased (as on 09/01/09: SGPT-90, SGOT-70, S.alK. PHOS.-878(has decreased 234), Total Serum Billirubin-0.4, Uric Acid-3.00, Calcium-10.6, Phosphate-5.6(range 2.5-4.8). All of the credit goes to “APO-HEPAT” formula of PEKANA. I used this only for 20 days to my son and it’s really working! NO Side Effects! Now I am giving it continuously with more strictly following Fructose-Free Diet. See the link and read carefully www.askariel.com/product_details.asp?ItemID=109 When I told the doctors (GI) about it, he was totally surprised. But they never believe in HOMEOPATHY. (APO-HEPAT-this Homeopathic-remedy is being manufactured by ADEL in INDIA and my father found it, he is an experienced Homeopathy RMP). After physical examination, Doctors didn’t find any liver enlargement and replied that it may be possible that liver has been decreased to the normal size.
Now my main hurdle is the Vitamin & Minerals Supplement? I came to know that regular use of Vitamin C & E is perfect for HFIiers. But I couldn’t find any fructose-free product till now.
Is there anyone, who has information about [glow=red,2,300]VITAMIN & MINERALS[/glow] etc. for HFIers .Please give any detail or Hint?

rasash2008
CAN DO-WILL DO

[colormist]

Hi Rasash2008,
I take these vitamins everyday: www.basicvitamins.com/scripts/prodView.asp?idproduct=1772
It's for adults, but they're pretty small (like the size of an ibuprofen). I have yet to find a multivitamin for kids that is fructose-free.

*colormist

[ljs678]

My baby has been prescribed a multivitamin supplement and also a separate vitamin C supplement.

The consultant who we saw for the DNA test (still waiting on the results) checked the ingredients of the multivitamin supplement from their reference book and said it is safe for HFI.

It is called Oranol and is made here in Switzerland by Bayer AG.

He also has an additional vitamin C supplement (they said that if he had enough of the multivitamin to give him the vitamin C he needs he would get too much of some of the other vitamins).

This has to be made up specially as there was not a commercially available one that is safe for HFI. The pharmacy order it and it comes in capsules which I break open and mix the powder in with a bit of food.

I should mention that he doesn't like the taste of the Oranol but will eat it if I mix it with just 1 spoonful of food and give it to him before his breakfast first thing.

I hope this helps. Bayer are a big company so their products may be available in your area, or if not they may be able to ship it to you.

Just as an aside, the vitamin C is covered by our insurance but not the multivitamin. I am waiting on the DNA test results before I have a fight with the insurance company about this one...

[rasash2008]

Hi all,
Now my son is Ok. NO symptoms is appeared since july,08.he is on a very strict diet even we are not trying any type of vegetable.because there is no way to measure Fructose level in Products of Indian Origin.Fructose level in a natural product depends upon the nature & origin of the plants. In FEB,08 a blood test was performed,i found the decrement of hemogloubin(due to decerement of Iron). all the enzymes was raised due to our fault. I always insist to restict his diet at my level & also take the feedback daily from all members,but very small quentities of 7-8 questionable items are given to him. but no symptoms appeared.
Now i am giving 'PROKID' MultiVitamin plus Zinc tablet(half in a day) after confirmation from [glow=red,2,300]frusano[/glow],on the other hand [glow=red,2,300]APO-HEPAT[/glow] is going on. this formula is really working without any symptoms and small child always asks for it.
Little baby also liked the taste of Choc-late from frusano. i found their products very good,while shipping time to my place is about 5 weeks!
For rising Iron level in Blood (for hemoglobin) i started 2 drops/2 times in a day of [glow=red,2,300]'Lecithinum 30'[/glow]. after some times i am going to manage another blood test scheduled by Big Hospital.

yours
rasash2008
(CAN DO-WILL DO)

[ljs678]

Great to hear that your son is now doing so well!

Your post reminded me to ask about iron deficiency anaemia.

My baby has it, seems yours does too. Is it common with HFI?

Does anyone know why? At the moment we are giving an iron supplement but it would be good to know if there is any other solution.

[julienc]

Apr 6, 2009 5:41:23 GMT -5 ljs678 said:

Your post reminded me to ask about iron deficiency anaemia.

My baby has it, seems yours does too. Is it common with HFI?

Does anyone know why? At the moment we are giving an iron supplement but it would be good to know if there is any other solution.

I don't know if it is coincidence or not, but I have always trended on the anemic side. It never gave me too many problems except during my pregnancies when my iron levels dropped significantly. I took iron supplements, but that's all I've ever done to mitigate the problem.

[meaniejean]

Iron deficiency anemia here too. Calvin was anemic at his last test - but we finally found him a multivitamin and he looks so much better - don't know if the anemia has resolved, but he doesn't have the dark circles under his eyes anymore.

I have always tended towards anemia as well - and as julienc stated - it was much worse during pregnancy and after childbirth - the after childbirth part was at least partly due to postpartum hemorrhages.

At my last blood draw I was no longer anemic - I have never seen my lab results look so normal! Amen to fructose restriction!

[meaniejean]

Oh - the vitamin we found for Cal is called PediaNutrients by Pure Encapsulations. It is a little capsule and he is able to swallow them like a big boy! They also have a flavored powder but it has natural fruit flavors.

[CK]

I too have had iron deficiency anaemia, but my doctors thought it was from Celiac Disease. I never thought of it for this too.

[Stefanie (Ziba)]

Thank goodness I found all of you!!
Hepatologists only want to scare us and do invasive procedures. My 16 mos. Old son was born anemic and has had all HFI symptoms yet we've been up and down Florida and very little progress has been made. Olivia ' mom, who ordered the genetic testing for Olivia? We are waiting to get into Duke.
Has anyone had cysts on the liver?

[sarosh]

Dear Rasash, My name is Sarosh, I am a 59 yr old woman from India but live in Dubai . I have been diagnosed positive for HFI first by Boston university and then in India. Sorry, I haven't been active on the forum for some time. You could e mail me for information on where to get the mutation of your baby checked and for safe Indian food.


Oops sorry. Looks like i replied to a very old post.

[stefaniebillette]

Olivia mom, thank you for the info. Remy's liver problems started after he was given sucrose in the NICU. He has not had a biopsy, but his alt/ast are usually in the sixties as well. His bilirubin has been up at times probably when he was exposed to too much for a long period of time. His ultrasound also revealed signs of steatosis and fibrosis, and they called areas cystic or lesions. They seem to have hyperechoic and hypoechoic areas to them. His liver is large his swing is on the upper limit of normal. I know your post is old, so my question is whether the diet over the course of several years help your daughters liver heal. It is an extremely difficult diet as you know. I just wonder if his liver will heal over time. Thank you.