Suspect HFI - help?

[Alisha]

Hello Nicoleh.

I understand you completely. I have two older kids who leave crumbs on the floor and I feel like I don't have time to constantly keep a close eye on them and on my daughter. It's been very overwhelming for us. It seems like your daughter is ok with not havnig any sweets or veggies but with my daughter, lately, she has been very upset, banging her head on the ground and throwing a fit if she doesnt get to eat whatever her brothers are eating. She wants to have candy, sometimes my older kids will drop something on the floor and before you know it, it's in her mouth and I'll try to fish it out of her mouth but it's impossible. She has not had any diarrhea lately, instead she's been very constipated, could it be lack of greens? . She'll also try to grab my celery, cabbage and any veggies that I'm eating out of my hand. I've caught her eating cabbage from the garbage can (she's 15 months now) and It's been frustrating and upsetting for me to watch my daughter go through this. I now feel like I'm not certain she has HFI anymore. But I still wonder what could be causing her elevated liver enzymes? and her fatty liver. No one seems to be able to help me with this. We did a liver biopsy basically for nothing. It ruled out alot of other liver related conditions but couldnt tell us if she had HFI or any other condition. I am to a point where I feel very desperate. I know alot of ppl say 'oh it's ok, she doesnt need chocalate or cookies or sweets, but what about fruits and veggies? They are supposed to be good for her. Her metabolic Dr and her liver specialist think we should still keep on a HFI diet which i'm ok with. But I'm not very happy about it because she never showed any signs or symptoms of HFI as a baby. She was actually growing fine weight and length wise. We're gonna run more tets and currently waiting on the dna test for Cholesterol Ester Storage Disease. It's been 4 weeks so I hope to hear back soon. Today, we have to check her liver enzymes again and I'm super nervous. We havent had this checked in 2 weeks, since her liver biopsy. I just pray that it goes down.

Anyhow, sorry for rambling on and on there. To answer your question about my daughters BUN and Creatinine and electrolytes, they've all ben normal except for one time when her bun was at a 24 (normal range was between 0-19, in the U.S). I came across this one site once, where I read that sometimes after the stomach flu, it can really mess up your system and your liver enzymes. My daughter had a severe case of the stomach flu before this nightmare happened (back in March) and I sometiems wonder to myself if she's really ok and she doesnt have any of this? But then the fatty liver comes up and then I'm back to square 1. Maybe it's time to fly her to see Dr Tolan??

I really hope you get answers for your daughter Nicoleh.

[khethma]

Is she anemic? Eating paper and other odd things, like dirt and ice, are symptoms of iron deficiency anemia, which is a common problem for people with malabsorption disorders. I've had iron deficiency anemia off and on my whole life, and I used to love to chew paper as a kid (I didn't usually swallow it, though, at least not once I got older). As an adult when I get anemic I can't eat enough ice. I just crave it like mad.

Heidi

[nicoleh]

Hi Heidi,
she has low transferrin saturation but otherwise normal iron studies. I'm 99% sure that it's not a sign of anything other than being 12 months old. She just hasn't grown out of that 'put everything in mouth to check it out' stage. She doesn't actually seem to like eating paper, she does try to spit it out but is often unsuccessful at that and just swallows it. Doesn't stop her from 'trying' the next piece though! My kids do a lot of craft and it just gets spread everywhere.

Alisha I'm glad someone else is having the same trouble with older siblings! I've banned eating anywhere other than at the table and haven't allowed guests with kids to eat anywhere other than outside (as other people's kids seem to be even messier than my own).

It sounds like it might be time to put your older kids on a candy free diet too! It would certainly only do them good - fructose/sugar is bad for everyone - just not on the HFI level. That might help your DD not to be so jealous of what they're eating.

For the constipation and also the ear infection you could try a fructose-free probiotic. I'm sure you could find one somewhere without much trouble. priobiotics are really good for gut symptoms and have also been shown to help with any infection, including ears, often with better results in the long term as there isn't the cycle of infection, antibiotics, re-infection; replacing the offending organisms with healthy bacteria stops them from constantly getting hold. the antibiotics don't get rid of the bacteria that cause infections, they just kill most of them and then the ones that remain become more resistant and stronger and thus re-colonise more quickly than before. and if you do get her on antibiotics the probiotics are even more important as the antibiotics kill the good bacteria in her gut which worsens gut symptoms AND future infections. You would want to keep her on a daily dose for at least 6-8 weeks whether or not you use antibiotics. anyway this isn't the ear thread so I'll head over there later!

Did your daughter have the needle biopsy rather than the open liver biopsy you use for HFI?

[charlie]

Hi Alisha,

I'm sorry you are having such a struggle, trust me we have all been there. Luckily for me I didn't have other kids and Meg never tried anything she couldn't have at home, at others and at nursery and people used to comment on it............ guess I was very lucky there .......... until the chocolate episode when she was 8............. and then curiousity got the better of her, luckily by then her system was improved and could cope better with the occasional hiccup.

You typed:
am to a point where I feel very desperate. I know alot of ppl say 'oh it's ok, she doesnt need chocalate or cookies or sweets, but what about fruits and veggies? They are supposed to be good for her.

In this modern age we are obsessed about the 5 portions of fruit and veg a day, yes for most people it is healthy, it adds fibre, vitamins (although how many is debatable when it is packaged, flown round the world then sits on a supermarket shelf for a week..........) but it also pads out the meal to keep weight down. Do you absolutely need it, well meg from age 4 had no fruit, no veg at all, in any shape or form and she is on the 75th centile for growth and the 60th for weight.......... everyone comments how "well" she looks (mind you, they don't see her on a reaction day). As long as you source good quality meats, fish and dairy, adapt recipes to suit her so she can have as close to her siblings as possible and find a good safe multivitamin supplement then for the short term stopping fruit and veg altogether while you figure out what is going on will be just fine. Isn't it so frustrating though, you have thousands of kids that refuse fruit and veg, then the one that can't, craves it............

I can assure you, we all go through the same thought processes you are at the moment, it takes a while to get the message, but it is very doable, you will need the cooperation of the rest of the family though to adapt their lifestyle a bit too. But take it step by step, as your daughter gets healthier you will find it easier to cope with, just at the moment it seems like you have hit a brick wall, but it sounds like you are in good hands medically and they are looking at all the possibilities, of which there are quite a few.

Take care, keep battling and enjoy the challenge of inventing new dishes.................

[ukbill]

I second Charlies comments about the Obsession to eat more fruit and vegetables.. mostly its to reduce the amount of highly processed foods we are eating.. if you have a piece of fruit instead of a cake , cookie or chocolate then the fruit is VERY good for you (in comparison)

If you eat a lot of veg in your main meal you are less likely to eat a large sweet / fatty pudding after etc.

However I have to point out vegetarians and Vegans in particular get such a poor diet they have to take many vitamins and minerals they do not get from the Fruit and vegetables, which we are told is "good for us" because they are "healthy".

I cannot take any multi-vitamin because it overloads my body with vitamin A and iron.. giving me real problems when I do.

I think this is because I eat so much meat and animal fat that contains high levels of vitamins (most vitamins are oil soluble) other than vitamin C I manage very well.

My cholesterol level should be very high (my fathers was) particularly on this meat and fat diet of mine.. however its not.

5 years ago I had a cholesterol test and came back with a 1, last year the same test came back with a 4 and my Doctor got agitated about this..

6 is the level where they start to "think" about statins etc

Being honest I had put on some weight.. so over 6 months I lost 25lbs and was retested and came back lower.. (I was not told how much lower I will find out when I see the Dr next)

Well I feel much better for having lost the weight and I do not now need to buy all new trousers (pants in American speak) because the buckle did not meet in the middle anymore

I can confirm that Megan is the very image of a healthy bright young lady, like her mother.



DO not panic about not eating a "Healthy" diet because you are not eating fruit and vegetables, meat and animal fats are very healthy!

What is not healthy is foods which are high in sugars and fats and very refined carbohydrates.

Cookies, cakes and chocolate are certainly by far the most unhealthy things out there, and are VERY addictive!

A test was done recently with some Monkeys for two weeks they were taken off their "normal" diet and put on a highly refined very sweet diet.. they were then put back on their "normal" diet and went on hunger strike for a week..

Which was just as well because they had put on a lot of weight, they wanted to be back on the cakes and chocolate diet!

So be thankful you do not eat them!