Child with HFI and liver problems

[raluca]

Hi Ziba,

My daughter also had high fever recently. We gave her supository with Paracetamol - this is completely safe. She cried a lot because of it, but fever went away quickly, it's even more efficient than oral medicine..

[raluca]

Hi Ziba,

My daughter also had high fever recently. We gave her supository with Paracetamol - this is completely safe. She cried a lot because of it, but fever went away quickly, it's even more efficient than oral medicine..

[stefaniebillette]

Jul 16, 2015 14:59:14 GMT -5 heatherp said:

Hi! I have a two year old with HFI and perhaps can help with diet related questions. We started with white rice and chicken and milk to drink. Plain and simple. From there we have expanded to noodles, cheeses, butter, beef, homemade breads and pizza doughs, pancakes, some mushrooms, and we have a meat processor specially make sausages and bacon for us. He is too sensitive for some things that others eat - lemon, cucumbers, celery, pickles, even the malted barley in flour seems to have a bad effect over time, so we are very cautious and conservative with his diet. He also has a Congenital Lactase Deficiency and so can not have lactose. Also, my little one takes a vitamin called Nano VM which you can buy online. It is made by Solace Nutrition. His vitamins are followed via blood work, as are his liver enzymes. This is pretty much it for follow up. We go in to his very wonderful specialists whenever they feel it is necessary, but in the end, it is really the diet that is required, and they can not make that happen, only we can. So we do! He is currently doing very well. He was diagnosed around 15/16 months. It is kind of a miracle to see how healthy he is now, because at diagnosis he was in rough shape. Ask away! You are in good company. I would guess that, if you are strict with diet, you will see liver improvement and perhaps other health improvements also. I know we have!

Thank you! Our stories seem very similar. I appreciate your input.

[stefaniebillette]

Dec 9, 2015 9:13:30 GMT -5 colormist said:

Glad I can help! Were you the person that commented about the White Pasta on the HFI Food Pyramid? If so, I never even thought about how that was not the actual name for that type of pasta. I think I just got carried away with listing "white" in those ingredients.

I'm really impressed that you thought to check the ingredients on medicine! It's so hard to keep things sugar-free nowadays as producers tend to want to sweeten everything.

If he does come back positive for HFI, honestly you are past the worst hurdle. This group can help you with almost anything else.

I just saw this one! Yes, I did comment about the white pasta because I wasn't exactly sure about the distinction.

[ukbill]

Just a quick note to offer my help if I can.

1st a LONG and happy life is easy once the diet is followed.
2nd after about 6 months your child's liver should recover and regenerate so long as you keep the Fructose intake as low as it is possible to get. Charissa's lad James was very ill indeed with a fatty liver, after 6 months of him being very ill but on a Fructose free diet he suddenly recovered and he is now a fit healthy and VERY lively , fun lad of 6 years old and is growing fast.
3rd when your child is old enough to learn how to cook, teach cooking because almost all pre-prepared foods will contain added sugar even if not listed in the ingredients list. "Natural flavourings" usually being used to cover up the addition of sugars in foods. The labels on foods which state sugar content are highly misleading and should only be used as a very very rough guide. Pasta for example usually states that it is quite high in sugar yet is always 100% safe (if cooked in a lot of water).
Also products from Multi National companies do not have the same formulation in every country so always check yourself.
Have you been given a "safe food" list?
Varieties of vegetables can vary wildly in their sugar content so in some countries Avocados are tolerated by some HFI'ers because they can get a local variety not internationally available.
Tolerated is not the same as "SAFE" ever!

If you have a list of foods send it to me and I will take a look at it I live in the UK and have traveled in Europe and know a little about the foods available to us over this side of the ocean.

Above all DO NOT PANIC! all will work out fine. Your child could have any number of far worse conditions.

[Stefanie (Ziba)]

Can you all help me understand what your or your child's liver damage looks (or looked) like on ultrasound?
My son's liver is always described as having "increased echogenicity with multiple hypoechoic lesions". His liver is enlarged as well.
I am very curious how HFI livers are described in Ultrasound reports.

Many thanks!

[ukbill]

Mine is fine however I have heard of the lesions being replaced by fatty layers withing the liver.
In extreme cases this stops the normal blood flow through the liver and the blood vessels go around the outside of the liver stopping it being able to grow new undamaged areas..

The liver is a fantastic organ and can repair itself very well given 1/2 a chance. It takes at least 6 months for a person with a damaged liver to start to recover even on a very low Fructose diet. however over a week or two once it starts it "kicks back in" and the response in children is incredible.

They go from poor sickly lethargic children to total bundles of fun and mischief as they should be. Its literally like a switch being turned on.

[rysmom14]

Hi Ziba!

my son has had 2 ultrasounds scans with elastrography. the first was a baseline and the second was last December. They both looked similar, but it was hard for them to see all that much because he was throwing a fit. But his liver is 5 times the size of a normal liver for a kid his age, and his findings also says that the liver is "diffusely increased in echogenicity". he also has bulbous contours.

The echogenicity is showing that there is a difference in the normalness of an organ. in the liver's case, if it is damaged it could appear brighter or darker on the screen depending on what the normal state is. In both of our cases, the "increase in echogenicity" is showing that their livers are brighter than normal so that is showing damage. as far as the lesions, they also would be described as brighter or darker than what would be expected, but its harder to learn anything from ultrasound based on those.

So with all that mumbo jumbo, basically it is hard to tell all that much from an ultrasound. it is very helpful to detect size, but beyond that it is good for showing that damage is there, but it cant really say what the damage is without a biopsy. the Dr would like him to have this on a yearly basis to look at overtime, but it is more of a comparison tool.

Like Bill Said, the liver is amazing and given a chance, can repair and function normal. With the fructose free diet, the new liver tissue that will grow as he gets older will be healthy and functioning tissue. After my son came home from the hospital he was on diuretics and had fluid restriction. He had a lot of free floating fluid in his abdomen because of the liver functioning so poorly and all his labs being screwy. He was only on the meds for a couple months before things started to correct themselves. He doesn't need meds or fluid restrictions and hasn't for years. so his liver is functioning normal, even with being larger and damaged. it seems the other parts of the liver kind of pick up the slack to work the way it needs to.

[Stefanie (Ziba)]

Thank you, UKBill and Rysmom14!

[marialia]

Jul 7, 2015 15:01:43 GMT -5 raluca said:

Hello everyone! I am new in this community and I am so very glad that I found you. My name is Raluca, I live in Bucharest (Romania) and I have 2 daughters (one is 10 and the other one is 2 years and 7 months old). My little one, Amalia was very recently diagnosed with HFI, after the genetic blood test.

We also did several other blood tests in parallel, and a few of them came out with small deviations: lower copper in plasma (77) and a few others. In order to eliminate the possibility of another genetic liver disease (Wilson), our local doctor recommended biopsy. Results came today and we are terrified… Steatosis level 3, signs of fibrosis...and some other things which I cannot translate in English, but I will have them translated soon…



Our doctor told us to wait. Diet, waiting and monitoring once in 3 months… But we are so scared and we have millions of questions.

Reality is that there is no relevant local experience with this disease. We were lucky to have it diagnosed so quickly, but from that moment, we feel that we are somehow alone; it’s not clear for us what are the necessary follow-ups (if any) and how can we manage this situation.



We need to know from somebody who has a small child with the same disease what are the steps to be taken further… for example if there are any additional tests to be done in order to cover all basis.

Or what are additional implications / effects within Amalia’s body, especially now when she is growing. What we should monitor constantly and what we have to do in order to keep things under control and help her have a normal as possible life? We desperately need a specialized nutritionist to help us with the diet… We are reading a lot on-line, but we still have many questions and need ideas.



So, is there anybody in this group going through the same experience? Somebody having a small child (around 2-3 years old) diagnosed with HFI? Or an older child, but who was diagnosed around this age?

Thanks a lot for feedback!

[marialia]

Hello, I am new to the group, I am from Panama. My daughter is Two years old and she is the First with IHF, she is not taking vitamin C, since I can not get it, I would like to know more about this new world that I do not know please. My girl has a large fatty liver, Fibrosis has liver damage due to her poor diet, we follow a strict diet but I would like to know more. Thank you. This is my email [e-mail]

[Stefanie (Ziba)]

I found a site that ships internationally and this Vitamin C would be safe for your daughter:
www.vitaminworld.com/vitamin-c-crystals-5000-mg.-0070003162.html
* I would give her 1/5 of a scoop per day. If she gets diarrhea, reduce the dose

You should have no trouble ordering online or by phone and having it shipped to you. Let me know if you have problems. 

[Stefanie (Ziba)]

OK, I just saw that product is on backorder and I called and they don't know when it will be back in stock.

I found this one instead:

www.vitaminshoppe.com/p/c-1000-powder-1000-mg-17-oz-powder/vs-1018

[ukbill]

From the NHS web site on vitamins.

Vitamin C, also known as ascorbic acid, has several important functions.

These include:

helping to protect cells and keeping them healthy
maintaining healthy skin, blood vessels, bones and cartilage
helping with wound healing
Lack of vitamin C can lead to scurvy.

How much vitamin C do I need?
Adults aged 19 to 64 need 40mg of vitamin C a day.

You should be able to get all the vitamin C you need from your daily diet.

Vitamin C cannot be stored in the body, so you need it in your diet every day.

What happens if I take too much vitamin C?
Taking large amounts (more than 1,000mg per day) of vitamin C can cause:

stomach pain
diarrhoea
flatulence

[marialia]

Hello
My daughter has grade 3 and 4 fibrosis.
She has 2 years old
Can you help me.
They have been through this difficult situation
I need advice please thank you
My e-mail is: [e-mail]

[ukbill]

Hi sorry it has been so long between you commenting and getting an answer.
Get her on a safe diet and she will be 100% fine.
I will send you a safe foods list. the less fructose the quicker she will recover.
Keep smiling all will be fine.