Liver failing?

[danielle]

Hi everyone,
I am new on the board but have been reading for a while.

My son is 14 months old and has been suffering since a couple of week after he was born from something we are still trying to determine. Dies has been screaming in pain since 3 months old and after introducing bottle feeding ended up in hospital because he also started vomitting. Nothing could calm him down, but eventually the Neocate did. But then food introduction started and the whoile nightmare of creaming vomitting and now sleep started again. Although he did not eat and vomitted a lot he looked very chubby and bloated but he didn't grow in hight. I have always found that weird and scary, docters said it was the Neocate. After several months of reintroducing foods Dies was finally taken into hospital for research, he had severe esof*gitus and candida all over. He was tested negativ for EoES. Docters said it was all due to the reflux. Intervenus medication helped and he could finally swollow some food, but the vomitting stayed. I then found that is was the fructose and sugar that made him vomitt. Lactose was breath tested negative. I pushed the docters to DNA test him for HFI. He is now on a fructose diet for 2 months and the vomitting stoped and he finally sleeps through the night. But sometimes the reaction is there again so I am very uncertain if I need to cut down on more in case it is HFI.
His reactions now become clearer, because he grows older? because he is on the diet? Reactions are always at night, he vomitts and has severe reflux, fever for a day, diarriea the next day. He doesn't eat for a couple of days and then recovers.
Yesterday I got frightened, he looked very pail, dark circles under his eyes, and he almost passed out. I got him to stay awake and in the end he creid through the night, vomitting even water. I am afraid it is HFI but we are still waiting for the results. In the meantime please advice; Does this sound like HFI to you? IF it is, what does liver failure look like? How should I react?
Please help
Regards Danielle

[ukbill]

You need to talk to Carissa Gunnlow jdsmomey2009 she is on Skype and here with that name.

What your child is suffering sounds like JD

She is in Florida email [e-mail]

Welcome to the board I hope we can all help.

[danielle]

Thanks for this! I will try here.
Can you tell me what JD stands for and what it is?

[ukbill]

Its his initials James.. D

I cannot say for certain if your child has HFI or not, by what you have written..

Although it looks like a strong possibility, was he perfectly OK on breast milk?

The problem you have is if he's HFI then his liver by now having been fed countless amounts of fructose in the formula he has been eating, will be in a very poor condition. (like JD's)

So he will be ultra sensitive to Fructose but will show similar symptoms even if he has not eaten any Fructose for days, weeks, months.

An explanation of HFI in a nut shell.

The initial effect of eating sugar (or Fructose) is our blood sugar level drops as a result of Hypoglycaemia.

We feel VERY ill during this phase, shakes, vomiting, poor brain function etc..

After about 1 to 4 hours we start to recover, however the brake down products from the Fructose, are for a few days causing damage to our liver, kidneys and in extreme cases brain.

During this phase we are generally just feeling really "foggy" and have various pains (kidney pain is common). This can last for days to more than 6 months depending on the amount of Fructose consumed.

We can also go Hypoglycaemic if we are hungry and do not have any food to digest and so are running on internal food resources (body fat) which when converted back into energy for the body to use convert both into Glucose and Fructose.

Some of us can go Hypoglycaemic if we get thirsty as well, the reason for this is yet to be explained but could be something to do with the kidneys.

In a "normal" (if there is such a thing) HFI person we still produce a very tiny amount of the enzyme needed to break down Fructose in our kidneys.. however about 20% of us are 0-0 double zero.. IE we produce None of the enzyme at all.. for those with that condition HFI is a different beast and quite a challenge.

The Good news is IF your child has HFI then a long and happy life is available providing the amount of Fructose ingested is kept to a very low level.

However the first step is diagnosis. Do not have the chalenge test! or the test for DFI which would make your child drink a quantity of fructose solution before doing a Hydrogen Breath test!

This test should only be done if HFI is completely ruled out, the test is highly damaging to HFI people.

The current Genetic test only proves positive in 80% of cases so just because the test is Negative it dose not mean that HFI is not the problem.

Liver Damage unfortunately can be a cause of acid reflux as can a lot of things, so been as he is only sick after being put down to sleep might be a result of the acid reflux not HFI.. a "normal" HFI will vomit shortly after eating anything sweet.. although this might not happen in some cases where the only foods available are predominately sweet.

How is he on cows milk, cheese etc. ?

[hfimomof3]

I am not so sure that this is HFI. For example, you say that after you put him on a fructose-free diet, his symptoms stopped, but that sometimes the reaction is there again. It sounds like you are thinking this is because his diet is not sufficiently free of fructose (and I suppose that could be the case) but couldn't it instead be that his symptoms stopped for some other reason and the fructose is not the problem?

In terms of whether you need to cut out more fructose, what are you feeding him? It is true there could be hidden fructose, for example whole grains have more fructose than refined grains. So brown bread will be more of a problem than white bread, and brown rice more than white rice. But, if he had symptoms even on Neocate, I am not so sure it is HFI.

You also say that he has periodic fevers and that yesterday he almost passed out and looked very sick. This is very concerning. I am not so sure this is liver failure at all but I'm not a doctor so I wouldn't be willing to take a guess as to what is going on here. To be honest, I would be taking him into the hospital with the symptoms you describe. If it is liver failure then they can do tests on his liver. If you wait and he recovers, then perhaps those tests will appear normal and you will not get the information you need. If it is not liver failure, then it could be something else that, again, having him seen WHEN he is having the symptoms will help them diagnose him.

In either case, a child who is feverish with diarrhea and lethargic like that needs to be seen by a doctor right away.

[Tammy]

I agree with HFImomof3. He really really needs to see the Dr immediately when this happens.
I also don't know whether it is HFI or not, and it is NOT for us to decide.
Some people with HFI get sick just as Bill describes, others differently. For some it takes a day or two until they get sick. So if the DR does prove it is HFI, you also have to look at what he may have had up to a couple of days ago.
I have never heard of fever being a sign of HFI. Also, it would be any time of day, not just at night.

If it would be liver damage, the dr would still be able to tell, even now after the episode. Please take him and tell the dr about it.

Also, where are you located? When you say what he is eating, it makes a difference as things that are safe in one part of the world may not be in another.

[jdsmommy2009]

hi and yes like bill said u sound like ur going throught what I went through and at times still deal with

[charlie]

Yes, i agree with the others, due to his young age do take him to the doctors. As tedious as it may be you need to take him every time he is ill just to get taken seriously. My daughter as a baby was perfect until we started weaning and then the vomiting and diarhea, skin rashes and being poorly started. She was really bloated, we used to call her little buddha. she also does seem to get a fever if she is having a bad reaction but it is sometimes hard to tell as sometmes there are bugs going round she just gets worse than anyone else.

I took her to the doctors the first few times but got nowhere so tried to manage it at home. Then things got too bad, like the screaming pains at night so I called ambulances a few times, then they started to take me seriously, luckily her doctor had heard of HFI.

But to start with they said just take her off fruit and sugars but vegetables were OK so she improved but not completely. It wasn't until I eventually took her off absolutely everything on the list that she improved. So do make sure , if it is HFI that things aren't slipping through.

But firstly do get a proper diagnosis quickly to make sure no further damage is being done.

Good luck, and welcome to the board.

[danielle]

Thank you so much for all your concern and answers.

Dies is doing a bit better today...the fever just lasted one day...but he doesn,t eat... We eat out Saturday night and I am afraid it was the chips. We thought this would be safe.... Or like some mothers said you think....maybe it is a bug. But he had the same thing two weeks a go, when I know he got hold of his brothers cup with juice.

As for replies to your questions;
- We are from Holland;
- Dies is on a fructose diet from the dietician, I searched and googled and discussed HFI with the doctor on my own initiative. He gets no fruit and no sugar. I make sure there is no sugars in his bread, cookies etc. I don,t give him to much tomato and no red pepper. But he does get vegetables and brown bread.
- We took him off his reflux medication, because it had sugar in it. He doesn,t suffer from reflux now, only when he has a reaction I hear him thrive, swallow and cough, followed by vomitting, rumbling tummy and diarrea the next day.
- He was restless on breastfeeding, but escalated on formula.
- He calmed down on Neocate but we found out that is was the Neocate porridge formula that made him vomit.....this had sugar in it;
- We just reintroduced lactose products because his hydrogene test was negative...started with yoghurt on wednesday...he was bad on Saturday? Back to basics now and reintroduce?
- He has calmed down a lot on the diet but sometimes gets hold of his brothers apple or cup with lemonade....then he has reaction within a few hours...takes a few days to calm down.

I find it very hard to get the right diet for him. I feel we are very close as he does now sometimes sleep through the night and is happy.
I could use some help on what a HFI diet looks like so I can try to also rule out sugars from vegetables and bread.

I speak to our doctor tomorrow. What else can I ask the doctor to do, other then wait for the DNA results? Check his liver function?

Thank you so much for your input!
Regards D

[hfimomof3]

Tomato, brown bread, and some vegetables, could definitely be problems if your child has HFI. You probably want to talk to a dietititian about finding vegetables that are free of sugar. Does your child still take Neocate? My understanding is that children can be on Neocate for years as it is designed to meet the nutritional needs of children with severe dietary restrictions. Did your doctor discuss this with you?

You say he eats cookies? Are these cookies you make at home? Also, how did you find a brown bread that is free of sugar? I have never seen such a thing, they are usually quite sweet. Even white bread is often sweet, and I find there are only a couple of brands I can tolerate. Did you know that if you have HFI you are not supposed to eat sorbitol?

I am a little worried that you are suggesting a lot of the management to his doctor. If you trust his doctor, then you need to let the doctor diagnose the problem instead of doing the diagnosis yourself. The fact that he is having repeated fevers is concerniing and you don't want the doctor to miss an important puzzle piece because you are pushing HFI as the diagnosis. I am not saying he does not have HFI, just that you need to let the data drive the diagnosis, not the internet.

I would do as Charlie describes, every time he gets sick talk to the doctor and let the doctor gather the information about what things seem to be in common. If you feel like your family doctor or pediatrician is not managing it actively enough, then maybe you can see a GI specialist. But, don't try to be the doctor, you will miss things that may be important.

[ukbill]

I agree with all of the above..

Holland is a lovely country and you do make very good beer too

From my experience of eating in Holland and living 5 minuets from the Dutch border for a year.. the food is very HFI compatible you should have no problems with his diet once its stabilised.

It might be an idea to keep a log of the food he eats and what the reactions are so you can eventually narrow down the things he can and cannot eat.

Tomatoes are usually out for all of us on HFI diet as are all root vegetables like carrots , turnips beetroot and things like peppers etc..

There are VERY few vegetables we can tolerate.

You stated "started with yoghurt on wednesday" I presume this was plain unflavoured yoghurt??

All of the flavoured Yoghurt have added sugars even the plain Vanilla flavour, also it might be that the production line had just come off a sweet yoghurt and there was a little contamination possibly?

I have had no reactions to any bread I have eaten in Holland, France, Belgium, Germany or the Czech Republic. I do know by experience which to avoid and my Liver is generally in good condition so I am less sensitive these days.

Some of the heavy brown or Black breads have molasses added but many are not too bad.. until you factor in the effect of the fibre breaking down in the gut..

For HFI you also have to avoid digestible fibre as found in brown breads.

This converts to sugar in the intestine inflaming the bowel and causing diarrhoea and possibly a slow HFI reaction sometimes days later.

The Hydrogen breath test is not for Lactose intolerance unless I've missed something its for DFI which stands for Dietary Fructose Intolerance. (I think).

At least Charlie and I are nearly in the same time zone as you are.

It sounds like you are getting somewhere anyway, but his diet will need refining some more yet in any event even if he is not a "normal" type HFI if there is such a thing. ;D

In any event we are all here to offer our advice and help to you. So take heart help is at hand..

[danielle]

Hello again,

Today I started addapting his diet to no vegetables, no brow bread untill he has calmed down. I will also discuss this with my dietitian and doctor but like I said they do not know a lot about this.
Replies:
- yes Dies is still on Neocate Active. He couldn,t do without it as he does not really eat a lot.
- The cookies I found for him in the health store are made with rice syrup, barley malt syrup or corn malt syrup, all glucose molucules sweetners. I make ice creams with this for him from syrup and water. And I make cakes from rice milk with almond flavour and rice syrup.
- Dutch bread is very low on sugar but what they do add here is what the call bread improver; E451 I believe and this contains sugar. So I get him bread from the health shop. I will switch to wite bread.
- He was first on a lactose and fructose diet, but he just had a lactose hydrogene breath test .Just like the fructose test you get a cup of lactose to see if you produce hydrogene in your breath. We will not do the fructose test untill we get the results from the DNA test.
He was tested negative for lactose so I am now sure it was the vanilla and sugar in the yoghurt when we used to give him this a couple of moths a go. Back then we tried feeding him solid foods a couple of times but this never lasted more then a few days. The vomitting, crying and screaming would increase over 3 to 4 days and he would stop eating himself and would only drink Neocate. We now realise it was the fructose.

Doctor said to wait for the results from DNA test and for now assumes it was a virus......

[Tammy]

Switching to white bread would be a good thing. Just always double check the sugars.
The cookies and cake are fine if that is all that is in it. The important thing is to find things he will eat that doesn't make him ill. Don't worry about what you consider "healthy" until you get an answer from the Dr. If he is HFI, then throw all your "healthy" ideas right out the window. What will be healthy for him is pretty much opposite of what you are used to. Keeping track in a food diary like someone else advised is a good idea. It makes it easier to look back and see what he had.

My son lives in Amsterdam. He's been there 6-7years now. We've been over several times to visit. It turned out to be easy to feed my daughter as long as we cooked. Eating out in the restaurants was a different matter. They just didn't want to be bothered. Now I know I was an American, but My now-ex daughter-in-law, who is Dutch did all the ordering in Dutch to make sure they understood. We still had to send stuff back. She finally got mad, and they got it right. When we asked her what she told them, she said fine, we'd keep it like it was but they better send someone out to clean the puke off the table after she ate it. lol. Then they made her a new one. Imagine that!

[hfimomof3]

so he can drink milk? Why not feed him milk instead of yoghurt? I think that will be simpler in terms of keeping sugar out because usually there will be some kind of sweetener in the yoghurt.

I am not a doctor so I do not want to lead you wrong and please talk to your nutritionist and/or doctor before you do anything suggested on this board. However, I don't think kids need cookies to survive so personally I would cut out all cookies, ice cream, and any sweeteners, even those that seem safe. You say your child steals his big brother's applesauce. Children who are HFI will typically develop a natural aversion to sweet tastes and before HFI was an official medical condition that is probably how most of them survived. If you are giving your child sweet foods then I think this will slow the development of his body's conditioned Pavlovian response to sugar.

I would also cut out the rice syrup as if it is made from brown rice then it may have fructose. I would cut out carrots and onions definitely. Garlic might be ok but will likely make him gassy. Personally my only veggies are spinach, mushrooms, cucumbers (I do eat onions for taste in cooked food but it is a tradeoff of taste versus energy). I also make homemade lemon juice occasioanlly for the vitamin C but it is hard on the teeth. Your child will need vitamin A and vitamin C if he is not eating fruits and vegetables but your Neocate should handle that.

Once you have everything all sorted out and he is symptom-free for an extended period, perhaps you can consider adding in safe types of sweeteners. (I personally wouldn't but there are people on here who are comfortable doing that.) But for now, since you are having symptoms that to me seem very concerning, then I think your priority should be on doing everything you can to eliminate these symptoms for an extended time, and make sure that there is not some other condition also going on here.

[charlie]

Hi Danielle,
at the risk of bogging you down with info so you wish you never asked!!??

If you are heading down the right path and they decide it is HFI then the quickest way to get an improvement is to clear his system out completely for a month or two if you can. This is what I did with Megs and then gradually added things in slowly and kept a note of the reaction. It is important to keep a diary as very soon you find your mind going mushy and you can't remember what you have and haven't tried and what the reaction is. It can also take several days for lesser amounts of fructose to cause a reaction so you need to check back.

The lactose breath test is to test the reaction to milk sugars exactly the same as the reaction to fructose it produces high levels of hydrogen if they can't tolerate it, I was very relieved when Meg was negative to that as she lived on milk.

To start with cut out all fruit, veg and sugars. As Tammy has said, throw out the five a day rule book, Megs hasn't had any for four years and is one of the tallest (and of course the most beautiful!!) in her year, has a healthy sun tan and most times a healthy colour to her cheeks, When she has a reaction she turns grey - almost literally.

Use only white rice and flour - brown grains store more fructose in the husk. therefore for now also cut out the rice syrups etc as they are made from the whole grain. feed as much plain bland food as you can. Search some of the recipes. whenever meg has a reaction I put her on a chicken and rice diet to clear her out and soothe her gut. Home cook as much as you can as processed foods hide sugars in the making process. You can still make it fun for liittlies by making shapes with the food etc. They don't know at that age that it isn't very colourful or varied.
Use old potatoes, not new. avoid processed chips as they may be dipped in sugar solution before being frozen!! Also supermarket meats here are injected with sugar water to plump them up, thats what makes them so much cheaper. Once you start looking, boy will your eyes open wide.

If it turns out to be Fructose Malabsorbtion which is diagnosed by a breath test then once his system is settled you can try the FODMAP diet which is what they have started Meg on as they are convinced that is what she has (mainly cos her liver tests were OK). Then you can use certain types of fruit and veg and table sugar as the glucose/fructose ratio is even but you can't use wheat, onions, garlic etc due to fructans.

Anyway, get a clear diagnosis first then we can send you down the right path, but if your instincts are that fructose isn't helping then steer clear for a short while and see what happens

Keep posting.

[ukbill]

Tammy "When we asked her what she told them, she said fine, we'd keep it like it was but they better send someone out to clean the puke off the table after she ate it. lol. Then they made her a new one. Imagine that! "

That sounds like a good line remember!

I might try that one day..

I 100% agree with what is being advised here..

Charlie I have not had a problem with normal supermarket meats in the UK.. only "ready basted chicken" I know "brining" is common in meats in Australia and so I understand nearly universal in the USA, but in Australia it seems to be only pre-packed chickens and Frozen chickens mainly.

I did not get any reactions to Australian supermarket meats .

Bacon is always one to be careful of of course, I have to watch what is is. Smoked bacon (my favourite) now is far too sweet for me even when the packaging lists no added sugar!

PS. Potatoes can be a problem. white floury varieties are best avoid all red skinned, yellow fleshed, waxy varieties.

To reduce the amount of sugar that will still remain no matter what the variety, make mashed potatoes or if making home made chips soak the cut chips for an hour or more in salted water before cooking. This reduces the amount of sugar remaining in the potatoes.

[danielle]

Good tips thanks. Dies loves chips and the white mashed potatoes...he doesn,t really eat a lot more...and then still chews it and spits it out.

Spoke to the docter. I asked if they have ever checked his liver function. the answer was no...and we have been to 4 hospitals. she also said they do not do the fructose hydrogene test anymore, didn,t know the reason. she would now check if there is a point for me to come to hospital next time he gets bad.....i am so frustrated about these docters! she was not sure what to advice me about his diet. i will just have to find out myself. she did see that he was not bloated anymore, finally growing in lenghth but now loosing weightn so does want to keep seeying him. well that is something.....

[hfimomof3]

So it sounds like you need to get permission to go to the hospital? Where I live, fever plus vomiting plus lethargy such as you describe would get me permission to go to the hospital.

Is this doctor a pediatrician? A GI specialist? What is she going to do about the weight loss? Have you told her about the fevers? If you have fixed his diet, even if not perfectly, then why is he losing weight? How much Neocate is he taking? How much did the doctor suggest he take? What were her thoughts about his weight loss? What tests does she want to do, if any?

I would suggest you go to www.kidswithfoodallergies.org. You do not have a food allergy (a known one anyway) but they will know what kinds of things you should expect when your child is on Neocate, etc.

In terms of his diet, at this point I would focus on determining whether he can indeed tolerate dairy products. If he can, then that gives you a lot of ways to gain him weight. I would skip the yogurt, it doesn't taste that good and is low fat by definition. But, you could start frying everything in butter and it would give him lots of new tastes and also help him gain some weight. You can also get a lot of nutritional mileage out of whole milk

[ukbill]

Try him on White rice cooked in milk.

Preferably the short grain "pudding" or arborio (risotto) varieties as they make a thicker softer meal.

You can blend it with a hand blender to a paste and let it down with milk to make it palatable for him.

[marleen]

Hi Danielle,
I've lost this board out of sight. But today I came round here, because tomorrow my daughter is going to the hospital to see how she is doing again. The doctor thinks for sure she is HFI, but DNA didn't show.

I 100% agree with what is being advised here.

I react to your posts because I'm also from Holland. You say you have a hard time to find someone over here who knows HFI. Our doctor is specialist in HFI. You can find her in Nijmegen.
If you want more details you can send me a PM.

About the bread in Holland. I have found white bread with no sugar, not even bread improver. It is called "scheurmik". I buy it at a bakkery in my city.
If you have questions, just ask.