Suspected HFI Dad (also gluten & casein intolerant)

[Justin M]

This is my first post on the forum. Hi All!

Our son appears to be extremely sensitive to fructose (we suspect HFI), has a severe milk protein allergy, and also reacted to gluten. He has had diarrhea since birth but we noticed the fructose at about 12 months old. In the 15.5 months he's been with us he has probably only woken up a handful of times not screaming in pain first thing in the morning. Right from the beginning we had problems, he didn't seem to tolerate breast milk and at 8 days old we ended up switching him to Similac Advance, 2 days later he ended up in hospital with a UTI, 7 days after that he was diagnosed with a rare condition called Necrotizing Enterocolitis. Since then we have been fighting an uphill battle to figure out why he has permanent diarrhea and seizure-like episodes (starting at 8 months) and is in constant pain and is continuously exhausted. One of the other big things is he would cry out in pain sometime between 8-10pm EVERY night, our naturopath suspected something with the liver as that is a key time of the day for it.

After being released from hospital for NEC and it was believed he had a milk sensitivity he was put on nutramigen A+, being a partially hydralized formula they thought he would be able to tolerate it. He had continuous problems after this, based on our research we suspected he had a milk protein allergy but none of the doctors wanted to beleive it at 3 months we forced them to try switching to another one (alimentum) as we had heard it had better results for most babies with milk sensitivities and thought it would be a goods step before going all the way to something like neocate which is extremely expensive and is not covered by insurance.

At first things got a little better with the switch but he was still having diarrhea 12-15 times a day. His weight was extremely low (3% on the chart, he started at 35%). To try and boost his weight they recommended introducing solids, we tried with an all-organic, as "free" as possible brown rice cereal. He had a severe reaction (hives lasting 3 days) and we discovered the cereal may have been processed in the same facility as gluten. After fighting with the doctors for a few months we finally got referred to a GI specialist who finally prescribed neocate.

After being put on the neocate it seemed like things were getting better. In the first month he jumped up to 15% on the charts and things seemed to be slowing down, however after a while his weight gain slowed down again and the diarrehea started building up again as the amount of solids he ate went up (we weren't able to isolate to a single food source). My wife's aunt who is coelliac and sensitive to fructose then suggested maybe the GFCF diet was not enough and that we should try cutting out fructose, we tried going to the FM diet and we saw some improvements in both his mood and bowel movements but we were still seeing 5-8 bouts of diarrhea a day. about ~3 months ago we took him to a HFI level diet and within about 7-10 days we started seeing improvements. He started sleeping in a bit (7am instead of 3:30-4:00 am wake ups) he also stopped crying out at night and for the first time ever actually woke up happy a few times in the morning. Since cutting out all fructose his weight gain has increased considerably, he jumped from 15% to 50%.

The most recent case of fructose by accident was he had a fluorscopy and the barium solution contained significant amounts of sorbitol and he has been a basket case for the last 10 days again. He also started crying out again in pain and waking up screaming in the night again which he hasn't done in months.

So far his blood sugars, enzymes, etc have seemed to be nearly-normal however any time he has even a little fructose it can be a disaster for days or weeks. The "main 3" mutations came back negative. He has always been a difficult child to diagnose with anything, all his tests come back normal or near-normal. Even with the UTI it was just our suspicion that something was wrong that got him diagnosed, his temperature only spiked for a few minutes (was normal at the hospital) and even the culture came back as barely above the threshold. The NEC incident happened while they were preparing to discharge him and it was us saying continuously "somethings not right" and him having a bloody diaper in front of the doctors that finally got them to look deeper.

He also seems to have several sensory processing issues and the doctors just seem to think that he has autism (we've only been saying this for the last 12 months) and extreme sensitivity to change and that he is reacting to slight internal changes and that there is no medical basis for his problems. They even encouraged us to start re-introducing all the foods we've worked so hard to cut out. What they don't seem to realize is the only reason he is doing as well as he is, is because of all the cuts we've had to make and the impact on his mood/health.

Its very difficult for us to truly determine which foods he can tolerate and can't as we have never had a "baseline" to compare to. We've also been avoiding red meats (due to inflamation and damage to the bowel from NEC) and soy (due to risk of allergic reaction).

I guess my question to the forum is for those who have had children diagnosed with HFI, what were they like as a newborn-1 yrs old?
What resources do you use to find out what foods are safe?
Working with my wife's aunt we've created a pretty extensive list of foods and their fructose content using various online databases. In cases where the fructose content isn't known we've assumed any sugar is bad sugar. In total we've compiled a list of about 204 foods, of which we've found less than 50 that he can tolerate to some degree.

[ukbill]

Hi Justin welcome to the site I have not had time to fully read and digest the problems you are having.. I sympathise with your situation.

How a baby is with HFI depends on how much sugar and fructose they are getting.. with No fructose we can appear 100% normal all the way to limp semi comatose critically ill with liver and renal failure, not to mention throwing fits and seizures as well.

There seem to be several close cousins to the HFI condition which include multiple food issues (casein and lactose + others)which may also be involved here.

I have just come off a night shift so do not have time to read and research more just now. need my bed ..

We are a friendly family and like any family we occasionally argue between ourselves.. do not be put off with this, its good as it keeps us all on our toes and learning all the time

Keep smiling.. if we can help here we will.
Bill A.

[nicoleh]

Hi Justin,
I haven't got long right now but I will point you to a few things to look in to.

GAPS (gut and psychology syndrome) may be just what he needs for his gut. very very hard to do completely fructose free, but some elements can be done at least and if he tolerates any veg at all it is possible.

you could also see if your doctor can test him for MTHFR. this is strongly associated with autism, inability to detoxify things and also gastro problems. I recommend mthfr.net for good information. we have just found out my daughter has 2 copies of this mutation and it explains a lot. multiple sensitivities etc.

My dd is probable hfi which seems to be on top of the dramas caused by mthfr - my other two daughters don't have hfi or fructose issues but both seem to have mthfr (test pending). so I really can't tell you what she was like as a newborn without giving the disclaimer that she has issues caused by mthfr and other stuff as well as the fructose stuff. but basically - yep, I hear you. much high-pitched screaming and diarrhoea and no tolerance of gluten or dairy in my breastmilk for a very long time there. for us, me removing those foods but also removing vitamin B6 helped a lot - have to give activated B6 (P-5-P) instead. Interestingly the fructose made her sweaty, weak, floppy, vomit, diarrhoea, hiccoughs, excessive thirst and urination. the high pitched screaming was more to do with other issues I think. Other people on here with kids that "only" have hfi can give you a better guide. I do know that if his formula was fructose free then he shouldn't have had any signs or symptoms related to hfi at that time he was on it alone.

as an NEC baby his gut will be very raw as you note. probiotics can help restore balance if they are tolerated (Check the full contents) and pure glutamine and glycine supplements are also highly recommended for gut healing. these are things to discuss with a holistic doctor or well qualified nutritionist. if you tell us where you live I can possibly give you a name of someone to try.

these babies can make life difficult but I'll tell you that the GAPS and other work we've done with both my older girl's food intolerances have completely turned their health around. and my youngest DD is now doing really well as well. there's hope! The first step is probably to make certain that he doesn't have HFI - to the extent that this is possible. Are you considering the liver biopsy or full gene screen?

[Justin M]

Bill,

Thanks for the well wishes, we try to stay positive but its difficult when he wakes up every morning screaming in pain (he was up at 4:45am this morning).

Nicoleh,

Thanks for pointing out the sweats, he was always an extremely hot baby (one of the reasons a fever is difficult to identify with him). He also had excessive urination, the one morning he had 8 soaking wet diapers in < 5 hours. He constantly was sweating, to the point that holding him for more than a few minutes would leave you a nice big stain on your shirt. In the winter we were outside in -40oC weather and he was perfectly happy, in -20oC he would go outside with just a long sleeve shirt when we needed to calm him down. We are considering both but probably the full gene screen first. One of the arguments against HFI from the doctors is it should have started with the introduction of fruits/veg, however after the NEC we had to go onto a variety of formulas, looking at them in retrospect they all had fructose, he also was on TPN which has a history of causing liver damage.

Feeding history:

Day4-Day15 – Similac Advance – 55g Lactose per 100g
nutritiondata.self.com/facts/baby-foods/10031/2
Day15-Day25 – TPN – dextrose only
• TPN liver disease or damage. TPN increases the risk of having liver disease and damage. Infants and young children on TPN are more at risk for liver disease than older children and adults. The organs of infants and younger children are still developing. They are not as capable of handling the burden and strain that TPN puts on them. Children who are on TPN for a long time are also more at risk than those who are on TPN temporarily or for a short time. Some children who require an intestinal transplant may also receive a liver transplant at the same time due to liver disease associated with their use of TPN.
Month 1-2.5 Nutramigen A+ - 41g carbs, source: modified corn starch (aka HFCS) & corn syrup solids – can’t get an exact % on how much is sugar
www.enfamil.ca/en/products/feeding-solutions/Pages/nutramigen-a.aspx
Month 2.5-8 Alimentum – 4.4g sucrose per 100g
nutritiondata.self.com/facts/baby-foods/457/2
Month 4+ - solids, fruit within a few weeks??
Month 8-16+ (current) Neocate – glucose only

We'll take a look at the MTHFR as well. One thing that is interesting is he had to wear a helmet for plagicephaly, when he finally graduated from it the orthotics specialist noticed that there was a section of his skull which had not fully closed up from it (one of the signs of the MTHFR), also my wife has glaucoma which is another.

[claudiarivas]

Hello Justin!
I understand your fustration , especially when there is not a determine diagnosis. My son has HFI, he was diagnose by genetic testing when he was 5 months. He didnt show any symptoms ( breastfed and on enfamil premium as formula) until he was 5 months and they change his milk to similac sensitive ( which doesnt have lactose,but instead fructose ) and introduce fruits. His symptoms were: extremely lethargic, he slept all day, he vomitted after feedings, gag at the food/milk and when tested his liver enzymes were highly altered.
I am no expert and far from being a doctor, so I can 't tell you what to do, but if you want to try an HFI diet this is the place to learn, in the ingredient source section there is a lot of HFI safe foods. In my case, my kid is doing great and he hasnt had any symptoms , I put his diet on an earlier post. I would recommend you start with WHITE rice, potatoes, oatmeal and chicken. About his milk, check the ingredients, my baby did great on Enfamil premium and now at almost 2 yrs he takes similac go and grow.If I were you I will keep pushing for a diagnosis ( I know thats what you are doing) but I promise that when you know what your baby has , it would get easier!!! so just hang in there and have hope.
Have a good day!!

Claudia

[charlie]

Hi Justin,

welcome to the board to all your family. I hope we can be of help.

I read your post earlier and have delayed answering while I had a think about it. So where to start, please correct any of these that are wrong:

Your son is 15.5 months old?

Has had the DNA test for HFI? that came back negative. That doesn't mean absolute negative as I assume you already know as there are possibly unknown mutations but makes it less likely.

But he shows in your opinion what appears to be very clear reaction to fructose.

OK, of paramount importance is to keep it simple and only one step at a time. At this stage as his body is so reactive at this age things seem alot more complicated than need to be. Plus he has already had lots of things going on with his digestive system. But it sounds like you are in good hands medically and they are on the case.

The problem is that very little is really known about these sugar reactions and the parameters change almost daily as the child grows and develops, is subjected to other germs and bugs, the climate, the weather, teething, learning to put the wrong things in their mouths etc, etc, etc. So you need to take one step at a time as sometimes things are affected by a build up of reactions too. Keep a very clear diary of foods eaten, include brand for ready prepared stuff, his moods, his poos, his weeing, sleeping pattern etc. It takes a very long time for the full picture to build up. When you introduce a new food leave at least 4 days before introducing another while he is so small to really get a clear picture and build up a basis of safe foods first, don't be tempted like so many do to try and build up variety in his diet, for these kids it just doesn't work and you end up getting very confused.

It is becoming more and more obvious the more people join these boards with kids (and I myself have been down this route) where it looks so obviously one thing but the tests keep drawing negatives. It ends up looking as though there is a problem with multiple sugar absorption problems, not just one and these are usually the polysaccharides - sucrose (and alot of foods containing sucrose also have fructose hence the confusion), lactose, maltose, etc and their bowels do not produce enough enzymes (dissacharides) or any at all to break them down properly so they end up sitting in the large intestines and ferment, draw water to them and cause this vile watery, smelly diarrhea. I used to have to change Megs nappies really fast or they burned her. This also then alters the balance of sugars being absorbed properly into the blood to fuel them through their day leading to hypoglycemia. The osmotic balance is altered leading to metabolic acidosis and at the end of the day the poor kid is fed up and trying to function through a fog. Interestingly also alot (including my daughter) end up looking as though they also have autistic spectrum disorder so there may well be a link. As your son had NEC early on then there may well have been considerable damage caused to his absorptive processes via the villi which may take years to recover from.

But the process of diagnosis is slow and takes alot of guess work so simple steps is best. Claudia is probably a very good guide on the HFI diet as Carlitos is close in age and he is positively diagnosed. there are a few undiagnosed, suspected but possibly nots that are not such a good guide. Pop over to the FM board too, I am in the process of updating that for food ideas on the FM diet as those produced seem good for the adults but too much for the children that have had it from birth. Avoid the temptation of looking up too many other syndromes, there are so many unusual conditions that people have been through but you can end up driving yourself (and your doctors) mad following them all up as possibilities.

Your lad is at the ideal age for you to control what he eats and therefore be able to use the elimination diet, there is no reason why at this age if he likes it he shouldn't have chicken and rice three times a day as a base diet. It is only us as adults that think we need variety.

As for food databases, looks like you have them all covered already, in fact you have passed one on I didn't know about so thanks for that.

Good luck, keep asking, we are here to help.

Charlie

[Sherri s.]

Wow I had so many memories come flooding back as I read your post.....my son is only a fructose malabsorption patient and not even tested. His pediatrician suspected the condition, had us try the diet and was dead on so we haven't even pursued it beyond controlling his diet. There is little that can be done beyond that anyhow!
I just want to encourage you not to give up:) it's so interesting how a child with fructose issues has such distinct relief after its removed from the diet! For them at least, it really is evil stuff!
Also, I would encourage you to keep researching but like someone mentioned, try not to go crazy doing it:) I wobbled on the brink of it:) as more and more digestive issues surface, there will be more and more data to interpret!
So sorry I don't have any breakthrough info or useful suggestions:( just a bit of moral support! It's tough!
Hang in there!
Sherri