HFI and now Kidney Disease

[donna]

My 26 year old son has just been diagnosed with kidney disease. He and his brother were diagnosed with HFI when he was 4 and they have followed a strict HFI diet ever since.

22 years of his high protein diet and 2 years with protein shakes recommended by his personal trainer and his kidneys are most unhappy. His body has swelled up with oedema. He spent one week in hospital and has been lying down since then (2 weeks). Before that he was very fit and went to the gym 4 times a week.

The renal dietitians have not been able to offer any help yet and are still at the hindrance stage, and can't give an appointment till the end of January. At least they are listening to me now about HFI. At first they were telling him to eat fructose containing foods because they believed he was Fructose Malabsorbant, never having heard of HFI. They have me second guessing what he should now be eating, giving me lists of 'okay' and 'sometimes' foods that disagree with what we have practised all these years. We are desperately trying to reduce his sodium and his protein while keeping fructose at bay. But it is impossible. His world has been turned upside down and we are endeavouring to keep his spirits high while we try and sort his diet out.

22 years ago we decided it was better for the kids to not get a sweet tooth as we (doctors and us) believed they would therefore be able to recognise unsafe food and reject it. So the boys have had a bland diet since then. We never gave them aspartame foods (diet pepsi, cakes, etc.). Now any sweet taste is foul to them.

I have just discovered this forum and see that some of you refer to HFI being more and less severe in different people, I was unaware there were different degrees of HFI. I also see differences in what vegetables if any can be safely ingested. Back then they only talked in terms of ingesting 'tiny' amounts which could be passed out of the body, and 'large' amounts which could induce a coma. They could never state an amount, can they now? The 'Minimal Fructose Diet' the dietitian gave me suggests ingesting 2g of fructose per day. How do I know if that is safe or not? Are the dietitians still thinking he has fructose malabsorption and not HFI? I can't trust them yet.

I have an old printout from USDA (US Dept. Agriculture) which shows individual sugar content in foods and it is most helpful for HFI. I have a similar one for protein content in foods. But it doesn't matter how many labels I read or lists I look at, I am not getting anywhere. Short term we have to reduce his sodium, fluid intake and protein to reduce the oedema and get him up off the lounge and hopefully back to work and his life (possibly 3 months). Long term we need to reduce his protein.

He is now suffering cramps in feet and hands - perhaps reduced sodium or reduced fluid (only 1.5 litres per day). And he suffers stomach pain if he eats a normal sized meal, so is having smaller feeds more often. I don't know how to feed him something low in protein that isn't high in sugar and/or sodium. We are yet to sit down with the kidney specialist having only seen him in hospital. But we have an appointment with him this week. I'm confused what will happen and I'm really just having a whinge - but thanks for listening.

[ukbill]

Donna, welcome to the site!

I am sorry your lad is suffering and I might be able to offer some help.

The only person we know who has been accurately tested for tolerance levels of Fructose had an ability to tolerate 100mg (1/10th of a gram) a day..

This is far lower than the 2g your dietitian recommends.

How is his liver enzyme level?

If the problem is HFI related there will be damage there also.

Personally I would shoot the fitness trainer who recommended "protein drinks" well not really but certainly give him a serious talking to.. I am English after all

Have the drinks been checked for things like sugars?

All the "protien deinks" I have seen are ususally full of the stuff and occasionally steriods as well if they are not a reputable make!

I am glad to hear you took the sensible way with his diet, believe me you have given him a much better chance at life doing it that way!

There is another posible cause thinking about it..

When we exercise if we convert body fat back into blood sugar.. this includes FRUCTOSE!

This is why dieting for an HFI is so difficult.

Being sweetness free in the diet is not "bland" though you should try some of my curry!

You are right labels only get you so far.. taste buds get you the rest and keep you far safer than a label ever can.

I have great difficulty with food labels they are so inaccurate as to be next to usless..

Wishing you a better xmas and sending you best wishes for the new year.

[ukbill]

Just had another thought the "Protein drink" did it have sorbitol in it??

That dose not cause an HFI reaction but breaks down into Fructose in the intestine and I think kidneys.. but not sure on that one.

All the top athlete type people now just drink skimmed milk with added milk protein it works better than ANY bought concoction apparently, and is not full of nasty chemicals!

Not to mention a devil of a lot cheaper!

Worked quite well at the Olympics this year anyway!

[donna]

Thanks ukbill,

we checked the protein drinks to make sure they were HFI friendly, and we often told him he didn't need them as his diet was pretty much only protein anyway, and we often told him that skimmed milk on its own has been proven to be of equal benefit to protein shakes - but to no avail. But that is all in the past now anyway as he will NEVER take them again. He evidently had the kidney disease for some time and they just finally got annoyed enough to react. I have the same kidney disease (diagnosed 11 years ago) but they never told me there was a chance my kids could get it. Doh!

Thank you so much for your comment on the 100 mg fructose per day, it would be good to hear of any other folk who have a confirmation on this or another measure. I'm pushing to see a specialist on HFI (if one exists) and then maybe we can get a number. I'll take on board other info you and others have mentioned. When I read of HFI now I notice mention of kidney disease as being on the list of possible outcomes - I had never been aware before, or maybe I had and had forgotten. Seems I passed more on to my boy than I wanted to.

[ukbill]

Hay don't go knocking yourself out about what you have passed onto him, its a lottery for everyone.

Which part of the world do you come from or more accuratly which part are you in now?

I know some and more importantly who knows the rather good specialists in Australia, USA and the UK.

Wishing your son all the best and a speedy recovery.

Bill A.

[charlie]

Hi Donna, welcome on board.

Like Bill says, don't worry about what you passed on, I blame my partner, I say he showed up, contaminated my genes then b******d off....................... not my fault at all!!!!

Fill us in a bit more on your sons history so maybe we won't go over old ground for, if you fill in your location in your profile you may find someone else in your location spots it and will know the local good specialists. also you may find food differs from country to country.

First and foremost has your son had a positive diagnosis for HFI or just by guesswork and elimination. If not then getting a gene test would be your first step to getting clear answers.

[ukbill]

I can confirm when I met Charlie I could see nothing wrong with her genes or what was filling them My Dyslexia kicking in a bit there maybe

So I blame her partner as well

Although Blame is not the right word.. If you ever meet Megan you will see, she is a wonderful bright fun kid.

I know Charlie has a more than a few problems trying to get Megans diet sorted not to mention the NHS and schools!.. but tell me a parent who hasn't had problems..?

And as such her problems, put in perspective, are not as bad as some I know of who's children are Genetically "Normal" whatever that means?

I know Charlie is very Proud of her daughter and so she should be. she could have done a awful lot worse I can tell you!

On the Family history front can I add my request for information too please?

Also I would love to know how your lads feel about being HFI how are they dealing with everyday life?

Many people think they will have been "deprived" by not having sweet foods.. I for one could not disagree more.

I think what you have done is absolutely perfect to give the lads a good set of protective skills which will help them through all of their long lives.. but that's just me I would love to hear their points of view on the subject.

I bet they have never thought about it and just get on with living

Other than your lad having Kidney problems have they had any other problems? how did they manage through school etc?

Sorry bombarding you with questions.

I hope he is on the mend now .

Keep smiling

PS cornflour skimmed milkshakes are very good for long slow glucose release into the blood stream, great for avoiding Hypo's as a result of too much exercise and help you recover real quick too!

PPS Only just found out about them from Charlie and they really work a treat! Wish I knew about them 45 years ago! (or even 55 years ago!)

[donna]

We have seen the kidney specialist who of course knows nothing of HFI, but we are now going to be referred to a specialist (probably a gastroenterologist?) to: a) confirm HFI diagnosis; b) check out his liver size; and c) see where we go from there.

History:
From birth, my son Tom (now 28) had a tough start and feeding never went well till I found tinned brains. We had tried every food we came in contact with and the doctors were suggesting he had a problem swallowing. The very last tin we tried in babyfood was brains. He loved them and had them often. His diet was funny and we couldn't work out his taste preferences. Our local doctor wanted me to try hiding veges in his food! On a few occasions, to be social, Tom tried to eat like others and each time threw it up straight away. So when this happened when he was 5 I took him to the Children's Hospital in Sydney. I was met by a rude doctor who was sick of people 'looking for miracle cures', but who did examine and found he had an enlarged liver - but she didn't tell me. She referred us off to a gastoenterologist for some other reason I cannot remember now. I chose not to go because I didn't want to be abused by a doctor again, but the hospital asked me to come back. And coincidentally, the doctor we got to see had done research on HFI, so when he listened to the history he knew what it was before testing. They did a liver biopsy on Tom then when he was 5 and his liver was about 2.5 times what it should have been. 4 year old Shane's liver was also enlarged at that time so they said he was HFI too. Up till then we just assumed Shane was copying Tom with his food dislikes. Both had blood taken for testing as did their oldest brother. The doctors said they were looking at perfecting, in conjunction with England, a blood test to detect HFI, and I seem to remember that some time later they said they had.

Immediately following diagnosis I put both the boys on a very strict diet for (I think) 3 months with NO fructose and both of them had their livers return to normal size - something the doctors had not expected. In discussion with doctors we decided to keep them away from sweet tastes (aspartame etc.) as a method of protecting them from the outside world and it worked. Their taste buds were and are so sensitive that they detect unsafe foods from smell, or from taste. When younger they may start to eat something (eg. bread) but after a few bites would realise it wasn't safe and reject it.

I taught them from the time of diagnosis what was happening, but as they were young I had to keep it simple and serious. Whether I said it that way or not I don't know, but their short-cut answer even these days is 'Sugar will kill me' - and that often stops budinskys who try to feed them the wrong stuff. Anyone they came in touch with - friends, friends' parents, relatives, teachers - were all told that the boys were intolerant and bottom line was 'The boys MUST be listened to in regard to what eat' - and 'Even when the boys start to eat something they may stop eating it whenevery they want'. This kept them safe when at friend's houses, or at school camp etc. Of course they sometimes ate unsafe food but usually only in small quantities. They can both relate incidences of 'feeling sick/queasy' after eating unsafe foods. But never more than headaches or upset tummies - that I know of. Generally speaking the only fructose they did ingest is from potatoes which I did limit to only 2 or 3 times a week. Unless of course, we have been misinformed all this time and they have been eating more fructose than we believe. They will both on occassion eat a cherry tomato (if we have them) although they take about an hour to 'savour' it (it's only the size of a marble!).

I remember the specialist at the time telling me to only serve them 'new' potatoes as they were high in starch which converted to sucrose as the potato aged. We don't seem to have 'new potatoes' in the shops any more and back then they were a lot dearer. But in my recent reading I am seeing that they should only eat aged potatoes. So that's a biggy, I'd like an answer on that one. Also, the listings I have show that cooked spinach has no fructose, but neither of the boys like it. And they both drink Vodka - after we discoverd it contains no fructose. They took their first taste of vodka about 4 years ago and drink it with chilled water with lemon or lime in it. I always believed they could have lemon juice but not the lemon and not lime - but current reading makes out that is wrong as well.

As for exercise - Shane has been working out for over 2 years now and never reported any problems. Tom has often taken on bouts of exercise and never reported any problems, but I will raise the issue with him and get some feed back. I have sent the link to this forum to Tom but I expect he won't bother with it.

They have just learnt to live with HFI and not make a fuss about it, and they hate people treating them as interesting science experiments.

Way back then, I put a short note in magazines asking for other HFI people to contact me. I remember one reply from someone whose daughter was allowed sweetened food, but the best response came from a lady whose 66 year old husband had similar 'tastes' like my boys. We met them, and she had made morning tea. My boys tasted her pikelets and refused to eat them. She had been feeding her husband tiny amounts of fructose in every meal because they didn't actually understand what it was he couldn't have. The boys specialist met with him and confimed his HFI. So his wife changed his diet and he had the first days in his life without headaches. He was soooo happy. His mother had abandoned him when he was young because he had weird food preferences. We lost touch with those people over the years.

[ukbill]

Donna I am coming over to Australia in August next year on a sponsored motorcycle ride.. maybe we could meet up ?

Re. new potatoes..
Yes they are far too sweet to eat.. old floury potatoes are the best but still not as safe as Rice or pasta.
Lemon Juice is good for me but Lime is very iffy.. although it might be a variety thing again I once had a lemon which was very full looking (if that makes and sense) it was very round and the normal deeply textured skin was far smoother than normal (more like an orange) this was far too sweet for me.. UUK nasty.

Spinach is one of those things you either like it or don't..

When I have had tinned and frozen Spinach and it is never good far too sweet. Fresh spinach is OK but I pull off all the stalks and only eat a little once a week at most.

I like it best just cooked in softened unsalted butter and a sliced clove of garlic in a fry pan, and just the green leaf parts freshly washed, no water a finger of sea salt and a little black pepper,

It is lovely put on top of a soft topped omelet with a good slice of smoked Salmon and folded over so the salmon is in an omelet sandwich so to speak (with no bread) Eat it with fresh baked white bread and butter .. Pure Magic! ( great as an xmas morning treat!)

I am SO pleased to hear your story it is exactly what I keep telling parents here..

Feeding Sweet tasting food to an HFI child is just wrong!

In OH so many ways and for far too many reasons to list!

I wish you could post your story of how you coped with not feeding them sweet foods and the excellent result you have in the children's section.. because most parents think their children will "miss out" if they don't get sweet foods when in reality the opposite is true.

I am so glad when I go into a cake shop I am not feeling like I am missing out on anything the same in a sweet shop.. It would be terrible if I actually liked the stuff (like chocolate) but could not eat it..

Many Doctors and Medical professionals read this forum as well and hopefully they will get the message too!