HFI liver and kidney issues?

[karine]

Hi,

Glad I found this forum. I'm 36 and was diagnosed with HFI following a nearly fatal illness as a baby. Both my brother and I have HFI, although he has it less severely. I try to follow a very low fructose diet and am very rarely ill.

I was followed very closely in France as a child, then since moving to the UK about a decade ago I have not really been followed at all (my choice, I don't get on with doctors...). However, a few years ago I started to get abdominal pains and did not know the source. My GP referred me to have a scan, which showed that my liver and kidneys were "bright" with fatty deposits.

Neither the GP nor the person interpreting the scan knew what this meant aside from having some fatty deposits, and they were confused when I told them that I don't drink at all. Neither of them had heard of HFI. The pains got better and I wasn't followed up. Around this time I found out that Sorbitol was bad, so I stopped having the sorbitol-containing chewing gum that I eat every day.... Probably not a coincidence.

During pregnancy a year later, I was referred to a specialist in metabolic diseases in London where I had frequent blood tests, showing my liver function to be normal. However, when I mentioned the liver and kidney scan results, they didn't know what it meant and just said "don't worry, it's probably ok." No follow up was offered. Having read some of the posts on here, it does sound like this was not good advice... Does anyone have any thoughts on this? Are there any specific HFI specialists in the UK?

Thanks,

Karine.

[Tammy]

Hi Karine and welcome to the board.

I agree that the sorbitol you were chewing everyday was probably at least part of your problem with your liver.

Here is wonderful chart to check and see if any of the other sugars are sneaking though.
www.bu.edu/aldolase/HFI/treatment/sugar_table.htm

As far as Doctors in the UK, I'm sure that Charly and Bill will have some advice for you.

Here is a great blog that one of our members started. She and her brother also both have HFI (well, she's pretty sure about the brother).
fructose-free.tumblr.com/

Good luck and glad you found us.

[colormist]

Hi Karine,
I don't really go to the doctor about my HFI. They don't seem to know much at all about the condition and they tend offer advice that is unhealthy.

I also get kidney pain. I knew the accidental fructose ingestion was causing it, so I would make sure to eat super-safe for a week following the incident and drink plenty of water.

I'm not sure what the doctors would suggest about fructose causing the fatty build up in your kidneys. I tried to research it online, but can't find any specific information. I guess you could always mention it to your doctor and ask how to best deal with the situation in the future.

[charlie]

Hi Karine,

welcome to the board. Nice to have another UK resident on here too. Britain is slowly waking up!!

Yes, doctors - don't even go there!!! Which specialist did you see in London, We see Dr Champion at the Evelina in St Thomas' who is lovely but if your tests don't fit into his remit then that is it, he doesn't think outside the box. I think they are slowly getting more info about fructose related problems but it is very slow. You will get much more useful info from this site. I'm not sure how well your organs will repair but if you can now eliminate as much fructose as possible you hopefully will limit any further damage.

Good luck, there is lots of info to use on this board and feel free to ask anything, no matter how trivial.

Where in the UK are you, if you are near Bill he may offer to take you shopping!!!! Now there's an offer you can't refuse!!!!

[karine]

Thank you very much for your answers, it is a real problem that so little is known of that condition. And what doesn't help is that doctors don't seem to help much nor listen to us much! I am waiting to hear from my brother in France as he is just about to have a scan too and we'll see what his specialist says, as he said that she seems to be good.

To answer your question Charlie, I am seeing Dr Murphy at the neurology and neurosurgery hospital in London. She is very nice but what surprised me is that she didn't touch my liver to check if it was swollen. Each time I only had a blood test to check the liver function....It's great but given that my scan wasn't good. I wished she had helped more. The other thing that surprised me is that when I went, I was pregnant and was worried about my body and in particular my liver and kidneys and how they would be affected by it as I know that pregnancy is rather harsh on the body and the organs. The other things was the baby of course, and I wanted to know if he could grow properly with my diet or if he'll miss certain things. Lastly, the pregnancy I had was difficult in many ways but I got a massive constipation problem and she wasn't able to sort me out. Many laxatives cannot be taken during pregnancy and the few remaining ones that can be taken are sweet.....So, I was stuck and I had a horrible time...Now, what I found again surprising is the fact that she had never come across a pregnant woman with HFI before......Surely, I am not the only one? And I am surprised that as a specialist she hasn't dealt with any...That wasn't reassuring really.....Has anyone else had a pregnancy?

You asked me where I am located, I live in Berkshire, so a way away from Sussex.

[Sarosh Rahimtula]

Dear Karine,

I have HFI and gave birth to two healthy, intelligent children many years ago- my son is 31 and daughter is 28- I had not heard of HFI then! Except for terrible morning sickness and constipation, I did not have many problems during pregnanacy. I was advised to take dextrose before a meal to keep my food down and took psyllium husks for constipation- in India we always have psyllium husks at home - you will find it in any health store now.
My first delivery was a caesarian section and the second a normal birth - I recall that I reacted to a pain reliever during the second delivery - please check all medications, injections, IV drips before you go into hospital- more for sorbital and its compounds like polysorbate which is common in pain relievers.
One interesting fact is , and I don't know if this is related to my being HFI , is that both my children turned out to be prize winning artists - they are both very intellingent and creative and there is no artistic talent in our family! But, the downside is that they have very poor hair - agian our family is known for long, lusturous locks! I attribute the hair problem to some vitamin deficeincy in the foetal environment so I hope you are taking your vitamins particularly vitamin C.
Good luck.
Sarosh

[Diet and Disease]

Hi Karine:

I'm new here so haven't really posted anything - but I couldn't help but wonder if it might be beneficial to go with another type of doctor. In the UK one of the "big" diseases over there is Glycogen Storage Disease (GSD). This disease affects the liver (big time).

GSD patients cannot have sucrose, fructose or lactose - so I call this disease a "cousin" to HFI. A GSD doctor would understand the ramifications of fructose and the effects on the liver and may be able to provide you with with the correct doctors who work with these diseases on a daily basis.

Here is the link for the Glycogen Storage Disease Association-UK. Give them a call to find a doctor near you. www.agsd.org.uk/

I have also found this which might be helpful to you from Patient.co.uk about fatty livers and patients of metabolic disorders. www.patient.co.uk/doctor/Steatohepatitis-and-Steatosis-(Fatty-Liver).htm.

I hope this helps!

[karine]

Thank you to both of you. Regarding pregnancy, I have already given birth now, my son is one and luckily he hasn't got HFI. However, if I ever get pregnant again, your recommendation regarding psyllium husk might come handy! Thanks for that.....I am amased that the doctors didn't know about that......Something so basic as helping constipation.....Yes, I was very ill too and had to be signed off work for a while as my morning sickness were all day and night and very strong. I couldn't get up..... I ended up having an emergency c-section, so as you said checking everything before hand is vital! We luckily did anticipate that it might all go wrong and I am glad we did some reasearch and we did told to anaesthitists and others before hand, otherwise I don't know if I would still be here..... Maybe it's me but having been followed during my pregnancy I would have expected the doctor to enquire about all the risks linked to my condition......Surely, they should be in charge and it should be their job to make sure that you are not at risk??

Thanks also for considering the fatty acid problem on another angle and for giving me all the websites linked to various liver disease problem. We are going to take the time to read all that in the near future and no doubt I will ask a few questions to the doctor who is folling me since I am due to see her in February....

Thanks a lot all of you!

Karine.

[ukbill]

Psyllium Husk is part of my daily diet (here in the UK) for the last 6 years and so far although I've not been pregnant.. it was a real life changer for me it has stopped almost all the cramps and bloating issues I was having and keeps me nice and regular

I take a slow release Vitamin C and Zinc supplement every day along with a 75mg aspirin to reduce the risk of cancer.

Other than that I find no other supplements are needed for me .

[karine]

Thank you very much! Many of you seem to be using Psyllium Husk, I am quite amased that the doctor I see in London never mentionned it to me, especially with the constipation problem having seriously got worse during pregnancy!!!! How come they don't know that? I already don't trust doctors much but the more I see them the more I find faults and lack of knowledge or advice from them.....This doesn't reassure me much....something as simple as easing the digestive system....

Anyway, thank you all!

Karine.