Looking for guidance: possible HFI

[tipper]

Hi, 
I'm in my mid 30's and am currently undergoing tests for inborn carbohydrate metabolism disorders. I have been sick with a variety of complaints on and off throughout my life, and have experienced hypoglycemia and urinary problems from a young age. I have thought for a long time that the hypoglycemia was linked to carbohydrates in general and have had myself on a low-carb / ketogenic diet for years. After straying from this diet recently, I realized that I wasn't nearly as sick as I used to be when I occasionally indulged in something like a chocolate croissant. I had also added savoury carbs into my diet, and was eating vegetables and berries. I was actually eating a lot of (low carb, leafy) vegetables, but also starting to realize that things like bagels gave me a lot (like, a lot) more energy and made my thinking significantly (like really, very significantly) clearer. About 5 weeks ago I was sent for tests and I decided that I was going to eat like everyone else did to make sure that the tests actually showed something, and it wasn't all in my head. The day before my tests I indulged in a bag of yam chips and an apple. Within 10 minutes of that apple, I was sure I was going to die. I didn't vomit (haven't since I was little), but my body fell apart and I experienced a fever, chills, incredible lethargy and body pain severe enough that I had to call my husband to come home from work for the first time in our almost 9 year relationship. Since that day, I've been able to eat nothing except meats and fats, and the only 'vegetable' is lightly brined olives. White rice in limited portions is ok, but more than a bite or two a day starts causing an extremely painful psoriasis on my hands to start (which I had once before in my early 20's, but has been entirely absent for the last 14 years). Vegetables don't even make me hypoglycemic feeling anymore (ie. shakes, sweats); now they just make me feel like I can't stand, can't think, and I just cry. My husband brought me a saltine when this happened and it was immediate relief. But bread is destroying me as well - the psoriasis is brutal. I have crazy upper quadrant pain when I've tried to eat other things, and a host of other body pain issues that I haven't experienced in years or ever, including urinary tract pain and incontinence issues. I've been perusing this site a bit, so I know there is potential of HFI, and I am going for a series of tests tomorrow. I'm wondering if anyone can let me know if this sounds like something they've gone through, or if it sounds like something completely different from HFI. If anyone has any advice about the tests I'm going for tomorrow morning (short notice, I know) that would also be great: acylcarnitine spectrum, fasting lactate paired with glucose, transferrin isoelectric focussing, aldolase B activity. My preliminary acyclcarnitine has already come back abnormal, although I'm not sure how. I recently went back to school to do a second degree in computer science and I was struggling badly with being able to think clearly before the last 5 weeks, but the last 5 weeks have pretty much destroyed me (and possibly my chances of surviving this semester). The only respite has been the meat / fat diet that I've been on; things have actually been easier in a lot of respects, and I look a million times better than I have for years, but I am starving. So hungry. Is there a light at the end of this tunnel? 

[antonia]

Were you ever tested for GSD/Glycogen Storage Disease? I discussed with my daughter's genecist a form of GSD (there are many different types) that can mimic HFI in that sugars cause hypoglycemia. The main difference being that with this type of GSD, ALL sugars will cause metabolic distress. HFIers have no issues with lactose (sugar in milk) or glucose based carbs (such as plain, white rice) because they don't contain fructose. So this is something to consider if you feel all sugars, including glucose and lactose, make you sick or cause you to become hypoglycemic.

If you suspect HFI, the best place to start is with a DNA blood test. In all my years of searching for a diagnosis for my daughter, we've never uncovered any kind of metabolic test that is diagnostic for HFI. The best indicator would be elevated liver enzymes. But you won't see significant elevation unless you've recently consumed fructose.

[tipper]

I did some research on the tests that are being done this morning, and it looks like one of them is an indicator for GSD. Good to know there is no test that is diagnostic for HFI out there. It sounds like I will be getting a DNA test done at some point, but I am in Canada, and funding needs to be applied for through the Province, so it's a lengthy process. I currently am not sure what sugars I have problems with because eating in general is hard. Dairy is ok, I think, because I am definitely adding butter to my foods to keep my calories up, and I have been able to keep some plain yogurt down.

[jenn123]

23 and me tests for the most prevalent mutations of HFI and a few Glycogen Storage Disorders. It is 200$ and is returned in 4-6 weeks. Our mutation of HFI shows up in this test. I wish it had been available when my child with HFI was younger as it would have saved us a lot of money and time. Not all mutations show up though.

[tipper]

Thanks Jenn123, that's good advice.

[tummyache]

Antonia, it is not true that all HFI'ers can tolerate lactose. Perhaps it is better said "most" or "many", especially when young can tolerate and enjoy dairy. There actually is quite a range in what those of us on this site can eat safely. I don't mean to pick on you --but we all need to remember that there are variances in tolerances in this disease. Some with HFI are a lot less tolerant than others. We need to be extra careful when very young when we are most sensitive. Some adults can only eat white rice...others might be able to tolerate whole grain brown rice in small amounts at times. I'm saying this not so much to you, as to others, who are not always gentle in their comments.

[tipper]

Does anyone find that their tolerance for foods changes with how well (or not well) they have been following their diet?

[antonia]

Tummyache, sorry if you took my comment to mean that HFIers can’t have other food intolerances unrelated to HFI. My daughter can’t eat wheat but this is not related to HFI. I only meant since lactose contains no fructose an intolerance to it would not be due to HFI. That’s my understanding of the medical facts. And when discussing which sugars my daughter was intolerant to with a geneticist, the fact that she tolerated lactose helped us rule out a particular form of GSD that can mimic HFI. My comment about rice was only that white rice is a form of almost pure glucose and to help tipper determine if she had an intolerance to all sugars/carbs or just fructose. I realize that many HFIers do eat brown rice, yes, and tolerance does vary.

I was not advising tipper on what she should eat either. (I usually refrain from advising anyone on what's safe or not safe anyway since my daughter is not diagnosed.) Only discussing what I've learned about HFI in our personal search for diagnosis. 

[jenn123]

Antonia, just for general knowledge, which form of GSD were they exploring ? We had had a liver biopsy for GSD Hers Disease/Disorder. Just wondering. Thanks

[antonia]

Sorry, I don't remember what type he said it was. It was a conversation at the end of our visit revolving around, if not HFI than what? And, per usual, came up with nothing.

[ukbill]

Yes the cleaner you get the more sensitive you get to nasties and the better health you have..

IF you had GSD I think you would be dead by now.. its a very unforgiving condition.

It is posible to have all sorts of problems once your liver is compromised.

If it's HFI your liver should start to recover if you have a strictly HFI safe diet for about 6 months. I must stress NO glucose or DEXTROSE these are far too contaminated for a compromised liver to take and the simple act of trying to control blood sugar levels after hitting your system with either of them will stress even a healthy liver.

Keep us informed of the results please, shared knowledge is useful for us to help each other and others like yourself who have multiple symptoms that confuse medical Professionals.

[tipper]

I will definitely keep this board informed. Thank you all for the responses; this board is very helpful. I introduced some rice today and have had no feelings of hypoglycemia, although it was a bit tough coming off the keto diet (I was super sleepy for a while). I've also had relatively clear thinking capacity all day, which is very welcome. I *think* the negative symptoms I'm still experiencing are kidney related, and I'm still having a fun pinching feeling in my upper quadrant (is that your liver?), but they're decreased significantly since the last time I tried rice.

ukbill: by no glucose or dextrose, do you mean no rice? Because I've clearly already made that mistake, if so.

Is a 'strictly HFI safe diet' composed solely of white rice, white pasta, sugar-free white bread, meats, and dairy? I see spinach and arugula are OK for some people, but do people generally include them on a 'healing / strict' diet?

[ukbill]

No only meaning the manufactured variety.

Rice is a long-chain Glucose molecule its the manufacturing process that brings in Fructose contamination.

Spinach is fine in small quantities for me, however I remove the stalks from the leaves before cooking or eating.

Watercress is fine also if you like its sharp spicy flavour.

The green leaves of cabbage (only the darkest of green leaves) is fine also although I also cut the veins out of the leaves (the thick large ones only) I have good reason, and experience, that the plants store of sugars is kept in the white parts of the plant.

[ukbill]

Arugula (rocket), Lambs lettuce, soft green lettuce leaves, Oak leaved lettuce, Cress, Mustard and Cress and others of a similar structure are fine in small quantities.

Onions are unsafe however small quantities of Chives and Scallion leaves may be OK.

The leaves of beetroot and similar plants should be fine in very small amounts however Beetroot itself is far too sweet as are almost all root veg.

With many vegetables its both the variety and how they are cooked that makes the difference between healthy for us and really unhealthy.

Some Potatoes can never me safe for HFI people in particular yellow fleshed waxy varieties, some pure* varieties of potato in particular floury white varieties used to make Mashed potatoes can be made safe.

To reduce the amount of Fructose in potatoes peel, cut small and soak in water for an hour or more before changing the water and cooking.

Boiling or par boiling (if making Roast potatoes) in plenty of water also helps.

Fructose seems to behave like a salt and wants to equalise its concentration out in whatever volume of water the fructose finds itself in.

This is why soaking is so effective at removing the sweetness out of the potatoes.

Instant Mashed potatoes while not tasting very nice or having the right "mouth feel" is generally fine however although watch closely the label to check for unwanted additions like "natural flavours" which usually means added sugar.

Hope this helps?

regards

Bill A.
Edit * all agricultural seeds in the UK and the EU work as follows.
The patent holder of the particular variety produces basically a clone crop where everything is closely controlled and the genetic purity of the seed is guaranteed pure, Thi si sold to farmers as 1st year Pre-basic.
This seed is very expensive and the resulting crop usually is sent to make seed for subsequent years. Seed Merchants buy this back from farmers (if they have grown the crop with the proper bio security safeguards)
This is then treated and sold to farmers as C1 seed this provides the most pure variety you can get. Some of the smaller potatoes or seeds can be kept back and resowed by the farmer or the seed merchant as C2 and C3 in following years.
After C3 there is no purity left and the seed has degenerated so much with cross fertilisation with other varieties of corn or compatible weed seeds that it cannot now be sold as a "name" brand.
All plants cross pollinate with as many varieties of compatible plants as is posible to add to their own genetic code any genes the plant wants to try out for itself.. its called Evolution and it go's on all the time.
Because the C1 seed costs a lot more than C2 or C3 occasionally someone will try to pass off some C4 as "xyz" variety and it might look the part.. however its sugar content might be way different for example.

Sorry for this boring stuff but I spent 3 years working in this industry and it was fun.

[ukbill]

Herbs and most spices are fine also. I also use Celery seeds in my cooking to add the flavour of Celery but not the high sugar content of the stalks.

Fennel, Cardamom, Cinnamon bark, poppy seeds, cumin, coriander seeds etc are all fine in the quantities you are likely to eat them in occasionally.

Fresh (and dried) Herbs like Parsley, Fennel, Coriander leaves, Bay leaves, Sage, Marjoram etc etc are also all fine.

So boring flavoured food is only the result of not experimenting or adding enough Parsley and seasoning.

A rich meat stew with dumplings and lots of herbs is a real treat in colder weather!

Good rich stock can be made using off-cuts of meat and or bones. I would recommend getting a cookery book form a Michelin Stared Chef.. one that shows how to make the stocks and sauces (which is where the Star comes from) that will teach you a lot about how to make your food explode with flavour.

There is no need to follow the recipes too closely however, miss out the root veg and onions of course.

I think the outer dry skins of onions might be safe to use in a stock.. not tried it yet but will soon.

Making a good stock can be expensive a short cut can be to use the dark red sticky residue from under a joint of roast meat, pour off the fat and then pour hot water on to the dried juices and stir and scrape them up into a quick stock.

This is making me feel hungry!

A good beef stock can be made by buying the cheapest stewing beef (a genuine butcher is far better than supermarket or store bought) roast the meat off until brown then transfer all the meat and juices into a pan with a lid (or a slow cooker) add hot water and a few bay leaves (not too any or it gets bitter flavoured) and then slow cook for a few hours, skim off excess fat, strain the meat off and then reduce to make your stock to the strength you want.

This works on all dark meats (Beef, Venison etc, chicken, pork (with much more fat!), Mutton and a few cuts of lamb, its a waste to use most of the Lamb cuts to do this, even really good Fish stock can be made like this.

Hope this helps?

Enjoy

Bill A.

[tipper]

ukbill this helps so much. Thank you.

[Stefanie (Ziba)]

I read that many HFIers are also lactose intolerant. Also, since wheat contains fructans, even non-celiacs and non-gluten sensitive HFIers could have a reaction when consuming too much wheat due to the fructan content. I notice this with my son so it may be a reaction only present when young and tiny. Just my experience...

[tipper]

Thanks ziba. I see you answered the follow-up question I posted on the White Bread thread right here.

[ukbill]

A damaged liver can cause lots of side effects and false roads to travel.

I almost live on milk and milk products.. only problem with them is they do have a tendency to increase my body fat levels.

This was an advantage when young and underweight.. but not now. ;(

[tipper]

Stefanie (Ziba), I noticed on your signature that your son has a carnitine deficiency. I have had two tests that have come back with 'irregularities' in serum acylcarnitine profile which were then replicated in my bloodspot acylcarnitine profile. I unfortunately don't know how they were irregular, except that the patten observed is not specific to a known condition. We are all currently waiting on genetic testing to be approved for myself, but in the meantime, the only information I can find on acylcarnitine profile is that it is used for biochemical screening of disorders of fatty acid oxidation and organic acid metabolism. Do you know if carnitine is the same as acylcarnitine? How did your doctor know to link your son's deficiency in carnitine to an inability to metabolize fructose? Incidentally, I believe in one of your other posts you noted that your son is on an almost ketogenic diet. I have also been experimenting with ketogenic diets for years; it is the only way I can keep myself from being very sick, and is what I am currently trying to treat myself with since the 'event' that made me sick about 6 weeks ago.