Adults diagnosed with HFI?

[antonia]

Rose, I’m assuming your ob gyn has ruled out gestational diabetes? Because the symptoms of hyperglycemia/high blood sugar can be similar to hypoglycemia/low blood sugar. You might google symptoms for high blood sugar, type II diabetes or gestational diabetes and see if they ring a bell.

My daughter gets low blood sugar and ketoacidosis after eating fructose, sucrose or sorbitol. Blood acidosis can be part of diabetes or HFI, the difference being high blood sugar with diabetes and low blood sugar with HFI. My daughter also tested negative for HFI through Dr Tolan’s BU lab and tolerated some fruit, without it making her deathly ill or instantaneously vomit, before going on the HFI diet. She actually became more sensitive to fructose after starting the diet, so I’ve no idea what her reaction to eating fruit would be now. Did she have an aversion to sweets? Yes, most definitely she was not your average child and rejected a whole lot of sweet foods. Recoiled and shuddered like she was eating a lemon as a baby. Later though she did try very hard to eat like everyone else. The operative word being TRY, because that’s not normal. The best I can describe it, as a parent observing, is that certain foods she wanted to eat, said they were delicious, but it looked to pain her at the very same time.

She is now very stable and thriving on a strict HFI diet. Been on it for three years now. Other than olives, she eats no fruit. The only vegetables she eats are leafy greens and rhubarb, and even then they are occasional foods in very small quantities, not everyday foods. She takes a multivitamin everyday, sometimes I give her extra vitamin C and D. She also has an allergy to wheat. If you’re interested in some wheat free, HFI safe recipes I would be happy to share them with you. Of course, work with your doctor to make sure you’re getting all the necessary nutrients you need for your pregnancy, but I don’t think following a HFI diet would be a detriment to you or your baby.

That being said, if you don’t have HFI, the diet won’t be a fix for you. There are a lot of glucose based grains and starches as well as dairy foods high in lactose (a HFI safe sugar) in the diet that would not sit well with you if you had CSID or diabetes. Those conditions would require more of a ketogenic or very low carb diet.

Additionally there is another condition known as FDPase or FBPase Deficiency that is considered a cousin to HFI. How close of a cousin depends on who you talk to. People with that are suppose to tolerate more fructose than HFIers, but when ill or their bodies are under stress for some reason their sensitivity to sugar increases and it mimics HFI. People with FBPase are not deficient in adolase b enzyme but some other enzyme further along in the metabolic pathway. I don’t really understand why they don’t tolerate fructose if they make adolase b, but unfortunately even less is known about it than HFI and I’ve found hardly any literature that explains it. But I did have my daughter tested for it and it was negative as well.

So I’ve rambled on a whole lot. I hope some of it helps you. For some of us diagnosis is a long road.

[Rose]

Thanks so much for your response!

I have had my liver tested and the Bun/Creatinine ratio has been red-flagged as high, both recently and ten years ago (I'm 33.)

I stopped eating gluten some time ago to see if that would help me, and I consistently notice when I try eating it again that I have depressive symptoms for about 24-48 hours afterwards. Then I feel like myself again. So I have said that I'm gluten free, but I guess I'm not really sure if it's gluten that causes the problem.

Milk/Ice cream/cheese has always given me stomach aches, and I avoided them as a kid.

I tried to do the HFI elimination diet that you suggested above to Matthew, and the dairy was consistently giving me brain fog and stomach pain about 10 minutes after eating it. When I cut out dairy, it was so limiting that I didn't succeed at continuing the diet. It's probably time to try again. What rice products are HFI friendly? Is air-popped popcorn safe?

I also have always hated rice and starchy potatoes. Maybe it's possible I could have CSID, I guess. Do you have any idea if the hypoglycemic symptoms are also part of that condition?

Thanks for your help!

[Rose]

Antonia, I didn't see your post for some reason and my above response was to Colormist. Thanks so much for taking the time to respond in depth.

I have to say that it stopped me in my tracks when you said that your daughter has experienced ketoacidosis before. I have been to the ER twice recently with ketoacidosis and was told repeatedly that it was a mystery for me to be having it since I don't have diabetes (I have been tested a couple times.) When I went to the ER with ketoacidosis, it was because my doctor recommended taking a glucagon shot (basically of glucose) when I was feeling hypo to see if that made me feel better. Clearly it did not make me feel good at all (couldn't sit or speak, shaking convulsively.)

When I took a fasting glucose test for diabetes, my blood sugar levels dropped. I started with blood sugar at 102, dropped to 73 at the first hour, and then dropped to 56 at the second hour (at which point I was lying down, shaking and thinking I was going to pass out.) My doctor was baffled, as they said the numbers were supposed to go up.

Thanks also for the info on the FDPase/FBPase Deficiency. I will be definitely look to that as well. But I have the feeling that I'm really in the ballpark of what the problem is. Either HFI or something similar or related. When my HFI test came back negative, I was ready to give up on the whole thing, but all of your comments have definitely made me reconsider.

Thank you so much for all your help with this!

[antonia]

Rose, I found this:

res.mdpi.com/d_attachment/nutrients/nutrients-09-00356/article_deploy/nutrients-09-00356.pdf

Only had time to skim through on my phone, but I think you’ll find it very interesting. Especially note the affects of pregnancy on FBPase Deficiency.

[Rose]

Thanks Antonia. I read up a little on FDPase/FBPase Deficiency, and I'm not sure that it would be what I have, only because (if I understand correctly) someone with that deficiency suffers from severe episodes of medical hypoglycemia (having actually hypo numbers, not just the symptoms.) I wore a blood sugar monitor for a month and (except for the Fasting Glucose Test, at a different time) I haven't ever recorded an actual hypo number below blood sugar 70. I just have the hypo symptoms.

I've heard this can be the case in HFI, where you have the symptoms but not the low numbers. But in FDPase/FBPase Deficiency you might have the low numbers also, which might cause hypoglycemic comas etc. Does that sound like I understood the literature correctly?

If I had FDPase/FBPase Deficiency, I would have to be carefully monitoring my blood sugar numbers while pregnant to prevent slipping into a hypoglycemic episode that might cause harm to the baby.

Out of curiosity, does anyone know if there's a test for CSID that doesn't involve drinking a bunch of sugar? I'm reluctant to do that if I actually have HFI instead.

[tummyache]

Rose, I may have misunderstood, or did you say the doctors treated your hypo incident with glucagon and that your blood sugar level kept going down? If so, that is a sign of Glycogen Storage Disease.[Agents/circumstances to avoid in liver type GSD: Excessive amounts of simple sugars; glucagon administration as a rescue therapy for hypoglycemia]. Glucagon causes a "flat curve/line blood glucose level reaction". Lactic acidosis and hypoglycemia are very diagnostic -- along with an enlarged liver in some cases. There are many types of GSD which can affect the liver and/or muscles. Some types are more serious than others and some types are frequently confused with HFI (especially types Ia, III, and milder types VI, IX). So, here's something else to consider. [The doctors have questioned if I might have GSD-VI , for instance, instead of FBPaseD as symptoms are so similar]

I too react very strangely to injected sugars...get dizzy; black out (have had seizures when little but mostly just tremors now). I use the GSD trick of uncooked cornstarch drinks to even my blood sugar release as need, snacks in between meals to keep from having hypo incidents, eat meals every 2 - 4 hours, and eat protein just before I go to sleep at night. I'm 76 and have been doing most of this since college days. The cornstarch is a rather new addition as no one knew about that way back when! I have had 3 oral glucose tolerance tests, all about 10 yrs apart (the one done in college they did 2 x's, the 2nd time twice as long with an endocrinologist), all because of them thinking I was a diabetic -- all were "flat curve" results.

By the bay, I have been dairy intolerant since birth. GSD-Ia has that as part of it's symptoms + GSD-VI is considered a milder version of GSD-Ia.

[antonia]

Isn’t glucagon also bad for HFI? I think I’ve heard that before but never understood why.

[antonia]

Rose, I was also going to mention GSD. Our geneticist mentioned one form of GSD that can mimic HFI that would also include an intolerance to lactose. And since my daughter has no issues with lactose it was ruled out for her. She does seem to have GI distress when she eats too much dairy, but it does not affect her metabolically. GI distress with dairy is so common, so it would be worth while discovering if your issues with dairy are GI related or metabolic.

On that note CSID is also a GI condition. It can only be diagnosed through an endoscopy/biopsy of the GI tract as far as I know. Although there might me hypoglycemia and ketosis, I don’t think it would elicit the kinds of symptoms you describe. Hypo and ketosis would occur simply because carbohydrates are not well digested and, therefore, never enter the blood stream. Anybody who restricts carbohydrates will produce ketones and go into ketosis. But ketosis will not make you as sick as you describe. My daughter produces 8-10 times the number of ketones someone might produce due to carb restriction or fasting. When it’s that high, and makes you sick, it’s called ketoacidosis. This high level of ketones is dangerous. It is what type 1 diabetics must watch very carefully because it can be fatal to them. I’m by no means an expert on CSID and could be wrong. But I’ve never heard or read that CSID causes this level of metabolic distress.

[antonia]

Rose, I finally had time to sit down and read that article. Granted a lot of the technical information is over my head. But my understanding of FBPaseD is that the high sensitivity (HFI level) to fructose, sucrose and sorbitol occurs when the body is depleted of plasma glucose and must dip into its glycogen stores. This can occur due to fasting, exercise, illness, etc. And apparently pregnancy puts high demands on your body for glucose, so would be one of the conditions that would increase your sensitivity as well. You said that one grape or berry can cause you to react, this seems very sensitive to me.

As far as hypoglycemia is concerned, I feel you have to take what you read in the literature with a grain of salt. These diseases and their reactions are not as cut and dry as the literature depicts them to be in my opinion. There are a lot of variables that can affect blood glucose. And these diseases are so rare. Doctors have not taken the time to study them and all that goes on in the body when one has a reaction. If you have all the symptoms of hypoglycemia this is more important than the actual number. Also the literature doesn’t seem to discuss acidosis nearly as much as they do the hypoglycemia. I know when my daughter has severe reactions the two always go hand in hand. I believe the acidosis is what makes her feel really poorly. Getting blood glucose up alone does not fix her. The acidosis needs to get under control too. HFIers can experience both lactic acidosis and/or ketoacidosis. Google either of those and you will see the symptoms associated with them and they are not very pretty.

[tummyache]

Aug 28, 2019 19:20:15 GMT -5 antonia said:

Isn’t glucagon also bad for HFI? I think I’ve heard that before but never understood why.

I don't know the answer to that, but I thought they give an IV drip of glucose for hypo episodes. Glucagon is injected intramuscularly, I think. [Wikipedia: Glucagon is a peptide hormone, produced by alpha cells of the pancreas. It works to raise the concentration of glucose and fatty acids in the bloodstream, and is considered to be the main catabolic hormone of the body] So, evidently it doesn't work the same as an IV glucose drip at all. Sounds like it could be bad for HFI too. Bet UKBill knows.

[Rose]

Aug 28, 2019 16:24:40 GMT -5 tummyache said:

Rose, I may have misunderstood, or did you say the doctors treated your hypo incident with glucagon and that your blood sugar level kept going down? If so, that is a sign of Glycogen Storage Disease.

Thanks for all your comments and help!

I think some of my original comments are confusing. My blood sugar levels kept going down when I took the Fasting Glucose Challenge Test to see if I was diabetic. (Basically, you drink a cup of glucose and see if your numbers shoot through the roof if you're diabetic. Mine went down to 55, which is my only recorded hypoglycemic blood sugar level.)

A few months later, I asked my doctor if there was a medication that could be used to treat the moderate hypoglycemic symptoms that I have (basically every day.) He wasn't sure, but said that when a diabetic is hypo, you can inject a shot of glucagon into your arm that is hyperglycemic and cause your blood sugar to go up. He said to try it and see if it would help. So I drank a glass of orange juice, felt terrible, and took the shot of glucagon. My blood sugar did go up and then after about two hours it went back to normal (around 100 for me.) Then I began to feel severe nausea, couldn't speak or stand etc. I was taken to the ER and diagnosed with ketoacidosis (blood sugar levels were still 100.)

When I say I have hypo symptoms every day, I mean shaky hands, cold sweats, fatigue, mental confusion. I don't go into a coma or have severe symptoms; in fact, I've never passed out.

I was tested for some Glycogen Storage Disease types: 1a, 1b, 2, 3, 4, 5 and 7. They were all negative.

Thanks for your help!

[Rose]

Aug 29, 2019 6:30:26 GMT -5 antonia said:

Rose, I finally had time to sit down and read that article.
Thanks for researching more, Antonia. Do you know if there's a way to test for FDPase/FBPase Deficiency? Is there a difference in diet/treatment as opposed to HFI?Thanks so much!

[Rose]

Sorry, my post above was confusing as I figure out how to use this message board. I was trying to quote Antonia as saying, "Rose, I finally had time to sit down and read that article. "

And then I wanted to respond: Thanks for researching more, Antonia. Do you know if there's a way to test for FDPase/FBPase Deficiency? Is there a difference in diet/treatment as opposed to HFI?Thanks so much!

Eventually I will figure out how to post like a pro!

[antonia]

No worries Rose. I’m incredibly techno challenged. It’s amazing I accomplish any form of communication through this thing they call the internet.

FBPase Deficiency is a DNA test just like HFI. My daughter was tested through our local children’s hospital through their biochemistry/genetics department. I know Tummyache was tested by Baylor University. If I had to do it over again I would have tested my daughter through Baylor, because I think they test for some of the more rare mutations. But be prepared that DNA tests are not diagnostic when negative. My daughter has tested negative for both HFI and FBPaseD. Yet the HFI diet has turned our life around and completely stabilized her metabolism. This is all the proof I need to know she has HFI or something similar. A piece of paper confirming it would be a nice thing to have, but not necessary. She is happy and healthy and that’s all that really matters.

You’ve definitely got something metabolic going on. If I were you I would try sticking to a dairy free, wheat free, HFI diet. If it helps you then both HFI or FBPaseD are possibilities. If it doesn’t then that indicates you’ve got something else going on. As far as I know, someone with FBPaseD can tolerate more fructose than HFIers. But until you know otherwise or your body has had a chance to heal and your metabolism has completely stabilized, I would stay as fructose free as possible. I just really hope that you can start to feel better. Especially with a baby on the way.

Following the HFI diet means you need to cook for yourself. So hopefully you like to cook or have someone in your life that will cook for you. There’s a learning curve to cooking HFI safe foods for sure, but I’m actually able to make some pretty delicious food for my daughter. She’s also wheat free and I’ve started to reduce her dairy intake as well. I figured out last spring that when she ate a lot of fresh dairy products it would give her diarrhea and nausea. So I can share with you some of her favorites. I still bake with dairy, but if you find a safe milk replacement (Tummyache can probably help you with that), or just use water instead I think most recipes would still work. Baked goods made with butter you can replace with vegetable shortening.

[Stefanie (Ziba)]

My son also can't eat gluten= (respiratory symptoms). He eats: oats, meats, fish, white rice, white rice crackers, homemade oat crackers (I can send you the recipe), eggs and romaine lettuce (stalks removed). He can eat hard cheeses but no milks (abdominal pain from lactose). It is a limited diet, but it is do-able. He also tested negative for HFI but clearly has it (literally every symptom in the book.

[Stefanie (Ziba)]

My son also can't eat gluten= (respiratory symptoms). He eats: oats, meats, fish, white rice, white rice crackers, homemade oat crackers (I can send you the recipe), eggs and romaine lettuce (stalks removed). He can eat hard cheeses but no milks (abdominal pain from lactose). It is a limited diet, but it is do-able. He also tested negative for HFI but clearly has it (literally every symptom in the book.

[tummyache]

DAIRY REPLACEMENTS...I use unsweetened rice milk or almond milk to cook with or drink on occasion; otherwise just drink water or unsweetened tea, with the occasional Le Croix sparkling water. I've been 100% dairy free since the 1970's and take 1500 mg calcium + 3000 IU vitamin D daily to make-up for the lack for dairy. Before that I wasn't being as careful and not doing very well. If you are in the USA, Earth Balance Margarine is wonderful stuff -- it comes in soft-tub and hard-tube for baking. I use the one in the lightest yellow lid tub without pea protein (because I'm allergic). There is Tofutti sour cream and cream cheese (a problem if you can't take soy) without pea protein unlike most all the other non-dairy brands. If you do have FBPaseD, rather than HFI, it is possible that you may be able to tolerate a very small amount fructose in foods before having symptoms/problems according to the literature in medical journals. It must be a very, very small amount, however, I am finding! Hope this helps you all who are dairy intolerant too.

ADDITION: Forgot to add my two favorite non-dairy cheeses that are really pretty good and melt well for grilled cheese sandwiches!
(1) CHAO Vegan Slices
(2) Follow Your Heart Smoked Goyda Style Slices