Maturity Onset Diabetes of the Young Group
MODY Support Community
MODY3 - Tealas Family
Our family has a MODY3 mutation (mutation of HNF-1alpha regulatory protein).
I was first diagnosed "borderline" diabetic at age 13 (1976), but for years before that I had seen sugars in my urine and asked my mother if I should go to the doctor. For many years I did not know what the heck kind of diabetic I was, and I used all kind of diets, herbs, supplements, exercise and acupuncture to control it.
Finally in 2000 my mother and I met a doctor (Dr. Stoller of Rush Hospital in Chicago) that told us we were probably a MODY family. I did research and in 2002 got a hold of the folks at Exeter University. I was sequenced as MODY3 (W267NX/N) in spring of 2002. My family finally had a "name" for what the heck we had. My mother, her father and his mother have all had this MODY3 "disease". So far everyone in my family has died of kidney related disease or complications at a young age. Great-grandmother died at ~33 of "Bright's Disease". My grandfather died at 56 of nephritis complications and a heart attack. My own mother died at 63 from end stage renal failure triggering a stroke. My son has inherited the gene and has no sign of expressing it so far. Because MODY3’s have a much lower renal threshold – the easiest way to screen him for post-meal blood sugar spikes is urine testing. I try to test him 1-2 times a week. My hope is to be very proactive with him, so that he learns how to use diet, exercise and medication to avoid spikes as much as possible.
Before I had my son at age 41, I was able to manage my MODY with the diet, exercise, herbs & acupuncture. Then I began using Humalog insulin sliding-scale shots with meals. In Dec 2008 I tried Byetta, however the side-effects on my GI system and immune system were too intense for me to continue. My LDL levels have begun to be a concern for me - especially since the folks at Exeter have indicated that higher HDL levels in MODY3 "may not" be protective (from their initial research). MODY3 is really a syndrome of potential issues with the pancreas, liver, small intestine and kidneys (all organs in which HNF-1alpha plays an important role). MODY3's have lower levels of APOM and other lipid transforming factors due to lower levels of HNF-1aplha. Our raised HDL levels are likely not so much protective as they are a consequence of not metabolizing all lipids properly. So I think it is safe to say that MODY3 itself presents cardiovascular risks beyond just being a "diabetic".
In Mar 2009 I tried eating only vegetables at dinner in an effort to lower average blood sugars and see if it could help my LDL levels. It did slightly, but then I stumbled into trying a predominately raw foods diet. I have lost 10 lbs "insulin" weight, and feel much better. My 14 day averages run about 105mg/dl now and I only need insulin a few times a week if I am eating cooked starches or a lot of fruit. I would call this diet 75% Raw/Paleolithic, as I am combining concepts from both areas, as well as D'Adamo's "Blood Type" diet. I'll have my first blood tests in June, and hope my HbA1c will go into the 5's and that LDL will go under 100 with these diet changes.
I was first diagnosed "borderline" diabetic at age 13 (1976), but for years before that I had seen sugars in my urine and asked my mother if I should go to the doctor. For many years I did not know what the heck kind of diabetic I was, and I used all kind of diets, herbs, supplements, exercise and acupuncture to control it.
Finally in 2000 my mother and I met a doctor (Dr. Stoller of Rush Hospital in Chicago) that told us we were probably a MODY family. I did research and in 2002 got a hold of the folks at Exeter University. I was sequenced as MODY3 (W267NX/N) in spring of 2002. My family finally had a "name" for what the heck we had. My mother, her father and his mother have all had this MODY3 "disease". So far everyone in my family has died of kidney related disease or complications at a young age. Great-grandmother died at ~33 of "Bright's Disease". My grandfather died at 56 of nephritis complications and a heart attack. My own mother died at 63 from end stage renal failure triggering a stroke. My son has inherited the gene and has no sign of expressing it so far. Because MODY3’s have a much lower renal threshold – the easiest way to screen him for post-meal blood sugar spikes is urine testing. I try to test him 1-2 times a week. My hope is to be very proactive with him, so that he learns how to use diet, exercise and medication to avoid spikes as much as possible.
Before I had my son at age 41, I was able to manage my MODY with the diet, exercise, herbs & acupuncture. Then I began using Humalog insulin sliding-scale shots with meals. In Dec 2008 I tried Byetta, however the side-effects on my GI system and immune system were too intense for me to continue. My LDL levels have begun to be a concern for me - especially since the folks at Exeter have indicated that higher HDL levels in MODY3 "may not" be protective (from their initial research). MODY3 is really a syndrome of potential issues with the pancreas, liver, small intestine and kidneys (all organs in which HNF-1alpha plays an important role). MODY3's have lower levels of APOM and other lipid transforming factors due to lower levels of HNF-1aplha. Our raised HDL levels are likely not so much protective as they are a consequence of not metabolizing all lipids properly. So I think it is safe to say that MODY3 itself presents cardiovascular risks beyond just being a "diabetic".
In Mar 2009 I tried eating only vegetables at dinner in an effort to lower average blood sugars and see if it could help my LDL levels. It did slightly, but then I stumbled into trying a predominately raw foods diet. I have lost 10 lbs "insulin" weight, and feel much better. My 14 day averages run about 105mg/dl now and I only need insulin a few times a week if I am eating cooked starches or a lot of fruit. I would call this diet 75% Raw/Paleolithic, as I am combining concepts from both areas, as well as D'Adamo's "Blood Type" diet. I'll have my first blood tests in June, and hope my HbA1c will go into the 5's and that LDL will go under 100 with these diet changes.
Replies to This Discussion
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Permalink Reply by sofaraway on -
thankyou teala for adding your information. I am also from a MODY 3 family. I found it interesting to read about the renal problems your family has faced. In my family the cardiac problems have been clearly evident but I don't know of anyone with the renal complications. I will ask my nan when i see her about the extended family that i don't really know or ever see.
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Permalink Reply by tealas on -
I think it is a bit of a chicken and egg question. Both my mother and her father technically died of cardiovascular events - however that was after years of nephritis or end-stage renal failure stresses. Was the kidney damage merely due to higher blood sugars, or is it more directly connected to the changes in transport factors in the kidney due to the lower levels of HNF-1alpha? In mice studies there are something like 15-20 kidney co-transport mechanisms that are impacted by lowered HNF-1alpha. That is why MODY3's have lowered renal glucose thresholds, because lower HNF-1alpha means the kidney doesn't reabsorb glucose correctly. Which is in the short term probably "protective" in that average blood sugars don't run quite so high, however in the long term I think it means some level of kidney damage. There are also some electrolyte transports affected (potassium I think?).
I also think that the whole LDL/HDL situation is part of the picture. HNF-1alpha and I think APOM have something to do with LDL and the renal tubules. I know one of my mom's doctors thought her blood flow to her kidneys might be blocked. So maybe the lipid issues that lead to the cardio events are also part of the damage to the kidneys? Probably a negative synergistic cycle after a certain point in the expression of MODY3.
But to make it more practical - I strive to keep my blood sugar below my renal threshold and avoid spilling sugar in my urine. I think it is just "smart" and protective of my kidneys.
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Permalink Reply by sofaraway on
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I have asked the question to a couple of Dr's, (the top MODY researchers) here if there is any damage caused to the kidneys by spilling glucose and the answer has been 'no'. But like you I am not completley convinced. What is your renal threshold? I don't know what mine is exactly but I have probably tested negative for glucose only a handful of times over the past 9 years I've had diabetes. I seem to spill glucose when my blood sugar is 5/ 90 so it's almost impossible for me to get under that.
How did you find byetta at controlling your blood sugars? Would you have continued if you didn't suffer from the side effects?
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Permalink Reply by tealas on
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Since I have a biochemistry background, I have two levels on which I approach these questions - both the scientific theory and also the practical/empirical/intuitive of being a human being dealing with a disease. On neither of these levels do I think it is "free" to run your blood sugars high enough to frequently spill into the urine.
My renal threshold varies from about 130mg/dl up to about 160mg/dl. It appears to go lower the more often I am spilling sugar in my urine. Practically speaking, have you ever noticed that if your blood sugar goes up really high one day, that it might be primed to more easily go high for another day or two until you get your diet, exercise &/or meds back in balance? I think similar mechanisms are in play with MODY3's renal threshold.
One question I would ask you is how closely do you watch your post-meal sugars? Do you test your urine frequently? I test blood sugars about 5-6 times a day - and any time I am concerned about spiking after a meal I watch my urine for the next few trips to the loo. Normally if I just eat a little too much carbs with lunch (low glycemic), I'll see just a little sugar in my urine if my post-meal reading is about 150 or higher. If I eat high glycemic carbs (white rice for instance), and take 5-7 units of humalog, I might have a good post-meal number like 120-130, but then I see about 100-250 in urine. I assume at that point that I have "spiked" over 150 for some transitory amount of time before the insulin really kicked in and when I took my post-meal reading.
Certainly you know your body - but it may be worth trying a some meals with only meat/tofu and non-starchy veggies and seeing what your post meal readings are, and whether there is any sugar in your urine. If you are eating normal levels of higher glycemic carbs (grain, potatoes, breads, pastas) - I would bet that your renal threshold isn't 90, but that you are spiking frequently enough that it appears that way.
There is a biochemical reason why MODY3's spill sugar at a lower level, and even if the mere act if "filtering" extra glucose doesn't hurt the kidneys, the regulatory cascades (which cause MODY3's to spill sugar at a lower level) probably do affect our kidney's (and other organs) over time. I have seen articles about feedback loops and how the good copy of the HNF-1alpha gene can get "shut-off" over time. I am personally very certain that keeping blood sugars below about 130 or 140 is important for avoiding those negative feedback loops.
RE: Byetta. It seemed amazing for the first week. Kept my blood sugars around 120 post-meal. But if I ate substantial carbs, I would get "breakthru" spikes about 2am, so it wasn't like I could eat anything with impunity. If I didn't have serious side effects, I would have probably continued. But I also needed 1/2 the normal starting dose. I had to draw the 1 unit out of the 5mg Byetta pen with a syringe twice a day. It was a bit of pain to be honest, but maybe someday they will allow variable dosing. I had heard from Jenny Ruhl (http://www.bloodsugar101.com) that many MODY3's do seem to do well with Byetta, that is why I tried it.
I guess it is just my fate - but the only way I have ever truly been able to truly avoid post-meal spikes is to do it with dietary control. I'm very excited with the "Byetta-like" results I'm getting with the 75% raw foods diet.
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Permalink Reply by tealas on
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Dang, typed more into that reply below that got lost :(
You live in England, at least in London. What kind of foods do you eat Nikki? Ever since my late 20's - I have really noticed a beneficial impact of eating greens, sprouts and green juices. I used to call it "rebuilding my pancreas". There are also a few supplements that have tended to lower my average blood sugar - mainly chromium and pancreatic enzyme supplements.
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Permalink Reply by Mody sculptor on -
Hi Tealas,
I am wondering how you are? Please post and update when you have a chance. I am still on Byetta 10 mg twice a day. I have great numbers and I am still losing weight, down to a 7/8 and I feel wonderful. It just happens to work well for me.
Hope to hear from you soon.
MB Mody Sculptor
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Permalink Reply by tealas on
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Hey there! We are still doing well!
I had my first HbA1c in normal ranges! 5.9 last June. I'm doing about 75% raw foods, with about 1 quart of fresh made green smoothies a day, and in general following the Blood Type Diet/Paleolithic diet guide lines. I still take very small doses of Humalog insulin, but only if I am going to eat cooked starches at a social gathering (pasta, cake, french fries etc) - which is only about maybe 1-3 times a week. I saw my son have a high blood sugar in June (153), but so far that is the only time I have seen with blood or urine testing (I test urine a couple times a week, since that is very easy with MODY3 lower renal threshold).
I think I have someone at work that is a mystery to his endo and he likely has MODY2 (and an agressive version of it like you). I may put you two in touch if he and his wife are interested?
Cheers,
Teala
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