Should You Be Tested for MODY?

Diabetes is sometimes misdiagnosed. For example, people are often told they have Type 1 or Type 2, when they actually have MODY, also called maturity-onset diabetes of the young or monogenic diabetes. Might you be one of these people? And should you care?


A company called Athena Diagnostics thinks you might benefit from knowing your real diagnosis. At the American Association of Diabetes Educators conference last week, one of their experts shared some good information with me. So I did some more research on my own.

What is MODY?
MODY is a group of at least six genetic defects that damage insulin response. Each type of defect has different symptoms and may benefit from different therapies. Some cause severe high blood sugar levels; others damage the kidneys more; still another can wipe out a person’s whole pancreas, not just the beta cells. On the therapy side, some can be managed with lifestyle, some benefit from sulfonylureas, and others need insulin.

It is estimated that as many as 5% of all diabetes cases are MODY. People of Asian Indian descent may have more MODY. In a UK study, MODY was nearly as common as Type 2 among white youth.

Unlike Types 1 and 2, which are caused by multiple genetic and environmental factors, MODY is the result of a defect in a single dominant gene. So you have a 50% chance of passing it to children. Probably, environment and behavior don’t have much to do with it.

Signs Your Diabetes Might Be MODY
According to the brochures I was given, a person diagnosed with Type 1 might have MODY if they:

  • Were diagnosed before 6 months of age
  • Have a family history of a parent having diabetes (only 2% to 4% of people with Type 1 have an affected parent)
  • Have detectable insulin production three years or more after diagnosis
  • Have no immune antibodies to their islet cells, especially at diagnosis

A person diagnosed with Type 2 might have MODY if they:

  • Are not markedly heavy or sedentary
  • Never had acanthosis nigricans: a dark, velvety discoloration in skin folds and creases, such as in the neck, armpits, and groin
  • Have no evidence of insulin resistance, with fasting C-peptide levels (a marker of insulin production) within the normal range

Why Would You Be Tested?
Since MODY is 50% likely to be passed down to a person’s children, it would be worth knowing if you had it before having children of your own. Also, knowing you have MODY might allow you and your health-care team to provide more appropriate medical treatment.

I especially worry about the people with Type 2 who actually have MODY or LADA (latent autoimmune diabetes of adults). Many of them may have been denied insulin that would have saved their lives or made them better. However, people with Type 2 are often being prescribed insulin earlier now, so maybe that isn’t as much of a problem.

Those with MODY misdiagnosed as having Type 1 might be able to switch from insulin to a sulfonylurea in some cases. It would depend on which type of MODY they have, which requires genetic testing to find out.

This genetic testing is what Athena Diagnostics (my chief informants) sell, so you have to take that into account. The whole panel of tests could run you about $3500. Your medical insurance might pay for the tests, but some plans have genetic testing exclusions, meaning they might not pay for the MODY tests. If you can show that the testing would affect your treatment, they might have to cover it.

My own take is that, if your doctors are treating you well, based on your personal symptoms, history, and numbers, you might not need to know if you have MODY. If they are treating you as a diagnosis, according to a generic formula, then you would benefit from finding out. If you are a so-called “thin Type 2,” you might clear up some confusion by testing. And if you are thinking of starting a family, testing would be a very good idea.

I learned lots more at the AADE conference. I’ll be reporting in the coming weeks on driving, blood glucose awareness, mindful eating, and interesting new ways to change behaviors. My own talk on sex and diabetes went quite well, I thought. I’ll have to see the participant evaluations to be sure.

I really don’t like Las Vegas. The strip was like one gigantic shopping mall on top of one gigantic casino. Loud music playing everywhere. But I think I was in a minority — most people I saw seemed to be having a good time. Maybe someone can explain it to me.

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  • jim snell

    Interesting feedback. Science marches on.
    I remain a little skeptical that while some answers await, treating the folks in here and now need some better regimenes.

    I would not stop researching as many paths partial and blind allys need to be walked to winnow out solutions.

    You comments on Las Vegas – dead on target, I am sensitive person to whom loud noise and the din of the one arm bandits drives me nuts. I never happier as when I get away from that crap.
    I find that the unrelenting din making life miserable and it totally distracts my over the horizon radars.

  • jim snell

    David; a slight diversion (similar topic) but based on your earlier blogs:

    The words you shared on the bariatric surgery/lap band etc. raise interesting issues. A recent article from England where rather than any extensive surgery, they simply put a plastic liner tube inside small intestine for 2o inches from duodenum and results were equally as startling. i.e. results called remission/cure.
    My guess is that these changes drop the amount of glucose that can be absorbed from food to a point where body can properly control glucose without having to invoke insulin resistance to choke off feed to cells.
    I say this based upon my own case of reducing diet to 1200 calories for last 4 years and finally getting leaky liver shut up. Yes I needed a small boost of Humalog Lispro -4 units with every meal to off set the reduced out put but after 7 months on this approach I suddenly saw my body pick up and decide to use its own insulin I suspect was lurking around all this time . I ended up yanking all the starlix, stop using 26 units of 75/25 insulin once a day, shot actos fast to combat super lows.
    My best guess is that those of us on older hunter gatherer gene set optimized to burn very low grade fuel but choke out on the glucose generated on all the newer super grains, rice, corn high fructose corn sugar etc. To cope, the body turns on the insulin resistance to choke off excess presence of glucose all the time. When young, exercise burns off all the excess and Type 2 not usually seen at early stages(except those getting insufficient exercise). As one ages, physical activity decreases and couch potato computer tools, games, wide screen TV drops that further and voila, the old gene set body is now operating outside the margins on glucose it can handle and insulin resistance starts up blocking the body’s own insulin. Cut that glucose back using lap band, surgery, aggressive diet and the body goes back to working properly – cured – no, body just operating at proper point on the glucose operating range for that body.
    My ignorant guess is that the volume of glucose generating foods for these older hunter gatherer gene sets need to be carefully scaled back ( you mentioned starvation) to ensure glucose supply sized appropriately to what body type can manage and may need additional assistance – drugs to combat degeneration and medical failure of body/gene corruption.

  • Natalie Sera

    Since I live in Reno, Las Vegas was more of the same x 100! Ugh!! I went home on sensory overload, with my ears ringing, and seeing visions with my eyes closed. And the smoke gave me asthma attacks. ūüôĀ

    The presentations I attended were on Food, Fat, and Satiety, and Fructose: A Not-So-Sweet Connection. They were both fabulous. If you can download the powerpoint slides as a pdf they are well worth reading.

    Looking forward to reading more of your reports!

    Natalie ._c- <– that’s a duck, named Duck Fiabetes!

  • David Spero RN

    Jim, I agree with your analysis regarding the mismatch between modern carbs and some gene sets. I would only add that, in my opinion, it’s not just reducing the amount and rate of carbohydrate absorption that helps, it’s changing WHERE in the intestines the carbs absorb from. They need to get down to the ileum to trigger hormones like GLP-1. Modern carbs don’t make it that far. hat may be why that British “sleeve” approach works so well.

    I’m really glad that what you’re doing now seems to be working. I think your plan would be helpful for millions of people.

  • jim snell


    Excellent feedback – here again, all us old gene sets with digestion system to match has not caught up to modern day super grains, advanced milling and processing and foods.

    Instead we seem optimized for scrawney rabbits, sinuous deer, game, fish and nuts and berrys.

    Your comments on the hormone issues appear to back that up.

    What a heart break. Have a great day.

  • Siri Greeley

    David: Glad to see people like you trying to raise awareness about the unusual forms of diabetes such as MODY and neonatal diabetes, which we (and others) refer to as monogenic diabetes. We just published a new website to provide more information and have a number of ongoing studies for anyone interested:

    Siri Greeley, MD, PhD
    University of Chicago