Halle Berry: Poster Girl for MODY?

There's been a huge outcry online now that Halle Berry, previously a poster girl for Type 1 diabetes, has told the press that she has been able to wean herself off insulin.

A lot of people with Type 1 diabetes are very upset with this for the very understandable reason that it is impossible to go insulin if you have Type 1 diabetes unless you get an experimental pancreas or beta cell transplant--and even those are iffy. So there are a lot of people with Type 1 who are feeling betrayed and that is making for a lot of anger.

In fact, what happened to Berry has happened to quite a few people who have emailed me over the years since I put up my web page about monogenic diabetes (MODY).

None of them are TV or movie stars, so their experiences didn't hit the media. But all of them were diagnosed with Type 1 diabetes in their late teens or early 20s only to find out years later that they actually had a genetic form of diabetes that keeps beta cells from secreting--a form of diabetes where it is possible to reestablish beta cell secretion using sulfonylurea drugs like Amaryl or, more recently, Byetta instead of, or in combination with, insulin.

While some forms of MODY, like the one I appear to have, are mild enough to be misdiagnosed as Type 2 diabetes, as mine was, others can be quite severe and easily confused with Type 1 diabetes. Here's a case history of just such a case:

Identification of MODY: the implications for Holly
Journal of Diabetes Nursing, Jan, 2004 by Jo Dalton, Maggie Shepherd

The main things hinting that MODY might be at fault here were that insulin doses remained in the "honeymoon" range, years after diagnosis and that the patient did not develop DKA. Note also that the patient's father was diagnosed with "Type 2" later in life, but actually had a much milder form of the same genetic diabetes. The virulence with which these gene express is modified by many environmental factors science does not yet understand.

Many doctors still believe, incorrectly, that a person cannot develop MODY unless one parent has been diagnosed with diabetes. But even though neither of Ms. Berry's parents was diagnosed with MODY it does not rule out that she might have inherited the gene from one of them. In addition, as in the case of Holly's father, the gene may spontaneously mutate and appear in a person with no relatives with diabetes.

That there are "silent" carriers of these genes scattered through the population was only realized recently when scientists started testing family members of people diagnosed with MODY via gene tests. They discovered that there were other people in the families who were carrying MODY genes but whose blood sugar abnormalities had escaped diagnosis--probably because, like mine, the gene defect affected only post-meal blood sugar levels and were not detectable using a fasting plasma glucose test.

It is also worth noting that scientists who study genetic diabetes believe there are many more genes out there causing insulin secretory disorders than the six that have been so far identified. So it is possible that there are a lot more people diagnosed as Type 1 diabetics who have one of these not yet diagnosed genes.

However, it is also very important to note that whatever the cause of the defect, these MODY forms of diabetes are every bit as capable of wreaking havoc on eyes, nerves, and kidneys as is Type 1.

As far as Ms. Berry's situation goes, I hope that she isn't settling for the 7%-8% A1c that so many doctors consider good enough for someone with Type 2. As exciting as it might be to be able to give up insulin, trading shots for blood sugars high enough to cause blindness, amputation, and dialysis is not such a smart idea.

Ms Berry still has diabetes, it still has the potential to ruin her life, and she still needs support from the rest of the diabetes community in learning how best to get her blood sugars down into the normal range so she can avoid developing complications.

And we all need to realize that Ms. Berry's situation points out how misleading are the current diagnostic criteria which lump hundreds of different genetic and metabolic disorders into one of two bins--Type 1 and Type 2.