Know Your MODY (it doesn’t rhyme with body)
March 17th, 2008 by Steve
This is the next in a series of article excerpts by me discussing all forms of diabetes and metabolic syndrome. This article was originally published November 5th, 2007 in the Caller Times and reposted here with permission.
By now most of us have heard that there are two types of diabetes: type 1 and type 2. By far, the majority of all persons with diabetes (about 85%) have type 2 diabetes. This used to be called adult-onset diabetes or non-insulin dependent diabetes. These latter two terms are outdated and no longer relevant. We now know that kids can get “adult” diabetes even before they become teenagers, plus persons with type 2 diabetes often require insulin injections to survive the longer they have the condition.
But there is another form of diabetes that the public is incredibly unaware of. This type of diabetes is estimated to account for up to 5% of all cases of diabetes. It’s called Maturity Onset Diabetes of Youth, better known as MODY. MODY is a genetic form of diabetes that is passed down from one generation to the next. A mother or father with MODY diabetes will pass it to either a son or daughter. Because it is a dominant genetic condition, this means there will be a direct chain of inheritance up the family tree (grandparent to parent to child). Persons with MODY can be of any weight, even though originally it was described in only thin persons. Our obesity epidemic has made diagnosis of this form of diabetes a lot trickier.
The reason why patients should know if they have MODY is that there is a 50% chance of passing the condition on to their children and grandchildren. That information alone could be invaluable to the future health of the child in regards to earlier diagnosis and treatment which would lower the risk of long term diabetes complications. Once you know whether you have a MODY form of diabetes, the MODY gene tests can be done long before diabetes ever occurs in a child.
Last 5 posts by Steve
- Halloween and Diabetes CAN mix! - October 5th, 2009
- Introducing Healthy Families of South Texas - October 5th, 2009
- Complications 101 - About Kidney Failure - August 23rd, 2009
- The Sleep Bolus - August 18th, 2009
- The Dirty Truth Behind DCCT - August 16th, 2009
April 29th, 2008 at 12:31 pm
I have heard of MODY, but you’re so right; it’s not talked about much at all. Good idea to blog about it and help awareness!
May 5th, 2008 at 7:27 pm
I have heard of MODY, but the girl I knew had what she called MODY3. What does the number stand for?
May 11th, 2008 at 8:33 pm
Hi Emmy - my best guess is that your friend was referring to a genetic mutation form of diabetes. This is the most common form in those w Euro ancenstry. The MODY link at the end of the article takes you to Wikipedia. There is a nice description of MODY3 which includes a heads up that your friend might not have to use insulin early on but will most likely end up on insulin.
From Wikipedia:
http://en.wikipedia.org/wiki/Maturity_onset_diabetes_of_the_young#MODY_3:_hepatocyte_nuclear_factor_1.CE.B1
“This is the form of MODY which can most resemble ordinary type 1 diabetes, and one of the incentives for diagnosing it is that insulin may be discontinued or deferred in favor of oral sulfonylureas. Some people treated with insulin for years due to a presumption of type 1 diabetes have been able to switch to pills and discontinue injections. Long-term diabetic complications can occur if the glucose is not adequately controlled.”
May 15th, 2008 at 6:04 pm
She DOES live in Canada…
And she was hypoglycemic before being diagnosed with MODY 3 Thanks!!
June 5th, 2008 at 5:01 pm
My husband at the age of 35 passed away from complications from MODY. We didn’t know at the time
that he had MODY….I had 4 of my kids genetically tested after he died one of my 4 children tested positive.
He was 11 and was asymptomatic, now he is 15 and his A1c’s have been increasingly rising. We have not
treated him with medications yet, he is trying to control it with diet. We know that medication will be
inevitable in the future.
I am soooo thankful that they discovered this type of diabetes and that they understand the complexities
of it. It is unfortunante that my late husband was treated like a regular type 1 diabetic, I believe that
contributed to his early demise. I am learning everything I possibly can about this terrible disease, my son
is a 5th generation MODY carrier and I will do anything I can to change the course of our families history.
I am very knowledgeble about this diease from first hand experience….but I get VERY frustrated when work
ing with my sons doctors, I don’t think they understand. I would love to have a chat with somebody who
really understands this so I can prevent all of the complications my husband had to go through with my son.
June 6th, 2008 at 10:37 am
I’m glad this is getting discussed. I’m a very experienced endocrinologist and even I must tell many of my patients when their diabetes is first discovered that I may not have a solid diagnosis of the type or form of diabetes for several weeks, maybe longer. The biggest challenge is getting docs to be more aware that MODY can be tested for when the circumstances call for it. I have seen patients get off insulin for years once they were diagnosed, and managed with oral agents. But as many of you know, some MODY types are progressive and insulin (and unfortunately complications of diabetes) could still lie ahead.
Janelle, if you want to speak with me, I can be reached at “Stephen.Ponder @ dchstx.org”
September 12th, 2008 at 9:06 am
Both of my kids have MODY 2. Thankfully we are close to a great Childrens Hospital that is on the cutting
edge with research of MODY. It is good to hear from people who are in the same boat as us, because
when I try and explain this to people, (even in the medical community) they act like I am crazy! We are
lucky in that we have the MODY2 mutation, and is not known to be progressive. I am not that great with
computers, but often wish I could start up a support group for people with MODY online. I have searched
and not found any…(except this page). If I knew how to create a blog I would!! Anyway, if there is anyone
else with the same interests, or with children that would just like to email back and forth with ideas, and
just have someone to talk to that knows what your talking about let me know!
September 12th, 2008 at 8:34 pm
My husband and so far 1 of our children have MODY. My husband was diagnosed at age 16 and our daughter at age 10. I feel the frustration that Jayme stressed in dealing with people even in the medical field that think you are crazy when you tell them that a child has type 2 diabetes. I go round and round with our school nurse too. My husband as been on Lantis for about 1 year along with his oral meds. His A1C has come down but he is so sensitive to carbs. It is so hard for him eating his meals and watching carbs. Our 10 year old just takes oral meds now. I hope she keeps a good A1C on them. Over the past two years her numbers have been slowly climbing. Honeymoon faze may be over. We have two other daughters and not a day goes by that I don’t worry about them also getting it. I watch for signs and symptoms constantly. I would love a support group online too. I would love to email Jayme or anyone else with kids or spouses that suffer from MODY too. Thanks so much.
September 17th, 2008 at 8:03 pm
With great help from this site, I started a support web page at:
http://www.modysupport.ning.com
Please come and join in!
Also feel free to email me at: modymom@comcast.net
June 26th, 2009 at 7:59 pm
I just read Janell Ponte’s story and I was sorry to hear about her husband. My 13 year old son was diagnosed with type I a year and half ago. Within the first year his A1C dropped to great numbers and he started needing less insulin and now none. He follows a good diet and with some exercise he seems to be doing great. The doctors said this was just a honeymoon and will all end about three months. Well a year and a half later and still no insulin needed except a unit or two on occasion. The doctor told me today that now he thinks this might not be type I but possibly MODY. He has had some adnormal kidney test, which concerns us. We were told he may have to start kidney medicine to prevent failure. After his appt I came home and started searching the computer for this MODY. I found that kidney and heart failure is common in some of the MODY’s. He wants to wait 6-8 months before we do the gene test. My concern was after reading her husband died at such a young age should I be concerned? My father was Type I and died at 65 from kidney and heart failure so now I can’t help but to think what is going on with my son. Is this adnormal kidney test have anything to do with MODY?
June 29th, 2009 at 1:03 pm
Sue:
MODY is suspected based on the presence of 3 classic clinical features: 1) multi-generational family history
of diabetes (patient, parent, grandparent..all directly related), 2) ability to manage off insulin for at least 2
years, and 3) being lean. This last element has been altered by our national obesity epidemic.
I routinely measure diabetes autoantibodies at diagnosis to verify T1 diabetes. Only if this were negative
would I seriously consider MODY. Most docs won’t try any patient off insulin for 2 years like him, so
the negative autoantibody test is a better criterion to include. There are multiple forms of MODY and the
Athena Labs will perform a screening panel. But the cost is very high and your doctor might need to
write a letter of medical necessity to get it covered by your insurance. Still, there may be out of pocket
costs. Last time I checked, the price was between 2 and 3 THOUSAND dollars. Still, knowing the answer
can be considered “priceless”.
The kidney matter should have been done by a 24 hour timed collection and not after strenuous exercise.
You were probably given a medication whose name ends in “-pril” or “-artan”. Both are actually helpful
to the diabetic kidney and should be taken indefinitely. Between 4 and 8% of all teen diabetics may have
some microalbuminuria and this may or may not be directly due to the diabetes. It can worsen with poor
control and should be followed by your doctor on an annual basis, or by a nephrologist. I hope his blood
pressure is ok. Both the aforementioned meds are actually BP meds. If you want to see the cardiologist,
that’s not a bad idea if you want to be assured of his heart status. I hope you get a clean bill of health
on that.
MODY or not, keep the A1c as low as possible to lower risk of complications, plus keep BP under good
control.
Please let me know if the MODY test comes back positive.
Good luck, I hope this helps.
August 5th, 2009 at 10:44 am
My son has not been diagnosed with MODY, however he has been diagnosed with Type 1 Diabetes for
6 years. He recently returned from Diabetes camp where the doctor on duty told me she thought he might
have MODY. His A1c is as of the July 24, 2009 is 5.3. They got him down to 1 unit of insulin a day. I have
not been able to talk to his endo.
Melinda