Dear Diabetes Educator,
My sister told me my thirteen year old niece has a condition called MODY diabetes. My grandmother had diabetes and my sister and brother have diabetes. Can you tell me what this is and where I can get more information about it?
MODY is the acronym for Maturity Onset Diabetes of the Young. It is a form of diabetes that is relatively rare. It is thought that approximately 1-5 percent of all people with diabetes have MODY and it is a genetic form of diabetes. According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) a child of a parent with MODY has a 50-50 chance of developing this disease. As with your niece, it is usually diagnosed during adolescence or a little later but it has been diagnosed from one year of age up to age 50. It is caused by a genetic mutation that targets the pancreas resulting in lack of enough insulin. Many people with MODY may think they have type 1 or rmaybe type 2 diabetes. Medical therapy depends on which gene is affected as there are different types of MODY. Some people with MODY need to take insulin and some need oral medications to help make more insulin. Genetic testing is used for confirmation of MODY. As with your niece, MODY is usually present in many generations of a family. Ask your physician if any family member should be tested to see if they are likely to develop this type of diabetes. For more information please go to the National Diabetes Information Clearinghouse (NDIAC) at: http://diabetes.niddk.nih.gov/dm/pubs/mody/
Kayla please also contact the Kovier Diabetes Center at the University of Chicago. The Kovier Center s has a MODY registry. To be included in the registry one needs the following:
(This information was taken directly from http://monogenicdiabetes.uchicago.edu/mody-registry/ )
Diagnosis of diabetes after 12 months of age and before 50 years of age AND at least one of the following criteria:
Presumed diagnosis of type 1 diabetes with one or more of these features atypical of type 1 diabetes
Negative autoantibodies (no antibodies to GAD 65, insulin, islet cell, or ICA-512/IA-2)
Detectable c-peptide >1 year after diagnosis
Adequate diabetes control on unusually small doses of insulin and/or no significant illness with missed insulin doses >1 year after diagnosis
Presumed diagnosis of type 2 diabetes with one or more of these features atypical of type 2 diabetes
Diagnosis before age 25 years, especially in ethnicities with a low prevalence of type 2 diabetes (non-Hispanic white, Asian, South-east Asian)
Not significantly overweight (body mass index <30 kg/m2)
No signs of insulin resistance on examination or blood tests
Incidental diagnosis of mild but persistent high blood sugar that does not worsen over time
Diabetes in 3 or more successive generations of family members with a pattern consistent with a mutated gene being passed from a parent to their children
Known genetic cause of diabetes in you or your family
The information above was taken from:. Please call the registry information staff at (773) 702-0829 to get more information or go to the site.
Thanks for asking such a great question!
Clara Schneider MS,RD,RN,CDE