My nine year old nephew was diagnosed with diabetes when he was six. At the time of diagnosis, he was in critical condition with blood sugars running in the 500s mg/dl [27.8 mmol/L]. Recently, my brother had some tests run on him. It seems that his pancreas is still producing insulin, and that his body isn’t allergic to the insulin. The specialist is looking into the possibility of him having MODY. His blood sugars are high a lot of the time (between 200-260 mg/dl [11.1 – 14.4 mmol/L]). He has also been gaining weight steadily for the past couple of years. His mother comes from an adopted home and does not know her biological family history. What ways are there to find out exactly what type of diabetes he has? If he does, in fact, have MODY, is this news good, bad, or indifferent?

It’s hard to interpret your nephew’s story without a good deal more information, but here are some of the possibilities. First of all, until quite recently, nearly all diabetes that developed acutely in childhood was called type�1. Recently, this has become divided into Type 1A (autoimmune)which is a disorder of the immune system and might at that time have been confirmed by getting a positive antibody test. There is also type�1B diabetes, where the onset is also acutely insulin dependent, but the antibody test is negative, and insulin production can become restored: It is rather uncommon in Caucasian families, but very common in Hispanic and African American children. The acute onset makes it unlikely that he has type�2 diabetes even though the residual C-peptide level would support this. However, if he became overweight, after developing clinical diabetes, not before, this may be due to giving rather high doses of insulin to control blood sugars. This issue is important though because, if he is still producing insulin as well as getting it by injection, and blood sugars are consistently abnormal, then some of the rare forms of insulin resistance need to be considered.

Maturity Onset Diabetes of the Young�(MODY) is a possibility, though as the name implies it doesn’t usually start acutely with blood sugars in the 500 mg/dl [27.8 mmol/L] range.. There are five main variants of this uncommon form of diabetes. Type II or hexokinase deficiency is the most common and the easiest to get diagnosed, but only in Type III, in which there is a deficiency of something called HNF or hepatic Nuclear Factor, is there likely to have been such a dramatic onset.

Specific diagnosis means enlisting the help of a laboratory that can do these tests, and often, because of the inconvenience and expense, this is not pursued because in the end, the aim in all forms of diabetes, is to avoid hypoglycemia, but to keep blood sugars near to normal limits to prevent long term damage to small blood vessels, especially in the eyes and kidneys.

I hope this gives you some idea of the possibilities that your nephew’s doctor must be thinking about.

DOB