What is MODY Diabetes

Diabetes is not a one-size-fits-all diagnosis. There are several types of diabetes with type 1 and 2 being the most common, but there are other types of diabetes that each has their own symptoms, treatments, and considerations for the people that have them. It can be frustrating to find information on the types of diabetes that are not common which is why we at Genteel are offering basic facts on the lesser-known variants. This time we’re examining MODY diabetes which stands for maturity-onset diabetes of the young.

MODY is actually a collection of diabetes types that fall under the name monogenic diabetes meaning it stems from the mutation of one gene, unlike polygenic diabetes which results from mutations in more than one gene. Type 1 and type 2 diabetes are polygenic diabetes. Because it involves just one gene, MODY is a fairly rare form of diabetes.  The rarity means it has been researched less which makes a misdiagnosis of type 2 diabetes more common. In fact, the name MODY stems from the fact that MODY mimics type 2 diabetes which is most often found in adults, yet MODY usually develops in adolescence or early adulthood. 

The gene mutation can be inherited from one or both parents, but it can also occur spontaneously. Similar to type 1 diabetes, with MODY the body cannot produce enough insulin which can lead to increased blood glucose levels. Because it is genetic, risk factors like lifestyle, weight, and even ethnicity do not play a role in who receives a MODY diagnosis. The genetic mutation appears to carry across generations so it is likely if a child is diagnosed, a parent, and even a grandparent will be shown to have the same mutation and MODY diabetes. 

Common Types of MODY

There are different types of MODY that are classified by their genetic causes. They have scientific names but are also designated as MODY1 through MODY5, with the most common being MODY3. The list includes:

• HNF1 Alpha (MODY3) occurs in 50-75% of cases
• GCK (MODY2) also called glucokinase, occurs in 30-50% of cases
• HNF4 Alpha (MODY1) occurs in 5-10% of cases
• HNF1 Beta (MODY5) also known as renal cysts and diabetes (RCAD) syndrome, occurs in 5-10% of cases

And while this may seem like a comprehensive list, there are at least 10 other types of MODY that have been identified, although each is quite rare. 

Signs & Symptoms of MODY Diabetes

So what’s the difference in MODY types?

Both MODY1 and 3 develop slowly over time as the pancreas slowly stops producing enough insulin to keep the body functioning normally. The two types have similar symptoms to other diabetes such as high blood sugar, frequent urination, fatigue, weight loss, and excessive thirst. However, there are particulars to each. Abnormally low blood sugar and/or a weight of 9 lbs. or more in newborns can be a sign of the gene mutation for MODY1, while the development of noncancerous liver tumors can be a symptom of MODY3. MODY3 most often appears in adolescence or early adulthood. 

MODY5 is most often found in combination with other issues unrelated to diabetes such as cysts in the kidneys, abnormalities in the liver or pancreas, or gout. These symptoms may appear very early on in a child’s life, but diabetes may not develop until later. 

The mildest form of MODY diabetes is GCK. The gene mutation in this type is that the gene responsible for recognizing and sparking a response to high blood glucose levels isn’t functioning and therefore allows blood sugars to rise. Despite this missing role, some people will have no symptoms and only have slightly elevated blood sugar levels. The good news is that complications from this type of diabetes are rare. 

Testing and Treatment for MODY

As with other forms of diabetes, testing for MODY is done predominantly through blood tests that look for pancreatic antibodies and C-peptide, as well as blood glucose levels. The doctor is looking to find how much insulin your body is producing and if any auto-antibodies are being made in your blood. Low insulin and no auto-antibodies can rule out type 1 diabetes, but the diagnosis should not then automatically turn to type 2 diabetes. Genetic testing can also be done to see if the gene mutation for MODY is present. 

The treatment for MODY is very much case-dependent so working closely with your health care provider is important. 

Some forms of MODY are treated with oral medications or insulin injections, and some forms may not require any treatment. The specific treatment may vary depending on what genetic mutation caused the condition. Generally, most patients with MODY1 or MODY3 will not need to take insulin, but will likely be prescribed medication that is similar to that used for patients with type 2 diabetes. For those with MODY5, insulin and adopting a healthy diet and lifestyle will be needed for management. There are also instances where testing blood sugar will need to be part of your daily life. To make testing as painless and trouble-free as possible, we suggest trying Genteel’s Lancing Device, the only one with vacuum technology enabling the right amount of blood to be drawn each time. It’s simple to use and no mistakes mean fewer pricks.  

When to Seek Help

If you suspect you or your child has MODY diabetes, educate yourself. Because the disease is rare, being your own advocate is important. Learn the signs and symptoms and be ready to ask for specific testing. If you are diagnosed with type 1 or 2 but you suspect you may have MODY, find a second opinion. Take control of your health to assure a long and happy life.