What Is MODY? What You Need to Know

In the United States, type 1, type 2, and gestational diabetes account for nearly 98% of all cases of diabetes. About two percent of people have what is known as ‘other diabetes.’ These include different types of monogenic diabetes, cystic fibrosis-related diabetes, and diabetes caused by rare syndromes. One type of diabetes that many people are unaware of is maturity-onset diabetes of the young, more commonly known as MODY.

So, what is MODY, and what should parents know about it? Below is everything you need to know, including the symptoms and risk factors associated with MODY.

What Is MODY?

MODY is a type of diabetes that is caused by a mutation (or change) in a single gene. It is a very rare type of diabetes that typically runs in families. According to a study published in Diabetes, Metabolic Syndrome & Obesity: Targets & Therapy (DMSO), MODY accounts for only 1 to 2 percent of diabetes cases and is almost always first misdiagnosed as either type 1 or type 2 diabetes.

“It is often difficult to distinguish MODY from these two forms,” explains the study because the attributes of MODY include key aspects of both type 1 and type 2 diabetes.
Like type 1 and type 2 diabetes, MODY affects the way your body uses and stores the sugar (glucose) it receives from food. When our bodies don’t produce enough insulin, it can increase blood glucose levels. This increase in glucose levels eventually leads to diabetes.

MODY is very similar to type 1 diabetes in the sense that the body struggles to produce enough insulin, and it often develops early in adolescence or young adulthood. Where the two differ is in how it is inherited.

While type 1 diabetes can be seen in multiple family members, it’s more often only found in one. MODY, on the other hand, is almost always seen in multiple family members and is likely to continue to be passed down to future generations.

Another aspect that makes MODY different from type 1 and type 2 diabetes is the way it is treated. MODY isn’t usually treated first with insulin, much like type 2 diabetes. However, unlike type 2 diabetes, MODY isn’t related closely to body weight or obesity, research has found that an obese person with an undiagnosed MODY gene mutation may develop the condition earlier in life compared to someone with a healthier body weight.

Risk Factors for MODY

MODY is typically inherited, which means your odds of developing it are significantly higher if you have family members with the disease. If you have a parent with MODY, your chances of developing the disease are around 50%. However, MODY has been noted to cross more than one generation. In some cases, can affect a grandparent, parent, or grandchild.

MODY typically occurs in teens and young adults under the age of 35. Unlike type 2 diabetes, MODY is not linked with obesity, high blood pressure, or other medical issues. Most people with MODY are at a healthy weight, and may not have any significant health concerns.

Symptoms of MODY

The signs and symptoms of MODY can differ from person to person depending on which genes are affected. In some instances, there may be no symptoms at all, however, this is quite rare. For the most part, symptoms of MODY will come on gradually, and will typically manifest before the age of 35. Some people may have high blood sugar for years before they notice the signs of MODY.

Common symptoms of MODY include:

Feeling thirsty or hungry more often than usual

Urinating more frequently

Blurry vision

Skin or yeast infections

Weight loss

Chronic fatigue

The Different Types of MODY

The type of MODY you have depends on the gene that is being affected. There are 11 different types of MODY that are caused by mutations in one of four genes. Treatment for MODY depends on what type you have and can range from simply managing diet and exercise to medications that can help regulate insulin levels.

Let’s take a closer look at the different types of MODY.

MODY 1, 3, 4 (HNF1-alpha gene)

MODY 3 is the most common type of MODY, which accounts for nearly 70% of all cases. This mutation of the HNF1-alpha gene causes the pancreas to significantly decrease the amount of insulin it produces. This type of diabetes typically develops before the age of 25.
MODY 1, 3, and 4 are often managed by taking a type of diabetes medication known as sulfonylurea that helps increase the amount of insulin your pancreas produces.

MODY 2 (GCK gene)

MODY 2 is usually treated by changing your diet and ensuring you get adequate amounts of exercise. Most people with MODY 2 will not require further treatment, and their condition will usually not worsen.

MODY 5 (HNF1B gene)

MODY 5 is caused by a mutation of the HNF1B gene, which usually results in other medical issues aside from high blood sugar levels.

MODY 7, 8, 9, 10, 11 (HNF4-alpha)

While the HNF4-alpha gene doesn’t account for all types of MODY 7 through 11, it is one of the more understood types in this overall rare group.

All types of MODY apart from glucokinase carry a risk of the long-term complications of diabetes so you should follow a healthy balanced diet and keep physically active as this helps to maintain good blood glucose and cholesterol levels which in turn reduce the risk of complications.

Why Diagnosing MODY Early Is Important

It is important to recognize and diagnose MODY early, as the behavior of this type of diabetes is different from the most common type 2 diabetes. The onset of MODY is usually during adolescence or early adulthood, which means parents should take note of any abnormal changes that may resemble the signs of diabetes. The signs and symptoms of MODY can vary depending on what gene is affected, but most people will display some hallmark symptoms of diabetes.

Because the symptoms of MODY can mirror that of type 1 or type 2 diabetes, you and your doctor may not realize at first that you or your child have MODY. Your doctor may diagnose high blood sugar levels as diabetes. The next step is to figure out what kind of diabetes you have.

Your doctor may ask about your family history of diabetes and may order blood tests that can help rule out other types of diabetes besides MODY. Based on the results, your doctor may suggest genetic testing to confirm the presence of MODY.

Getting a proper diagnosis is extremely important, especially when dealing with MODY. Since there are 11 different types of MODY, finding out which one you’re dealing with can help start you on a treatment plan sooner rather than later. In nearly every instance, an early diagnosis of any disease will typically result in a better outcome.

If you suspect you or your child has MODY, talk with a health care provider as soon as possible. If you have more questions like “what is MODY?” you may find that speaking with an endocrinologist to be helpful.

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