MODY

Hereditary diabetes

There are more types of diabetes than just type 1 and type 2. Some forms are hereditary, such as monogenic diabetes—also known as MODY.

This type of diabetes is often diagnosed in young people, but it can also develop later in life.

GOOD TO KNOW

Monogenic diabetes can be identified by several characteristics. It sometimes resembles type 2 diabetes but typically begins at a younger age.

If a parent has monogenic diabetes, there is a 50 percent chance that their child will also develop it.

Diabeter pays attention to signs that someone may have monogenic diabetes. The Milkshake Test helps with this.

The treatment for monogenic diabetes is often different from that for type 1 or type 2 diabetes.

About MODY

MODY stands for Maturity Onset Diabetes of the Young. In Dutch, this roughly translates to “age-related diabetes in young people.” The name originated in earlier times when researchers had limited understanding of hereditary diabetes.

Today, we know that the name is actually misleading. That’s why it’s now referred to as monogenic diabetes. “Mono” means “one,” and “genetic” refers to the genes.

Monogenic diabetes is a form of diabetes caused by a single genetic mutation.

Characteristics of Monogenic Diabetes

Monogenic diabetes is not always recognized, as it can closely resemble type 1 or type 2 diabetes. However, there are several distinguishing features:

Too young for type 2 diabetes: Monogenic diabetes often develops at a younger age. Many people are diagnosed before the age of 25, and rarely after 40. It is frequently discovered during significant life stages, such as puberty or pregnancy. In contrast, type 2 diabetes typically occurs in older individuals.
Diabetes across multiple generations: Monogenic diabetes is caused by a genetic mutation passed from parent to child. If several family members have diabetes, it may be monogenic.
Unusual patterns for type 2 diabetes: Some people diagnosed with type 2 diabetes require less insulin than usual or do not have excess weight. These atypical traits reduce the likelihood that it is truly type 2 diabetes.

What does hereditary mean?

Monogenic diabetes is caused by a genetic mutation. It develops due to a single gene in the body that is involved in insulin production and glucose regulation. In people with monogenic diabetes, this gene differs from how it normally functions.

There are different types of monogenic diabetes. The location of the genetic mutation determines which type a person has.

Passing Genes from Parent to Child

Monogenic diabetes occurs in certain families because parents pass their genes on to their children. If one parent has monogenic diabetes, there is a 50 percent chance that the child will also develop it. This is called dominant inheritance.

If a child inherits the diabetes gene from one parent and a healthy gene from the other, they will still develop monogenic diabetes. That’s because the diabetes gene is stronger—dominant.

Diagnosing hereditary diabetes

It’s important for someone with monogenic diabetes to receive the correct diagnosis. This ensures timely and appropriate treatment. Additionally, some types of monogenic diabetes are associated with other physical conditions.

For example, MODY 5 is more often linked to kidney problems.

How Is Monogenic Diabetes Diagnosed?

Getting the correct diagnosis for monogenic diabetes can sometimes take years. It is often initially mistaken for type 1 or type 2 diabetes.

The official diagnosis can only be confirmed through genetic testing. It may take up to three months before the results of this test are available.

Early Testing for Monogenic Diabetes at Diabeter

Diabeter can conduct early testing for monogenic diabetes when there is a strong suspicion that this type of diabetes is present. For example:

If someone has type 2 diabetes but is slim
If diabetes occurs across multiple generations in a family, without the presence of antibodies in the blood that indicate type 1 diabetes
If a child develops diabetes before the age of one

To diagnose monogenic diabetes, Diabeter performs a Milkshake Test. This test determines whether there are still cells in the pancreas that produce insulin. During the test, the person drinks a special, thick beverage—the milkshake—which causes blood glucose levels to rise.

They also receive special tablets containing the substance repaglinide. If insulin-producing cells are still present, they will respond to the repaglinide and begin producing insulin.

Improved treatment

Thanks to this specialized testing, Diabeter has already revised the diagnosis for dozens of individuals. This means that people who were previously diagnosed with type 1 or type 2 diabetes were later found to have monogenic diabetes.

With a more accurate diagnosis, these individuals often receive a different—and better—treatment.

Tablets Instead of Insulin

For some types of monogenic diabetes, it may be possible to switch from insulin to tablets. These are the same tablets used in the treatment of type 2 diabetes. To determine whether this is suitable, various tests are required.

The appropriate treatment for monogenic diabetes often depends on the life stage a person is in. During puberty or pregnancy, the body typically needs more insulin.

These are also periods when monogenic diabetes is more frequently diagnosed. In such cases, treatment with tablets may not be sufficient.