Everything You Need to Know About Maturity-Onset Diabetes of the Young (MODY)

Maturity-onset diabetes of the young, also called MODY, is a type of diabetes that is caused by a mutation in…(continue reading)

Maturity-onset diabetes of the young, also called MODY, is a type of diabetes that is caused by a mutation in one of the genes that regulate insulin production.

This type of diabetes usually develops during adolescence or early adulthood, but can also develop in adults.

These forms of diabetes caused by mutations account for about 2% of all cases of diabetes. In this article, we will discuss everything you need to know about MODY including what it is, who can get it, how it is treated, and the complications associated with it. 

What is diabetes?

Diabetes, also called diabetes mellitus, is a disease that is caused by problems with the hormone insulin.

When your body breaks down food it produces glucose, which is also called blood sugar. Insulin is needed by your cells to process glucose.

If your body doesn’t produce enough or doesn’t know how to use insulin, it leads to high blood glucose levels and medical problems.

Diabetes, when it is left untreated, can cause kidney damage, eye damage, liver damage, blood vessel damage, and cardiovascular problems among other medical issues.

What are the different types of diabetes?

Diabetes is actually a group of diseases and there are several types of it.

The three most common types of diabetes are type 1, type 2, and gestational diabetes which all have different causes.

Let’s take a closer look at each form below.

Type 1

Type 1 diabetes is an autoimmune disease where your autoimmune system mistakenly destroys the beta cells, or insulin-producing cells, in your pancreas.

When you have type 1 diabetes, your body decreases or stops making any insulin at all and will require daily treatment with insulin either through insulin injections or an insulin pump in order to stay alive.

You can get type 1 diabetes at any age but it is usually diagnosed in childhood through early adulthood.

Type 1 diabetes is also called juvenile diabetes or insulin-dependent diabetes and it is not preventable.

Type 2

Type 2 diabetes is a metabolic disorder that causes your body to develop insulin resistance.

This means that your body can make insulin but it doesn’t work as well as it should.

When you have type 2 diabetes, your blood sugar levels are usually high and over time this can damage your organs.

Type 2 diabetes is most often diagnosed in adults but it can also be diagnosed in children. Type 2 diabetes used to be called adult-onset diabetes but this is no longer the case because more and more children are being diagnosed with it.

It is the most common form of diabetes and is usually preventable through lifestyle changes such as diet and exercise but sometimes medication or insulin treatments are needed as well.

Gestational Diabetes

Gestational diabetes is a type of diabetes that only pregnant women get.

It happens when your body can’t make enough insulin to deal with the extra glucose in your blood during pregnancy.

It usually goes away after you have your baby; however, both you and your child will have an increased risk of developing type 2 diabetes later on in life.

What is maturity-onset diabetes of the young (MODY)?

MODY is a rare form of diabetes that is most commonly diagnosed in teenagers and young adults although you can get it at any age.

MODY is a form of monogenic diabetes, which means it is caused by a single gene mutation that controls how the body uses or produces insulin.

As a result, blood sugar levels can become too high.

MODY Diabetes: NorthShore University HealthSystem

What causes MODY?

MODY is caused by a mutation in one of your genes that controls how the body uses or produces insulin.

This genetic mutation can be passed down from parent to child or it can occur randomly.

If you have a parent with MODY there is a 50% chance that you will develop it too.

There are several different genes that can mutate to cause MODY and, depending on which gene mutates, can determine how serious diabetes may affect you and also dictate your treatment options. The other form of monogenic diabetes is neonatal diabetes. 

Who can get MODY?

MODY diabetes can affect anyone at any age, but most people are young adults or an adolescent at the age of onset.

If you have a strong family history of diabetes and specifically MODY, it is important to be screened for MODY even if you don’t have any symptoms due to the chance of it being inherited from a parent.

What are the symptoms of MODY?

The symptoms of MODY can vary depending on which gene mutation you have. However, the common symptoms of MODY are similar to the symptoms of diabetes in general and include:

  • Excessive thirst
  • Excessive hunger
  • Frequent urination
  • Blurry vision
  • More frequent infections, especially skin and yeast infections
  • Weight loss despite eating more
  • Fatigue

If you are experiencing any of these symptoms please talk to your doctor or health care provider.

What are the complications of MODY?

MODY can have the same risk of complications of diabetes in other forms due to high glucose levels. The diabetes-related complications include:

  • Skin problems, such as infections
  • Heart disease
  • Nerve damage
  • Eye damage and even blindness if left untreated
  • Foot problems that can end in amputation if left untreated

How do doctors diagnose MODY?

MODY is often misdiagnosed at first as a more common form of diabetes.

The condition has the same symptoms and it’s difficult to determine the cause. Diagnosis of diabetes usually requires a blood test to determine your blood glucose levels but more testing will be needed for the diagnosis of MODY.

Usually, doctors will rule out other forms of diabetes first before performing genetic testing using your blood or saliva which is the only way to receive the correct diagnosis.

How do you treat MODY?

There is no one-size-fits-all treatment for MODY and your treatment will vary depending on which gene is mutated to cause MODY.

Please see below for the different mutations and treatment options.

MODY 1 and MODY 4

If you have these mutations you will usually need to take different diabetes medications, most often sulfonylureas, to help your pancreas make more insulin.

Insulin therapy may also be prescribed in some cases.

MODY 2

The treatment for this form of MODY is similar to the treatment for type 2 diabetes in that it usually is treated with diet and exercise.

MODY 3

Eating a healthy and balanced diet is the most frequent treatment for this form of MODY.

Sulfonylureas may also be used in conjunction with a healthy diet to help your pancreas produce more insulin too but isn’t always necessary.

This is the most common type of MODY for people with European ancestry. 

MODY 5

The treatment for MODY 5 is similar to type 1 diabetes in that you will require insulin treatment.

MODY 6

This form of MODY requires insulin injections or an insulin pump too and is usually diagnosed after the age of 40.

If you have MODY it is important to work closely with your doctor or health care provider to create a treatment plan that works best for you.

Living with diabetes can be difficult, but by following your prescribed treatment plan and monitoring your blood sugar, you can live a long and healthy life.

If you think that you or someone in your family may have MODY please talk to your doctor or health care provider. Your health care provider will be able to help screen for it and get you the optimal treatment.

Summary

MODY is a monogenic form of diabetes meaning that it is caused by a single mutation to one of your genes.

People with MODY are usually a teenager or young adult at the age of diagnosis although it can occur at any age.

The symptoms are the same as other forms of diabetes with the most common being excessive hunger and thirst and frequent urination among others.

Similar complications to other forms of diabetes may occur including heart disease, kidney disease, and eye damage although others may occur also.

Doctors can only diagnose MODY using genetic tests of your saliva or blood for a genetic diagnosis and treatment will be determined by which gene has the mutation.

If you have any more questions or think you may have MODY please talk to your doctor or health care provider.

References and Sources:

NIH

American Diabetes Association 

Fact Checked and Editorial Process

Diabetic.org is devoted to producing expert and accurate articles and information for our readers by hiring experts, journalists, medical professionals, and our growing Diabetic.org community. We encourage you to read more about our content, editing, and fact checking methods here. This was fact checked by Erik Rivera and medically reviewed by Dr. Angel Rivera. 

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