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Maturity-Onset Diabetes Of The Young (MODY) is a rare, genetic form of diabetes. This article outlines the symptoms, diagnosis and treatment of MODY.

Diabetes is a disease that is characterized by high levels of sugar in the blood. The most common forms of diabetes are type 1 and type 2 diabetes, but there are rarer types of diabetes including one known as maturity-onset diabetes of the young (MODY). This kind of diabetes is genetic in nature and runs in families. Similar to how type 1 and type 2 diabetes develop, MODY is caused because the body is no longer able to functionally metabolize and store sugar. However, treatment for MODY is different and therefore, it is vital to get the right diagnosis.

As MODY is rare, it affects about one to two of all people with diabetes, and is often not even recognized in the early stages.

MODY: Causes and risk factors

As mentioned above, MODY is genetic in nature. Therefore, it is caused by mutations in your genes and can be inherited. This is a unique type of diabetes as both type 1 and type 2 are caused by a mixture of lifestyle and genetic factors while MODY is caused exclusively by genetics. There are several different forms of MODY that are caused by mutations in different genes involved in making insulin. Therefore, mutations of genes that are involved in insulin production lead to a deficiency in the volume of insulin produced.

People with one parent with MODY have a 50 percent chance of getting it. MODY can also be a multi-generational disease with a grandparent, parent, or child having the disease.

People whodevelop MODY usually do so when they are teenagers or at least below the age of 35, although it can develop later. There aren’t specific risk factors associated with MODY (besides genetics). This is unlike type 2 diabetes, which is more likely to develop in people who are obese, have a sedentary lifestyle or have high blood pressure. In fact, even people with a healthy body weight can develop MODY.

Symptoms of MODY

Symptoms of MODY can differ depending on which genes are mutated. In some people, there may be no symptoms not all. However, in most cases, certain symptoms appear gradually. These are the some of the classic symptoms associated with diabetes, including MODY, and can develop after a long period of time in which you have high blood sugar levels:

  • Excessive thirst
  • Excessive hunger
  • Frequent urination
  • Impaired vision
  • Blurry vision
  • Skin infection
  • Yeast infection
  • Weight loss
  • Fatigue or tiredness

Complications of MODY

Similar to other forms of diabetes, uncontrolled high blood sugar levels can lead to a wide array of complications including:

  • Neuropathy (nerve damage)
  • Cardiovascular disease (heart disease)
  • Eye disease
  • Foot problems
  • Skin issues

Diagnosis: How is MODY diagnosed?

Since getting the right treatment for MODY is dependent on the subtype of MODY you have, it is important to get the right diagnosis. It might be hard to just diagnose you with diabetes as many people don’t present with symptoms right away. Once you do present with symptoms that suggest a diagnosis of MODY, your doctor will send you for blood tests to confirm the diagnosis and determine you blood glucose levels. The types of tests they may administer include:

  • Hemoglobin A1c test
  • Fasting blood glucose test
  • Random blood glucose test
Based on the results of your blood tests, if your doctor suspects that you condition may be genetic then they will send you for genetic testing, which will look for mutations in genes that are known to play a role in MODY. If the genetic testing comes back positive then you will be diagnosed with MODY.

Treatment: How is MODY managed?

While MODY is similar to other types of diabetes, the treatment differs based on the subtype of MODY you have. To date, six subtypes of MODY have been identified and people are treated accordingly. These are the different treatments for MODY:

  • MODY 1. MODY 1 is generally treated using sulfonylureas, which is a type of diabetes medication. Sulfonylureas encourage your pancreas to produce more insulin. Some patients with MODY 1 may need to take more insulin.
  • MODY 2. MODY 2 is a type of diabetes that is managed with a combination of diet and exercise. Hence, patients don’t usually have to take medication.
  • MODY 3. MODY 3, at first, is treated through changes in diet. However, as time goes on patients may need sulfonylureas and subsequently insulin.
  • MODY 4. MODY 4 is treated using sulfonylureas and some patients may need to take insulin.
  • MODY 5. MODY 5 is treated using insulin. This is a rare type of MODY that can harm your other organs such as the kidneys and you may need treatment for MODY-related complications.
  • MODY 6. MODY 6 develops later on in life, generally around the age of 40 and is treated using insulin.
After diagnosing the right type of MODY, doctors can prescribe the best possible treatment to help manage the disease.

  • Shields, B. M., et al. "Maturity-onset diabetes of the young (MODY): how many cases are we missing?." Diabetologia 53.12 (2010): 2504-2508.
  • Vaxillaire, Martine, et al. "A gene for maturity onset diabetes of the young (MODY) maps to chromosome 12q." Nature genetics 9.4 (1995): 418.
  • Winckler, Wendy, et al. "Evaluation of common variants in the six known maturity-onset diabetes of the young (MODY) genes for association with type 2 diabetes." Diabetes 56.3 (2007): 685-693.
  • Photo courtesy of SteadyHealth

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