Q: Why does my urine smell like maple syrup? 

A: If you notice a very distinct sweet smell as you urinate, this could mean one of two things: maple syrup urine disease or diabetic ketoacidosis (DKA).

Maple syrup urine disease is a metabolic disorder that causes certain amino acids to build up in the body. This disease is typically diagnosed in infancy and can be harmful. It must be closely monitored by a metabolic specialist.

DKA is a potentially life-threatening complication of diabetes. It occurs when your body's blood sugar spikes from a safe level of 70 to 100 milligrams to a dangerous level of over 250 milligrams, and remains there, untreated. DKA is a result of an insulin shortage, which prevents the body from processing sugar properly. In response, the body instead burns fatty acids that produce acidic ketone bodies.

A warning sign of DKA is a person's breath may develop a specific, sweet, fruity smell.

Other symptoms may include vomiting, abdominal pain, shortness of breath, increased urination, generalized weakness, and confusion. Onset of symptoms is usually rapid.

DKA happens most often in patients with type 1 diabetes, but can also occur in those with type 2 diabetes under certain circumstances. Triggers may include:

  • Infection
  • Not taking insulin correctly
  • Stroke
  • Certain medications that increase blood sugar, such as steroids

It is typically diagnosed when testing finds high blood sugar, low blood pH, and the presence of ketoacids in either the blood or urine.

DKA is treated primarily with intravenous fluids and insulin. Blood sugar and potassium levels are regularly monitored during treatment. Usually, potassium supplementation is needed to prevent the development of low blood potassium. Antibiotics may be required in those with an underlying infection.

Although smelling your urine may not be the first thing on your to-do list, it could alert you to a larger issue.  In some cases, diabetes can manifest with few visible symptoms. Therefore, DKA can serve as the first clue to a diabetes diagnosis.

If you have been diagnosed with diabetes, it is important to follow your treatment plan closely to avoid DKA. Make healthy lifestyle choices such as eating a diet high in fiber and low in fat and calories, prioritizing physical activity, and monitoring your blood sugar levels.

If believe you are experiencing symptoms of DKA, speak to your doctor right away as this condition requires immediate medical attention.

Melissa Bertha, DO, is family medicine specialist at Mercy Primary Care at Roosevelt Boulevard.