Cystic fibrosis-related diabetes (CFRD) is a type of diabetes common in people living with cystic fibrosis (CF). It occurs as a complication of CF, which damages the pancreas. Medication and lifestyle changes can help manage the condition.

CF is a genetic condition that occurs when the body produces mucus that does not function as usual. CF mainly affects the lungs and digestive system but can also cause complications, such as diabetes. This is due to mucus damaging the pancreas, which is where the body produces insulin. This hormone plays a vital role in helping to regulate blood sugar levels.

This article discusses CFRD, including potential causes, symptoms, and treatment options.

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CFRD is a type of diabetes that occurs as an extrapulmonary complication of CF. This term means CFRD is a complication of CF that occurs outside the lungs. It occurs when the thick, sticky mucus common in CF leads to damage and scarring of the pancreas.

CFRD is common in those living with CF. Some evidence suggests that more than one-third of people over 16 years with CF receive treatment for CFRD. However, other research indicates a lower incidence, with one-fifth of individuals with CF having CFRD.

While CFRD may present similarly to type 1 (T1DM) and type 2 diabetes (T2DM), it is a distinct type with its own causes, symptoms, diagnosis, and treatments. It is also possible for a person living with CF to develop another type of diabetes.

CF occurs due to an alteration in the CFTR gene. These variations can cause a variety of issues throughout the body, including the pancreas.

CFRD is a complex condition, and researchers are still studying the underlying mechanisms of CFRD. Evidence suggests that it occurs due to inflammation and scarring of the pancreas. This damage may occur due to how changes in the CFTR gene affect the exocrine pancreas. This term refers to the role the pancreas plays in helping digest food.

These changes may attract immune cells to the pancreas, which can result in damage to pancreatic tissue. This can affect the beta cells of the pancreas, which are responsible for producing insulin. With a reduced insulin production, the body will experience difficulty regulating blood sugar levels, resulting in symptoms of diabetes.

In many cases, people will usually receive a diagnosis of CFRD before they notice symptoms. However, some people may experience symptoms such as:

  • increased thirst
  • the need to pass urine more frequently
  • blurry vision
  • reduced lung function
  • fatigue
  • weight loss

According to CFRD guidelines, it is advisable for individuals with CF, ages 10 and older, to test annually for the condition.

Typically, a doctor will be able to diagnose CFRD using an oral glucose tolerance test (OGTT). This involves a person fasting for 8 hours and then drinking a liquid that is high in sugar. A doctor will then compare blood samples before and after the person ingests the liquid. If a person’s blood sugar levels are high, this may indicate diabetes.

A doctor may also use other screening tests to measure if a person has high blood sugar levels. They may then order further tests to confirm the CFRD diagnosis. While CFRD may present with similarities to T1DM and T2DM, these diagnostic tests can help differentiate it from other types of diabetes.

Currently, there is no cure for CFRD. However, with proper treatment, a person can help manage the condition. Typically, this involves a person managing their blood sugar levels. Not only does this help to avoid potential complications of diabetes, but it helps them gain weight and increase muscle mass.

A person can help manage CFRD by:

  • using medications such as insulin
  • monitoring blood sugar levels regularly
  • continuing eating a high calorie diet to help manage CF
  • staying physically active

In the short term, potential complications of CFRD may include:

  • weight loss
  • malnutrition
  • decrease in lung function
  • more severe lung infections
  • thicker mucus due to high blood sugar levels

In the longer term, a person may be at risk of developing complications often associated with diabetes. This may include:

Cystic fibrosis-related diabetes is a type of diabetes and potential complication of cystic fibrosis. It occurs as a result of damage to the pancreas due to sticky mucus causing scar tissue to form on the pancreas. This scarring can prevent the organ from producing enough insulin, which can make it challenging to manage blood sugar levels.

Symptoms of CFRD are similar to other types of diabetes. Treatment of the condition involves managing blood sugar levels. A person can do this through the use of medication and regular exercise.