by Liz Revilla
Vertex Education Ambassador, Fitness
Cystic Fibrosis Related Diabetes (CFRD) is the most common complication of CF that is unrelated to the lungs. CFRD is a unique condition that is different from Type 1 or Type 2 Diabetes, although it contains elements of both. Currently about 40% of adults with CF will be diagnosed with CFRD in their lifetime, and it is more common in those who are hospitalized, taking steroid medications, pregnant, receiving tube feedings, or after lung transplant. Early treatment is important to prevent decreased lung function, unintentional weight loss, and other undesirable outcomes.
Since CFRD can often begin with no symptoms, it is important for people with CF to be screened regularly. Starting at age 10, everyone with CF should have an Oral Glucose Tolerance Test (OGTT) once per year. They must not have anything to eat or drink for at least 8 hours before the test. An OGTT is done by having a fasting blood sugar level taken, drinking a specific amount of an orange flavored soda called Glucola, waiting either one and/or two hours, and then checking another blood sugar level. The CF care team will review the OGTT results (which could be either normal, pre-diabetes, or CFRD) and decide on the next action steps that are recommended.
The primary problem in CFRD is lack of adequate insulin production, so it makes sense that the primary treatment is providing supplemental insulin. In people with CFRD, insulin therapy can improve lung function and nutritional status very quickly. When someone is newly diagnosed with CFRD, they will need quite a bit of education on how to check their blood sugar, how to administer the correct dose of insulin, and possibly how to count carbohydrates if blood sugars tend to be high after meals. They will also learn how to identify the signs of high and low blood sugars and how to treat each one, as well as the complications of unmanaged CFRD and how to avoid them.
The main goal of nutrition management in CFRD is to ensure adequate nutritional intake while maintaining healthy blood sugars. Sometimes people might feel afraid to eat or try to eat less to keep their blood sugar numbers low, but it is important to continue to eat a well balanced diet (including plenty of lean protein, whole grains, fruits and vegetables, and nuts, seeds, and other healthy fats). Your body needs all of these foods to keep your immune system and your lungs in tip top shape!
The management of CFRD should be a top priority for all people with CF and their care teams. As a person with CF, you can advocate for yourself by making sure you get your OGTT done every year and/or checking your blood sugar and taking your insulin as prescribed. If you have any questions, please reach out to your CF care team!