Cystic Fibrosis-Related Diabetes Blood Test

What is Cystic fibrosis-related diabetes?

Cystic fibrosis-related diabetes (CFRD) is a unique type of diabetes that develops in people with cystic fibrosis, affecting up to 50% of adults with CF. It is caused by thick mucus blocking the pancreas and preventing insulin production, combined with increased insulin resistance during CF lung infections. The Hemoglobin A1c with eAG Estimation is the most important test for monitoring CFRD because it tracks average blood glucose control over 2-3 months.

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What causes cystic fibrosis-related diabetes?

Cystic fibrosis-related diabetes is caused by thick, sticky mucus that blocks the pancreas and damages insulin-producing cells over time. In CF, the defective CFTR protein causes mucus to accumulate in the pancreas, leading to scarring and destruction of both the cells that produce digestive enzymes and the beta cells that make insulin. Unlike Type 1 diabetes where the immune system attacks insulin cells, or Type 2 where the body becomes resistant to insulin, CFRD develops because the pancreas physically cannot produce enough insulin due to CF-related damage.

What is the best test for cystic fibrosis-related diabetes?

The Hemoglobin A1c with eAG Estimation is the most important test for monitoring cystic fibrosis-related diabetes because it measures your average blood glucose control over the past 2-3 months. This test shows what percentage of your hemoglobin has glucose attached to it, and the eAG number translates this into an average glucose reading you can compare with your daily meter checks. For people with CF, keeping your A1c in a healthy range is essential because good glucose control helps maintain lung function and nutritional status, which are critical for overall CF health. Your CF care team will use this test regularly to adjust your insulin or other treatments and prevent complications that could further impact your breathing and nutrition.

When should I get tested for cystic fibrosis-related diabetes?

You should get tested if you have cystic fibrosis and notice unexplained weight loss, increased thirst or urination, or declining lung function despite good CF care. Annual diabetes screening is recommended starting at age 10 for all CF patients, but you should test sooner if you experience frequent respiratory infections that are harder to recover from, fatigue that interferes with daily activities, or if you are taking medications like corticosteroids that can raise blood sugar. Getting tested regularly helps catch CFRD early so you can start treatment before it impacts your lung health and nutritional status.

What are the symptoms of cystic fibrosis-related diabetes?
Cystic fibrosis-related diabetes symptoms include unexplained weight loss despite maintaining your usual diet, increased thirst and frequent urination, and declining lung function with more frequent pulmonary exacerbations. You might also notice persistent fatigue, slower recovery from CF-related infections, and difficulty maintaining your nutritional status even with pancreatic enzyme supplements. Some people with CFRD do not have obvious symptoms initially, which is why regular screening is so important for anyone with cystic fibrosis.
Who is at risk for cystic fibrosis-related diabetes?
Anyone with cystic fibrosis is at risk for developing CFRD, but risk increases significantly with age, affecting about 20% of adolescents and up to 50% of adults with CF. Risk factors include having severe CFTR gene mutations, a history of frequent lung infections requiring corticosteroid treatment, pancreatic insufficiency requiring enzyme replacement, and being female with CF. People who have had liver disease related to CF or who require tube feeding for nutrition support also face higher risk of developing diabetes.
What happens if cystic fibrosis-related diabetes is left untreated?
Untreated cystic fibrosis-related diabetes leads to accelerated decline in lung function, increased frequency and severity of respiratory infections, and progressive weight loss that makes it harder to maintain the nutritional status needed for CF health. High blood sugar levels create an environment where bacteria thrive in the lungs, making infections worse and harder to treat. Over time, uncontrolled CFRD can also cause typical diabetes complications like nerve damage, kidney disease, and vision problems, which compound the challenges of managing cystic fibrosis and significantly reduce quality of life and survival.
Can cystic fibrosis-related diabetes be diagnosed with a blood test?
Cystic fibrosis-related diabetes is typically diagnosed using an oral glucose tolerance test, but blood tests like the Hemoglobin A1c are essential for ongoing monitoring and management once CFRD is identified. The A1c test shows your average glucose control over 2-3 months and helps your healthcare team adjust insulin doses and evaluate how well your treatment plan is working. While the initial diagnosis requires the glucose tolerance test, regular A1c blood tests are the cornerstone of long-term CFRD management to protect your lung function and overall health.
How is cystic fibrosis-related diabetes treated?
Cystic fibrosis-related diabetes is treated primarily with insulin therapy, as this helps maintain both glucose control and the high-calorie nutrition needed for CF health. Treatment focuses on keeping blood sugar in a target range that prevents complications while supporting adequate caloric intake and weight maintenance, which is different from typical diabetes management where calorie restriction may be recommended. Your CF care team will work with an endocrinologist to adjust insulin doses based on your A1c results, daily glucose monitoring, nutritional needs, and lung function, often coordinating timing with meals, snacks, and tube feedings to maintain both glucose control and nutritional status.
How can I prevent cystic fibrosis-related diabetes?
While you cannot completely prevent cystic fibrosis-related diabetes since it results from CF-related pancreatic damage, you can delay its onset and reduce complications through excellent CF management. Maintaining aggressive treatment of lung infections, adhering to your pancreatic enzyme replacement therapy, and working with a dietitian to optimize nutrition help preserve pancreatic function longer. Getting screened annually starting at age 10 allows for early detection and intervention, which can slow the progression of glucose problems and protect your lung function and overall health.
What can I do at home to manage cystic fibrosis-related diabetes?
At home, you can manage cystic fibrosis-related diabetes by monitoring your blood glucose as directed by your care team, taking insulin with meals and snacks as prescribed, and maintaining the high-calorie diet needed for CF health. Continue all your regular CF treatments including airway clearance therapy, inhaled medications, and pancreatic enzymes, as good CF management helps stabilize blood sugar levels. Keep a log of your glucose readings, insulin doses, and symptoms like fatigue or increased thirst so your healthcare team can adjust your treatment plan at regular visits, and stay consistent with follow-up A1c testing to track your long-term glucose control.
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If you have any questions, please text us at 754-799-7833 or email support@privatemdlabs.com and we'll gladly help you.
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Hemoglobin (Hgb) A1c With eAG Estimation
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