What is Cystic Fibrosis-Related Diabetes (CFRD)?
Cystic Fibrosis (CF) is an inherited medical disorder that leads to severe damage of the lungs, digestive tract, and other organs of the body.
Specifically, CF affects the cells that produce mucus, sweat and digestive juices. While these fluids are slick and slippery in healthy individuals, in those with cystic fibrosis they become thick and sticky. This “thickening” occurs due to a defective gene and leads to extremely challenging medical conditions.
Because CF alters the fluids the body needs to lubricate, instead of doing their jobs the fluids turn into serious problems. The thicker mucus begins to plug up tubes and ducts and passageways in key organs like the lungs. The symptoms of CF include a persistent cough that produces thick mucus, wheezing, activity intolerance, repeated lung infections, inflamed nasal passages, and recurrent sinusitis.
Cystic Fibrosis and the Pancreas
One of the organs the cystic fibrosis can impact is the pancreas. When this happens, the thickened and sticky mucus caused by CF begins to block the pancreatic duct. These blockages prevent pancreatic enzymes from reaching the intestines, which leads to scarring or “fibrosis” of the pancreas. Once the pancreas is scarred, it becomes far more difficult for the organ to produce and secrete insulin, the hormone the body’s cells require to transform blood sugar into energy.
Cystic Fibrosis and Diabetes
Diminished insulin production, caused by damage to the pancreas resulting from cystic fibrosis, means that the body can no longer process blood sugar properly. The result is a gradual build of glucose in the blood, which eventually leads to a diabetes diagnosis – what’s called cystic fibrosis-related diabetes or CFRD.
CFRD is not Type 1 diabetes, nor is it Type 2 diabetes. Though the signs and symptoms are similar, the disease itself is medically different. For instance, like those with Type 1 diabetes, those with CFRD don’t necessarily have any contributing risk factors to developing the disease. For those with Type 1 diabetes, it is an autoimmune disorder that leads to the condition. For those with CFRD, it is cystic fibrosis. In both cases, nothing could have been done to prevent the onset of diabetes.
CFRD resembles Type 2 diabetes only in the sense that in both cases the pancreas may still produce some insulin. However, Type 2 diabetes is usually brought on by a combination of genetic and lifestyle factors such as family history, age, obesity, diet, and a lack of physical activity, whereas CFRD is solely caused by damage to the pancreas because of the disease.
How Common is CFRD?
According to the Cystic Fibrosis Foundation, CFRD is the most common comorbidity in cases of cystic fibrosis, occurring in up to 20% of adolescents and up to 50% of adults with CF.
Symptoms of CFRD?
The symptoms of cystic fibrosis-related diabetes are very similar to those experienced by individuals with Type 1 and Type 2 diabetes. They include:
– Frequent urination
– Feelings of exhaustion
– Unexplained weight loss
– Increased thirst
Those with CFRD are also likely to experience an unexplained decline in lung function, which is not in line with the symptoms of Type 1 and Type 2 diabetes. This is a result of CFRD exacerbating the conditions associated with cystic fibrosis.
Screening for CFRD
CF-related diabetes is diagnosed through an oral glucose tolerance test (OGTT) given by a physician. The test is usually performed in the morning, after a person has fasted and has had nothing to drink for 8 to 12 hours. Blood samples are taken before and up to 2 hours after the patient drinks a specific amount of glucose liquid. The test after 2 hours measures the body’s response to sugar and can indicate a diabetes diagnosis (or not).
CFRD is a serious problem given the fact that cystic fibrosis, itself, is an extremely serious disease and anything that would exacerbate it further complicates the lives of patients. The earlier CFRD is detected, the better the outcomes. For this reason, cystic fibrosis care guidelines recommend testing for CFRD in people with CF ages 10 and older every year using an OGTT.
Treating and Managing CFRD
The primary goal for managing CFRD is the same as it is for managing any form of diabetes, and that’s keeping blood sugar at normal or near normal levels. Like those with Type 1 and Type 2 diabetes, those living with CFRD take insulin injections, usually by syringe or insulin pen, to keep blood sugar under control. This, of course, also means testing blood sugar regularly (particularly as the disease progresses) using a glucose meter and test strips, or, perhaps a continuous glucose monitoring (CGM) system, such as the Dexcom G7 or FreeStyle Libre 3.
One of the big differences between Type 2 diabetes and CFRD is the need for a high calorie diet. Because Type 2 diabetes is often associated with obesity and inactivity, losing weight can be a benefit because it reduces insulin resistance. However, those with CFRD have a different regimen. This is because CF can make it hard for a person to maintain a healthy weight – so those with CFRD are encouraged to eat a high-calorie, high-protein, and even high-fat and high-salt diet, to prevent unwanted weight loss. This is a very different protocol than with other forms of diabetes.
Ultimately, it is important for anyone with cystic-fibrosis and CFRD to work closely with their doctors, endocrinologists, and care teams to develop a diabetes management plan designed around their individual condition and needs.
If you or someone you love is currently living with cystic fibrosis and experiences any of the symptoms of CFRD, contact your physician right away. The sooner a diagnosis is made, the sooner treatment and effective management can begin.
We hope you found this post informative and insightful. At Diabetic Warehouse, we’re committed to helping those with diabetes control blood sugar and avoid diabetes-related complications with a complete selection of testing and treatment supplies at prices up to 65% less than those found at most pharmacies and suppliers.
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