Hemoglobin A1c levels may appear low or even low-normal in patients with cystic fibrosis, but this test will miss symptoms of impaired glucose tolerance in this disease. Although the test is not an accurate predictor of diabetes in patients with CF, it can measure treatment response. Nevertheless, a high level of suspicion should be maintained for any diabetes involving cystic fibrosis.
Common complications of cystic fibrosis related diabetes
Patients with CF are often at a higher risk of developing a form of cystic fibrosis related diabetes (CFRD). This disease primarily results in accelerated decline of lung function and a greater likelihood of acute pulmonary infectious exacerbations. Lung transplants are often associated with an increased risk of CFRD and diabetes. Patients with CFRD have lower FEV1 in general and the proportion of CF patients with low FEV1 is greater in men than in women. Although the degree of lung function loss associated with CFRD is unclear, estimates range from 0.85 to 2.1% of predicted FEV1 per year.
CFRD is a comorbid condition that affects forty to fifty percent of adults with cystic fibrosis. Although common, it is often difficult to predict the impact of CF-related diabetes on a patient’s prognosis. While CF-related diabetes varies in severity, it is associated with increased morbidity and mortality. The early detection of CFRD is critical for allowing treatment to begin as early as possible.
CF-related diabetes is distinct from type 1 and type 2 diabetes and has many features common to both. The main cause is relative insulin deficiency associated with the destruction of pancreatic islets. Other factors, such as insulin resistance, may also contribute to the development of this disease. It may lead to an acute exacerbation of CF and chronic progression of the disease. In the meantime, treatment for CF-related diabetes should focus on prevention and management of the condition.
Diagnosis of CFRD is difficult, as it typically occurs in childhood, during the adolescent phase of development when children are still learning to cope with chronic conditions. However, the disease can interfere with the patient’s autonomy and participation in routine CF care. If properly managed, cystic fibrosis-related diabetes can improve a patient’s quality of life and encourage participation in routine CF care.
In addition to CF-related diabetes, people with CF often have problems digesting food. They often need a high-calorie diet and frequent snacks. Diabetes is associated with high blood glucose levels, which can lead to weight loss, increased chest infections, and deterioration of lung function. If left undiagnosed, the condition can cause complications in the eyes, feet, and heart. Continuous glucose monitoring and an oral glucose tolerance test may be used to detect CF-related diabetes.
In patients with CF and CFRD, the most common type of treatment is insulin. Studies have shown that insulin therapy has improved glycemic control, increased weight gain, and improved pulmonary function. People with CFRD should start insulin therapy while still on a similar caloric intake as they did before the diagnosis. They should begin insulin therapy if they are at risk of fasting hyperglycemia.
Treatment options for cystic fibrosis-related diabetes
Insulin therapy is the preferred method for treating cystic fibrosis-related diabetes. Insulin therapy has been associated with improved glycemic control, weight gain, and pulmonary function in patients with CFRD. Patients should maintain the same caloric intake as before diagnosis. Once diagnosed, insulin therapy should be initiated as soon as possible. Rapid-acting insulin can be used for meals and premeal doses should be calculated according to insulin-to-carbohydrate ratios.
Other treatment options are available. Insulin-replacing tablets are a promising alternative to insulin injections. Researchers are continuing to study the effects of CFRD on lung function and disease severity. Further, studies are needed to examine the effectiveness of these new therapies, and to assess the degree of patient compliance. Further, anti-inflammatory tablets may be used in conjunction with insulin. But more research is needed to determine the optimal treatment regimen for patients with CFRD.
People with cystic fibrosis should visit their healthcare team immediately if they notice any of these symptoms. Symptoms of high blood sugar include frequent chest infections, worsened lung function, and high blood pressure. Moreover, diabetes can cause complications in the eyes, feet, and heart. Diagnosis of cystic fibrosis-related diabetes can be done through continuous glucose monitoring and oral glucose tolerance tests.
For the management of CFRD, a patient should follow a healthy diet and exercise program. Regular exercise helps improve lung function and the response of the body to insulin. People with CFRD should closely monitor blood glucose levels and keep themselves active. They should also monitor their blood glucose levels and follow a high-calorie, high-protein, and fat diet. Taking insulin regularly can help prevent hypoglycemia and other complications of diabetes.
Treatment options for cystic fibrosis patients include special diets. The body cannot absorb certain nutrients from food, which makes them malnourished. To help the patient absorb more nutrients from food, they may be fed through a feeding tube. These feeding tubes are either temporary or surgically implanted in the abdomen. This provides them with extra calories throughout the day. A feeding tube is a convenient way for cystic fibrosis patients to eat.
A variety of oral medications are available. These include metformin and sulfonylureas. However, oral insulin sensitizing agents with anti-inflammatory activity may provide duel benefits for patients with CF and diabetes. The Cystic Fibrosis Foundation and the hospital and university institutional review boards approved the study protocol. Patients elected the treatment after receiving informed consent. The study protocol included a list of potential side effects, including hepatic toxicity and metabolic acidosis.
As the CF population ages, insulin sensitivity decreases, and patients should adjust their insulin dosage accordingly. For adults, insulin dosing should be 0.5 to 0.8 units per kilogram of body weight daily. Children and adolescents may need less insulin than adults, but do not require any special diet. A close partnership between the patient and his doctor will ensure optimal care. It is important to stress the importance of monitoring glucose levels regularly.
Diagnosis of cystic fibrosis-related diabetes
Despite a long history of improvements in the treatment and survival of cystic fibrosis patients, the disease still remains a serious complication. The progression of the disease, and the presence of diabetes, is a strong predictor of worse clinical outcomes. The prevalence of cystic fibrosis related diabetes (CFRD) increases with age. Over half of CF patients develop the disease at some point. Fortunately, early diagnosis is associated with increased body weight, reduced pulmonary exacerbations, and improved overall survival.
The symptoms of CF-related diabetes are similar to those of Type 1 and Type 2 diabetes. The primary cause of CFRD is relative insulin deficiency due to the destruction of pancreatic islets. Additionally, insulin resistance may play a role. The disease may also be associated with uncontrolled CF lung function, acute illness, pregnancy, or steroid medications. In addition to these symptoms, a person with cystic fibrosis may experience excessive fatigue, weight loss, and unexplained decline in lung function.
Treatment of CFRD should aim to maintain a normal blood glucose level, while providing adequate nutrition and weight maintenance. In addition, insulin is usually given to patients with cystic fibrosis, as this treatment has been associated with better glycemic control, weight gain, and improved pulmonary function. However, if an individual is unable to take insulin, they may receive oral hypoglycemics. Diagnosis of cystic fibrosis related diabetes becomes more complicated once corticosteroids are prescribed to control symptoms of the disease.
A diagnosis of CF-related diabetes is challenging, particularly in children and adolescents. Often, the disease develops during the adolescent years, when children are developing independence and learning to deal with chronic illness. Despite the challenges of managing CF-related diabetes, some patients are able to integrate the disease into routine CF care. When this is done, the patient may be more likely to engage in routine CF care and develop healthy habits.
The deterioration of pulmonary function and nutrition in patients with CF is often insidious, occurring a couple of years before the diagnosis of cystic fibrosis-related diabetes (CFRD). The incidence of this disease is higher than in the general CF population, and it correlates with the degree of insulin deficiency at baseline. Recently, a study in the UK confirmed that CF patients with diabetes also had worse lung function and higher BMI than those without the disease.
The authors of a consensus conference report on the diagnosis and management of cystic fibrosis-related diabetes reviewed the evidence for screening for the disease. Fasting plasma glucose concentrations and two-hour plasma glucose concentrations were found to be diagnostic methods for diabetes. The study also included multiple choice questions regarding the importance of collaboration in the management of cystic fibrosis-related diabetes. Although these tests can be helpful in assessing symptoms and managing cystic fibrosis-related diabetes, they should not be used to determine the cause of the disease.
