Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
As early cystic fibrosis-related diabetes (CFRD) may be clinically silent, these guidelines highlight the importance of regular screening. Management recommendations focus on insulin therapy and ongoing care by a team with knowledge of CF and diabetes.
Clinical Care Guidelines for Cystic Fibrosis-Related Diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33(12):2697-2708.
Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in cystic fibrosis and occurs in up to 20 percent of adolescents and 50 percent of adults with CF. A diagnosis of CFRD has a negative impact on lung function, nutrition, and survival. As early CFRD may be clinically silent, these guidelines highlight the importance of regular screening in various clinical contexts. A diagnosis of CFRD is made based on standard American Diabetes Association criteria, but may also be made after detection of intermittent hyperglycemia during illness or gastrostomy feedings. Management recommendations focus on insulin therapy and ongoing care provided by a multidisciplinary team with knowledge of CF and diabetes.
The latest Cystic Fibrosis-Related Diabetes (CFRD) Clinical Care Guidelines from 2010 summarize screening, diagnosis and management recommendations resulting from a joint collaboration of the Cystic Fibrosis Foundation, American Diabetes Association (ADA) and the Pediatric Endocrine Society. The report references the ADA Standards of Medical Care, published annually in Diabetes Care for all people with diabetes, but focuses on aspects unique to the care of CFRD. The process for developing these guidelines included an: expert committee, specific questions, systematic literature review, recommendations drafted, committee vote, final recommendations, and grade (US Preventative Task Force [USPSTF] and ADA Schemes). Find additional information about the USPSTF grading definitions.
Recommended test is a 2-hour 75g-Oral Glucose Tolerance Test (OGTT).
ADA - E
ADA - B
USPSTF - B
HbA1c as a screening test for CFRD is not recommended.
ADA - B
USPSTF - D
Self-monitoring blood glucose (SMBG) -- if elevated, confirm by central laboratory glucose measurements.
Gestational diabetes screening is recommended at both 12-16 weeks and 24-48 weeks gestation with a 2h 75g-OGTT and 0, 1, and 2h glucose measures.
During a period of stable baseline health, the diagnosis of CFRD can be made according to standard ADA criteria (below). Testing should be done on 2 separate days to confirm, unless there are unequivocal symptoms of hyperglycemia (polyuria, polydipsia) or symptoms and the presence of random glucoses ≥ 200 mg/dl.
A diagnosis of CFRD can be made in patients with acute illness (hospitalized on IV antibiotics or systemic glucocorticoids) when FPG ≥ 126 mg/dl or 2h post-prandial glucoses are ≥ 200 mg/dl and persist for more than 48 hours.
A diagnosis of CFRD can be made in patients on enteral continuous drip feedings when mid- or post-feeding glucose levels ≥ 200 mg/dl on 2 separate days.
A diagnosis of gestational diabetes should be made based on International Association of the Diabetes and Pregnancy Study Groups recommendations where diabetes is diagnosed based on 0-, 1-, and 2-hr glucose levels with a 75-g OGTT if any one of the following is present:
The onset of CFRD should be defined as the date a person first meets diagnostic criteria, even if hyperglycemia subsequently abates.
Patients should ideally be seen quarterly by a specialized multidisciplinary team with expertise in diabetes and CF.
Patients should receive ongoing diabetes self-management education from programs that meet national standards.
Patients should be treated with insulin therapy.
ADA - A
USPSTF - B
Oral diabetes agents are not as effective as insulin in improving nutritional and metabolic outcomes in CFRD and are not recommended outside the context of clinical research trials.
ADA - A
USPSTF - D
Patients on insulin should perform SMBG at least 3x daily.
Patients should strive to attain plasma glucose goals as per ADA recommendations for all people with diabetes.
HbA1c measurements are recommended quarterly.
HbA1c treatment goal is < 7%.
CF Foundation evidence-based guidelines for nutritional management are recommended.
Moderate aerobic exercise for at least 150 minutes per week is advised.
Monitoring for complications:
Since 2010, additional data have been published reporting higher rates of glucose abnormalities in children with CF aged younger than 10 years than previously reported. More recent insights into CFRD pathophysiology suggest a potential role of CFTR mutations in the beta cell, and these defects may contribute to early impairments in insulin secretion detectable in young children. Therefore, screening of high-risk children aged younger than 10 years may also be warranted.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Christine L. Chan, M.D. (University of Colorado) and Antoinette Moran, M.D. (University of Minnesota)
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