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
- Antoinette Moran, MD1,
- Carol Brunzell, RD, LD, CDE1,
- Richard C. Cohen, MD2,
- Marcia Katz, MD3,
- Bruce C. Marshall, MD4,
- Gary Onady, MD, PHD5,
- Karen A. Robinson, PHD6,
- Kathryn A. Sabadosa, MPH7,
- Arlene Stecenko, MD8,
- Bonnie Slovis, MD9 and
- the CFRD Guidelines Committee
- 1Division of Pediatric Endocrinology, University of Minnesota, Minneapolis, Minnesota;
- 2Kaiser Permanente, Portland, Oregon;
- 3Department of Medicine, Baylor College of Medicine, Houston, Texas;
- 4Cystic Fibrosis Foundation, Bethesda, Maryland;
- 5Wright State University Boonshoft School of Medicine, Dayton, Ohio;
- 6Department of Medicine, Johns Hopkins University, Baltimore, Maryland;
- 7Department of Community and Family Medicine, The Dartmouth Institute for Health Policy and Clinical Practice, Lebanon, New Hampshire;
- 8Department of Medicine, Emory University, Atlanta, Georgia;
- 9Department of Medicine, Vanderbilt University, Nashville, Tennessee.
- Corresponding author: Antoinette Moran, .
Cystic fibrosis–related diabetes (CFRD) is the most common comorbidity in people with cystic fibrosis (CF), occurring in ∼20% of adolescents and 40–50% of adults (1). While it shares features of type 1 and type 2 diabetes, CFRD is a distinct clinical entity. It is primarily caused by insulin insufficiency, although fluctuating levels of insulin resistance related to acute and chronic illness also play a role. The additional diagnosis of CFRD has a negative impact on pulmonary function and survival in CF, and this risk disproportionately affects women (2–4). In contrast to patients with other types of diabetes, there are no documented cases of death from atherosclerotic vascular disease in patients with CFRD, despite the fact that some now live into their sixth and seventh decades.
These guidelines are the result of a joint effort between the Cystic Fibrosis Foundation (CFF), the American Diabetes Association (ADA), and the Pediatric Endocrine Society (PES). They are intended for use by CF patients, their care partners, and health care professionals and include recommendations for screening, diagnosis, and medical management of CFRD. This report focuses on aspects of care unique to CFRD. A comprehensive summary of recommendations for all people with diabetes can be found in the ADA Standards of Medical Care, published annually in the January supplement to Diabetes Care (5).
In 2009, CFF in collaboration with ADA and PES convened a committee of CF and diabetes experts to update clinical care guidelines for CFRD. Investigators at Johns Hopkins University conducted evidence reviews on relevant clinical questions identified by the guidelines committee. The reviews were provided to the committee to use in developing recommendations. Where possible, the evidence for each recommendation was considered and graded by the committee using the ADA (5) and the U.S. Preventive Services Task Force (USPSTF) (6 …