Objectives: Our aim in this study was to identify challenges and gaps in Canadian practices in screening, diagnosis, and treatment of cystic fibrosis-related diabetes (CFRD), with the goal of informing a Canadian-specific guideline for CFRD.
Methods: We conducted an online survey of health-care professionals (97 physicians and 44 allied health professionals) who care for people living with CF (pwCF) and/or CFRD (pwCFRD).
Results: Most pediatric centres followed <10 pwCFRD and adult centres followed >10 pwCFRD. Children with CFRD are usually followed at a separate diabetes clinic, whereas adults with CFRD may be followed by respirologists, nurse practitioners, or endocrinologists in a CF clinic or in a separate diabetes clinic. Less than 25% of pwCF had access to an endocrinologist with a special interest or expertise in CFRD. Many centres perform screening oral glucose tolerance testing with fasting and 2-hour time points. Respondents, especially those working with adults, also indicate use of additional tests for screening not currently recommended in CFRD guidelines. Pediatric practitioners tend to only use insulin to manage CFRD, whereas adult practitioners are more likely to use repaglinide as an alternative to insulin.
Conclusions: Access to specialized CFRD care may be a challenge for pwCFRD in Canada. There appears to be wide heterogeneity of CFRD care organization, screening, and treatment among health-care providers caring for pwCF and/or pwCFRD across Canada. Practitioners working with adult pwCF are less likely to adhere to current clinical practice guidelines than practitioners working with children.
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http://dx.doi.org/10.1016/j.jcjd.2023.02.002 | DOI Listing |
BMJ Open
December 2024
Division of Endocrinology, Metabolism, and Lipids; Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
Introduction: People with cystic fibrosis (PwCF) are at high risk for developing cystic fibrosis (CF)-related diabetes (CFRD), which worsens morbidity and mortality. Although the pathological events leading to the development of CFRD are complex and not completely understood, dietary factors may play a role. For example, habitual intake of dietary added sugar (i.
View Article and Find Full Text PDFIslets
December 2024
Center for Diabetes and Metabolic Diseases, Indiana University School of Medicine, Indianapolis, IN, USA.
Background: Knockout (KO) ferrets with the cystic fibrosis transmembrane conductance regulator (CFTR) exhibit distinct phases of dysglycemia and pancreatic remodeling prior to cystic fibrosis-related diabetes (CFRD) development. Following normoglycemia during the first month of life (Phase l), hyperglycemia occurs during the subsequent 2 months (Phase Il) with decreased islet mass, followed by a period of near normoglycemia (Phase Ill) in which the islets regenerate. We aimed to characterize islet hormone expression patterns across these Phases.
View Article and Find Full Text PDFPLoS One
December 2024
Department of Medicine, National Jewish Health, Denver, CO, United States of America.
Background: The duration of time a person with cystic fibrosis (pwCF) spends on the lung transplant waitlist is dependent on waitlist and post-transplant survival probabilities and can extend up to 2 years. Understanding the characteristics involved with lung transplant and survival prognoses may help guide decision making by the patient, the referring CF Center and the transplant team.
Methods: This study seeks to identify clinical predictors of lung transplant and survival of individuals with CF using 29,847 subjects from 2003-2014 entered in the Cystic Fibrosis Foundation Patient Registry (CFFPR).
Horm Res Paediatr
November 2024
Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins School of Medicine, Baltimore, Maryland, USA.
Introduction: Cystic fibrosis-related diabetes (CFRD) is linked to worsening pulmonary function and increased mortality in people with cystic fibrosis (CF). Because of this correlation, early recognition of CFRD is important. Current recommendations for detecting glucose abnormalities using an oral glucose tolerance test (OGTT) can be difficult to achieve in a busy clinical setting.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
November 2024
Cystic Fibrosis Center Copenhagen, Department of Infectious Diseases, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark.
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