About this trial
Diabetes is a major co-morbidity in pancreatic insufficient cystic fibrosis (PI-CF) and associated with worse outcomes. While reduced β-cell mass contributes to the insulin secretory defects that characterizes cystic fibrosis-related diabetes (CFRD), other modifiable determinants appear operative in the emergence and progression of abnormal glucose tolerance towards diabetes. Identifying interventions to preserve β-cell function are crucial for delaying and potentially preventing CFRD development. In this study, we hypothesize that weekly administration of the long-acting glucagon-like peptide-1 (GLP-1) agonist dulaglutide will improve defective early-phase insulin secretion and improve glucose tolerance during a mixed-meal tolerance test.
Eligibility criteria
Qualifiers
1. Male or female, aged ≥18 years on date of consent
2. Confirmed diagnosis of CF, defined by positive sweat test or Cystic Fibrosis transmembrane conductance regulator (CFTR) mutation analysis according to Cystic Fibrosis Foundation (CFF) diagnostic criteria.
3. Pancreatic insufficiency defined by clinical requirement for pancreatic enzyme replacement.
4. Abnormal glucose tolerance defined by OGTT criteria for EGI, IGT, or CFRD, or diagnosed CFRD.
Disqualifiers
1. BMI <19 kg/m2
2. Presence of first-degree atrioventricular block or other evidence for cardiac conduction system or structural heart defects
3. Pregnancy or lactation; a negative urine pregnancy test will be required at enrollment
4. Known allergic reactions to any GLP-1 agonist, and any history of severe hypersensitivity reactions (anaphylaxis or angioedema)
Trial design
Treatments tested in this trial
- Dulaglutide 0.75Mg/0.5Ml Inj Pen
Treatment groups
Sponsors and collaborators
University of Pennsylvania
Lead sponsor
Children's Hospital of Philadelphia
Collaborator
Children's Hospital Colorado
Collaborator