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We were unable to enroll eligible subjects for the study.
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The purpose of this research study is to find out if the combined therapy lumacaftor-ivacaftor affects glycemia in patient with cystic fibrosis.
This is a single center, open label study. Patients will have 1 visits at the Diabetes Research Center (DRC), or Clinical Research Center (CRC).
The participants will have been previously screened to make sure they are candidates for the study. These patients will be contacted prior to their first visit to discuss enrollment in the study.
At the study visit the participant will come to the CRC or DRC for a research visit. The following will occur at this study visit: informed consent; brief medical history; weight and height; vital signs and blood pressure; blood draw for DNA extraction, A1c and an extra research tube for storage. This will be scheduled at a time that is convenient to the patient.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Lumacaftor-ivacaftor | Experimental | Subjects will be monitored for glycemic changes before and after starting lumacaftor-ivacaftor. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| lumacaftor-ivacaftor | Drug | Drug given for cystic fibrosis |
|
| Measure | Description | Time Frame |
|---|---|---|
| Change in glycated hemoglobin (hemoglobin A1C) | A blood test will be used to determine the hemoglobin A1c change while on the medication. | 1 year |
| Change in units of insulin used over a period of 6 months to 1 year. | Using chart review, the change in insulin units used per day will be calculated | 1 year |
| Measure | Description | Time Frame |
|---|---|---|
| Change in glycemia contingent on genetic risk score | The investigators will examine how change in glycemia is dependent on genotype at variants associated with type 2 diabetes and insulin secretion using genetic risk scores. | 1 year |
| Pulmonary function test (PFT) forced expiratory volume at one second (FEV1) measurements |
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Inclusion Criteria:
Exclusion Criteria
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| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 25981758 | Background | Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, De Boeck K, Flume PA, Konstan MW, McColley SA, McCoy K, McKone EF, Munck A, Ratjen F, Rowe SM, Waltz D, Boyle MP; TRAFFIC Study Group; TRANSPORT Study Group. Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med. 2015 Jul 16;373(3):220-31. doi: 10.1056/NEJMoa1409547. Epub 2015 May 17. | |
| 23952705 |
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| Type | Date | Date Unknown |
|---|---|---|
| Release | Jul 29, 2019 | |
| Reset | Sep 6, 2019 |
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| Release Date | Unrelease Date | Unrelease Date Unknown | Reset Date | MCP Release Number |
|---|---|---|---|---|
| Jul 29, 2019 | Sep 6, 2019 |
| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| D003920 | Diabetes Mellitus |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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| ID | Term |
|---|---|
| C000599212 | lumacaftor, ivacaftor drug combination |
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The investigators will compare how PFT measurement of FEV1 are related to changes in glycemia |
| 1 year |
| Background |
| Bellin MD, Laguna T, Leschyshyn J, Regelmann W, Dunitz J, Billings J, Moran A. Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: a small pilot study. Pediatr Diabetes. 2013 Sep;14(6):417-21. doi: 10.1111/pedi.12026. Epub 2013 Mar 13. |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |
| D044882 | Glucose Metabolism Disorders |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D004700 | Endocrine System Diseases |