Metabolic Efficiency of Combined Pancreatic Islet and Lung Transplant for the Treatment of End-Stage Cystic Fibrosis (PIM)

This is an interventional treatment trial for Cystic Fibrosis focused on measuring Cystic Fibrosis, Diabetes Related Cystic Fibrosis, Lung transplantation, Pancreatic islets, Insulin-dependent diabetes Read More

Inclusion criteria:

• Patient with cystic fibrosis
• Patient able to respect the protocol procedures
• Patient with end-stage respiratory insufficiency indicating a lung transplant
• Clinical history of cystic fibrosis related diabetes and/or insulin-dependent diabetes, with no residual insulin secretion (C-peptide < 0,5 ng/mL) and/or no response to IV glucagon stimulation: [peak stimulated C-peptide (T6min)/basal plasma C-peptide(T0)] < 2. The absence of insulin secretion will be verified 2 times before inclusion
• Evolution of diabetes for over 3 years
• Patient whose glycaemic control obtained with insulin therapy is not satisfactory and could significantly alter quality of life (HbA1c > 7% and/or MAGE index > 1,25). This situation is assessed by a diabetologist.
• Social Security membership or benefit from Social Welfare
• Patient who received the results of the medical evaluation required

Non-inclusion criteria:

• Patient with contra-indication for undergo a lung transplant
• Patient with an indication of heart, liver or kidney transplantation
• Patient for which poor therapeutic compliance is expected
• Patient under oral antidiabetic drug
• In women of childbearing potential: pregnancy test (urine and / or blood) positive, lactation, or unwilling to use effective contraception for the duration of the study until 3 months after the end . Men: procreation project during the study period up to 3 months after its end, or unwilling to use effective contraception patient.
• Active infection, including hepatitis B, hepatitis C, HIV
• Any history of malignancy within the past 5 years, with the exception of cutaneous basal cell carcinomas completely resected. The history of breast or melanoma cancers are an absolute contraindication
• Alcoholic intoxication or drug addiction
• Anemia (hemoglobin Hb <10g / dL in women and Hb <11 g / dL in men), lymphopenia (<1000 / uL), neutropenia (<1500 / uL) or thrombocytopenia (<100,000 / uL).
• Persistent elevated liver enzymes at baseline
• Portal hypertension identified by oesophageal varice and/or hypersplenism (platelets < 120 000 /mm3) or Child-Pugh score > 6.
• Use of a medical treatment under investigation within 4 weeks before inclusion
• All medical situation assessed by an investigator which could interfere with the good management of the project
• Patient restricted of freedom or unable to give his consent
• Patient has been included in another study that could interfere with the results of the study
• Contraindications to the use of experimental drugs (Cellcept®, prednisone, Prograf, prednisolone, methylprednisolone, and pancreatic islets)