INTRODUCTION:Insulin remains the only recommended medical treatment for cystic fibrosis related diabetes (CFRD) Whilst there is an established role for orally bioavailable incretin mimetic agents such as the dipeptidyl peptidase-4 inhibitors (DPP4-I) in Type 2 diabetes mellitus, there exists little data on their utility in CFRD. AIM:To examine the use of DPP4-I therapy in patients with CFRD at a single large adult cystic fibrosis center. METHOD:People with CFRD prescribed a DPP4-I were identified from our specialist CFRD clinic and records were retrospectively examined for indication for therapy, tolerability and effectiveness. Analysis of continuous glucose monitoring data Libre 2 was done for these patients (CGM) pre and at least 3 months post therapy was performed. RESULTS:23 people with CF (PwCF) with a mean (SD) age of 35.0 ± 2.4 years were included in this analysis . In 21 patients DPP4-I was prescribed as a monotherapy and it was given in combination with insulin in 2 others. Indications for therapy included reactive hypoglycaemia (n = 10) post prandial hyperglycaemia (8), insulin avoidance (3), metformin intolerance (1) and unclear (1). Therapy was well tolerated with no discontinuations due to adverse effects. Significant improvements were noted in Time in Range- Pre vs Post: 78.0 [67.5 - 84.0] vs 89.0 [79.8 - 96.0]%, p = 0.005, Time above Range -Pre vs Post: 19.5 [12.5 - 30.8] vs 6.0 [2.5 - 16.5]%, p = 0.006 and glucose variability Pre versus Post: 28.3 [25.4 - 31.1] vs 26.9 [23.1 - 31.3], p = 0.021, Of the 10 subjects who initiated therapy for hypoglycaemia, 7 reported an improvement in symptoms. No significant difference was found in weight pre and post: 61.5 ± 15.0 kg vs 62.5 ± 15.3 kg, p = 0.326 or Hba1c pre vs post: 41.0 [36.0 - 53.3] mmol/mol versus 40.5 [36.8 - 47.3], p = 0.727. CONCLUSION:DPP4-I is well tolerated in CFRD and can lead to an improved glycaemic control in these patients with significant improvement in validated CGM metrics.

Introduction: Insulin remains the only recommended medical treatment for CFRD with little data on the clinical use of incretin mimetic agents such as the dipeptidyl peptidase-4 inhibitors ((DPP4-I) in CFRD despite recent paradigm shift in CF with modulator therapy.

Oral glucose tolerance testing (OGTT) is the recommended screening for cystic fibrosis-related diabetes (CFRD). Increasing evidence for benefit of early detection of CFRD has led to utilisation of continuous glucose monitoring (CGM). One-hour plasma glucose (1-h PG) during the oral glucose tolerance test (OGTT) is known to be an accurate predictor of type 2 diabetes.

Facilitating education and supporting behaviour change in patients with Cystic Fibrosis Related Diabetes (PwCFRD) in traditional CF multidisciplinary clinics can be challenging due to their lengthy and busy nature. To combat this, a stand-alone CFRD virtual clinic was pioneered, delivering bespoke education for PwCFRD who required specialist support, and for those with a new diagnosis of CFRD. Education was delivered by the specialist CFRD team alongside new resources developed specific for CFRD.

Patients with CFRD are typically asymptomatic for many years and annual glycemic screening by 10 years of age is recommended.We aimed to review the current status of CFRD in the Bulgarian population and to compare the data of those patients to our other CF patients.From a total of 210 patients with confirmed CF (sweat test and genetic mutation), 16 have CFRD.It should be noted that 3 of them developed diabetes after being lung transplanted.The mean age of the patients with CFRD is 24.21 yrs (from 7 to 41), while the other 194 CF patients mean age is 16.59 (but it should be taken in account that in the first group there are no children under age of 7).The gender prevalence in CFRD group is male: female 6:10, while in the total population is in reverse with prevalence of males 113:81.As expected CFRD group shows lower FEV 1 53.1% ± 20.52 vs. 70.54%± 24.3 for non-CFRD patients.The same trend is noted for z-score of BMI with better results for non-CFRD -0.95 ± 1.5 vs. -1.68 ± 2.3.All CFRD patients are diagnosed early in life (mean age of diagnosis 1.01) while diabetes diagnosis was done around late teens (mean age 19.85 yrs).Only one child was diagnosed by a routine laboratory check at the age of 7 years.All but one patient with CFRD are with chronic Pseudomonas aeruginosa, and only two were on constant inhaled corticosteroids.None of them had oral corticosteroids in the therapy.Although we still have less than 10% CFRD prevalence in our population, with improved medical care and initiation of new CF therapies, it is crucial to identify CFRD as early as possible to prevent possible complications of non-treated diabetes.Since our youngest patient is under the age of the recommend screening we advocate for lowering this age, especially in patientd with chronic Pseudomonas aeruginosa and symptoms of the disease in the first year of life.

during the year from 6,1 ± 1,9 to 3,6 ± 1,1 ( p < 0,001), while in group2 no significant change was seen.

Background In our large adult CF centre, we adhere to national guidelines on the management of cystic fibrosis (CF) but have noticed that even the best ‘standard CF care’ does not suit all patients. We have a cohort of patients that deteriorate rapidly despite receiving excellent advice from the CF MDT. These patients are typically non-adherent to medications and physiotherapy, have low BMIs, frequently exacerbate, rely on intravenous antibiotics and have accelerated lung function decline. Furthermore, these patients often have psychological difficulties and struggle to communicate optimally with the MDT. Objectives We aimed to develop an outpatient based service to provide intensive multidisciplinary support to these patients with key objectives being to improve: communication and engagement with the CF MDT; adherence; nutrition; lung function and to give enhanced psychosocial support. Methods The CF BOOST (Cystic Fibrosis Better Outpatient Outcome Support Team) service was developed consisting of 1 consultant, 2 specialist nurses, 2 physiotherapists, 1 dietician and the psychosocial team. Each enrolled patient receives intensive home support using a combination of phone calls, texts, emails and home visits. Weekly MDT meetings are held to discuss progress, problems and patient feedback and priorities. A summary of discussions and proposed individualised action plan is immediately discussed with the patient. Results 7 patients are now enrolled in the service. The first patient to enrol has now completed 12 months of CF BOOST support. This patient is now taking all oral medication and regular nebulised antibiotics for the first time in their life and has had a sustained 10% (absolute) increase in FEV1. BMI has risen from 16.2 to 21.8 (without enteral feeding or supplements), intravenous antibiotic days have halved, anaemia and hypoalbuminaemia have resolved and mean CRP is the lowest it has been in a decade. All 6 more recently enrolled patients are also showing favourable outcomes e.g. improved lung function, BMI, adherence and engagement and decreased intravenous antibiotic frequency. The use of text messaging has hugely improved all patients’ engagement and communication. Conclusions Using an alternative approach with rapidly deteriorating, non-adherent, disengaged patients can result in significant improvements in patient outcome and satisfaction.

Results: 31 patients with CFRD attended an annual review in 2016.Median age was 31 years (range 18-53).23 were pre-lung transplant and 8 postlung transplant.Median FEV 1 %-predicted in the pre-transplant group was 53% (30-111) and median BMI 20.6 kg/m 2 (15.7-32.7)compared with 80% (54-103) and 23 kg/m 2 (17.3-28.0) in the post-transplant patients.Mean total daily dose of insulin was 37 units (SD 27.4).Median HbA1c was 48mmol/l (28-86).There was a positive correlation between TDD of insulin and HbA1c (r = 0.61).There was no significant correlation between TDD and BMI or FEV 1 .Mean (SD) TDD in patients treated with steroids in the preceding year was 36.4 (28.9) units compared with 33.3 (24.8) units in patients not receiving steroids ( p = 0.78).Duration of insulin therapy was categorised as <1 year (n = 2), 1-5 years (n = 9), 5-10 years (n = 10) or >10 years (n = 10).Median (range) TDD was 10.5 (4-17) units in the <1 year group; 24 (5-84) for 1-5 years; 19 (1-76) for 5-10 years and 51.5 (6-109) for >10 years.Conclusion: TDD of insulin was positively correlated with HbA1c and increased in patients with longer duration of insulin therapy.Larger longitudinal studies are needed to determine the long-term significance of TDD and its correlation with clinically significant CF morbidity.