1. CFRD GUIDELINES UPDATE Dr Nigel Paterson, respirologist Tracy Gooyers, nurse case manager Pat Leggatt, dietitian

2. Overview  Introduction  Epidemiology  Screening  Diagnosis  Management  Complications

3. Epidemiology  Prevalence  40-50% of adults with CF  20% of adolescents with CF  Rare in childhood  In London CF clinic  18/80 (23%) CFRD  14/80 (18%) IGT  Untested/infrequently tested (~5%)  Some intermittent IGT

4. Introduction – CF Related Diabetes  A distinct clinical entity Neither Type 1 nor Type 2 Diabetes Risk for microvascular, not macrovascular disease Significant impact on pulmonary function  Shares features of both T1DM/T2DM Insulin resistance Insulin insufficiency  Variants CFRD with Fasting Hyperglycemia (+FH) CFRD without Fasting Hyperglycemia (-FH)

5. Screening  Significance  Often clinically silent  Nutritional and pulmonary consequences Weight loss Protein catabolism Lung function decline Mortality

6. Screening  General Recommendations × HbA1C(insensitive, poor +ve predictive value) × CGM(intermittent high BG non-diagnostic) × FPG(fails to detect CFRD –FH) × SMBG(devices are inaccurate) √ OGTT(recommended method)

7. Screening  Annual screening  Annual screening in asymptomatic patients  2h 75g OGTT  After 10 years of age in all pt with CF  Other indications  Acute pulmonary illness requiring IV antibiotics or glucocorticoids  Continuous enteral feeding  Pregnancy  Transplant patients

8. Recent Admission  2 week admission  1 st week daytime BG 3 – 5s Before evening tube feeds 2 – 4s During feeds 4 – 7 (one 12.1)  OGTT mid stay 5.0 & 12.6 mmol/L  2 nd week daytime 3 – 7s Once established on tube feeds (2500 – 3000 kcal in evening) 9.8 prior to feed 7 – 13s mid feed ( one 5.8) 4 – 10 post feeds

9. Screening - Inpatients  Significance  8 pt admitted with pulmonary exacerbation  Normal random FPG  2 hr IVGTT 14.5 mmol/L (intermittent glucose intolerance)  Baseline FEV1 predictor of poor glucose tolerance J Cyst Fibr May; 9(3): 199-204  Look for hyperglycemia in unwell CF patients!

10. Diagnosis  Based on standard ADA criteria  2h 75g OGTT > 11.1mmol/L  FPG > 7.0mmol/L  *HbA1C > 6.5%  Casual BG > 11.1mmol/L + symptoms of hyperglycemia  Insufficient data for lowering these thresholds in CF

11. Acute Pulmonary Exacerbation  Acute illness requiring IV antibiotics or systemic glucocorticoids can lead to hyperglycemia  Screening  First 48hr  Fasting and 2hr post-prandial blood glucose  Diagnosis  Diagnostic criteria persist > 48hr  Correlated with risk for microvascular disease

12. Pregnancy  Pregnancy is a state of insulin resistance  Screening  Women planning pregnancy or are pregnant  For GDM: 12-16 weeks and 24-28 weeks  For CFRD: 6-12 weeks post-partum if GDM  Diagnosis of GDM by 75g OGTT  FPG > 5.1mmol/L  1hr > 10.0mmol/L  2hr > 8.5mmol/L

13. Enteral Feeding  Screening  Mid and immediate post-feeding plasma glucose  At initiation of gastrostomy feeding  Monthly by SMBG (Confirm by laboratory)  Diagnosis  If criteria are met on two separate days

14. Transplant Patients  For CF transplant patients  Universal need for insulin peri-operatively  Many need insulin long-term  Screening  Pre-operatively by OGTT

15. Management  Care team  Regular meetings  Multidisciplinary approach  Experts in CF and diabetes  Education on self-management  Communication between providers

16. Management – CF Diet Doesn’t blend well with group DM classes Carb counting: insulin best approach for many No significant change to CF diet High Calorie, High Protein, High Fat, Liberal Salt intake Variable intake with focus on eating as much as wishes Adjust simple carbohydrates through day or with food Some have developed poor eating behaviors Artifical sweeteners not usually recommended Use of supplements (Scandishake, Ensure Plus e.g) as indicated by BG

17. Management - Diet  CF Diet  2500 - 7000 kcal/d  Up to 7 – 9 carb choices per meal, 3 or more for snacks  For those using pancreatic enzymes assume a malabsorption factor about 10 – 15%

18. Management  Pharmacological  Individualized insulin therapy FH positive - basal and MDI or pump FH negative – MDI Effects of adding basal are unknown  Oral agents less effective than insulin

19. Management  Goals same as other diabetes patients  A1C < 7%  FPG/preprandial PG4.0 – 7.0 mmol/L  2hr postprandial PG5.0 – 10.0 mmol/L 5.0 – 8.0 if A1C > 7%  Monitoring  SMBG 3x daily if on insulin  Quarterly A1C  Exercise encouraged

20. Complications  Acute  Hypoglycemia  Rare: DKA, hyperosmolar hypoglycemia  Chronic  Microvascular – more common in FH positive screening  Pulmonary  Gastroparesis

21. Complications  Screening  Annual screening Spot Albumin:Creatinine Ratio (ACR) Dilated eye exam Neurologic assessment and foot exam Regular BP measurements Lipid profile if sufficient exocrine pancreas or risk factors for cardiovascular disease  Begins 5 years after date of onset if known  Otherwise, immediately upon first diagnosis