1. The Evolution of the Canadian Cystic Fibrosis Registry Apps for Health Mohawk College April 28, 2016 Anne Stephenson MD, PhD Respirologist, Adult CF Program, St. Michael’s Hospital Director, Canadian CF Registry

2. OUTLINE Overview of cystic fibrosis (CF) Canadian CF Registry –History –Summary data –How the CF Registry can be used –Limitations and goals

3. HISTORY OF CYSTIC FIBROSIS 14th century folklore: “Woe is the child who tastes salty from a kiss on the brow for he is hexed, and soon must die.” 1938: Dr Andersen first describes cystic fibrosis as a disease of the pancreas

4. 1989: Discovery of CF gene by Tsui & Riordan at Sick Kids Cystic fibrosis breakthroughs…a Canadian success story CFTR gene cloned Most common CF mutation identified (delta F508)

5. MULTI-SYSTEM DISEASE Cystic fibrosis Multi-organ disease Respiratory system most common cause of morbidity The majority of deaths are due to progressive lung damage

6. Specialized CF Centres 42 accredited CF centres in Canada –Pediatric and adult clinics Multidisciplinary CF team –MD, RN, PT, RT, SW, Pharm, admin Multisystem care Respirology, GI, Endocrine, Obstetrics, Rheum, Psychiatry Long term, primary care

7. HISTORY OF THE CYSTIC FIBROSIS REGISTRY 1957: Dr. Leroy Matthews, Cleveland –Mortality at CF clinic at Babies and Children’s Hospital was less than 2% –National mortality at the time was ~20% 1964: US CFF gave $10,000 to Dr. Warren Warwick to collect stats from 31 CF centres –Median survival was 7 x higher than any other clinic in the US!

8. CANADIAN CF REGISTRY Median survival 19 yrs in US and 23 yrs in Canada

9. CANADIAN CF REGISTRY > 7,000 individuals with CF in the registry > 4,000 subjects are followed annually Annually collects important clinical variables: –Pulmonary function (e.g., FEV 1 ) –Nutritional status –Microbiology –Complications: diabetes, transplant, etc.

10. WEB- BASED APPLICATION

11. UNIQUE CLINIC DASHBOARD PAGE

12. Annual Registry report Epidemiologic trends Clinic-specific data on website Research Quality improvement initiatives USES OF CF REGISTRY DATA

16. September 2010 Powerpoint Presentation Fonts and Styles 16

17. Current median age of survival is 51.8 years

18. STRENGTHS AND LIMITATIONS OF CF REGISTRY Strengths Comprehensive, longitudinal data Low rate of lost to follow up; few duplicates Data relatively clean: rules for data entry Limitations Data only when patient comes to clinic Time consuming for clinic to enter data

19. FUTURE GOALS Increase patient engagement Fill in the missing gap when patients are not in clinic Develop a mobile app and/or wearable device –To capture data and help researchers obtain a complete picture of the health of CF patients Develop a patient portal –To provide feedback and to encourage patients to monitor and track their own health data

20. SUMMARY CF registry has comprehensive longitudinal data on all Canadians with CF Used to understand the CF population and track epidemiological trends over time Can be used to evaluate important CF outcomes

21. ACKNOWLEDGEMENTS Cystic Fibrosis Canada Dr. Denise Mak, Program Manager, Healthcare Jenna Sykes, Statistician Patients and Families living with CF CF Clinic staff Inovex Inc.