2. Brief History in USA & UK
Cystic Fibrosis Foundation, USA:
1955 – Foundation was established
1980 – Patient registry established
– became a national summary & anonymous
2001 – centres got their own data
2003 – open to centres & public (transparency)
Cystic Fibrosis Trust, UK:
2007 – UK joined PORT CF

3. Brief History in NZ Timeline:
2009 – PORT CF (big ups to CFNZ & CFF USA)
2009 – Standards of Care (minimum requirements)
IT housed in Christchurch
Christchurch Earthquake
CFA NZ support small centres to enter data
Annual review & backgrd demographic data

4. 48.3% adults
54% male / 46% female

5. PORT CF: Demographics 2014 USA UK AUST* NZ Total patients 28,676
10,583
3,235
443
Mean age
20.2 yrs
19 yrs
20 yrs
18.1 yrs
% adults
50.7%
57.9%*
49.9%
48.3%
Mean FEV1
76.2%
73.5%
85.1%
Predicted survival
39.3 yrs
40.1 yrs
???
Australia – 59% children have normal lung function, 15% males & 17% females as adults have normal lung function
* Australia registry is 2013
* 16yrs vs 18 yrs

7. Lung Function trends over time
North American Cystic Fibrosis Foundation
Patient Registry Annual Report 2012

8. 75% -80% are oral supplements with about 12% gasotrosmy feeds – similar across paeds & adults

11. < 16 yrs
n = 155
Number of individuals employing the technique at least once per year
No physiotherapy - in children across the years has increased from 2.3% to 3.9%.
No physiotherapy - in adults it is higher at 13.3% but has dropped over the years from 16.1%
Using exercise – 13.5% children & 35.5% adults

12. Number of individuals employing the technique at least once per year
No physiotherapy - in children across the years has increased from 2.3% to 3.9%.
No physiotherapy - in adults it is higher at 13.3% but has dropped over the years from 16.1%
Using exercise – 13.5% children & 35.5% adults
> 16 yrs
N = 203

13. PORT CF: Medication use
2014
USA UK
AUST* NZ
DNase
88.7%
54.5%
41.6%
26%
Macrolide
67.3%
52.5%
35.3%
25%
H/Saline
66.3%
26.1%
Neb Antibx
71.4% (T)
41.8% (Az)
88.6%
(49.8%)
12%
IHCS
43.8%
28%
Some variances here – UK say number with chronic PsA infection, USA has > 6 year age

14. PORT CF: Medication: Ivacaftor
CFF, USA Registry report:
Ivacaftor use in those with an eligible mutation: 80%

15. Survival In 2014 – current life expectancy - 39.3 years
CF Foundation Patient Registry Report, USA 2014

16. Median FEV1 in Paediatric Centres (children >6 years age)
UK CF Trust Registry
Each bar represents a different centre

17. Median FEV1 in Adult Centres
UK CF Trust Registry
Each bar represents a different centre

18. % with chronic Pseudomonas infection in Paediatric Centres
(children >6 years age)
UK CF Trust Registry
UK mean
Chronic infection – whereas our question is ever in the last year
Each bar represents a different centre

19. % with chronic Pseudomonas infection in Adult Centres
UK CF Trust Registry
UK mean
Each bar represents a different centre

20. Have we made progress? 2011 2015 Persons with CF registered 415 443
Median age in years
15.7 yrs
18.1 yrs
PWCF aged > 16 years
49.6%
55.8%
Genotype known
87.7%
96.8%
Median FEV1
< 16 yrs
>16 yrs
80.5%
91.6%
70.7%
85.1%
97.7%
78%

22. CF Data Network

23. Changes for the future? ABILITY TO INPUT DATA ACCESS TO DATA
Questions have been modified
Age brackets altered
Comparison to other registries