Cystic Fibrosis Demographic Challenges for Europe

J Stuart Elborn Centre of Infection and Immunity, QUB and Adult CF Centre, Belfast HSCT

- Austria - Belgium - Czech R - Denmark - France - Germany - Greece - Hungary - Ireland - Israel - Italy - Netherlands - Poland - Portugal - Romania - Slovakia - Spain - Sweden - Switzerland - UK

Source: © European Communities, [2007] http://cordis.europa.eu/regions/home_en.html

1182

8500

550

434

1275

1055

4533

939 115 4099

282

917

8000

428 433 153

Data provided by the national CF associations and/or the CF Registry and/or CF specialists.

987

800

340

238

Courtesy from Gita Mehta, EuroCareCF, and CF Europe

Total: 34,619

420

Improvements in survival

• Median age at death has increased dramatically

• In the UK children born in the 21st century are estimated to have a median survival of over 50 years of age (Dodge 2007)

• “under the influence of factors such as improved treatment and management, improved nutrition and better living conditions”

Adapted From 2005 Annual Data Report to the Center Directors. Cystic Fibrosis Patient Registry, Bethesda, MD http://knol.google.com/k/jerry-nick/cystic-fibrosis/UtI7gr91/HU2bIw# (24 April 09)

Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. European Respiratory Journal 2007;29(3):522

1940’s 1980’s

1990’s

REMARKABLE CHANGE IN PROGNOSIS FOR PEOPLE WITH CF

Ben is now the poster boy – the new look, if you will – of cystic fibrosis (CF), the incurable life-threatening genetic condition Read more: http://www.dailymail.co.uk/health/article-2193518/Could-male-pin-Ben-Mudges-incredible-pack-really-key-conquering-cystic-fibrosis.html#ixzz25IQqUCTc

Mehta et al 2010

Age distribution in Europe

Patients from Europe: 23693, US: 24487, Total: 48180 21 countries from Europe + US data shown Data from CFF Registry, ECFS & EuroCareCF

0

1000

2000

3000

4000

5000

<5 5 to 10 10 to 15 15 to 20 20 to 25 25 to 30 30 to 35 35+

Num

bers

of C

F pa

tient

s

5 year age groups

Europe / US Age Distributions

EuropeUS

Gender in Europe

Median FEV1(%predicted) among patients 6yrs and older by year since 2007

Trends in lung infections since 2007

Age distribution of deaths in 2010

Factors associated with worse survival

•Lung function/ FEV1 •Exacerbation frequency •P aeruginosa •B cenocepacia •Nutrition (BMI) •CFRD •CFLD •Female gender

de Boer K et al. Thorax 2011;66:680-685

Comparison of patient numbers in EU and non EU countries

F508del HZ

3212 patients (84%)

Metha et al 2010

Barr H L et al. BMJ 2011;343:bmj.d4662

Barr H L et al. BMJ 2011;343:bmj.d4662

©2011 by British Medical Journal Publishing Group

Expertise and capacity

• Capacity and Training – Europe additional 800

adults/year. – USA 600 additional adults/year.

• Future challenges for adult care! If we don't have the expertise we can’t deliver care.

24

26

28

30 32

34

36

38

40

'86 '88 '90 '92 '94 '96 '98 '00 '02 '04 year

Median survival

’06

Clinical Expertise

Virtuous triad of evidence based practice in Cystic Fibrosis

Registry Practice and outcomes

Guidelines Best evidence and practice

Peer Review Audit and accountability

People with CF

Summary and conclusions

•Survival in CF is increasing •Disease factors are important: FEV1, PEx, BMI and chronic PA •Social and economic factors have a major impact on outcomes •The majority of people with CF in Europe are adults •We need more capacity to deliver adult care: adult CF teams/centres?

Clinical Care & Research in CF