ECFS Book 2015

www.ecfs.eu

Living longer with Cystic Fibrosis

CHANGING EPIDEMIOLOGY: NEW CHALLENGES IN CF

CHAPTER 1

40 years of age, but it has only been in the

last 30 years that reaching adulthood has

become a realistic prospect for the majority

of individuals. We are now in an era where

the number of adults with CF will soon

exceed the number of children [2]. In regis-

tries where adulthood is defined as at least

16 years of age, rather than 18 years, this

has already occurred; for example, 57.6% of

all CF patients in the UK 2012 annual data

report were classified as adult [5].

The introduction of national and multina-

tional registries, which collect large volumes

of clinical data, has been instrumental in

improving our understanding of CF and the

changing demographics. The US CF Foun-

dation Patient Registry (CFFPR) contains

the data of nearly 30,000 patients and has

been running for decades. For the year

2012, the US registry reported that 49.1%

of the population were adults (≥18 years),

with an age range from birth to 82 years

and a median age of 17.7 years (mean

19.8 years) [3]. The European Cystic Fibrosis

Society Patient Registry (ECFSPR) is more

contemporary but now contains the records

of over 32,000 patients across 22 European

countries spanning a diverse sociodemo-

graphic and cultural spectrum [6]. Overall,

the European data are very similar to the

US data: 49.3% of patients were reported

as adults (≥18 years), with a wide age range

from birth to 80.1 years and a median age of

17.8 years (mean 19.5 years) (

Fig. 2

In some European countries the proportion

of adult patients is particularly high, such

as in Denmark where at least 56% of CF

patients are adults (≥18 years). Approxi-

mately 20% of the European registry popu-

Fig. 1

Cystic fibrosis patients under care at CF Foundation-accredited care centers in

the United States, who consented to have their data entered in 2012 - Median predicted

survival, 1986–2012 (using 5-year bands on the

-axis to reduce year-to-year variability).

(Reproduced with permission from Cystic Fibrosis Foundation [3].)

Median Predicted Survival Age, 1988-2012 In 5-Year Bands