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European Cystic Fibrosis Society
9
CHAPTER 1
Changing epidemiology:
new challenges in CF
Authors
Nicholas Simmonds, Barry Plant
Introduction
In the seven decades since cystic fibrosis
(CF) was first described, considerable
changes in both CF disease course and
survival of patients have been seen.
Improvements in our understanding of the
natural history of CF, as well as progress in
diagnosis and treatment have resulted in
the majority of patients now reaching adult-
hood and enjoying many of the employ-
ment, social and personal achievements
that their healthy peers take for granted.
While these changes should be celebrated,
we also need to acknowledge the chal-
lenges of an ageing patient population, and
ensure that we keep pace with the issues
of increased healthcare burden, increased
disease complexity in the face of comor-
bidities associated with getting older, and
the emerging antibiotic and infection chal-
lenges. This chapter discusses these new
challenges and suggests approaches to
future disease management in an adult CF
population.
1
The changing demographics
Since the first description of CF as a distinct
disease entity by the pathologist Dorothy
Andersen in 1938, the demographic char-
acteristics of the patient population have
changed beyond recognition [1]. At that
time, the diagnosis was devastating, with
more than 70% of children dying within the
first year of life, usually from meconium
ileus, severe malnutrition and/or respiratory
failure. The disease was originally termed
‘cystic fibrosis of the pancreas’, as pancre-
atic destruction was considered to be the
primary defect and the respiratory mani-
festations were thought to be a secondary
complication of malabsorption. Since then,
our understanding of the pathophysiology
and underlying disease mechanisms have
improved considerably; however, the ulti-
mate aim of a cure has yet to be realized.
Thankfully, death in childhood is now rare
and life expectancy has improved steadily
(
Fig. 1
) [2].
In the USA, the median survival rose from
14 years in 1968 to 20 years in the mid
1970s [4]. Median predicted survival in
many developed countries is now over