Survival estimates for adults with cystic fibrosis born in the United Kingdom between 1947 and 1967
a School of
Postgraduate Medicine, University of Bath, Bath, UK, b Department of Child Health, Queen's University,
Belfast, UK, c British Thoracic Society, London, UK, d Department
of Medical Computing and Statistics, University of Wales College of
Medicine, Cardiff, UK, e Royal
College of Paediatrics and Child Health, London, UK, f Cystic Fibrosis Trust, Bromley, Kent, UK
Correspondence to: Dr P A Lewis, School of Postgraduate Medicine, University of Bath, Bath BA2 7AY, UK.
Received 15 June 1998; Returned to authors 28 September 1998; Revised version received 10 December 1998; Accepted for publication 5 January 1999
BACKGROUND
The UK has
published observed cohort survival figures for subjects with cystic
fibrosis born since 1968. Prior to 1968 cohorts cannot be established
directly from routine data as cystic fibrosis was classified with a
number of unrelated conditions in ICD7. Reported here are interrupted
survival curves from 1978 for patients with cystic fibrosis born before 1968.
METHODSLife tables
for the three year cohorts born between 1947 and 1967 were constructed
by firstly estimating the numbers of patients with cystic fibrosis born
in each cohort from live birth data and the disease incidence. The
number of the estimated cohort that had survived to 1978 is known,
which enables the proportion surviving to 1978 to be calculated. The
survival of these cohorts after 1978 can be calculated in the usual way.
RESULTSThe survival
for each successive cohort was better than that of the previous one,
but most of the improvements appear to have taken place up to the age
of about 20 years. Only 3% of the 1947-49 cohort survived to 30 years
of age compared with 21% for the 1965-67 cohort, and 3% of the
1953-55 cohort survived to 40 years of age. For the later cohorts the
mortality rate for those aged between 26 and 30 years appears to be
about 50 per 1000 per year.
CONCLUSIONSWhile the
trend in the numbers surviving into later adulthood is upwards, the
mortality rates for these ages does not appear to be improving. It is
not possible to tell from these data whether the high mortality rates
in adulthood will improve with better resourced adult clinics or with
improved treatment during childhood.
Keywords: cystic fibrosis; survival
© 1999 by Thorax
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