Cystic Fibrosis mortality and survival in the United Kingdom, 1947 to 2003

¹ Dept Child Health, University of Wales Swansea, Swansea, UK
² Dept Mathematical Sciences, University of Bath, Bath, UK

^* To whom correspondence should be addressed. E-mail: j.a.dodge{at}btinternet.com.

	Abstract

Data up to 1995 have previously been published on the survival of three year cohorts of people with cystic fibrosis (CF) in the UK born between 1968 and 1992. This paper reports survival data up to the end of 2003 together with a 2003 population estimate.

All persons with CF born in the UK between 1968 and 1992 were identified by active enquiry, up to 1997, through recognised CF clinics and other hospital consultants. We have added information from the death certification authorities up to end 2003. Death certificates which could not be matched with UKCFS records were investigated and the data reconciled.

The observed survival to 2003 of CF subjects born in 1978 is males 55%, females 49%. For 1988 and 1992 the data are 91% and 88% and 97% and 96% respectively. The estimated 2003 mid-year CF population is 8284.

The continuing improvement in survival of CF subjects in successive cohorts means that the previous prediction of median survival exceeding 50 years for those born in 2000 continues to look realistic, even in the absence of proven effective therapy aimed at correcting the basic CF defect.

This article has been cited by other articles:

				A. Bush Treatment of Cystic Fibrosis: Time for a New Paradigm? Chest, November 1, 2009; 136(5): 1197 - 1199. [Full Text] [PDF]

				M Loeve, P T. W van Hal, P Robinson, P A de Jong, M H Lequin, W C Hop, T J Williams, G D Nossent, and H A Tiddens The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease Thorax, October 1, 2009; 64(10): 876 - 882. [Abstract] [Full Text] [PDF]

				M. Loeve, M. H. Lequin, M. de Bruijne, I. J. C. Hartmann, K. Gerbrands, M. van Straten, W. C. J. Hop, and H. A. W. M. Tiddens Cystic Fibrosis: Are Volumetric Ultra-Low-Dose Expiratory CT Scans Sufficient for Monitoring Related Lung Disease? Radiology, October 1, 2009; 253(1): 223 - 229. [Abstract] [Full Text] [PDF]

				M. Walshaw Routine OGTT screening for CFRD - no thanks J R Soc Med, July 1, 2009; 102(Supplement_1): 40 - 44. [Full Text] [PDF]

				Y. Yahiaoui, M. Jablonski, D. Hubert, H. Mosnier-Pudar, L.-H. Noel, M. Stern, D. Grenet, J.-P. Grunfeld, D. Chauveau, and F. Fakhouri Renal Involvement in Cystic Fibrosis: Diseases Spectrum and Clinical Relevance Clin. J. Am. Soc. Nephrol., May 1, 2009; 4(5): 921 - 928. [Abstract] [Full Text] [PDF]

				J Bott, S Blumenthal, M Buxton, S Ellum, C Falconer, R Garrod, A Harvey, T Hughes, M Lincoln, C Mikelsons, et al. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient Thorax, May 1, 2009; 64(Suppl_1): i1 - i52. [Full Text] [PDF]

				J. Goddard and S. J Bourke Cystic fibrosis and pregnancy Obstet Gynaecol (Lond), January 1, 2009; 11(1): 19 - 24. [Abstract] [Full Text] [PDF]

				J. Abbott, A. Hart, A. M. Morton, P. Dey, S. P. Conway, and A. K. Webb Can Health-related Quality of Life Predict Survival in Adults with Cystic Fibrosis? Am. J. Respir. Crit. Care Med., January 1, 2009; 179(1): 54 - 58. [Abstract] [Full Text] [PDF]

				C. Glasscoe and J. A. Smith Through a Mother's Lens: A Qualitative Analysis Reveals How Temporal Experience Shifts When a Boy Born Preterm Has Cystic Fibrosis Clinical Child Psychology and Psychiatry, October 1, 2008; 13(4): 609 - 626. [Abstract] [PDF]

				A. von Drygalski and J. Biller Anemia in Cystic Fibrosis: Incidence, Mechanisms, and Association With Pulmonary Function and Vitamin Deficiency Nutr Clin Pract, October 1, 2008; 23(5): 557 - 563. [Abstract] [Full Text] [PDF]

				S. C L. Hewer and J. Tyrrell Cystic fibrosis and the transition to adult health services Arch. Dis. Child., October 1, 2008; 93(10): 817 - 821. [Full Text] [PDF]

				C. H Goss Should we stop using intravenous gentamicin in patients with cystic fibrosis? Thorax, June 1, 2008; 63(6): 479 - 480. [Full Text] [PDF]

				M. M. Tunney, T. R. Field, T. F. Moriarty, S. Patrick, G. Doering, M. S. Muhlebach, M. C. Wolfgang, R. Boucher, D. F. Gilpin, A. McDowell, et al. Detection of Anaerobic Bacteria in High Numbers in Sputum from Patients with Cystic Fibrosis Am. J. Respir. Crit. Care Med., May 1, 2008; 177(9): 995 - 1001. [Abstract] [Full Text] [PDF]

				I. M Balfour-Lynn Newborn screening for cystic fibrosis: evidence for benefit Arch. Dis. Child., January 1, 2008; 93(1): 7 - 10. [Full Text] [PDF]

				J. C Davies, E. W F W Alton, and A. Bush Cystic fibrosis BMJ, December 15, 2007; 335(7632): 1255 - 1259. [Full Text] [PDF]

				W. Lenney The future for lung disease in children Thorax, December 1, 2007; 62(12): 1021 - 1022. [Full Text] [PDF]