Cystic fibrosis patients’ life expectancy increasing

January 01, 0001

Cystic fibrosis patients’ life expectancy increasing

The aim of this study was to assess mortality trends among people with cystic fibrosis (CF) in Australia. The researchers augmented Australian summary data for deaths from CF registered during 1979-2005 with information from Australian transplant centres on lung transplantation among CF patients for 1989-2005 to allow us to follow trends in all "mortality events" (death or lung transplantation).

Between 1979 and 2005, the mean age at death increased from 12.2 years to 27.9 years for males and from 14.8 years to 25.3 years for females. Overall, female deaths in childhood (0-14 years) occurred at an age-standardised rate of 0.40 per 100 000 during 1979-2005, which exceeded the corresponding rate for males of 0.24 per 100 000. Among 0-14- year-old boys, event rates declined markedly after 1989, but they declined later and more gradually for girls, with the result that the age-standardised rate for girls was 2.38 times that of boys during 1989-2005.

The researchers concluded: "The pattern of CF mortality in Australia has changed substantially. Mortality rates continue to be higher for girls than for boys, but death in childhood has become uncommon. Survival has increased since 1979, but females continue to have reduced length of life."

This really is an amazing difference. What implications does this have for the gene prevalence?


For the full abstract, click here.

MJA 195(7):392-395, 3 October 2011 © 2011 to The Medical Journal of Australia
Changes in cystic fibrosis mortality in Australia, 1979-2005. David W Reid, C Leigh Blizzard, Dace M Shugg, Ceri Flowers, Catherine Cash and Hugh M Greville. Correspondence to David Reid: vid.Reid@qimr.edu.au

Category: A. General/Unspecified. Keywords: cystic fibrosis, mortality, death registry data, journal watch.
Synopsis edited by Dr Stephen Wilkinson, Melbourne, Australia. Posted on Global Family Doctor 21 October 2011

Pearls are an independent product of the Cochrane primary care group and are meant for educational use and not to guide clinical care.

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