Cure4CF Update 

Life expectancy and the modulator era for CF

What does life expectancy mean?

Life expectancy is a commonly used measure and reflects the mortality level of a population.   It is calculated from age specific death rates and measures how long a person is expected live based on current age and sex specific death rates. These data are “statistics” and represent population or groups or people and are not always helpful for an individual.

How does your year of birth impact your life expectancy?

Variations in life expectancy are common across the world and are impacted by differences in public health and access to medical care and other socioeconomic factors. In 2021 the global average life expectance was 70 years.  However, as you can see in the infographic on the right, life expectancy is different depending on which country you are in.

Like general life expectancy, for people with CF life expectancy is also changing.  This is related to advancements in treatments and health care.

In the 2022 Report, the Australian CF Data Registry examined data from 3737 people with CF who ranged in aged from babies to adults more than 80 years of age. The data collected in the registry shows that those born between 2017-2021 have a median estimated survival of 58.2 years, and that for any individual we can be 95% confident that this will be somewhere between 53 and 60.6 years.  Conversely this also means that 5% of people born with CF between 2017-2021 will have shorter or longer lives than 53 and 60.6 years respectively.  This is still more than a 20-year gap when compared with estimates for non-CF Australians born during the same period who can expect to live to the age of 81.3 years (males) and 85.4 (females).  At Cure4CF Foundation we seek to bridge this gap.

The graph on the right shows some of the data from the 2022 report with each bar representing the median survival age.  This has increased over time in estimated median survival – from 47 years for a person born between 2008 -2012 to 57 years for a person born between 2016-2020.

Have modulator therapies impacted life expectancy?

A detailed analysis of CF survival in Australia was published in 2022 which showed that survival increased from 48.9 years for those born between 2005-2009 to 56.3 years for those born between 2016-2020. Some risk factors were identified for worse survival, these were low lung function and poorer nutritional status.  This suggests that these outcomes may be key factors for modification as part of future therapies for CF.

With the first modulators approved in Australia late in 2014, the data is still emerging and is likely to impact future statistical analyses.  As the modulator therapies continue to improve and access continues to be broadened it is likely that there will be ongoing gains in survival.  However, at Cure4CF Foundation we know that not everyone is eligible to receive modulator therapy and that in some people who are eligible this treatment is not suitable.  We strive for a cure for all people with CF.

Research has shown that both type of mutation and if it is present on one or both chromosomes can be associated with a lower probability of survival.  We know there is an urgent need for effective targeted therapies for people without F508Del mutations especially those with Class 1 mutations where no functional CFTR protein is formed.

If you want to know more about CF mutations, there is a great infographic that you can download from the Cystic Fibrosis Foundation here.