Health inequalities and cystic fibrosis

Cystic fibrosis is the most common serious inherited disease in white populations. Intensive support from family and healthcare services is needed from the time of diagnosis onwards, and most patients die prematurely from respiratory failure. Survival has dramatically improved over successive birth cohorts, such that British children born in the 21st century will have a median survival of over 50 years.1 However, disease progression and survival still vary greatly, mostly as a result of social and environmental, rather than genetic, determinants.2 It has been known for more than 20 years that people with cystic fibrosis from socioeconomically disadvantaged backgrounds die younger than those in more advantaged positions.3

In a linked observational study (doi:10.1136/bmj.d4662), Barr and colleagues used death registration data in England and Wales to show that this socioeconomic divide in premature mortality in cystic fibrosis has persisted with no substantial narrowing for over four decades.4 They also show that female patients continue to die at a younger age than men. What can we learn from this, and what are the implications …