Cystic fibrosis and survival in patients with advanced lung disease

BMJ 2011; 342 doi: https://doi.org/10.1136/bmj.d726 (Published 07 March 2011) Cite this as: BMJ 2011;342:d726
  1. Elliott C Dasenbrook, assistant professor of medicine and pediatrics
  1. 1LeRoy W Matthews Cystic Fibrosis Center, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA
  1. ecd28{at}case.edu

rhDNase slows progression, and is strongly recommended in treatment guidelines

When Dorothy Andersen provided the first comprehensive description of cystic fibrosis in 1938,1 survival was often measured in days and months. However, the introduction of penicillin resulted in children recovering from previously fatal infective respiratory exacerbations.2 Subsequently, antibiotics given in combination with aggressive treatment of malnutrition resulted in some people with the condition living into adulthood.3 Today, a myriad therapeutic strategies are directed at the infectious, inflammatory, and mucociliary defects in cystic fibrosis.4 Combined with other non-pharmacological advances, children born today with cystic fibrosis are expected to live into their 50s.5

Simon Fraser RVI/SPL

In the linked study (doi:10.1136/bmj.d1008), George and colleagues report on survival in a subgroup of adult patients with cystic fibrosis and advanced lung disease at the Royal Brompton Hospital, London, over 17 years (1990-2007).6 Median survival improved by four years in those entering the cohort between 2002 and 2003 compared with those who entered between 1990 and 1991. Their data suggest that the widespread introduction of recombinant human deoxyribonuclease (rhDNase) at the centre in …

View Full Text

Sign in

Log in through your institution