“Living into adulthood is now expected.”

Last month a cystic fibrosis course was held at the Royal Society of Medicine in London. Dr Nicholas Simmonds, Consultant Physician in adult CF at the Royal Brompton Hospital in London gave a fascinating presentation on ageing and long-term survival in CF, and has kindly summarised his talk for the CF Trust blog.

Dr Nicholas Simmonds

The demographics of cystic fibrosis, particularly median survival, continue to improve at a rate unparalleled to most other chronic conditions in medicine.

Within a few decades average survival has more than doubled and now stands at 41.4 years (CF Trust Annual Data Report 2010).

Children born with CF from the early 2000s are expected to live beyond 50 years of age and over 55% of the current UK CF population are adults. Living into adulthood is now expected – currently over 22% of UK CF individuals are in their 30s and over 7% aged at least 40! In paediatrics, the emphasis is now very much on when a patient will transition to the adult clinic (not if) and the aim is to transition with normal lung function with planning for a full and active life (e.g. college, work and relationships etc). 

My presentation at the Royal Society of Medicine highlighted this change in demographics and outlined some of the important reasons behind the improvements (e.g. keeping lung function and weight optimal from an early age, keeping Pseudomonas infection at bay by effective treatment, etc.). 

It also highlighted that all this good work is not straightforward to individuals with CF and comes at a cost – time, energy and effort – as treatment/physiotherapy is intensive and needs to be sustained throughout life. As clinicians and researchers we recognise this and are continuously looking for new and innovative ways to help (faster nebulisers, for example). 

Currently the majority of our adult patients are co-habiting/married and working – as a specialist adult clinic we aim to do all we can to support our patients to progress in life while at the same time providing effective specialist healthcare. The next few decades are likely to continue to bring exciting and important challenges – new drugs are being developed and the adult population will continue to increase. With this we recognise the need to continue to adapt and develop for the CF adults of the future.