Key findings from the 2023 UK CF Registry report

As we celebrate Cystic Fibrosis Trust’s 60th anniversary, we find ourselves as a CF community reflecting on our collective achievements, facing an optimistic yet still challenging future for CF. One of these achievements is the UK CF Registry which has recently released its annual data report. This valuable data shines a light on where we are today, embracing huge progress in CF care and outcomes, driven by the collective achievement of the whole CF community. It also helps to illustrate new challenges for people with CF and their clinical and research teams.

The report covers over 11,300 people living with CF in the UK and shows the expanding and changing age distribution of this population. Nearly two thirds of the UK CF population are over 16 years old and of these, nearly two thirds are in work or studying. Currently one in six people with CF in the UK are over 40 years old. Using Registry data, we are able to predict that half of people born today with CF in the UK will live to at least 64, which has increased from previous years. The gap between predicted survival of men and women with CF has also narrowed. This prediction assumes that CF treatments will remain the same for those babies over their lifetime.

The median age of survival can be helpful for us to look at change over our whole population with CF in the UK, but this statistical calculation doesn’t reflect individual, or personal characteristics, such as someone’s genotype or how well they might respond to different treatments. As a CF community we need to continue to develop how we monitor and care for a growing population who are living longer and healthier lives.

Over 8,200 people with CF in the UK were reported to be taking a CFTR modulator such as  Kaftrio, Kalydeko, Orkambi or Symkevi by December 2023. We have included new sections within the Registry Annual Data report (Sections 1.35b and 1.36) to illustrate modulator use by genotype group and demographics. 

We continue to see an improvement in the average lung function and a reduction in the percentage of people with CF requiring at least one course of intravenous antibiotics. As in previous years we see that in 2023, the number of people with CF with at least one sputum sample recorded in the last year was down and perhaps as expected, an increase in the number of cough swabs. 

This year, we have included new information within the report on physiotherapy techniques and exercise testing. It is interesting to note that more than one in 6 people with CF are currently using exercise as their primary airway clearance technique. The proportion of people with CF that remain on the combination of inhaled antibiotics, DNase and hypertonic saline or Mannitol has dropped again this year. However, three quarters of people with CF in the UK are currently on at least one of these inhaled therapies reflecting just one aspect of the ongoing burdens of CF care. 

More than one in four people with CF over 10 years of age are on CF diabetes therapies. As the CF population grows older this proportion is likely to increase and other comorbidities may occur. The nutritional status of the CF population is also changing with a smaller proportion now being underweight but an increasing proportion of adults being classified as overweight or obese. The Registry is evolving and assessing how this data can be captured and linked in a way that supports the CF community to perform research and to adapt care models to meet these challenges. 

The 2023 Registry data is a hugely rich information source which the UK CF community should be proud of, celebrate and use to drive forward improvements in CF care in the ever-changing landscape of cystic fibrosis. When looking at the Registry report it is important to remember that the annual reports summarise changes in populations as opposed to showing individual stories. We would like to express our gratitude to people with CF for consenting to have their clinical data recorded and the clinical teams for collecting and entering it into the Registry because “none of us is as smart as all of us”.

We hope you enjoy reading the report and we would love to hear your feedback. Please contact the Registry team on social media or by emailing [email protected] to let us know your comments and questions.

Read the latest UK CF Registry Report

For information or support with cystic fibrosis, Cystic Fibrosis Trust’s Helpline is available on 0300 373 1000, on email at [email protected] or on WhatsApp 07361 582053