Key findings from the 2022 UK CF Registry report

Blog - Professor N. Simmonds -

Improvements in lung function, reduced numbers of IVs and 140 women with CF having babies are just some of the highlights from this year’s Registry data report

The Registry team at Cystic Fibrosis Trust have released their latest data report, reflecting another year of rapid change in the CF landscape. The report, which covers 11,148 people with CF in the UK, shows the continued excellent progress that has been made, but, perhaps equally as important, it also shows where gaps still exist, shining a vital spotlight on areas that could be improved.

The rollout of CFTR modulators such as Kaftrio has continued, with almost 8,000 people in the UK now receiving them, including some people with rare CF genes, which has also now been approved by the NHS. This is a remarkable achievement, but it also highlights the need for other novel treatments so that all genotypes can be treated so that everyone with CF has the potential to benefit.  

Improvements in lung function and reduced numbers of IVs

The report shows lung function and intravenous (IV) antibiotic usage continue to improve. The latter is particularly important to people with CF, as IV antibiotics are disruptive to life, time-consuming and can be associated with side effects. The reduction in IVs is also positively impacting CF teams as services are adapting to reflect these changing needs. Overall, 22.3% of people with CF required at least one course of IVs in 2022, which is down from 39% in 2020!

Lung function – measured by FEV1, which has long been known to be associated with important outcomes in CF – is now on average 73.4% for adults and 94.6% for children over the age of six. This is a huge improvement on the figures from 10 years ago, which were 61.7% and 79.8% respectively.

Nutrition is another area where progress has continued with fewer people in the underweight category, but also the report highlights an increasing proportion of people moving into the high weight category since 2019. This is important as it is providing vital information for dietitians to develop new guidelines, so that nutrition can be optimised and tailored for everyone moving forward.

Median predicted survival age rises to 56

Nick Simmonds
Professor Nick Simmonds

Median predicted survival (the age at which half of the CF population born today would be expected to live to) also continues to improve and now stands at 56.1 years.

The rate of lung transplantation has fallen since the introduction of CFTR modulators, with 41 people evaluated in 2022 compared with 241 in 2019. These figures point towards a healthier population but also show more still needs to be done to improve this for everyone.

Opportunities and challenges

Undoubtedly, these are exciting times, with a rapidly changing landscape presenting opportunities for people with CF they may not have previously thought possible. For example, pregnancy rates continue to increase, with 140 women with CF having a baby in 2022 compared with 58 in 2019. But with this success we must ensure progress continues – over 15% of the CF population are now aged 40 years or older. We must ensure we stay ahead of the curve and manage their CF effectively as other medical conditions might develop.

Burden of treatment is also still high, with over 80% on at least one inhaled treatment. Studies such as CF STORM (a study which evaluates the safety of stopping certain daily nebulisers) will help us make progress here, but more research is required to work out which other treatments can be safely stopped.

These are exciting and changing times… but there’s still a lot more work to do!

Read the 2022 Registry report

Professor Nicholas Simmonds is associate director and consultant physician of the Adult Cystic Fibrosis Centre at Royal Brompton Hospital, and professor of practice (respiratory medicine) at the National Heart and Lung Institute, Imperial College London.

Cystic fibrosis (CF) is a genetic condition which causes sticky mucus to build up in the lungs and digestive system. It affects more than 11,000 people in the UK. One in 25 of us carries the faulty gene that causes it, usually without knowing.

Since 1964, we've supported people with cystic fibrosis to live longer, healthier lives and we won’t stop until everyone can live without limits imposed by CF.

Life with CF test 1

Sign up to our newsletter

Get the latest news on what the Trust and our supporters are doing - straight to your inbox

Your donation will make a difference:

Select amount
Select amount