Key findings from the 2021 CF Registry Report

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As we release the 2021 CF Registry Report, Gwyneth Davies (paediatrician, researcher, and member of the CF Registry Steering Committee) explores some of the key findings and what they mean for the CF community.

At a time of rapid change in cystic fibrosis (CF), the annual Registry report provides an insightful snapshot of the UK’s CF population. The 2021 CF Registry Report spans an eventful year, with the ongoing impact of the COVID-19 pandemic, but also real progress for people with CF, as access to Kaftrio increases across the community. Despite the ongoing disruption from COVID-19, it’s impressive over 10,000 annual reviews were captured – an excellent achievement and a testament to all, from patients to their clinical and administrative teams alike. 

Changes within CF Registry reports from year-to-year are often small, but the 2021 report suggests an acceleration in the pace of change. Over the past five years, mean FEV1 (this is the amount of air that a person can blow out of the lungs in the first second of a forced exhaled breath) has improved by 10% predicted in those aged 12 years and over, with more modest gains in those under 12.

There is further cause for optimism in the latest survival data, where data are analysed in five-year rolling periods (most recently 2017-2021) to provide a more accurate assessment. An increase in median predicted survival (i.e. the age that half of people born today are predicted to live to) to 53 years is driven by improved survival in both males and females. It is the improvement for the latter that has accelerated since the 2020 report – the gap between males and females appears to be narrowing. It’s also greatly encouraging to see a rise in the number of people with CF becoming parents. Over 100 women with CF had a baby in 2021 - a near doubling in number from 2020. 

The 2021 CF Registry Report spans an eventful year, with the ongoing impact of the COVID-19 pandemic, but also real progress for people with CF, as access to Kaftrio increases across the community.

Gwyneth Davies

New in the 2021 report is a summary of the nutritional status of the CF population over recent years, showing a continued trend in reduction in the proportion of people with CF who are underweight. Inclusion of such data in future reports will also allow the Registry to capture any changes occurring at the other end of the nutritional spectrum, increasingly important in view of changing demographics of the population. 

The infection data within the 2021 Report are harder to untangle - a further decrease in the proportion of people with chronic Pseudomonas aeruginosa, along with other important infections such as non-tuberculous mycobacteria (NTM), are welcome. However, they need to be treated with some caution until we better understand the impact of changes in airway sampling, reflecting different ways that CF care has been delivered over the course of the COVID-19 pandemic, and an effect of CFTR modulators. Across paediatric and adult populations, in 2021 the number of people with at least one sputum sample was down, with the opposite seen for cough swabs. 

For intravenous antibiotics (IV), the pace of change is rapid. There has been a sizable drop in the proportion of the CF population receiving IV antibiotics over the past 12 months, down from 39% to 24% between the 2020 and 2021 reports. 

Whilst the 2021 CF Registry Report is extremely encouraging overall, it’s important to remember that for many people, particularly for those who cannot benefit from CFTR modulators, things may not have changed so positively over the past 12 months. Registry annual reports summarise changes in populations, they don’t tell individual stories. But the Registry itself is constantly evolving, and changes are made each year to ensure that the data are inclusive and relevant for our evolving CF population and their needs. 

In summary, the 2021 Registry report is well worth a read. But then make sure you give yourself time to pause and reflect. In the words of Bob Dylan, ‘The Times They Are A-Changin'.

We won’t stop

In a time of profound change, this report brings so many useful insights that really can shape our work moving forwards. A predicted median survival age of 53 years is still far too young – and shows there is still much more work we need to do. We won’t stop until everyone with CF can truly live a life unlimited: mentally well, physically well, and personally fulfilled. 

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.

Finally (and most importantly), on behalf of the Registry team and Steering Committee, we want to extend our thanks to people with cystic fibrosis for continuing to support the UK CF Registry, as well as their families and their clinical teams for coming together to make this report possible.

Read the full report

Cystic fibrosis (CF) is a genetic condition which causes sticky mucus to build up in the lungs and digestive system. It affects more than 10,800 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.

Your donations help us fund vital research. Donate today to help everyone with CF live a life unlimited.

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