UK Registry Finds CF Community Living Longer, Becoming More Diverse

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by Forest Ray PhD |

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UK CF Registry 2019 report

People with cystic fibrosis (CF) in the U.K. have grown more numerous, older, and more diverse, according to the U.K. Cystic Fibrosis Registry’s annual data report for 2019.

The report, meant to present a snapshot of both the gains as well as the challenges still faced by people with CF in the U.K., found that patients had expanded in number to 10,655 in 2019 from 10,583 in 2014, and their median age was now 21.

“The insights included in this report are an important view into the evolving world of CF at what we may come to see as a landmark year for many in the community,” David Ramsden, chief executive of the Cystic Fibrosis Trust, said in a press release.

“For the first time the median age of people with cystic fibrosis has reached 21 years,” Ramsden added. “This is still far too young — the average age of the general population in the U.K. is approximately 40 years — and shows that we still have much work to do.”

According to the report, teenage years continue to be a vulnerable time for people with CF, involving sharp declines in weight and lung health. The Trust, for this reason, runs a number of youth programs to support this patient age group.

This year’s report features a new ethnicity section, showing changes in diversity of the CF population over the last 10 years. Since 2009, the proportion of whites with CF fell from 95.7% to 93.3%, while those of Asian backgrounds grew from 2.3% to 2.8%, and those of African descent remained steady at 0.1%.

Other findings included 29.3% of these people were diagnosed by newborn screening, and that patients less than 16 years old were diagnosed at a median age of 22 days in 2019. In comparison, 14.9% of adults in the registry were diagnosed as teenagers or beyond — at a median of 16 years old.

Life expectancy is a median predicted age of 49.1 years based on 2015–19 data, with men living almost six years longer than women (a median of 51.6 years vs. 45.7). More than half of the country’s CF population — 56.7% — were adults, defined as age 18 or older.

Chronic Pseudomonas aeruginosa infection was found in 39.4% of CF patients ages 16 and older. The median age of those with a chronic P. aeruginosa infection was 30 in 2019, compared with 25 in 2009.

The proportion of people using hypertonic saline or mannitol as mucus thinners grew to 36.8% in 2019 from 6.9% in 2009.

Over the same 10-year period, people being evaluated for lung transplants grew to 241 in 2019 from 144, and those receiving bilateral lung transplants rose to 50 from 19.

The annual report is meant to support CF care and research, and to provide evidence for pharmacovigilance studies that monitor the continued safety and real-world impact of new treatments after clinical trials.

“The information in this report will provide opportunities to improve the lives of people with CF now, and support essential breakthroughs in the future,” said Rebecca Cosgriff, director of data and quality improvement at the CF Trust.

This year’s findings are available in an “at-a-glance” version and as a full report.