Number of Adults With CF Likely to Grow by 28% Over Decade, UK Study Says

The number of adults with cystic fibrosis (CF) living in the U.K. is expected to increase by at least 28% in 10 years, and 36% of these adults will be older than 40, a study projects.

Findings highlight the need to make plans now to care for this changing population.

The study, “The changing demography of the cystic fibrosis population: forecasting future numbers of adults in the UK,” was published in the journal Nature Scientific Reports.

In recent decades, treatments for CF have improved dramatically. As the care available continues to improve, more and more people with CF are expected to live into adulthood. Making predictions about this anticipated increase can allow healthcare systems to better prepare and adapt, so that they can provide optimal care to everyone needing it.

Researchers at the London School of Hygiene and Tropical Medicine and the Imperial College London analyzed data from the UK CF Registry to construct statistical models that predicted changes in the CF population from 2018 to 2030. Of note, the last year with available data was 2017.

These models took into account factors such as the expected number of people of various ages who will be newly diagnosed with CF, how current CF patients are expected to age, and projected mortality rates based on the current standard of care.

From these projections, the researchers estimated that by 2030 the number of people with CF ages 16 to 17 will increase by 20%, to 578 from 483 in 2017. Over this same time, the number of adults with CF — those age 18 or older — is expected to increase by 28%, to 7,988 in 2030 from 6,225 in 2017.

Slightly different models were used to estimate the number of adults with CF who would require specialized care; these models predicted a 28% increase by 2030.

Age distribution is also expected to change by 2030. In 2017, 49% of adults with CF were over 30 years old, and 21% were over 40 years old. These proportions are expected to increase to 63% and 36%, respectively, by 2030.

These findings showed “how the age distribution of people with CF is expected to change in the coming years, such that there is expected to be a greater proportion of patients at older ages,” the researchers wrote.

Importantly, these projections were made assuming that current mortality rates hold steady for the next decade. Realistically, this is unlikely — as treatments improve, mortality rates are likely to continuously decline. Anticipating this, the researchers constructed additional models to account for changing mortality rates.

Between 2008 and 2017, age-adjusted CF mortality rates decreased by about 3% each calendar year. One model assumed that mortality rates would continue to decrease by this amount, and projected that the number of adults with CF would rise by 38%,  to 8,579 people, by 2030.

Another model assumed mortality rates would drop by half the rate seen between 2008–17 — that is, by about 1.5% each year. According to this model, the number of CF adults was projected to increase by 35%, to 8,390 people, by 2030.

Collectively, these data suggest how the CF population in the U.K. is likely to change over the next decade, highlighting healthcare system changes that will be needed in order to adapt to this changing population.

“Our findings suggest an urgent requirement to review health care provision for adults with CF in the UK as numbers increase, disease characteristics change and new therapies are introduced,” the researchers wrote.

“It will be important to ensure there is capacity to transition more patients from paediatric care, but perhaps more importantly will be the ability to care for a growing proportion of older, increasingly complicated adults, and also perhaps a more stable younger cohort who will predominantly require outpatient-based care,” they added.

The team believes that their findings provide a “vital impetus to act now to ensure care provision has the capacity and is fit for purpose for the changing needs of this adult CF population.”