The changing demography of the cystic fibrosis population: forecasting future numbers of adults in the UK

Abstract

Improvements in management of cystic fibrosis (CF) through specialist centres in the UK have been associated with a step-change in life expectancy. With increasing numbers of adult patients there is a need to review health care provision to ensure it is sufficient to meet future needs. We used UK CF Registry data to project the number of patients aged 16-17 and 18 and older up to 2030, and numbers therefore requiring specialist adult CF care. Survival modelling was used to estimate age-specific mortality rates. New-diagnosis rates were estimated using diagnoses observed in the Registry and national population figures. Uncertainty in projections was captured through 95% prediction intervals (PI). The number of adults (aged 18 and older) is expected to increase by 28% from 6,225 in 2017 to 7,988 in 2030 (95% PI 7,803-8,169), assuming current mortality rates. If mortality rates improve at the rate seen over recent years, the projected number increases to 8,579 (95% PI 8,386-8,764). The age distribution is also expected to change, with 36% of CF adults being over 40 in 2030, versus 21% in 2017. There is an urgent requirement to review adult CF health care provision, due to both increasing numbers and the changing care needs of an older population.

Projecting the impact of triple CFTR modulator therapy on intravenous antibiotic requirements in cystic fibrosis using patient registry data combined with treatment effects from randomised trials

“…We estimated how many days of IV antibiotic treatment in hospital and at home will be required by pwCF aged ≥12 years, and how this might change following the introduction of ELEX/TEZ/IVA. In previous work we have forecasted future patient numbers,12 which highlighted the need to plan for a larger adult CF population. This study increases our understanding of the future needs of this population and illustrates how combining registry and RCT data can enable estimation of population level treatment impacts.…”

“…Table 3 Estimated population totals for each outcome in 1 year following the 2018 visit, presented as N/1000 (95% prediction interval (95% PI)), and % reductions: with no treatment effects applied, with the effect of TEZ/IVA imposed and with the effect of ELEX/TEZ/IVA imposed Hospital-IV-days future patient numbers, 12 which highlighted the need to plan for a larger adult CF population. This study increases our understanding of the future needs of this population and illustrates how combining registry and RCT data can enable estimation of population level treatment impacts.…”

Cystic Fibrosis-Related Diabetes in Poland

“…Currently, it is estimated that a patient born in the last two decades of the 20th century in an economically developed country has an over 50% chance of reaching ca. 40 years of age [ 2 , 3 , 4 ]. Along with the prolonged survival of patients, the number and importance of diseases related to CF have increased [ 5 , 6 , 7 ].…”

Understanding the Experiences of How Mindfulness is Used by People with Cystic Fibrosis: Barriers and Enablers

“…Currently, four modulator therapies (Kalydaco, Orkambi, Symkevi, and Kaftrio) are available for 90% of the UK CF population. Following the advances of CF care and CFTR modulator therapies, the demography of the CF population is changing, enabling most people with CF to now survive into adulthood (Keogh et al, 2020).…”