Median survival has increased in people with cystic fibrosis (CF) during the past six decades, which has led to an increased number of adults with CF. The future impact of changes in CF demographics ...has not been evaluated. The aim of this study was to estimate the number of children and adults with CF in 34 European countries by 2025. Data were obtained from the European Cystic Fibrosis Society Patient Registry. Population forecasts were performed for countries that have extensive CF population coverage and at least 4 years of longitudinal data by modelling future entering and exiting flows in registry cohorts. For the other countries, population projections were performed based on assumptions from knowledge of current CF epidemiology. Western European countries' forecasts indicate that an increase in the overall number of CF patients by 2025, by approximately 50%, corresponds to an increase by 20% and by 75% in children and adults, respectively. In Eastern European countries the projections suggest a predominant increase in the CF child population, although the CF adult population would also increase.It was concluded that a large increase in the adult CF population is expected in the next decade. A significant increase in adult CF services throughout Europe is urgently required.
Cystic fibrosis (CF) is a genetic disease in which mutations in the gene encoding for the CF transmembrane conductance regulator protein result in a multisystem disease dominated by digestive and ...respiratory manifestations. In the mid-20th century, CF caused death within the first years of life. Over the past decades, advances in disease management, which includes systematic neonatal screening, multidisciplinary symptomatic CF care, lung transplantation and, more recently, highly effective CF transmembrane conductance regulator modulators, have transformed the prognosis of people with CF markedly. In most countries with well-established CF care, adults now outnumber children, and life expectancy is expected to increase further, narrowing the survival gap with the general population. However, marked differences in the prognosis of CF exist not only among high-, low-, and middle-income countries but also among high-income countries, based on the presence and quality of a specialized CF care provision network. Current evidence suggests that differences in patient clinical status and survival could be attributable not only to intrinsic disease severity but also to disparities in access to high-quality specialized care. Because CF is generally a progressive disease, adults with CF often show increased pulmonary severity and complications and increased occurrence of comorbidities, which highlights the need for specialized adult CF centers. This article seeks to describe the evolution of CF demography over the past decades, predict future trends, and anticipate the future provision of adult CF care.
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