The most comprehensive and up-to-date collection of epidemiological data for CF across Europe is the registry maintained by the European Cystic Fibrosis Society (ECFS) (www.ecfs.eu). Data are submitted to the registry by both national CF registries and individual CF centres throughout Europe. The registry collects data from 25 000 CF patients in 21 countries and produces annual summary reports.
The current report, covering 2008–2009, contains details on 18 999 patients. The 2007 report contains data on 20 204 patients. However, these datasets are likely to suffer from under-reporting. For example, the UK contribution of 4408 patients in the 2007 report (the third-largest in the registry) is likely to be at least 2000 short of the true figure. A high priority must be to resource this database so that data on all CF patients across Europe can be captured.
There are marked age-related changes in resource use, with older patients having more morbidity and requiring more expensive medications and other resources, including noninvasive ventilation. It is important to note that diagnosis by screening is associated with lower healthcare costs.
Prevalence and incidence
Figure 1 shows the prevalence of CF by country within Europe. Table 1 shows the number of patients reported to the ECFS Patient Registry in either the 2008–2009 or 2007 reports, and the age distribution of CF patients. Many countries have at least 90% case ascertainment, but in some countries this figure is much less. A more detailed analysis of the registry data between January 2003 and December 2007 by McCormick et al., 2010 (see Further reading), covered 29 025 patients, 25 126 from European Union (EU) countries (as of 2003) and 3809 from non-EU countries. In the EU cohort, 11 742 (47%) were aged over 18 years, emphasising that CF is increasingly becoming a disease of adults. However, only 1205 (5%) were aged over 40 years. There is therefore a pressing need to ensure that adult services are established all over Europe, offering the same high standards of multidisciplinary care as paediatric clinics. There were proportionately more ‘elderly’ CF patients in the 2003 EU countries compared with the non-EU countries (figure 2). This is not due to ascertainment bias (milder phenotypes being diagnosed in the EU): when the analysis was repeated just for CF patients homozygous for the severe mutation ΔF508 (deletion of a phenylalanine residue at position 508 in the protein), the findings were the same. Reasons for the better prognosis and lower mortality could include the lower median age at diagnosis in the EU, and better socioeconomic conditions.
It is intriguing to note that in countries where newborn screening for CF has been introduced, there has been a decline in the prevalence of CF. This may at least in part be due to diagnosis of the first CF child in the family before a second one is conceived, thus giving couples reproductive choices after the birth of a first CF child.
Table 1 – Cystic fibrosis (CF) in Europe: age distribution, 2009. EU: European Union. #: the estimated percentage of CF patients living in a country who are included in the national registries/national data collections. One individual centre might include almost all patients for some countries (e.g. Latvia and Serbia); ¶: national CF registry established; +: 2007 data reported for Bulgaria, UK and Belarus; §: 2008 data reported for Ireland; ƒ: although not officially a national registry, all centres in Israel participate and so considered a registry. Data from the European Cystic Fibrosis Society (ECFS) Patient Registry 2007 and 2008–2009 reports.
Mortality rate varies with age and is likely to be about 1–2% per year overall. In 2009, there were more than 800 CF patients across Europe living with transplanted lungs. There were 133 CF lung transplants performed in 2009, compared with 108 in 2007. However, it is thought that these numbers are likely to be an underestimate as in some countries patients are transferred to a transplant centre and so are not known to their CF registry. Data on liver transplantations performed each year are more difficult to ascertain, but they are performed significantly less often than lung transplants in CF patients – only seven liver transplants were performed in CF patients in 2009, making a total of 93 patients living with transplanted livers in 2009 across Europe.