It is difficult to obtain experience in special complications and rare CF genotypes, since knowledge of these on a national scale will often be of anecdotal origin. Therefore, it has long been the wish of European CF clinicians and researchers to have a Europe-wide CF registry.

Past History of the European Registries
At a meeting hosted by the French CF Association (Vaincre la Mucoviscidose) in 1995, the idea was first discussed in a larger forum. In the late 1990s, a registry (the Epidemiologic Registry of Cystic Fibrosis) emerged in connection with a clinical trial of Pulmozyme^®. This registry was funded by F Hoffmann-La Roche, Switzerland, and included patients from approximately 10 countries. Several reports and papers came from this registry, but unfortunately the funding was withdrawn and it was not – at that time – possible to find new funding, so the registry was dissolved.

In 2003, a few representatives from some of the existing national registries, headed by Dr Eitan Kerem, Israel, met in Amsterdam and started working on setting up a new registry, this time preferably one that would keep going on for longer. This group grew to include representatives from all the countries with their own national registry – the first task set was to try and merge the data already available – as a sub-committee of the European Cystic Fibrosis Society (ECFS).

Merging of Existing Registries
Merging of different databases with different definitions and different routines for data input theoretically poses a lot of problems. The first try was performed in 2006 using the data from 2003, since these were the latest that some of the countries could supply.¹ Seven countries contributed their data (Belgium, Denmark, France, Ireland, Italy, Russia and Sweden). Major differences in mode of registration were obvious from the start. For example, some countries recorded only the results of one sputum culture per year; others recorded only chronic lung infections. A lot of the agreed variables were not included in some of the registries and in others the number of ‘missing data’ was very high. Furthermore, there was the issue of reference values for lung function tests and height/weight. Each registry used its own, which made comparison difficult, but may still be the best way to do it, since there are definitely differences from Scandinavia to Southern Italy in general body proportions. The first report should therefore be seen as a pilot project and any conclusions should be interpreted very cautiously. Some of the issues can be solved easily, but others come from different modes of registration that make the data incomparable.

Many of the countries with national registries have used considerable resources in these registries and are not interested in changing to another common registry. Therefore, these data will continue to be merged, knowing that some difficulties will remain.

The sub-committee soon realised that it would have to offer some kind of software for the countries without existing registries in order to avoid the above problems. So far, 10 countries have their own registry and the prospect of trying to merge data from 25 different registries (in 20 different languages) seems almost impossible.

Alternative Funding
A new possibility arose in 2005 when the European Co-ordination Action for Research in Cystic Fibrosis (EuroCareCF) initiative, headed by Dr David Shephard, UK, achieved funding via the EU Framework Six package for different CF-related workpackages, among them the European Patient Registry Workpackage (headed by Dr Anil Mehta, UK).² The project of this part of EuroCareCF was to gather demographic data on CF patients from at least 22 of the 25 EU countries.

This opened up the possibility of a joint venture between EurocareCF (a time-limited initiative) and the ECFS. Since the demographic data would be preferably collected in a database, there was the opportunity to make a demographic and clinical database that could carry on existing even after EuroCareCF was terminated.

Considering the fiasco on relying on unilateral funding of the Epidemiologic Registry of Cystic Fibrosis, there was a general agreement in the Registry sub-committee that future funding should be multilateral and unconditional, preferably government/EU-based, with the addition of unrestricted grants from interested parties (patients’ organisations, pharmaceutical companies, etc.). This would make funding less vulnerable, but might also make it more difficult to obtain the money in the first instance, especially for the development of a common software program. Therefore, the authors were very happy to receive an offer from the US Cystic Fibrosis Foundation (CFF) to use its software, PortCF, and finance any special changes for the European version and the practical management of the European users.