Information for people with CF

We all use lists in our lives and a registry is another name for a list, a registry is merely a secure place where the list is kept safe. The ECFSPR is a registry holding data on people with CF throughout the European Union and some of the neighbouring countries over an extended period of time. The registry is a database, holding information related to the health of people with CF.

CF is a rare disease, which means that any particular single CF centre, even the largest ones, only have experience with a limited number of patients. ECFSPR data are collected for lots of reasons: to increase knowledge on CF, to determine clinical effectiveness of health care, to compare and improve standards of care for people with CF, to support health care service planning and for research purposes. Collecting data from many countries and centres gives us the opportunity to compare between CF centres and countries, but also to prepare the landscape for possible clinical trials once new potential treatments arise, for example to ensure a sufficient number of patients for research in rare complications, infections or specific genotypes.

Patient involvement is the key factor in the establishment of any patient registry. For the purpose of the above aims, we need as many patients in the registry as possible to

1.  make sure that data are representative of the whole spectrum of the disease and
2.  to have enough patients with e.g. a specific genotype, complication or infection to render research possible – research that may benefit you or other people with CF in the future.

Probably we don’t need to convince you of the need to advance improvement in care and treatment, and progression in research, when good standards of care or treatment are lacking!

We collect some demographic data: month and year of birth, sex, genotype, age and symptoms at diagnosis. We also collect clinical data which are updated once a year e.g. lung function, weight, height, infections, treatment and complications. The data are collected using a common set of definitions and codes so that the data are comparable from country to country. The full list of data and definitions can be found here.

We collect a set of semi-anonymous data; this means that we at the ECFSPR only know your month and year of birth (so we can calculate your current age), your sex and which centre or country you belong to. Your centre gives you a specific code, so they will be able to trace back to you in case we find problems with the data they entered, but only your centre can track this code back to your name. To protect your privacy even further, in our database all the centres have an EU-centre number, not their name. It is not possible to get back to you without your permission.

If researchers request data from the registry they will either get tables, graphs etc with total numbers or aggregated anonymised data.

All storage and use of data in the ECFSPR is regulated by Danish, Italian and EU data protection legislation. The database in Milan, where the data of the ECFCSPR are stored, is of course protected by all means of data security of both hardware and software. If your data are collected initially in a national database, your data are stored there in accordance with national laws and you can learn more about this from your centres. If your centre is using our online registry collection software, your personal data are stored at the centre, and only the anonymous data described above are sent to us. All data are encrypted and password protected.

You can participate via a national registry that collects data from the centres and sends us all the data from that country. This is the case for UK, France, Germany, Belgium, Denmark, Greece, Norway, Russian Federation, Hungary, Türkiye, Ireland,  The Netherlands, Italy, Czech Republik and Sweden. In these countries (almost) all centres participate.

In all other countries, the centres use our online software. New centres join us all the time; if you don't know if yours has already done that, ask at your centre or send an email to info@ecfregistry.eu and one of our team will get back to you.

Direct access to the data is allowed only to the CF centres. This means that only your doctor can enter and modify your data. The biostatisticians in charge of data management and data analysis can see the database but cannot modify your data and cannot identify you, because your identity is protected by the unique code known only to your centre.

If your data are entered directly from a single centre, the centres of a country can appoint a national coordinator who will then get access to anonymous data from all the centres in that country. This way your country will also have a national registry.

Researchers can apply for data for specific projects. The requests are reviewed by a Scientific Committee to ensure that your data are used in accordance with the legislation and the aims of the registry as stated in our guidelines. The scientific committee includes a representative appointed by the CF Europe patient organisation. If permission is granted, the data will be analysed in cooperation with the researchers and our biostatisticians.

The annual reports from the ECFSPR can be found here. You can also find a list here of the many published scientific articles that have made use of the data.

First check with your local CF centre. Maybe they are in the process of joining. In this case, be patient! Getting local data protection approval sometimes takes a very long time and you also need to be informed and give your consent (if you are a minor your parent/s or legal guardian will consent for you). We regularly contact centres that have not yet joined the registry, but we do not have contact details of everyone; you are welcome to send to us their your centre name and city, and we will contact them. It can be very powerful if people with CF urge their centres to join the registry. Please feel free to talk to your CF team about this.
For all enquiries, send an email to info@ecfregistry.eu.

There are CF Europe representatives in the Scientific Committee and the Executive Committee of the ECFSPR; they help to ensure that the actions and governance of the Registry are in the best interests of people with CF and also raise awareness of the Registry amongst the community of people with CF.