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Registry and cystic fibrosis in figures

registry

Patients suffering from cystic fibrosis in France

In France the number of cystic fibrosis (CF) patients is estimated at 7500 with as many men as women. Every year the French CF Patient Registry collects data to evaluate the characteristics of the population.
In 2016, 6,757 patients were recorded by the centres participating in the French CF Patient Registry (about 90% exhaustiveness).
 
The geographic distribution of patients shows a disparity among the departments with the majority of patients concentrated primarily in a north-west arch (the Nord-Pas-de-Calais, Haute and Basse-Normandie, Bretagne and Pays de la Loire regions), and secondarily, on an eastern arch (the Lorraine, Alsace, Franche-Comté, Rhône-Alpes and Provence-Alpes-Côte d’Azur regions).
The proportion of adults continues to increase: they represented 19% in 1992 and now account for 55% of patients (in 2016), even though the population remains structurally young (average age 21.9 years).

Age at diagnosis is less than 2 months for 50% of patients.  
In 2016, 177 new patients were diagnosed: 10 by antenatal diagnosis, 134 after neonatal screening and/or 41 due to other symptoms (primarily respiratory or digestive).

The worldwide scientific community has been active for several years now in terms of discovery of drugs treating the symptoms or attacking the disease at its source. Considerable progress has been made in the treatment of symptoms and current research aims to improve this strategy. At the same time, the concerted efforts in basic research are enabling the implementation of clinical trials on medicines intended to treat the source of the disease.

 

Cystic Fibrosis Registry history

The ONM (French Cystic Fibrosis Monitoring Centre) created in 1992 on the initiative of the association, became the French Cystic Fibrosis Patient Registry in 2006.

By supporting the creation and management of the Registry, the association aims to provide an exhaustive, high-quality database for researchers to facilitate studies and help in the advancement of research. More specifically, this tool has various objectives:

1. Descriptive and analytical epidemiology
2. Help for identifying priority issues in terms of care and research and for setting up clinical trials.
3. Evaluation of healthcare practices
4. To guide parents and patients in their personal choices and to guide the association and other institutional partners in their strategic choices
 
Data collection is carried out using a questionnaire comprised of three forms, filled out once per year by healthcare centres. The sending and receipt of questionnaires, as well the quality controls of the data, are ensured by the “Vaincre la Mucoviscidose” association. Analyses are carried out in conjunction with the INED (French National Institute of Demographic Studies).
 
A Steering Committee was established in 2009 with the aim of providing a strategic vision and arbitration in relation to scientific projects. 
 
In 2009, the Registry was qualified by the CNR-MR (French National Committee of Registries - Rare Diseases) for 3 years, renewed for 4 years in 2011. 

*The information provided on this website is intended to improve, not to replace, the relationship that exists between the patient (or visitor to the website) and healthcare professionals.

©Fotolia -  Sergey Nivens