EuroCareCF, led by Dr David Sheppard of the University of Bristol, provided a forum for groups from all over Europe to collaborate to improve the quality of life and life expectancy of individuals with CF.

As part of this collaboration, Dr Anil Mehta of the University of Dundee led a team from 35 countries that examined outcomes for almost 30,000 CF sufferers born in long-standing European Union member states compared to those born in countries who joined after expansion in 2003.

The resulting paper shows that despite similar population sizes and underlying gene frequencies for CF, the numbers of CF-affected children were lower in post-expansion EU member states.

Dr Mehta says this disparity can most likely be explained by the tragic fact that a lack of healthcare facilities in new member states means that the majority of children born with CF in these countries will die in very early childhood, a situation not encountered in the wealthier EU countries for many decades.

“We know that this disease occurs randomly in about 1 in 4,000 children born to healthy parents across the EU,” he said.  “Despite this, the team encountered many fewer people with CF in poorer countries.  CF patients there die far younger than in long standing EU countries.”

Nick Fahy, head of the health information unit in the Health and Consumers Directorate General at the European Commission, said, “Knowledge is key to improving health in Europe.  For these rare diseases, there are so few centres of expertise that only by working together across the EU can we enable all citizens to have access to the best possible care.

“This European cooperation also shows member states how they compare to best practice in Europe for different conditions, so that every health system can prioritise their resources to meet the needs of their patients."

Cystic fibrosis is an inherited chronic disease that affects many organs, particularly the lungs and digestive system.  CF patients carry a defective gene that disrupts the transport of salt across cell borders.  As a result, the body produces thick mucus that blocks ducts and tubes.

Blockage of air passageways causes chronic cough and lung infection; blockage of the pancreas prevents enzyme delivery to the intestine to break down food; and blockage in the intestine prevents food absorption.  Countries in Western Europe have committed significant resources to making the necessary treatment available to CF sufferers, helping them to live longer and fuller lives.

The study was completed in conjunction with Professor Milan Macek of Charles University, Prague, with support from the European CF Society through experts from universities in Denmark and Italy.  The work was funded by the European Commission Sixth Framework programme as part of the EuroCareCF Coordination Action for Cystic Fibrosis.

The median age of CF sufferers from established EU countries was 17 compared to 12.1 years of age in newer EU member states.  The team estimated that the current CF population of non-EU countries would rise by 84 per cent if they had a CF demographic profile comparable to those countries who were already EU members in 2003.

“We have to act to redress this imbalance if we are serious about ensuring the equality of healthcare for all EU citizens,” continued Dr Mehta.  “We need our politicians to act to end this death sentence for CF sufferers in the newer EU states.

“The research findings imply that children who live in countries that either are unable or unwilling to fund treatment, or who have prioritised other diseases due to a lack of funds, will likely experience a more negative outcome.

“Without appropriately targeted treatment, these CF children will continue to die in childhood in these new member states.  This used to happen in the UK before the proper treatment and healthcare was provided for children born with CF.”

Paper: Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis.  Jonathan McCormick, Gita Mehta, Hanne V Olesen, Laura Viviani, Milan Macek Jr., Anil Mehta on behalf of the European Registry Working Group Lancet 2010 Mar 19; 375:1007-1013.

EuroCareCF was a €1.75 million project funded by the European Commission (EC) Sixth Framework programme for 3½ years.  The major goals of the project were to improve the survival and quality of life of Cystic Fibrosis patients, and to optimise clinical management and therapy development.

Led by the University of Bristol, EuroCareCF has united groups in the UK, Belgium, Czech Republic, France, Germany, Italy, Poland, Portugal and The Netherlands.

Working with the European Cystic Fibrosis Society, participants of the EuroCareCF project, developed a European cystic fibrosis patient registry, a European cystic fibrosis clinical trials network, trained clinicians, healthcare professionals and researchers, distributed resources for research and patient care and developed best-practice consensus guidelines spanning all aspects of cystic fibrosis.

UK universities played a leading role in EuroCareCF.  Bristol provided scientific leadership; Cambridge contributed expertise in animal models; Dundee spearheaded the development of a database of European CF patients and Belfast led on standards of care for adults with CF.