Call for European Cystic Fibrosis healthcare gap to be closed

Pan-European research led by the University of Dundee has led to an impassioned plea being issued to close a healthcare gap amounting to a 'death sentence' for Cystic Fibrosis (CF) children born in Eastern Europe.

Dr Anil Mehta, from the University's Centre for Cardiovascular & Lung Biology, led a team from 35 countries that examined outcomes for almost 30,000 CF sufferers. The outcomes for those born in long-standing European Union member states were compared to those born in countries who joined after expansion in 2003.

The resulting paper, published in this week's edition of The Lancet, 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.'

The findings were welcomed by Birgit Dembski, Secretary of Cystic Fibrosis Europe, an organisation that campaigns for equal access to care for CF patients.

'Their study illustrates the daily experience of CF-affected families in Eastern European countries - care is underdeveloped and insufficient,' said Birgit, herself a CF patient.

'It makes me feel helplessly angry to know that the place where you were born determines whether you have a chance to live or not. To be born in certain countries is still a sentence for premature death.'

Karleen De Rijcke, CFE President, added, 'Politicians must become aware of and accept the special medical needs CF patients have and then start to implement appropriate treatment and care.'

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,' he said. '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 Fibrosisis a common hereditary disease which affects the entire body, causing progressive disability and often leading to early death. The name of the disease refers to the characteristic scarring (fibrosis) and cyst formation within the pancreas which is destroyed in foetal life. Difficulty breathing is the most serious symptom and results from frequent lung infections.

It is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which helps create sweat, digestive juices, and mucus. 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 led by Dr David Sheppard of the University of Bristol.

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% 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.'

Ian Hudghton, Member of the European Parliament for Scotland, also praised the research and called for action to be taken to close the CF healthcare gap, saying, 'This eye-opening study underlines that a great deal of work has still to be done to ensure that all EU citizens can have equality of opportunity.

'Armed with the detailed knowledge that this study provides, MEPs must work with EU institutions and the relevant member states governments to seek improvements in life-expectancy rates among Cystic Fibrosis patients.'

Case Study

Donna Todd (26) is mother to three-year-old Lucy and Emily (six months) as well as working as a primary school teacher. Despite being born with Cystic Fibrosis, the support of her family and the care she has received at Dundee's Ninewells Hospital, she has been able to lead a normal and fulfilling life.

She strongly believes that all children born with CF should be able to enjoy the same opportunities as she has had.

'I feel it is very important that no matter what your health is, you should be able to have the life you want to have,' she said. 'The treatment I had, both in paediatrics and in adult care, is very advanced and we're lucky to have the exceptional care available for us.

'It is only right that everyone has the same chances regardless of where they are from.'

Donna's sister Karen, also a Cystic Fibrosis sufferer, tragically died in 1992 at the age of 12. Their parents Shirley and Jim Wilson have been raising money for various CF charities since the girls were young children, and - along with Donna's husband Kevin and various other family members and friends - have now raised well over £30,000.

It was the efforts of the Forfar family that enabled Dr Mehta to set up the first database of CF patients in Tayside, which contributes to this latest research.

Tayside is home to a number of very active CF parents, many of whom lost children to the disease, who kept up their efforts to raise awareness and funds. It is thanks to their tireless efforts to raise funds, both locally and through the UK CF Trust, that Dr Mehta's team has been able to undertake work that has gone on to make an international impact.

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