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Babies with cystic fibrosis live longer in the EU

Better diagnosis and access to care means CF patients die later in the EU

OnMedica staff

Friday, 19 March 2010

Patients with cystic fibrosis (CF) living in European Union (EU) countries survive for longer than CF patients in neighbouring non-EU countries, shows a study published in The Lancet tomorrow.

One of the main findings from this large, multinational study is that there are disproportionately fewer than expected CF patients in non-EU countries. The authors suggest that if CF patients in these non-EU countries lived as long as their counterparts in EU countries, the CF population in non-EU countries would rise by an estimated 84%.

The authors of this study, Dr Anil Mehta and Dr Jonathan McCormick and colleagues at the University of Dundee, analysed data from 35 European countries on CF patients’ age, age at diagnosis, sex, and genotype. They looked for demographic differences between countries that were EU members in 2003, and non-EU member countries (many of which are now full EU members).

There was a large difference in CF patients’ median age – 17.0 years in EU countries and 12.2 years in non-EU countries. The proportion of patients older than 40 years was also significantly higher in EU countries (5%) than in non-EU countries (2%). The authors estimated that the CF population in non-EU countries would increase by 84% if their CF patients had a demographic profile comparable with that in EU countries.

The authors say: “Far fewer children and young adults have cystic fibrosis in non-EU countries than we expected. This finding is reinforced by the increased chance of patients surviving to 40 years in EU countries, even if they have the [most common and severe form of CF] mutation.”

They add: “The difference between the number of patients with cystic fibrosis between EU and non-EU countries is striking in view of the similarity of general population sizes and the expected disease prevalence [from genetic calculations]. This disparity in demographic indicators might be due to reduced availability of specialist drugs, equipment, and trained multidisciplinary staff in non-EU countries, rather than lower gene frequency, greater disease severity, or poorer treatment adherence than in EU countries.”

The authors note that diagnoses of children younger than 1 year are scarce in non-EU countries. They say that although this could be caused by insufficient data on disease incidence, they believe it is more likely to be a result of deaths from unrecognised CF – which used to happen commonly in the EU in the 1970s.

They add that as well as unrecognised CF cases, misclassification of infant deaths resulting from CF, and inequality of access to health care leading to early death, could be responsible for the profile of CF in non-EU countries. They conclude: “In our study, both factors are likely to affect new members of the EU, and these matters need urgent attention by governments.”

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