The largest study to date of cystic fibrosis (CF) says that people suffering from the disease live longer in some European countries than in others, mainly due to earlier mortality and differences in access to care and its quality. The research, which was supported under the Sixth Framework Programme (FP6), is published in The Lancet journal.
The study, undertaken by a research team at the University of Dundee, UK together with European colleagues, was carried out to determine if differences of care and available resources have a significant effect on the survival rates of CF patients. The results showed that there are disproportionately fewer CF patients than expected in countries that were not part of the EU in 2003. According to the findings, if these non-EU countries had similar demographic profiles to EU countries, their number of CF patients would rise by 84%.
There is no cure for CF, which is one of the most common inherited diseases. It is caused by a genetic mutation and can occur in an apparently healthy baby if both its parents are carriers of the defective CFTR (cystic fibrosis transmembrane-conductance regulator) gene.
Symptoms in children include slow growth and poor weight gain, chronic coughs and lung infections, insufficient absorption of nutrients, and bowel and intestinal problems. Many sufferers die young in their 20s and 30s, often from lung disease resulting from mucus build-up in the airways.
Researchers gathered data, including age at diagnosis, sex and genotype, on 29,025 CF patients from 35 European countries. This data was used to create a register to compare differences between patients in EU and non-EU countries and to estimate how many people in Europe have CF. Throughout the study, the team used a country's EU membership status as it stood in 2003 (when there were 15 EU Member States).
The study showed that the average age of CF sufferers throughout Europe is 16.3 years with a difference of 4.9 years between EU (17 years) and non-EU countries (12.1 years). The proportion of patients over the age of 40 was also higher in the EU countries (5%) than in non-EU countries (2%). In addition, the diagnosis of CF in children under one year of age is rare in non-EU countries, something that could be due to a lack of data or to unrecognised deaths of babies with CF.
The authors of the study said, 'We have shown that 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 added, '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 are concerned that both unrecognised CF cases and misclassification of infant deaths could be responsible for the low CF figures in non-EU countries. They concluded, 'In our study, both factors are likely to affect new members of the EU and these matters need urgent attention by governments.'
Countries included in the study that were already members of the EU in 2003 are Belgium, Denmark, Germany, Ireland, Greece, France, Italy, the Netherlands, Austria, Portugal, Sweden and the UK. In addition, the Lancet study focused on the following nations: Armenia, Belarus, Bosnia and Herzegovina, Bulgaria, Croatia, Cyprus, the Czech Republic, Estonia, Georgia, Hungary, Iceland, Israel, Latvia, Lithuania, Macedonia, Moldova, Romania, Russia, Serbia, Slovakia, Slovenia, Turkey and Ukraine.
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