It is time to celebrate more birthdays. Patients with cystic fibrosis, an orphan disease affecting fewer than 200,000 individuals throughout the world, are experiencing an increase in longevity. Estimates from a study out of Queen’s University Belfast in the United Kingdom predicts a nearly 80% increase by the year 2025 in the number of cystic fibrosis patients living into adulthood.
“The estimations we have made show very positive news for cystic fibrosis patients as the average survival age is increasing,” said lead author of the study, Dr. Stuart Elborn, Dean of the School of Medicine, Dentistry, and Biomedical Sciences, in a news release.
“Future Trends in Cystic Fibrosis Demography in 34 European Countries,” published in the European Respiratory Journal, was a unique study that forecasted the number of adult cystic fibrosis patients by 2025. Researchers in Belfast and their collaborators analyzed data from the European Cystic Fibrosis Society Patient Registry, with a specific focus on data from countries with extensive cystic fibrosis population coverage. With this data, the team compiled a model that indicated an overall increase of 50% in the cystic fibrosis patient population. This represented increases of 20% and 75% in children and adults, respectively.
Patient survival has been enhanced due to improved treatments within the last three decades. Previously, children had a low life expectancy, but many are now living into adulthood with an average age of approximately 40 years. In this next decade, 75% more patients are expected to survive into adulthood.
Although this is excellent news for cystic fibrosis patients and the research community dedicated to the disease, some experts are concerned about the preparedness of institutions that treat cystic fibrosis patients. “We are now concerned that there are insufficient specialist centers to provide optimal care to adults with the disease,” said Dr. Elborn. “It is crucial that we take note of these early predictions–which are conservative in nature and the likelihood is that the real figures will be higher–and adapt the [National Health Service] to this change.”
Since many centers are focused on pediatric care for cystic fibrosis, adults may lack the care they need. “Although this is good news in one sense, these figures pose a major challenge to healthcare providers in that adult cystic fibrosis services will need to be developed quickly and effectively to meet demand,” said Dr. Patrick Johnston, Vice-Chancellor of Queen’s University Belfast.
Fortunately, Queen’s University Belfast is equipped to face the challenges of advancing knowledge within the cystic fibrosis field and contribute to helping the increased number of adult cystic fibrosis patients. According to Dr. Johnston, “Queen’s was recently ranked amongst the Top 10 institutions in the UK for research intensity based on the results of 2014 Research Excellence Framework. This study is yet another example of how Queen’s researchers are continuing to advance knowledge and change lives.”