Differing National Healthcare Systems Affect Survival of Cystic Fibrosis Patients

Wednesday 17 April 2013, Amsterdam

National healthcare practices affect the diagnosis rate and survival rate of any disease, and CF patients can sometimes experience substantially different lives depending on the country they are born and treated in, states a new report by research and consulting a firm. The new report shows that CF patients in the US are more likely to live well into adulthood compared to several decades ago, and expect a median survival age of 37 years, showing a dramatic improvement of over the last 25 years. The CF patient population’s average survival age in the US has steadily increased due to early  diagnosis and treatment with all 50 states screening for CF in newborns. As a result of this stringency, the National Cystic  Fibrosis  Patient  Registry  reported  that  75.7%  of  patients with  CF  suffered  no  medical complications. Similarly to the other markets, Spain’s median age of death has increased in CF patients, with early screening and detection resulting in earlier treatment. The most common cause of CF-related death in Spain is respiratory failure, but improved treatment, lung transplants, and nutrition control is successfully tackling this problem.

In comparison, Germany boasts a relatively low crude mortality rate compared with most countries, but we believe that this may be due to CF deaths being underreported, undiagnosed, or undertreated. Chronic lung disease CF is caused by the defective cystic fibrosis transmembrane conductance regulator (CFTR) gene, which is more common in Caucasian populations and leads to thick, sticky mucus accumulating in the respiratory, digestive, and reproductive systems. Lung disease is the primary cause of death in CF patients, as the organs become susceptible to bacterial infections and inflammation, leading to respiratory failure. CF is widespread throughout the world, but is most common across the US and Europe. The World Health Organization (WHO) states that CF occurs in one in 2,000–3,000 births in the European Union (EU) and one in 3,500 births in the US. In comparison, African-Americans have a CF prevalence of one in 15,000. The UK and France have the highest prevalence proportion of CF, suggesting that CF-causing mutations originate from British and French ancestry, though the US has the largest disease population, with approximately 30,000 people affected by the disorder.However, diagnosed cases of CF have recently been increasing in Latin America, the Middle East, and the Indian subcontinent, likely due to the emigration of Western Europeans to these regions. Forecasts that total prevalent cases of CF in individuals in the 0–35 year age group will grow from 58,056 in 2012 to 68,373 in 2022, at an average annual growth rate of 1.78%.