Over the past number of years, the life expectancy of patients with cystic fibrosis (CF) in the Netherlands has improved considerably.
In addition, genes have been discovered that are responsible for less severe inflammations in CF, which results in less lung damage and improved life expectancy. Martijn Slieker describes this in his doctoral dissertation. Patients with CF, which is a life-threatening condition, suffer from severe respiratory infections and digestive problems. Only 10 percent of patients born in the 1950s lived to the age of 25. Since then, survival rates have improved considerably: 60 percent of patients who were born in the 1970s now reach this age.
Also, fewer and fewer babies are being born with the disease. From 1961 to 1965, CF was diagnosed in one out of every 3,600 live-born children. Between 1974 to 1994, this dropped to one out of every 4,750. CF is characterized by an excessive inflammatory response. A study of more than 300 CF patients in the UMC Utrecht shows that variations in the genetic material of a number of inflammatory proteins result in milder inflammatory responses, and because of this the course of CF is less severe. For example, lung function in patients with variations in the genetic material of the TLR4 protein is 18 percent better than that of patients without such variations.
Martijn Slieker will receive his PhD from Utrecht University on June 26. The title of his dissertation is “Prognosis in Cystic Fibrosis: Trends and Predictors.”