Life expectancy in cystic fibrosis

Assessment of the prognosis in cystic fibrosis

Although the Cystic fibrosis until today one incurable Disease, the life expectancy of patients has increased significantly in recent years. Since 1999 the average life expectancy has risen from 29 years to 37 years today. This is not least due to the numerous new and progressive therapy options.

Especially those Lung function the patient has improved significantly in recent years. While 20 years ago only about 30% of all cystic fibrosis patients still had an almost normal lung function, today about 60% of the patients still have a largely normal lung function when they reach adulthood.

This is based on the one hand on the general therapeutic measures for the treatment of cystic fibrosis, on the other hand on the numerous possibilities to prevent common infections of the bronchial system or to treat them effectively. Since in most cystic fibrosis patients the cause of death is directly an infection of the lungs or one of the resulting complications.

Also thanks to the generally improved Living conditions, a good one nutrition and the effective treatment of the frequently occurring Diabetes mellitus the life expectancy of patients can be increased further. Thanks to the continuously developing diagnostic possibilities, affected children are diagnosed at a very young age and can therefore be treated very early in specialized centers. For newborns born today with this disease, an average life expectancy of between 45 and 50 years of all things. Overall, women have a slightly lower life expectancy than men for reasons that have not yet been clarified. Most people with cystic fibrosis died in adolescence around 1980; only about one in 100 lived to see their 18th birthday. Today around half of those affected live to be 18 years or older. The mean life expectancy is currently between 32 and 35 years.

Thanks to today's therapy, only slightly ill patients can lead a largely normal life with a normal life expectancy and father children. The course of the disease, however, is very individual different and thus make an exact forecast very difficult. The most important measure for effective therapy and the highest possible quality of life lies in early diagnosis and consistent and special treatment in centers for cystic fibrosis.

Scientists are also currently conducting studies to try to replace the causal diseased gene with a healthy one. Through this Gene therapy one hopes to be able to cure the disease at some point and thereby to be able to considerably improve the life expectancy of the sick.