Assessment of the prognosis for cystic fibrosis
Although cystic fibrosis is still an incurable disease today, the life expectancy of patients has increased significantly in recent years. Since 1999, the average life expectancy has increased from 29 years to 37 years today. This is not least due to the numerous new and advanced therapy options.
Especially the lung function of patients has improved significantly in recent years. While 20 years ago only about 30% of all cystic fibrosis patients had a nearly normal lung function, today about 60% of 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, and on the other hand on the numerous possibilities to prevent and effectively treat common infections of the bronchial system.
Since in most cystic fibrosis patients, the cause of death is directly related to a lung infection or one of the complications resulting from it. The life expectancy of patients can also be further increased thanks to generally improved living conditions, good nutrition and effective treatment of the frequently occurring diabetes mellitus. Thanks to the constantly developing diagnostic possibilities, affected children are today diagnosed at a very young age and can therefore be treated in specialized centers very early on.
For newborns born with this disease today, an average life expectancy of between 45 and 50 years is even calculated. Overall, women have a slightly lower life expectancy than men, for reasons that have not yet been clarified. As late as 1980, most people suffering from cystic fibrosis died in adolescence, and only about one in 100 experienced their 18th birthday.
Today, about half of those affected live to be 18 years old and older. The average 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.
However, the course of the disease varies greatly from individual to individual and thus makes an exact prognosis very difficult. The most important measure for an effective therapy and the highest possible quality of life is early diagnosis and consistent and specialized treatment in centers for cystic fibrosis. Currently, scientists are also conducting studies in which attempts are being made to replace the causative diseased gene with a healthy one. It is hoped that this gene therapy will eventually cure the disease and considerably improve the life expectancy of patients.
