Cystic fibrosis - Description

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What is cystic fibrosis?

In mucoviscidosis (cystic fibrosis) the formation of various body fluids is disturbed by a genetic defect. It is a hereditary metabolic disease in which the secretions of the lungs, pancreas and other organs are more viscous than in healthy people. This thick mucus obstructs numerous passages of internal organs, among others, also the small bronchi of the lungs. That leads to massive disorders, especially in breathing and digestion.

Because a genetic defect is the cause of mucoviscidosis, it is not curable. Both the severity of the symptoms and the time of the diagnosis can vary widely. Frequently, the disease is immediately noticeable in children from birth. Sometimes it is also diagnosed only later.

Without treatment, the patient's health deteriorates continuously. An early therapy can significantly slow progress of the illness, if not completely stop it. The treatment methods have significantly improved over the years. Nevertheless, life expectancy is shortened compared to that of healthy people.

Mucoviscidosis is one of the most frequent congenital metabolic disorders in Europe and North America. 8,000 people are affected in Germany. The child falls ill only if both parents pass on a diseased gene. If only one defective gene is inherited, there are no complaints. About every 25th person has a predisposition for mucoviscidosis without knowing it.