Cystic fibrosis - Prevention & prognosis

Print Size

How can cystic fibrosis be prevented?

Prevention

Because cystic fibrosis is a hereditary disease, no prevention is possible. Early detection and initiation of treatment is important for a favorable course. Many complaints can be alleviated and complications (such as infections) avoided. Although cystic fibrosis continues to be lethal, life expectancy has risen significantly. Those affected often reach 40 years of age. Experts estimate that children who are born today with cystic fibrosis will be able to live until they are much older.

Prognose

The disease is incurable and leads to a reduced life expectancy and quality. Without therapy, patients usually die within a few years. A timely and targeted treatment significantly slows the progression of cystic fibrosis. Many patients turn 40 or even over 50 years old.  But even with optimal therapy complications can appear over and over again, most often it is acute respiratory distress through a poorly ventilated and sometimes even collapsed lung (atelektasis). Often accompanying diseases develop, such as:

  • Chronic bronchitis
  • Pulmonary infection (pneumonia)
  • Fungal infections of the lungs
  • Circulatory failure (due to fluid and electrolyte disturbances)

Other complications and secondary diseases are

  • Chronic liver disease, especially liver cirrhosis
  • Gall bladder infections and bile stones
  • Diabetes mellitus 
  • Chronic pancreatic inflammation
  • Heart disorders
  • Intestinal obstruction (ileus)
  • Intussusception (Invagination)
  • Malnutrition
  • Limited fertility in women, or infertility in male patients

If you have increased family-related risks and wish to have children, you should always ask for genetic counseling to be on the safe side. Here a genetic test is used to determine whether there is a change in the CFTR gene. The risk for the offspring can be calculated on the basis of the results.

Meanwhile, preimplantation diagnosis (PID) is also possible in Germany for mucoviscidosis. However, an approval from the ethics committee is always needed for this purpose. The eggs are fertilized outside the mother's body and only embryos without the problematic genes are used.