Cystic Fibrosis

Definition of cystic fibrosis

also known as: cystic fibrosis

Cystic Fibrosis Hereditary enzyme disease, in which a tough mucus is produced, which closes the ducts of glands and thereby leads to degeneration (remodeling) and inflammation of the affected organs. With a disease on 2,000 births, cystic fibrosis is one of the most common hereditary diseases. In Germany, about one in 30 carries the recessive cystic fibrosis gene and is therefore a potential carrier of the disease.

The mucus-producing glands (pancreatic, intestinal and bronchial glands) as well as the sweat and tear glands are particularly affected. Whereas 80 percent of infested children died from complications of the intestine and lungs within the first year of life, today more than half of the patients are over the age of 18, and in well-managed groups of patients as much as 80 percent. In all major cities, there are special outpatient clinics for children with cystic fibrosis.

The first indication of the disease may be the appearance of an intestinal obstruction (ileus) in the newborn through a plug of mycelium (meconium). Later, frequent, severe respiratory infections and pneumonia in the foreground, in which the patient can cough up the tough mucus only with difficulty. As a result, too little oxygen gets into the blood, which is reflected in a bluish discolouration (cyanosis) of fingertips, toes and lips as well as in piston-shaped widened finger tips, so-called drumstick fingers. Also in the pancreas creates a tough secretion that closes the excretory duct. In addition, voluminous, bad-smelling, shiny stools may occur. Male cystic fibrosis patients are infertile. Rarer symptoms are skin bleeding, which is based on damage to the liver, which produces in the healthy a substance essential for the blood clotting. In addition, cystic fibrosis is typical in that children with discomfort sweat profusely at the slightest effort and excrete large quantities of minerals that are urgently needed for many bodily functions. The consequences are low blood pressure, dizziness and fainting fits.

Diagnostically, changes in the abundant secreted sweat are characteristic: it has a greatly increased salt content, which can be determined by laboratory techniques. X-ray examinations of the lung show typical changes. The stool often contains undigested fat.

The treatment primarily serves to improve lung and pancreatic function. Already with the baby one can support the removal of the viscous mucus by special storage and tapping massages. Later, targeted breathing techniques, expectorant drugs and the inhalation of drugs that enhance the bronchi and thereby facilitate coughing help. For respiratory infections, antibiotics must be used in a targeted and timely manner. If the pancreas is impaired in its function, a high calorie diet with sufficient protein content is necessary, otherwise it can lead to significant nutritional disorders. The missing enzymes and the fat-soluble vitamins must be replaced by medication. Frequently hospitalizations are required, in which one tries with a complex care of the lungs, to avoid life-threatening complications or at least defer.

However, even with consistent treatment, sooner or later an uncontrollable lung distension occurs in most cases, and in many cases the course of the disease is complicated by cirrhosis of the liver. A common cause of death is infections with bacteria that are resistant to antibiotics. In heavily attacked lungs, a lung transplantation should be considered in young adulthood.

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