Why do I cough up chunky phlegm
Cystic fibrosis is an inherited metabolic disease that predominantly damages the lungs, pancreas, small intestine and liver. The cause is a congenital defect in the gland cells, whereby a tough mucus is formed, which can only be removed with difficulty via the respiratory and secretion transport routes of the other organs. Chronic cough and recurring infections up to the deterioration of organ functions are the result.
Cystic fibrosis is currently an incurable disease from which around 8000 people in Germany suffer. 1 in 2000 newborns is born with the disease. This corresponds to approx. 300 children per year who are born with cystic fibrosis in Germany.
Causes and origins
The cause of the disease is a genetic defect that is inherited in a recessive manner. Recessive means that both parents have the diseased gene in them (but neither must be diseased) and both must pass it on to their child in order for the disease to develop. About 4% of Germans carry this diseased gene inherited from one of their parents in their genetic makeup, but are healthy because only if the gene is present on both chromosomes (humans have two of each chromosome, one from their father and one from their mother cystic fibrosis develops.
Due to the genetic defect, channels of gland cells that are responsible for transporting chloride out of the cell are produced in a modified manner. Due to the outward transport of chloride and the associated transport of water from the surrounding tissue, the mucus produced by the gland cells is made liquid. If, due to the defective channel in cystic fibrosis, not enough chloride can be transported out of the cell, the glandular secretion remains thickened. This thick mucus cannot be transported properly from the lungs (or from the pancreas and the small intestine to the inside of the intestine) by the cilia of the trachea and bronchi, which leads to secretion congestion. This congestion damages the affected organs by reducing the removal of metabolic products, pathogens, etc.
The accumulation of secretion in the lungs causes chronic coughs and is a suitable breeding ground for bacteria and fungi. In particular, pathogens that the healthy lungs can fight off without problems can lead to severe and frequently recurring pneumonia in cystic fibrosis patients. Due to the high stress to which the lungs are exposed due to the increased inflammation, the disease can lead to increasing weakness of the lungs (lung insufficiency), which leads to shortness of breath and chronic oxygen deficiency in the body. In addition to the already mentioned shortness of breath, the patient can see the so-called watch glass nails (thickened and rounded fingernails) and drumstick fingers (fingers, with thickening in the terminal quarter) at this stage.
As already mentioned, other organs are also affected by the reduced evacuation of the secretion. The digestive enzymes produced in the pancreas cannot reach the intestines in sufficient quantities, which leads to malnutrition on the one hand and chronic diarrhea due to the ingested substances that remain in the intestine and draw water from the surrounding tissue. Furthermore, due to the weak pancreatic secretion, 50% of the sick adults develop diabetes mellitus. Cirrhosis of the liver, the increased occurrence of gallstones and infertility, especially in men, are symptoms of the disease.
A test that is specifically available for diagnosing cystic fibrosis is the pilocarpine iontophoresis test. With this the chloride content of the sweat is measured. If this is above 60mmol / l in adults (in newborns> 90mmol / l), it can be assumed that the disease is. Furthermore, nowadays the trypsinogen content in the blood can be determined for examination in newborns. In order to assess how severely the lungs are already affected by the disease, a lung function test is carried out in addition to measuring the oxygen content in the blood. The function of the pancreas and liver can also be assessed using suitable blood values.
By optimizing therapeutic care, the life expectancy of cystic fibrosis patients has increased significantly in recent years. Life expectancy, which until 20 years ago only extended into adolescence, is now around the fourth decade of life. Since cystic fibrosis affects the function of several organs and is sometimes not yet curable, each organ must be treated symptomatically individually. Regarding the digestive tract, patients are given high calorie food and digestive enzymes in tablet form to increase weight. To loosen the viscous mucus from the lungs, expectorants are administered and inhalations, as well as specially developed respiratory therapies (autogenous drainage, tapping massage) are prescribed. To avoid severe respiratory and lung infections, high-dose antibiotics are administered via the vein every three months for a period of 14 days. Furthermore, if a respiratory tract infection is suspected, targeted antibiotic treatment is given early on. Long-term oxygen therapy is recommended for the increasing weakness of the lungs.
Organ transplantation is possible in the case of advanced disease with life-threatening lung or liver and pancreas failure.
Find out more about lung transplants in Jena
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