Cystic fibrosis is perhaps the most common inherited disease on Earth. It was first described by an American pediatrician Dorothy Anderson in 1938. The official name is pancreatic fibrosis .
Cystic fibrosis: what is it?
The occurrence of the disease is associated with a protein mutation that performs the function of the chloride channel and is actively involved in the water-electrolyte balance of the cells of the respiratory organs, gastrointestinal tract, pancreas, genitourinary system and liver. With irreversible changes in the protein, the discharge of a large number of
glands of external secretion is greatly thickened, its secretion is much more difficult, violations occur in the internal organs. Cystic fibrosis often affects the bronchopulmonary system.
In the walls of blood vessels, severe inflammation of various forms of severity develops here, the connective framework is destroyed, and bronchiectasis is quickly formed. With constant blockage of fairly viscous sputum, bronchiectasis becomes more frequent, with time, hypoxia and pulmonary hypertension increase .
Cystic fibrosis: what are these and what are the symptoms?
It is worth noting that the symptoms of the disease may not appear immediately. Doctors often begin to treat ordinary bronchitis, but in the end it turns out that this is not at all, but cystic fibrosis. The help is then of a completely different nature. Suffering from this disease are characteristic of:
- significant lag in physical development;
- chronic diseases of the respiratory organs (including sinusitis and bronchitis);
- polyps in the nose;
- pancreatitis
- respiratory failure.
Cystic fibrosis: what is it and how is it diagnosed?
Of course, to make a correct diagnosis, you just can not do without a proper examination. It is better to approach the problem comprehensively. Tests that are prescribed by a doctor to detect cystic fibrosis:
- sweat test;
- chloride concentration;
- chymotrypsin in the stool;
- fatty acids in the stool;
- DNA diagnostics.
Cystic fibrosis: what is it and how is it treated?
At the moment, it is impossible to completely defeat this disease. But if help to patients with cystic fibrosis is provided on time, then even with this diagnosis a person can live a fairly long life.
All treatment of patients consists in a good diet and physical activity. Diet and load are set by a specialist individually. And then the treatment of cystic fibrosis proceeds based on the symptoms. The main goal of doctors here is to prevent obstruction of the lungs and avoid gastrointestinal obstruction. All patients are registered in the dispensary.
The main prescribed drugs: enzyme, antifungal and antibacterial agents. As physiotherapy, vibration massage of the entire chest and special gymnastics are often prescribed.
Is there any chance?
According to some reports, the average life expectancy of a patient in Russia is 15-18 years. In the USA, for example, with a similar diagnosis, the patient lives on average up to 28-42 years.
But medicine, as you know, does not stand still. Scientists around the world are carefully studying this disease. As many of them believe, to patients born in 2000, modern medicine may well guarantee 45-55 years of full life.