Cystic fibrosis – what is this disease This is a genetic pathology that is inherited according to the autosomal recessive type, that is, only in those cases when the defective gene is carried by both parents. Disruption of the secretion of exocrine glands is characteristic of the disease. With cystic fibrosis, the respiratory and digestive systems are mainly affected.
Mutation of genes causes a violation of the protein structure, as a result of which the secretion of exocrine glands becomes thick, its evacuation is difficult, and secondary changes occur in the organs. Sputum in the bronchi becomes viscous, a chronic inflammatory process develops, bronchiectasis is formed. Over time, these changes lead to hypoxia and the formation of a pulmonary heart.
The cause of cystic fibrosis — a defect in protein synthesis and, as a result, its inability to adequately perform the work of the chloride channel, due to which secondary changes occur in the organs. Cystic fibrosis is a systemic disease, but in 95% of cases the prognosis is determined by bronchopulmonary changes. The gastrointestinal tract, heart and other organs are also affected.
Symptoms of cystic fibrosis in children 1-2 years old are as follows:
- cyanosis;
- cough;
- shortness of breath;
- discharge of thick sputum.
With the pulmonary form of cystic fibrosis, a clinical picture of obstructive bronchitis, pulmonary emphysema, bronchiectasis, infection-dependent bronchial asthma develops over time. Read more about bronchial asthma on our website Dobrobut.com. In children aged 5-15 years, cystic fibrosis can be manifested by intestinal colic, flatulence, and paroxysmal abdominal pain. Multiple vomiting is possible.
Symptoms of cystic fibrosis in the pulmonary form of the disease in adults are as follows:
attack-like cough;
- shortness of breath;
- discharge of thick mucous-purulent sputum;
- signs of chronic rhinitis – in particular, nasal congestion;
- pale skin;
- weakness;
- decrease in working capacity;
- cyanosis of mucous membranes.
Extrapulmonary manifestations of cystic fibrosis – this is a violation of the pancreas, liver and bile ducts, gonads.
The choice of treatment method depends on the form of the disease. There is no single protocol for the treatment of cystic fibrosis. An individual treatment scheme is developed for each patient. When the bronchopulmonary apparatus is affected, therapy is aimed at eliminating inflammation and improving respiratory function.
For the treatment of the pulmonary form of cystic fibrosis in adults, the following are prescribed:
- breathing gymnastics;
- postural drainage;
- antibacterial drugs;
- inhalation bronchodilators;
- multivitamins;
- mucolytics.
In the terminal stage of cystic fibrosis, lung transplantation is indicated.
Enzyme preparations, multivitamins with trace elements, and laxatives are prescribed for the intestinal form of the disease. It is important to follow a protein diet with a restriction of fats and carbohydrates, to control body weight.
Treatment of patients with cystic fibrosis is carried out in specialized clinics. Patients regularly undergo medical examinations, pass tests, after which the doctor adjusts the treatment and rehabilitation regimen.
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