How dangerous is cystic fibrosis and what is it?
Seriously ill and learned that cystic fibrosis patients are interested in how to treat the disease. Cystic fibrosis is called cystic fibrosis - a genetic disease that disrupts the function of the glands. They begin to produce viscous secretion that leads to the defeat of all vital organs.
The main symptoms of the disease
What is cystic fibrosis and how it manifests itself?
Lungs of patient with cystic fibrosis at birth do not differ from the lungs of a healthy person. However, the violation of their functions can appear at any age. Viscous secretion of the bronchi obstructs their light, which causes inflammation. With the development of diseases of the bronchial walls thicken, the Airways are progressing infection of the pulmonary alveoli stick together. All this leads to stoppage of oxygen in the blood. Every fifth child with cystic fibrosis is marked intestinal obstruction. Meconium in such children is very thick, which hinders its passage through the intestines. Bowel obstruction leads to injury of its walls and volvulus.
First symptoms of cystic fibrosis in children are manifested in the form of malnutrition in the first months of life. A low production of secretions by the pancreas leading to malabsorption of nutrients in the intestine. The child's increasing belly, you receive frequent stools with a pungent odor. The lack of fat-soluble vitamins in the body leads to the development of anemia and rickets. Some children with cystic fibrosis notes rectal prolapse.
To go to the doctor parents makes a permanent cough and frequent colds. The cough may be accompanied by vomiting and insomnia. With the development of diseases of the chest bulges, lack of oxygen leads to a thickening of the fingertips and cyanosis. The maxillary sinuses are filled with thick secret nose polyps arise.
In adolescence, marked slowing of sexual maturation and decreased performance. Cystic fibrosis in adults manifests itself in the form of heart failure, appearance of blood streaks in the sputum, frequent infectious diseases. All this leads to the destruction of lung tissue and death from cardiac and respiratory failure.
Obstruction of the bile ducts causes inflammation of the liver, leading to cirrhosis. This increases the pressure in the hepatic veins. The consequence of such conditions is varicose veins of the esophagus, threatening massiveinternal bleeding.
In cystic fibrosis often develop infertility. Men almost not produced sperm, and also violated their elimination due to improper development of the VAS deferens. Viscous cervical secret in women prevent fertilization. Even if pregnancy occurs in women with cystic fibrosis high risk of complications. However, they have every chance of having a healthy baby.
In hot weather the patient is very much sweating which leads to dehydration and loss of salt. The child may show the salt crystals on the skin.
The causes of cystic fibrosis
This deadly disease affects mainly the representatives of the European race. Many cases do not survive to old age. The cause is a defective gene present in 5% of people. People with one defective gene symptoms not observed in the presence of two developing cystic fibrosis. This gene is responsible for the production of protein and supply of chlorine and sodium into the cells. If you have 2 defective genes, these processes are disrupted, which leads to dehydration and thickening of secretions.
Cystic fibrosis affects almost all the endocrine glands, disturbing their function. The dysfunction of pancreas leads to intestinal form of cystic fibrosis. Sweat and salivary glands produce a liquid containing a large amount of salt. Lung damage occurs due to production of dense mucus glands in the Airways. The severity of the disease depends on the degree of lung injury. The gradual deterioration of the body is inevitable, in the end it leads to death.
Current treatments extend the life of patients and improve its quality. 50% of patients live more than 30 years. Life expectancy in men somewhat more than women. Despite the fact that cystic fibrosis disrupts the functioning of many organs, patients can lead a normal life until the last days.
How to identify cystic fibrosis?
An important method for the diagnosis of cystic fibrosis in children is the determination of the amount of the digestive enzyme trypsin. However, on the basis of its results the final diagnosis is not put. Electrophoretic sweat test measures salt in the sweat. At high concentration of salt in the sweat and other signs of the disease the diagnosis is confirmed.
As the disease affects all organs, for diagnosticsthere are other methods. While decreased production of pancreatic enzymes revealed high content of fat in the stool. In violation of insulin production increases the level of blood sugar.
Parents of a sick child with cystic fibrosis want to know how high the probability of having a next baby is healthy. Genetic analysis of blood helps to identify the defective gene. If such a gene the parents are not found, they have every chance of having a healthy baby. In the presence of defective genes from both parents, the probability of having an affected child is 25%. To identify the cystic fibrosis lung in a child, perhaps even in utero.
Methods of treatment of the disease
The treatment of cystic fibrosis aimed at eliminating the consequences of the respiratory and digestive systems. The patient needs proper nutrition, moderate exercise and social support. The parents of the sick child needs to understand what is it and how is it treated.
To eliminate intestinal obstruction in newborns helps enema. If it turns out to be ineffective, the doctor decides on surgery. Taking medication, to establish a supply of the intestine with fluid, to prevent clogging.
The mainstay of treatment of cystic fibrosis is a digestive enzyme during the meal.
The diet should meet the daily body's need for calories and nutrients. We recommend taking double doses of multivitamins and water-soluble vitamin E. In hot weather and during exercise is necessary to increase the amount of salt. Newborn needs to obtain food in the form of a mixture on the basis of easily digestible fats and proteins.
In cystic fibrosis treatment of lung disease is aimed at cleaning up the bronchial tubes of phlegm and the elimination of infectious foci. The patient all necessary vaccinations, including the flu. Respiratory therapy includes percussion, drainage and removal of sputum. Adults can perform these procedures independently, and children helping their parents. Often prescribed bronchodilators and supplementary oxygen.
Are often used in aerosols, contributes to liquefaction of sputum. They facilitate its excretion and restore lung function. To relief the condition of children with severe inflammation of the bronchi which are used corticosteroids. To slow the development of the inflammatory process in the lungs are applied and non-steroidal anti-inflammatory drugs.
To prevent the spread of infection in the body help antibiotics. To identify the pathogen andthe purpose of a product out sputum. Antibiotics can be taken in the form of pills and spray. Severe pneumonia is shown by intramuscular administration of drugs. Some patients have to take antibiotics continuously.
Spadenie lung alveoli, chronic inflammation of the maxillary sinuses and bleeding from esophageal veins are removed surgically. The death of a patient with cystic fibrosis occurs most often due to respiratory failure, liver failure, internal bleeding and postoperative complications.