Mukovystsydoz (MV) – Kistoznyi fibrosis

Cystic Fibrosis (CF)

What is cystic fibrosis?

Mukovystsydoz (MV) It is a hereditary disease. His appearance is defective in certain cells of the lungs and digestive tract. Thus cells produce thick, sticky mucus, which can cause:

• Blockage of the lungs and airways;
• Problems of digestion and assimilation of nutrients from food.

Cystic fibrosis is a serious disease, that lasts a lifetime, but the severity of different people may differ significantly. The life expectancy of patients with cystic fibrosis is about 35 years. Some patients with mild forms of CF can live up to 60 years or longer.

Prichinы CF.

Cystic fibrosis is a genetic disease. Children, Patients with cystic fibrosis inherit the defective gene from their parents. Parents, which have the gene, Cystic Fibrosis, but they do not suffer from them, called carriers.

Faktorы risk CF.

Factors, which may increase the risk of cystic fibrosis include:

• The presence of the gene for cystic fibrosis parents;
• Brothers and sisters, with cystic fibrosis;
• Родители с муковисцидозом – в основном матери, as men, ill with cystic fibrosis often sterile.

Simptomы CF.

The abnormally thick mucus, generated in cystic fibrosis, blocks the functioning of some organs, which causes symptoms.

Symptoms of cystic fibrosis in children may include :

• Difficulty passing the first stool;
• Ileus, sometimes requiring surgery;
• Salty sweat.

Slime, plugging light, can cause the following symptoms:

• Coughing and wheezing;
• Breathlessness;
• The irregular shape fingertips;
• The distorted shape of the chest.

The mucus can also block the pancreas, which in turn blocks the production of enzymes, used for digestion. This may cause:

• Weight gain;
• Low growth;
• Retardation;
• Malnutrition;
• Dehydration;
• Bad smelling, плавающий стул – из-за плохого переваривания жиров.
• Diarrhea.

Other symptoms may include cystic fibrosis :

• Reduced fertility in women;
• Archoptosis;
• Poor sperm production in men;
• Nasal polyps;
• Chronic nasal obstruction from chronic sinusitis;
• Jaundice or other symptoms of liver disease;
• Excessive thirst and increased urination, which may indicate diabetes 2 type;
• Stomach pain and swelling of the intestinal blockage.

Girls suffer from cystic fibrosis more, than boys.

Diagnosis Cystic Fibrosis

The doctor will ask about your symptoms and medical history, and perform a physical examination. Suspected cystic fibrosis occurs in a child, if it observed the classic symptoms of the disease, especially cystic fibrosis sick brother or sister.

Cystic fibrosis is often diagnosed by the presence of symptoms, family history of cystic fibrosis or a positive screening in newborns. Diagnosis can be confirmed after the passage of genetic testing. Other laboratory tests, which can be used to confirm the cystic fibrosis:

• Sweat chloride test;
• Measurement of nasal transepithelial electrical potential difference (Nasal Potential Difference – NPD-тест).

The doctor may perform a test light, to find the symptoms of cystic fibrosis or determine the type of treatment. Tests may include:

• Chest X-ray and / or sinus;
• Testing of lung function;
• Isolation of sputum cultures.

Tests may also be necessary to check the functioning of the pancreas, to evaluate symptoms or determine the type of treatment.

Cystic Fibrosis Treatment

Cystic fibrosis can not be cured. Supportive treatment is aimed at:

• Improving the assimilation of food by the body;
• The prevention and treatment of diseases of the lungs and sinuses;
• Support for the functioning of the respiratory tract and lungs.

Treatment of cystic fibrosis include:

Power in cystic fibrosis

Proper nutrition can help improve your overall health, as well as the development of the child. Children, who scored a normal weight within two years after diagnosis have fewer episodes of cough and have better lung function. Some steps, that can help include:

• High-calorie diet, developed by a dietitian;
• Nutritional supplements, including reception soluble vitamins (D, E, and K);
• Acceptance of pancreatic enzymes with meals to improve digestion and absorption of nutrients;
• Drinking plenty of fluids and replenishing salts derived from the then, especially in hot weather or during illness.

Treating lung infections in cystic fibrosis

Accumulation of thick mucus in the airways increases the risk of respiratory infections. The infection can also cause serious complications with breathing, due to the accumulation of mucus. Treatment of recurrent infection often requires antibiotics. To prevent new infections used:

• Vaccination;
• Antibiotics (usually by inhalation).

Maintain airway and lungs in cystic fibrosis

Medications help to keep the airway open. Most drugs taken through an inhaler or nebulizer. Necessary medications may include:

• Бронхорасширители – чтобы расслабить мышцы и открыть дыхательные пути;
• Ингаляционные стероиды – для уменьшения отека и раздражения (only when necessary);
• Муколитические препараты – чтобы уменьшить выработку слизи и помочь ей выйти из легких.

Other steps, that can help bring the mucus from the lungs:

• The hypertonic saline is a special type of salt water. Special apparatus sprays the solution as a mist, which is then inhaled by. Fog can help reduce the production of mucus in the lungs;
• Перкуссия груди – ритмическое постукивание по груди. The procedure can help get the mucus from the respiratory tract.

When the disease progresses, may require oxygen therapy or mechanical ventilation.

Other treatments for cystic fibrosis

For the treatment of blockages in the gut may require surgical intervention. It may be considered an option lung transplantation and liver transplantation.

Support is very important for patients with cystic fibrosis and their families. Talk to your doctor about support groups or counseling psychologist.

If you or your child with cystic fibrosis, Follow your doctor's instructions.

Prevention Cystic Fibrosis

There is no way to prevent cystic fibrosis, if you have defective genes.

Before planning the conception of children, adults can be tested for the presence of the gene, calling cystic fibrosis. Prenatal testing can determine, if a child is ill with cystic fibrosis.