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The treatment of cystic fibrosis in children

Cystic fibrosis in children is a special pathology of the glands. This disease is also known as testaferros. It has a hereditary character. Certain types of glands of the respiratory and digestive systems of the patient begin to produce secretions (a substance produced by the cells of various glands and required for activity of the organism) with a high coefficient of viscosity.

problema mukoviscidoza u detej

Because of this, the ducts start to get clogged. As a result, the amount of mucus is constantly growing. This leads to disruption of the internal organs. In children with such disorders, disturbed digestion (the process is slowed), stopped, or slowed down growth and development. This pathological condition is currently considered incurable. However, there are some treatments that can slow the progression of the disease. Thanks to them the patient will be able to live a few years more.

The reasons for the development of the disease

Cystic fibrosis in newborns is a congenital genetic disease process. This disorder can be inherited only from the two parents. Thus passed the gene, which was previously distorted. The patient affected endocrine glands. The changes begin in the connective tissues. Then there are problems with water and electrolyte exchange processes. Because the affected gene start mutation with protein structures. This applies to the work of the liver, pancreas, digestive tract, respiratory system and reproductive organs.

harakteristiki mukoviscidoza u detejIn addition, violations of the chemical and physical composition of the mucus, which is excreted through the glands of external sector: lacrimal, sweat, glands that secrete mucus mucous membranes, and so on. The mucus becomes very thick due to the fact that it increases the level of electrolytes protein. It interferes with the excretion of mucus. Due to the fact that the secret becomes more viscous ducts become clogged and trying to become broader. They begin to form cysts. This is especially true of the bronchi, lungs and digestive organs.

Problems with electrolyte metabolism related to the fact that the secret contains large amounts of calcium, sodium and chlorine. Stagnant processes cause tissue glands begin to atrophy. The tissue of the glands begins to be replaced by connective fibers. This process is called fibrosis. In bodies, where there are glands, there are signs sclerosing phenomena. If there is infection, the tissues are inflamed, pus is formed.

If cystic fibrosis was struck by the child's system of bronchi and lungs, then there are problems with the excretion of mucus andof sputum. The functionality of the epithelium of the ciliated type is violated. Appears inflammation that becomes chronic. The patient had mucostasis. The bronchioles disturbed patency, bronchial gland type begin to grow. They protrude and block the passages from the bronchi. This changes the respiratory system.

Violations may not only affect the bronchi and lungs but also the liver, stomach, intestines, pancreas and other organs.


kashel pri mukoviscidozeThere are several forms of the disease. The patient may be pulmonary or intestinal form symptoms. In addition, there is erased, atypical and mixed forms. When mixed form simultaneously affected and the lungs, and the digestive organs. This classification is conditional.

Symptoms depend on the form of the disease. In pulmonary all starts with an ordinary pneumonia. In some cases, even before the development of this disease, your baby may cough frequently. Pulmonary syndrome will gradually improve.

Very often there are exacerbations. With intestinal manifestation in a patient frequent stools (up to 5 times per day), although sometimes there is constipation. This is due to lack of enzymes, causing food cannot be digested. The child begins to lose weight.

The treatment of cystic fibrosis

Treatment should start as early as possible. The sooner you start treatment, the greater the likelihood that the child will be able to survive. Therapy in cystic fibrosis is often only symptomatic, as treatments and medications help suppress the symptoms of the disease.

In other words, treatment helps to restore the functionality of the gastrointestinal tract and the respiratory system. Rules of therapy are required to observe throughout life.

nasledovanie mukoviscidozaIf the patient has a pulmonary form of the disease, the doctor selects the medication and treatments that will help to restore the patency of the bronchi and bronchioles, as well as to reduce the viscosity of sputum. In addition, it is necessary to treat the inflammatory processes in this area. The patient is assigned means from the group of mucolytics. For example, use such drugs as Mucosolvan or Acetylcysteine.

They are used for inhalation treatment or aerosol every day. In some cases, the doctor may prescribe medicines, which contain different enzymes. For example, can be used Chymotrypsin or Fibrinolysin.

In addition to medicines the doctor prescribes physiotherapy. Necessarily require postural drainage type, vibromassages in the chest. Doctors recommendto carry out reorganization of the bronchial tree. For this purpose, the bronchoscope and drugs from the group of mucolytics. In cases where the patient has acute disease appear pneumonia, bronchitis and other similar diseases, it is necessary to apply antibacterial medicine. In addition, the use of metabolic resources. They help to improve the functioning of the heart due to additional recharge. Assigned to glucocorticoids and cardiac glycosides type.

If the patient of the intestinal manifestation of the disease, must adhere to the rules of diet. You must consume foods that contain large amount of protein. These include cheese, eggs, meat, fish. In addition, the diet must include foods that contain organic types of carbohydrates and fats, but they must be easy to digest.

dieta pri mukoviscidozeFrom the diet should completely eliminate rough fiber. If you have a lactose deficiency type, it is better to refuse from milk. Be sure to drink plenty of fluids, preferably water.

The patient is assigned to a different vitamin and mineral supplements. Quite often, therapy is used to replace. In this case, the patient should take the drugs, which have in its composition a significant amount of enzymes that aid in the digestive process.

Examples of these drugs are panzinorm, Festal, Mezim-Forte. Doctor prescribes an individual dosage for each drug. Due to this patient's disappear pain attacks, stabiliziruemost chair, normal weight, fecal cease to be visible fats neutral type. To reduce the viscosity of the substances produced by the digestive glands, the patient is administered Acetylcysteine.

In some cases, intensive therapy. This is necessary when destructive processes in the lungs, hemoptysis and respiratory failure. If there is suspicion for the development of peritonitis, obstruction in the intestine or bleeding in the lungs, it is necessary to conduct a surgical operation.

Children are at the dispensary. It is better not to send them to preschools. As for schools and other institutions, it is decided depending on the condition of the baby. Parents must teach proper child care. Vaccination is carried out individually.

As a conclusion

Cystic fibrosis in children is a dangerous disease that leads to death.

This disease is genetic. As a result of increasing viscosity of the substances that produce cancer, clogged ducts, and organs begingradually refuse.

To completely cure the patient, it is impossible, but now developed techniques that help to improve his condition.