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The symptoms and treatment of cystic fibrosis

There is a hereditary disease that affects the organs that produce mucus in cystic fibrosis, the symptoms of which have already been established by the doctors. This disease is manifested both in childhood and in adulthood.

problema mukoviscidoza u detej

A little about cystic fibrosis

It is known that this is a disease that is betrayed by inheritance and affects organs such as the pancreas, liver, bronchial, sweat, salivary and intestinal glands. This disease is considered to be one of the most common among the hereditary diseases. However, he for a long period of time has not been given due attention. Despite the fact that cystic fibrosis has long been known and its symptoms are studied in Russia to fight with this disease began only in the late 20th century. The emphasis began to shift, it became clear that if you let the situation to chance, then nothing good will come of it.

medikamentoznoe lechenieThe basis of the treatment is correctly matched medication. The main drawback of the disease is that taking medications for life, and the symptoms of cystic fibrosis is not always possible to determine quickly. In addition, you need to constantly undergo preventive and diagnostic periodically to stay in the hospital. And yet, this disease is not a death sentence. It is possible and necessary to live. How to minimize the damage? How and what to treat the body adults and children?

Cystic fibrosis is manifested in many patients in the first days of their life. About 15% suffer from intestinal obstruction in the first days of life. The child may not have the chair, and you can see that retching and vomiting containing bile.


The basic cause of cystic fibrosis is a gene mutation transmembrane regulator cystic fibrosis. He is responsible for the transportation of electrolytes across cell membranes. Mutation of the gene leads to the fact that the functions and structure of the emitted substance is changing. It becomes very thick and viscous, and it damages all of the organs that produce mucus.

We can distinguish the following forms of the disease:

  • combined;
  • broncho-pulmonary;
  • intestinal.

osmotr rebenka vrachomAs the name suggests, what form of affect which organs. For example, in the case of broncho-pulmonary cystic fibrosis in children aged 1-2 years, problems with breathing apparatus. In the bronchi accumulates excessive amounts of mucus, and the possibility of separation is minimized. Quite often, patients worried cough with stubborn phlegm. In the case of seasonalor viral exacerbation can happen recurrence - long bronchitis, pneumonia, fever, increased shortness of breath and cough. The intestinal form, results in low enzymatic activity in the digestive tract. This is particularly noticeable when switching to another type of feeding of the child.

Nutrient absorption decreases and intestinal predominate over putrefactive processes involving accumulation of gases. Increased number of stools, and its volume can exceed the daily rate of 28 times. In addition, there may be cramps and bloating. As for appetite, he at first remains rather high, and sometimes higher. In case of violation of the digestive process in patients there is a lack of vitamins.

At the expiration of the 12-day period is worsening the condition of the skin, it becomes dry and pale stomach visible "figure vessel". Increased General anxiety and lethargy. Regardless of the form of the disease the child may develop the syndrome of “salt baby” in which the skin and armpits deposited salt. The skin becomes salty.

How to identify the disease?

If the symptoms of the disease were able to determine quickly that the attending physician can send to a specialised centre for combating this disease.

The diagnosis is actually not difficult. It is important to know the algorithm of actions. Disease indicate three main symptom: chronic process in the bronchi, genetic predisposition, unfavourable sweat test. Why you need a sweat test? It can be used to determine the presence and concentration of chlorides in sweat. If a child is sick, the test results are higher than normal, but it should also be noted that a favorable result can't guarantee 100% that no diseases.

If the child was delivered the diagnosis, the specialist can guide the entire family on the passing a special genetic analysis. He finally confirmed the diagnosis and also help in planning future offspring. A parent is required to always monitor the child's condition, and if there were any changes, it is necessary to pay attention and conduct a survey. If you revealed at least one of the above signs, it is necessary to immediately consult a pediatrician. The sooner you get diagnosed, the less negative effects will be the result.

In treatment huge role played by the parents themselves. The problem with this disease is that it requires constant inspection and care. Regardless of the state should follow the scheduled recommendations. The first disturbing call can worsening of mood of the child. He begins to behave passively, he lost appetite, increased body temperature, increased cough, and canbe problems with a chair. If the child's condition has deteriorated, it is necessary to immediately consult a doctor and hospitalize the child to the hospital.

A doctor

Before integrated therapy in the fight against cystic fibrosis are such objectives:

  • thinning and destruction of the bronchi viscous sputum;
  • activation of protective mechanisms in the fight against infectious diseases;
  • enzymatic support to help maintain and replace important enzymes in the pancreas;
  • liquefaction of viscous bile;
  • changing my diet and addressing issues with vitamins.

An important tool in the fight against disease for children and adults can be considered special breathing exercises - kinesitherapy. It allows you to eliminate from the lungs viscous sputum.

As mentioned earlier, a huge role in the treatment of a child are the parents. Only they are able to quickly respond to the slightest changes. As for the doctor, he can do complex assignments, remove some drugs or increase certain doses. The condition of the child treatment may hold differently. It can fit in a specialized Department, to be at home or in hospital.