Cystic fibrosis : what lies behind this word

Cystic fibrosis : what lies behind this word
Cystic fibrosis - an inherited disease caused by a gene mutation and affects many organs and body systems.

Disease name from the Latin is translated as the combination of two words - "mucus" and "viscous".

In patients with cystic

fibrosis disproportionately affects those systems of the body where there are glands secreting mucous discharge, - respiratory and digestive.

also disrupted exocrine part of the pancreas, sweat and salivary glands, the liver.

There are three main forms of the disease:

  • bronchopulmonary - manifested obsessive bouts of cough, chronic inflammatory diseases of the respiratory tract, shortness of breath;
  • E. - is characterized by bloating, frequent and copious stools;
  • mixed - a combination of respiratory symptoms and digestion.

Less common atypical form of the disease (for example, with cirrhotic liver disease or edema-anemic).

In infants the disease can take the form of meconium ileus, due to which the child does not depart original cal.

problems in patients with cystic fibrosis Russian

In Russia the average life expectancy of people with a diagnosis of "cystic fibrosis" is not more than 27 years in Europe - 38-40 years.This is due to the fact that the diagnosis and treatment of this disease in our country is not readily available, especially for patients older than 18 years.

For children, the necessary steps to identify the disease and provide necessary assistance, there are special centers and government programs, there are federal and treatment centers.

But adult patients may get help only a limited number of hospitals, not all regions of Russia, there are no centers for federal significance.Therefore, they have to wait their turn for treatment, and in some cases, any delay significantly reduces the life of the patient.

Cystic fibrosis - not lethal pathology in which it is impossible to help the person.In a systematic and adequate medical care, patients with this diagnosis may even reach retirement age and lead an active life as people with diabetes or asthma.

However, at the moment Russia does not have the necessary therapeutic standards to provide the necessary care for patients with cystic fibrosis.In most regions, these patients are treated symptomatically, paying no attention to the specifics of the disease and the characteristics of its flow.So often, people are dying, that, when access to modern methods could live for many years.

Russian patients are not available, many modern medicines, which in our country have not yet been registered or are not included in the list of preferential medicines.

Part of a new generation of antibiotics is neither produced nor sold in the Russian Federation.Many technical means, such as inhalers or oxygen concentrators required for normal breathing, not mandatory for the treatment devices, and patients have to find the money to buy them on their own.

Photo: shutterstock.com

treatment of cystic fibrosis in Russia and abroad

General principles of treatment of cystic fibrosis in Europe and Russia alike.However, in European countries and the United States are more than effective state program of care for patients, which is in the Russian Federation can only dream of.

Abroad constantly developing new drugs and treatment methods.There are specialized centers for lung transplantation or complex "heart-lung".

new word in the treatment of cystic fibrosis gene therapy becomes based on the restoration of the damaged gene function by introducing them missing genetic constructs using viruses.

fairness it should be noted that in Russia in 1998 also initiated a program for the gene therapy of cystic fibrosis.However, in our country, the level of care for patients, particularly adult patients are still significantly lower than in other countries.

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