Cystic Fibrosis: Symptoms and Treatment

Cystic fibrosis determines a genetic hereditary disease, accompanied by a specific systemic damage to the exocrine glands. Cystic fibrosis, the symptoms of which are determined on the basis of this lesion, is a chronic and incurable disease, accompanied by a violation of the respiratory system, as well as disorders associated with the functions of the digestive system, including a number of other related disorders.



General Description

Cystic fibrosis (CF) as a definition of the current pathology, is used in Europe (and actually in our country), while in Canada, Australia, the US and elsewhere it is defined as «cystic fibrosis of the pancreas» , And it is in this variant that the features of its morphological manifestations are revealed to the greatest extent. It should be noted that this disease is quite common, as evidenced by the frequency of its occurrence. So, only for newborns in the scale of European countries is the ratio of 1: 2500, which, in turn, indicates that cystic fibrosis for 10,000 newborns has chances for at least four.

Cystic fibrosis is affected with the same frequency regardless of gender, that is, both boys and girls are equally affected. Given the fact that it is a genetic disease, it should be borne in mind that the patients are already born, and it is impossible to catch cystic fibrosis. Meanwhile, the disease can last a long time without symptoms, so it is advisable to treat cystic fibrosis in adults. Symptoms of it are diagnosed in this variant in about 4% of cases, although the overwhelming majority of this disease manifests itself during the first years of life. Given the fact that children are born with it, it is often defined as hereditary cystic fibrosis or congenital cystic fibrosis.

Children get sick when they get one mutant gene from each parent. If only one such gene is inherited, then in this case its carrier («CF carriers») is considered, in which pathologies corresponding to the disease are not diagnosed. Given that the genes that are available for each person are paired, each of us, respectively, is the carrier of two copies of each of the genes (again, one gene from the mother, one from the father). Certain genes are deprived of the possibility of normal functioning in violation of their structure. With a similar disorder in the structure, the gene is changed. Therefore, in order to develop cystic fibrosis (as, in fact, and many other genetic diseases), it is necessary to obtain, as noted in the original example, two altered genes. With the carriage by the parents of MB, any child of a couple can get a modified gene from each of them with a 25% probability.

Approximately 70% of cases of the disease in question it manifests itself before the child reaches the age of two. Due to the relatively recent introduction of neonatal screening in medical practice, the detection of cystic fibrosis has significantly decreased over time.

Peculiarities of cystic fibrosis

Features of pathogenesis (the mechanism that causes the onset of the disease) are not completely determined. Cystic fibrosis manifests itself through the secretion of secretion by certain exocrine glands, this secret has an increased viscosity. Because of the difficulties arising in its evacuation, there is a blockage of the ducts of the mucous glands, glandular organs, the gastrointestinal tract and the bronchial tree, against which a background of secondary changes in the lungs, pancreas, intestine and liver (in particular, enzymatic disorders and processes of the obturation- Inflammatory). Due to the development of the inflammatory process in combination with fibrosis, a secondary form of organ failure develops. When studying the secretion of bronchial glands in an increased amount of phosphorus is found, in addition, the concentration of potassium and sodium is to be lowered, in view of which some authors are inclined to assume that this is precisely the reason for which mucus has an increased viscosity.

The dense secret in cystic fibrosis is almost not eliminated through the corresponding ducts, which we noted above. In turn, such a delay causes the formation of small cysts in the digestive and bronchopulmonary systems. Due to stagnation of mucus, atrophy of glandular tissue develops, as well as progressive fibrosis. Fibrosis in particular implies a gradual replacement of connective tissues of the gland tissues. In addition, the organs develop early sclerotic changes. With secondary infection, the situation is complicated, because this is accompanied by the development of purulent inflammation in patients. Accordingly, the bronchopulmonary system in cystic fibrosis is affected by the difficulties associated with the process of sputum evacuation (which is directly related to its viscosity, as well as the violation of the functions performed by the ciliated epithelium), with the development of mucostasis (which determines the stagnation of mucus), as well as with chronic The nature of inflammation.

As a basis for those changes that occur with respiratory organs in the disease we are considering, there is a violation of the patency of bronchioles and small bronchi. Filled with purulent-mucous contents, bronchial glands gradually increase in size, due to which their protrusion and subsequent overlapping of the lumen of the bronchi occurs. There is a formation of teardrop-shaped and cylindrical bronchialectas of saccate type, as well as formation of emphysematous areas of the lung. Bronchi in this case undergo complete obturation with sputum (i.e., complete obstruction that violates patency).

The main role in the development of cystic fibrosis is attributed to a gene mutation provoking a disruption in the structure (along with the functions) of the transmembrane cystic fibrosis regulator (CFTR, a specific protein involved in the transport of chlorine ions through the cell membrane, CFTR also defines the name for the gene through which the Encoding said protein).

Looking more closely at the processes that are relevant for cystic fibrosis, you can get the following picture of the disease. Thus, viscous sputum, the accumulation of which occurs in the lungs, leads to the development of inflammatory processes here. This is also accompanied by violations of their blood supply and ventilation. As a result, patients suffer from coughing, a manifestation of cough, one of the main and permanent manifestations of the disease of interest to us. In the future, the lungs are freely exposed to infection, and mainly it occurs due to Pseudomonas aeruginosa or staphylococcus, which is accompanied by the gradual development of changes in the destructive nature. The explanation for this is a violation of local immunity, in which the level of interferon, antibodies, phagocyte activity decreases, and the state of the bronchial epithelium is altered.

In patients often develop diseases such as bronchitis and pneumonia, moreover, their occurrence is of a recurring nature, and in some cases, such complications are noted in patients already during the first months of their life. It should be noted that the infection provides an even greater viscosity of sputum, so that the developing condition in this case determines a significant risk for the life of patients, because respiratory failure causes the death of a significant part of them (this applies to both children and adults).

Lack of pancreatic enzymes causes cystic fibrosis patients to encounter problems related to digestion of food (this, in turn, determines the lag in weight for them, which is actual even with increased appetite). Bile stagnation causes the development of cirrhosis in some patients or a factor that provokes the formation of gallstones in the gall bladder.

If you try to figure out how people live with cystic fibrosis, you can offer readers to imagine the need for a permanent stay in a gas mask. In a gas mask, which can not be removed, moreover, in a gas mask, which every day is worse dealing with the function that is predetermined for it. In a similar situation are patients with cystic fibrosis — their lungs cope with their functions by only 25%.




Forms of cystic fibrosis

The disease we are considering is characterized by a variety of different manifestations that are relevant to it, all of which are determined by the severity of the pathological changes, the urgency of the complications, and the age of the patient. Forms of cystic fibrosis can be the following:


  • Primarily pulmonary (bronchopulmonary or respiratory)
  • predominantly intestinal;
  • mixed (in this case, the defeat is at the same time and respiratory system, and the gastrointestinal tract)
  • meconium intestinal obstruction
  • erased and atypical variants of manifestations of the disease

This division of cystic fibrosis into forms is conditional, because the indication of predominantly pulmonary lesions is compared with actual disorders of the digestive system, while the intestinal lesion is accompanied by the development of appropriate changes in the bronchopulmonary system. It is also important to note that the disease is characterized by a variety of its clinical manifestations in each individual case of its occurrence. Many organs are affected, but the lungs, intestines, pancreas and liver suffer the most. Meanwhile, one of the most important features of cystic fibrosis is that the mental abilities of patients are in no way affected.

Erased and atypical forms of the disease can be identified as a result of a survey for the relevance of chronic sinusitis in a child. At an older age, cystic fibrosis can be detected with actual infertility for men. Infertility in men, regardless of the form of the disease is its concomitant pathology. It provokes azoospermia (the absence of spermatozoa in the seminal fluid), azoospermia, in turn, develops due to atrophy of the spermatic cord (i.e., due to a decrease in its size or due to the damage of its tissues, due to which are violated / discontinued Peculiar to him functions), because of his innate absence or as a result of obstruction (i.e., violation of patency). These disorders can also occur in those patients who are only carriers of the cystic fibrosis gene.

Cystic fibrosis in women is manifested in a decrease in fertility (the ability to reproduce offspring), is due to the increased viscosity level, which is actual for the cervical canal, which is separated by the uterine duct, which complicates the possibility of migration of spermatozoa.

Cystic fibrosis of the lungs: symptoms

In particular, in this case we will consider the pulmonary (or respiratory) form disease. The first symptoms of cystic fibrosis are manifested in the form of sluggishness of patients and general pallor of the skin. In addition, often even an increased appetite does not give an opportunity to gain weight (the same concerns and appetite is normal). The severe course is accompanied in some cases by the appearance in patients of a cough already during the first days of their life, over time, the cough begins to increase, due to which the character resembles a cough with whooping cough. The satellite of the cough becomes the joining of sputum, it is quite dense, and if it is layered with a bacterial flora (said staphylococcus, etc.), it gradually becomes purulent-mucous.

Because of the high degree of viscosity of the bronchial secretions, the mucostasis noted above is already developing with the concomitant blockage of bronchioles and bronchi, which in turn becomes a factor contributing to the development of emphysema. If there is a complete blockage of the bronchi, then atelectasis begins (which determines the decline of either the lung completely, or its individual parts). Cystic fibrosis in young children is accompanied by a rapid involvement in the pathological process of the lung parenchyma (located in the walls of the lungs air sacs (ie, the alveoli), through which gas exchange between the blood and atmospheric air is provided). In this case, a protracted and severe form of pneumonia develops, a feature of which is a predisposition to abscessing, which implies the development of purulent inflammation of the tissues, in which their subsequent melting leads to the formation of purulent cavities. The actual lung damage in any case is two-sided.

An objective examination determines the presence of wheezing, when tapping within the scope of such inspection, sound is defined as boxed.

In some cases, patients develop toxicosis (a morbid state in its manifestation, caused by exposure to the body of certain exogenous factors (eg, microbial toxins)) and even the symptom associated with shock. Shock, in particular, means a pathological process that occurs as a response to extreme irritation and accompanied by a progressive type of impairment. They, in turn, affect the most important functions of the nervous system, as well as the respiratory system, circulatory system, metabolic processes, etc. In other words, the shock that can develop acts as a breakdown of a number of compensatory reactions of the organism (ie, its adaptive reactions , Which usually arise as a response to tissue damage when subsequently performed by intact tissues of lost functions), manifested as a response to damage.

Returning to the connection between toxicosis and shock with cystic fibrosis, we note that these conditions can appear against a background of certain diseases, during which there is a high temperature, or in hot weather, in which there is a significant loss of chlorine and sodium from the body.

The actual pneumonia for the patients subsequently becomes chronic, the condition is combined with the development of the previously mentioned pneumosclerosis (a pathological process of proliferation in the lungs of connective tissue against the background of the actual disease for the patient, as a result of which the affected areas lose their inherent elasticity , Finding at the same time violations related to their gas exchange function) and bronchiectasis (in this case we are talking about the pathological expansion of the bronchi, their walls and structure). The subsequent development of pneumonia is also accompanied by the appearance of symptoms of the so-called «pulmonary heart» (when, due to the characteristics of the lung and bronchial lesions, the increase in blood pressure in the small circle of blood pressure caused pathological changes in the form of enlargement and enlargement of the divisions located on the right side of the heart). In addition, cardiac and pulmonary insufficiency develops.

The clinical picture, again, determines the characteristic changes in the appearance of patients. So, the skin of them has an earthy tint, there is a cyanosis of a general type and acrocyanosis (ie, cyanosis of the coloring of the mucous membranes and skin, cyanosis of the limbs). At rest, dyspnea also appears, the chest becomes barrel-shaped, the sternum deforms in a wedge-like fashion, the terminal phalanges of the fingers are also deformed, resembling the drumsticks («Hippocrates fingers»). Motor activity decreases, body weight decreases, appetite decreases.

As a rare form of complications of cystic fibrosis, pneumothorax and pyopneuromotorax, as well as pulmonary hemorrhage, are considered. Pneumothorax in particular determines the state in which gases or air accumulate in the pleural cavity, pyopneumotorax determines the state of simultaneous congestion with gases or air of pus in this area, and pulmonary hemorrhage, respectively, determines the flow of blood originating from bronchial or pulmonary vessels, due to which Blood begins to be released from the respiratory tract.

A more favorable form of the course of cystic fibrosis, in which its manifestation (the development of severe forms of clinical manifestations following the erased or asymptomatic course of the disease) occurs when the older age is reached, the manifestation of bronchopulmonary pathology occurs with a slow progression of the deforming bronchitis in combination with a moderate manifestation of manifestation Pneumosclerosis.

The long course of cystic fibrosis is accompanied by the attachment of nasopharyngeal pathologies to it, which manifests itself in the form of polyps of the nose, sinusitis, chronic tonsillitis and adenoid vegetations.

Intestinal cystic fibrosis: symptoms

In this case, respectively, the predominantly intestinal form of cystic fibrosis is considered. The manifestations of this form are determined by the actual secretory insufficiency, which is noted in the digestive tract. In particular, this type of violation takes on a pronounced character when the child is transferred to lactation or artificial feeding. It is accompanied by insufficient splitting and subsequent absorption of fats and proteins, insufficient digestion and absorption of carbohydrates occurs to a lesser extent.

There is a predominance of putrefactive processes in the intestine, which determines as a whole the decomposition of organic nitrogen-containing compounds (amino acids, proteins) in enzymatic hydrolysis, which, in turn, occurs under the influence of ammonifying microorganisms. As a result of this decomposition, end products with a high degree of toxicity are formed (hydrogen sulphide, ammonia, primary and secondary amines, etc.). The accompanying putrefactive symptoms are manifested in the form of accumulation of gases, due to which, in turn, patients suffer from bloating.

Defecation (stool, bowel movements) is getting more frequent in patients, and the infringement as a polyphecal that determines an abnormal increase in the frequency of defecation, which can indicate an excess of the age norm is doubled, is also topical (the increase in this frequency is also 8 times)


From the moment of accustoming a child with cystic fibrosis to a pot, he often has such a pathology as a prolapse of the rectum, which occurs in about 10-20% of cases. Under the prolapse of the rectum is meant a partial or complete form of turning the rectum out through the anus.

The patients also have complaints of dry mouth, which is due to an increase in the viscosity of saliva. Chewing dry food is difficult, in general, the process of absorbing food requires the simultaneous reception of liquid in significant quantities. During the first months of the manifestation of the disease, the appetite in patients, as already noted, is normal, in some cases, increased. Nevertheless, the subsequent development of disorders associated with digestive processes leads to a rapid development of malnutrition — a nutritional disorder against the background of such disorders, as a result of which the patients subsequently have a deficiency in body weight in varying degrees of manifestation. Polyhypovitaminosis also joins this state, which determines a combined form of insufficiency of the body’s supply with vitamins of various groups.

The muscle tone, as well as the turgor of tissues decreases (normally it is a stressed skin condition, due to which it looks elastic and elastic). In addition, patients have abdominal pain of various manifestations. So, with meteorism (gases) there are pains of a cramping character, after coughing — pains are muscular, with actual right ventricular failure — pain in the right hypochondrium.

If there are pains in the epigastric region, this indicates a lack of neutralization in the duodenum of gastric juice against a background of decreased secretion of bicarbonates by the pancreas. It should be noted that disruption of neutralization in this case of gastric juice can provoke the development of patients with peptic ulcer in the duodenum, the development of an ulcerative process in the small intestine is also not excluded.

As a complication of the form of cystic fibrosis that we are considering, a secondary form of disaccharidic insufficiency, a secondary form of pyelonephritis, intestinal obstruction, and urolithiasis, which develops as a result of metabolic disturbances, can act. A latent form of diabetes mellitus may also develop, which occurs as a result of a lesion in the pancreas of the insular apparatus. Because of the violation of protein metabolism, hypoproteinemia develops, as a result of which some cases of its manifestation lead to the development of edematous syndrome in infants.

Enlargement of the liver (hepatomegaly) is due to the development of cholestasis in patients (a disorder in which the duodenum receives bile in smaller volumes, which in turn occurs due to disorders associated with its formation, excretion and excretion ). When developing in patients with biliary cirrhosis, the symptomatology of cystic fibrosis is supplemented with manifestations in the form of jaundice and skin itching, portal hypertension (which implies the urgency of such a syndrome in which the pressure within the portal vein system increases).

Portal hypertension, in turn, is accompanied by a swelling of the abdomen and an increase in its size, the appearance of a feeling of heaviness in the right hypochondrium. In the presence of varicose veins in patients, the stool may change (it becomes black). Due to the fact that the liver can not normally cope with the blood-purifying function, some of the toxins in the blood are sent to the brain. Against this background, damage to his nerve cells occurs, which leads to the development of encephalopathy. The manifestation of this stage of portal hypertension on the background of cirrhosis in cystic fibrosis is the distraction of patients, forgetfulness. Because of the stagnation of blood in the spleen, its gradual increase in size occurs. The increased pressure in the portal system (against portal hypertension) leads to the accumulation of water in the abdominal cavity, and this becomes the cause of development in patients with ascites. Ascites are also defined as a dropsy, which also indicates the nature of the pathology, it lies in the fluid accumulation, it concentrates in particular in the abdominal cavity, and the volumes of such a cluster can reach 25 liters.

In some cases, cholestasis does not accompany the development of cirrhosis in cystic fibrosis.

Cystic Fibrosis: Mixed Form

The mixed form of the disease determines the disorders that are actual for both its pulmonary form and for the intestinal form, considered above. As a rule, already during the first weeks after birth, severe forms of repeated bronchitis and pneumonia are diagnosed in patients. For them, a protracted course is characteristic, as well as the appearance of a permanent cough. Also there are sharp forms of eating disorders and intestinal syndrome (manifested in the form of a loose stool and bloating, the feces do not lose their characteristic feces, but they have a liquid form, are abundant, the color changes to yellow-green or gray-green; The stool appears standard, from 3 to 5 times a day).

Clinic manifestations of cystic fibrosis differs in each case, because, accordingly, the variants of the manifestation of the disease and the peculiarities of its course differ. In addition, there is a relationship between the severity of the course of the disease we are considering and the timing of its first symptoms. Thus, the younger the patient’s age at the time of manifestation of cystic fibrosis (that is, the development of severe manifestations of cystic fibrosis after exiting beyond the erased version of his course or the course of the asymptomatic), the more unfavorable for him the prognosis. In view of this difference in the course of the disease in different cases, the severity of this course is determined on the basis of the degree and nature of the lesion in patients with bronchopulmonary system, for which the following stages are used:


  1. I stage. It is characterized by intermittent functional changes, combined with the appearance in patients with dry cough, without the addition of sputum. Dyspnoea manifests itself in a slight form, or in the form of a moderate, in both cases, its occurrence is accompanied by previous physical activity. The duration of this stage in patients is often about ten years.
  2. Stage II. At this stage, the chronic form of bronchitis develops, in which cough manifests itself with phlegm. Dyspnoea manifests itself in moderate form, its amplification is noted under tension. In this stage, the fingers are deformed (their terminal phalanges). In addition, there are «crackling» and wet wheezing when listening, breathing is characterized by rigidity. The duration of this stage can be on the order of two to fifteen years.
  3. Stage III. The stage is already under consideration here, within which progression of the bronchopulmonary process is combined with the development of a number of complications. In the lungs, zones of limited pneumosclerosis with diffuse pneumofibrosis form, bronchiectasises and cysts are also formed. Patients are diagnosed with a pronounced form of respiratory failure, which is combined with heart failure (previously noted «pulmonary heart»). The duration of the disease at this stage is about three to five years.
  4. IV stage. Within the course of this stage, patients develop a severe form of cardio-respiratory insufficiency. The duration of this stage is about several months, its completion determines for the patient a lethal outcome.

Meconium intestinal obstruction

This form of cystic fibrosis is also defined as meconium obstruction of the intestine. Its name determines the essence of the current state of patients with cystic fibrosis, respectively, meconium obstruction of the intestine indicates intestinal obstruction with original feces, meconium. Because of the increased viscosity of meconium, provoked by the disease of interest to us, cystic fibrosis, this blockage occurs.

This pathology manifests itself during the first days of a child’s life. Define meconium obstruction can be due to the fact that, in fact, does not depart meconium. Further, by the second day of life, the child’s anxiety is noted, frequent regurgitation occurs, vomiting appears, in which an admixture of bile is found. This also includes bloating of the abdominal child, a noticeable vascular pattern appears on it, the turgor of tissues decreases. In general, the skin becomes pale and dry. The former anxiety of the child replaces the state of adynamia (a condition characterized by a sharp decline in strength, the development of muscle weakness, a decrease, or even a cessation in the patient of any motor activity). It also increases toxicosis in combination with exsicosis (otherwise — dehydration, the pathological state of loss of significant amounts of salts and fluids by the body).

Meconium obstruction develops due to the absence of a specific enzyme (trypsin), as a result of which meconium accumulates in the region of the loops of the small intestine, becoming viscous and dense. Departure of the first feces in a newborn occurs mainly during the first 24 hours from the moment of birth, less often during the second day. If the child is sick, then, as is clear, meconium is not allocated.

An objective examination of the child reveals the presence of tachycardia (rapid heart rate) and shortness of breath. Treatment of this condition of the newborn is usually done through surgery. It should be noted that meconium obstruction is not related to the degree of severity in a sick child of cystic fibrosis. Only in rare cases, cystic fibrosis itself can not be a confirmed diagnosis by conducting laboratory and clinical studies.

Separately note that this condition can provoke serious complications, namely intestinal perforation in combination with the development of the child’s meconium peritonitis. Also, there is frequent adherence to 3-4 days of pneumonia, it is subsequently characterized by its own protracted nature of the course. The development of intestinal obstruction can occur even in older patients.

Diagnostics

The diagnosis of cystic fibrosis can be made after the results of the following studies:


  • conducting a general study of the early manifestations of the disease, the study of the hereditary factor in its possible appearance
  • conducting general tests (urine, blood)
  • microbiological examination (sputum)
  • coprogram (the feces are examined for the presence of fat and the volume of its content in it, as well as the presence / content of starch, muscle fibers and fiber)
  • bronchoscopy (allows to determine the presence of filiform sputum in the bronchi of a viscous and thick consistency)
  • bronchography (determines the urgency of bronchial defects, as well as the presence of bronchiectasis in them)
  • Radiography (the bronchi and lungs are examined for the presence of sclerotic and infiltrative changes in them)
  • spirometry (it makes it possible to determine the actual functional state in which the lungs stay, this is done by measuring the speed and volume of the exhaled air)
  • molecular genetic research (DNA or blood samples are analyzed for subsequent detection of mutations in the gene that causes cystic fibrosis)
  • sweat test (electrolytes of sweat are subject to examination, this analysis is the most informative in terms of identifying the disease of interest, it reveals the content of sodium and chloride in the sweat)
  • prenatal diagnosis (involves conducting a survey of newborn babies for the presence of congenital and genetic diseases, including cystic fibrosis).

Treatment

There is no effective treatment for the disease under consideration, today, the therapy for this disease is symptomatic. In particular, this therapy is aimed at restoring the functions of the gastrointestinal tract and the respiratory system, it is carried out throughout the life of the patient. Intensive care is required in case of actual respiratory failure within the II-III degrees of the disease, with «pulmonary heart», lung destruction and hemoptysis.

The prevalence in patients with intestinal disease is required to maintain a diet with a high content of proteins in it (eggs, fish, cottage cheese, meat), as well as restricting fat and carbohydrates in the diet (only easily digestible). Exception is subject to coarse fiber, lactose deficiency implies the exclusion of milk. Prescribed vitamins, digestive enzymes (the appointment is made on an individual basis, the severity of the lesion is the determining factor in the appointment of a specific dosage). The effectiveness of the prescribed treatment is determined on the basis of the state of the stool (more precisely, its normalization), the normalization of weight, the disappearance of pain, the lack of neutral fats in the stool.

The pulmonary form of cystic fibrosis in treatment is aimed at reducing the degree of viscosity of sputum in patients with simultaneous restoration of bronchial patency. Also an important measure in the treatment is suppression of the infectious-inflammatory form of the process. Patients are appointed mucolytics (inhalation, aerosols), and enzymatic preparations in the form of inhalations can be prescribed. At the same time, exercise therapy, postural drainage and vibration massage for the chest are used.

The urgency of acute manifestations, characteristic of bronchitis and pneumonia, requires the implementation of antibiotic therapy. Metabolism is prescribed to improve myocardial nutrition. Treatment of azoospermia in men with cystic fibrosis through the use of conservative techniques, which in particular implies hormonal therapy, determines the absolute lack of effectiveness.

A separate important point is a cough, by which sputum is removed. Its accumulation increases the likelihood of infection, cough is the main way to clear the lungs. Accordingly, the child should be accustomed to coughing to achieve this effect, he should not hesitate to do it during the day or during physiotherapy procedures.

As for the prognosis, it is determined on the basis of the severity of the course of the disease, which is especially relevant for pulmonary syndrome, as well as the time of the first symptomatology in the patient, the timely diagnosis and adequacy of the measures of the therapy used. Lethal outcomes are quite common, especially during the first year of life in sick children. As we have already noted, the earlier the disease was diagnosed and the corresponding therapy was started in its address, so, accordingly, its course is more favorable. The average life expectancy in Russia at the moment is 30 years, while in developed countries this figure is an order of magnitude higher — 40 years.




In cystic fibrosis, patients are observed at the pulmonologist, as well as at the treating pediatrician (therapist). Parents and relatives are compulsorily trained in the actions used for vibrating the patient’s massage, as well as the basic rules necessary for compliance to care for him.