Pulmonary cystic fibrosis – treatment and prognosis

Pulmonary cystic fibrosis is one of the forms of systemic cystic fibrosis, which is an inherited pathology. Unfortunately, despite the achievements of modern medicine, the prognosis remains unfavorable.

Pulmonary cystic fibrosis: signs

Pulmonary cystic fibrosis as an isolated form of systemic disease occurs in 1 of 5-6 patients, however, in any of the forms, the lungs are involved in the pathological process in 95% of cases.

The first symptoms appear, as a rule, in the first year of a child’s life, and the presence of meconium ileus in the neonatal period allows one to suspect pulmonary cystic fibrosis. Symptoms of the disease are caused by excessive production of bronchial mucus, obstruction syndrome and secondary infection. Pulmonary cystic fibrosis is characterized by the early appearance of pertussis-like cough, painful and paroxysmal, sometimes causing vomiting and accompanied by attacks of suffocation. The sputum is very viscous, it clears its throat with great difficulty, at first it has a mucous character, then (when a secondary infection is attached) it is mucopurulent.

Also, pulmonary cystic fibrosis can be suspected if:

  • the child is often ill with pneumonia bronchitis , there are indications of nasal polyps, sinusitis bronchial asthma ;    
  • the child lags behind in physical development, quickly gets tired and suffers from shortness of breath even with relatively minor physical exertion;
  • the skin of the child has an earthy tint, acrocyanosis is pronounced;
  • a barrel-shaped form of the chest is observed;
  • over time, the symptom of “drumsticks” and the symptom of “watch glasses” (changes in the final phalanges of the fingers and nails due to chronic respiratory failure) develop.

Pulmonary cystic fibrosis: diagnosis

Most often, pulmonary cystic fibrosis is detected by neonatal screening for genetic metabolic diseases. However, the standard for diagnosing this pathology is a sweat test according to Gibson and Cook, which is iontophoresis with pilocarpine and revealing several times the increased content of sodium and chloride in the sweat.

A further examination reveals the amplification and severity of the pulmonary pattern, lung atelectasis during radiography, a picture of purulent endobronchitis with bronchoscopy, bronchiectasis with bronchography, obstructive restrictive disorders with spirometry.

Pulmonary cystic fibrosis: prognosis and treatment

Unfortunately, lung cystic fibrosis remains an incurable disease with a poor prognosis. The average life expectancy of such patients is about 30 years (and in Europe is close to 40 years). Treatment is symptomatic and is aimed at improving the quality of life of patients. General regimen events and constant monitoring by a pulmonologist are also important.

To facilitate the course of cystic fibrosis and prevent the development of its complications, it is recommended:

  • high-grade, high-calorie, rich in proteins and vitamins nutrition;
  • breathing exercises;
  • vibration and percussion massage of the chest wall;
  • postural drainage (body position that promotes sputum discharge);
  • mucolytic treatment (endobronchial administration of mucolytics, inhalation with enzymes, glucocorticosteroids, hypertonic solutions, etc.);
  • with the addition of a bacterial infection – antibiotic therapy;
  • physiotherapy;
  • physiotherapeutic methods (microwave, uhf, electrophoresis).

At the last stage of pulmonary cystic fibrosis, lung transplantation is possible separately or in combination with the heart.

event_note March 31, 2020

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