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Грижи и психотерапевтичен подход на медицинската сестра към пациентите с муковисцидоза

Ст. Янчева, M. Нанкова, С. Борисова


Introduction: Cystic fibrosis (CF) is the most common hereditary, life-threatening condition in the European population that affects both children and adults. It is a result of a gene mutation (CFTR) that encodes a protein regulating transmembrane conduction and ion transport in the epithelial surface of a number of organs and systems in the body. The respiratory tract, the pancreatic ducts, the intestinal tract, the bile ducts, the sweat canals, and others are most commonly and heavily affected. Their function is limited by the formation and retention of dense, tough secretions, the removal of which is difficult and sometimes impossible. Restricting the work of these organs and systems leads to a progression of the disease and a significant reduction in the quality of life - especially with age.

Based on international data, the current predicted survival age of CF patients is 37.4 years. Unfortunately, in Bulgaria their average life expectancy is early school age - 12-13 years.

Although still falling into the group of rare diseases, it is determined to be the most common one in Bulgaria and it is predicted to soon leave the limitations of this group. This is due to the proven high number of possible mutations of this gene.

In Europe, the incidence of CF in different countries varies from 1/1350 to 1/25000, with an average of about 1/3500. The disease state follows an autosomal-recessive pattern of inheritance, which means that heterozygosity does not result in disease, but it may be transmitted to the offspring. The incidence of defective gene delivery responsible for the development of CF varies from 1/20 to 1/80.

In Bulgaria, every 33th person is the carrier of a defective gene that causes CF. Testing for the carrying such a defective gene is widespread in European countries and gives clear information about the risk of giving birth to a child suffering from CF.

The diagnosis of CF is considered to be certain if at least two of the following four criteria are present: a) family history; b) chronic diarrhea; c) chronic pneumonia; d) positive test.

Aim: To present nursing care and psychotherapeutic approaches to cystic fibrosis patients.

Materials and Methods: A documentary method was used in this scientific communication. The analysis of the documents was made in the period January - April 2017.

Results and Discussion: In recent years, medicine has progressed considerably in terms of CF, with a drastic improvement in the lifespan. Today, the newborn child is expected to survive 60 years. This is a challenge for the specialists and the nurse to outline the specific needs and care of the patients. Along with lung prophylaxis, prevention of exacerbations, physiotherapy, appropriate nutrition and prevention of complications, a psychotherapeutic approach is also needed for CF sufferers.

Conclusion: Through blockbuster treatment with the active role of the nurse, part of the pulmonary team can treat CF to reduce pulmonary infection. In addition to antibiotic treatment, physiotherapy - inhalation and respiratory gymnastics - plays a very important role. Bronchodilators are also used. Anoscillatory PEEP is also used. Feeding should be calories plus pancreatic enzymes. Due to heavy salt losses, the amount of salt should increase.

The nurse's individual psychotherapeutic approach in day-to-day care and patient meetings is essential for treating and improving the quality of life of the patient.


nurse; cysticfibrosis; health careandpsychotherapeuticapproach

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