THE EVOLVING MANAGEMENT OF CYSTIC FIBROSIS

Abstract

Introduction: Cystic fibrosis is a genetic pathological condition, affecting chromosome 7, which encodes the CFTR protein. This pathology is characterised by abundant mucus production, which in turn leads to blockage of the airways and blockage of the digestive tract.

Objective: The aim of this literature review is to gather the largest available amount of information related to cystic fibrosis and analyse it in detail, indicating the results and expectations that have been had with the use of some drugs over time in patients with cystic fibrosis. It is also intended to mention updates of procedures and their effectiveness.

Methodology: a systematic and complete search was carried out using the Cochrane Library database, with the term Cystic Fibrosis. The documents found were in Spanish and English. Some articles were excluded due to lack of updated information.

Results: Inhalation Dornase alfa alfa is a useful drug for airway improvement and mucus viscosity reduction, as are bronchodilators.  Pancreatic enzymes and ursodeoxycholic acid are beneficial in the gastrointestinal area, reducing intestinal symptomatology.  In patients with diabetes, Insulin is preferred. Only glutathione has a beneficial effect in patients with cystic fibrosis. Rehabilitation and management of postural abnormalities is vital to improve patients' lives. In patients with nutritional deficiencies, nasogastric tube placement or gastrostomy is preferred to improve their nutrition.

Conclusions: Cystic fibrosis is a disease that has an inevitable progression. The treatments studied here show that they only help quality of life and pulmonary and gastrointestinal functions temporarily. Further studies are needed to determine therapies that can significantly improve patients, both physically and psychologically; and who knows, some therapy or therapies that may lead to a cure for this disease.