Cystic fibrosis and current treatment options

Cystic fibrosis (CF) is a serious hereditary disease, which represents a significant burden for patients and their families, and shortens the life

of patients. The disease manifests with pulmonary and extrapulmonary manifestations, a characteristic symptom of CF is a high concentration

of chloride in the sweat of patients. Mucostasis, obstruction and chronic infection occur in the lungs. Treatment of pulmonary manifestations

has a major impact on the quality and length of the life of patients. This is especially good care of the airway through respiratory

physiotherapy and drugs that reduce the viscosity of mucus in the airways and in the treatment of serious lung infections with antibiotics.

In the digestive tract CF leads to failure of exocrine pancreatic function, consequently the absorption of fat from food is not possible and

it leads to malnutrition. Treatment of extrapulmonary manifestations includes substitution of pancreatic enzymes, especially nutrition of

patients, treatment of liver disease and early treatment of complications such as diabetes, osteoporosis, intestinal obstruction, and others.

As a clinical entity, this disease is known since the 40th of last century, originally a clearly fatal diagnosis in the first year of life. Thanks to

improved diagnostic methods and intense treatments are now up to 50% of patients live for the fourth decades of life. The purpose of this

article is to show the current CF treatment options with regard to the involvement of pharmacist in the care of patients with CF.

Keywords: cystic fibrosis, exacerbation, respiratory physiotherapy, pancreatic insufficiency, nutrition