Cystic Fibrosis: From Qualification to Lung Transplantation, a Single Center Experience

Abstract

Background Cystic fibrosis (CF) is congenital multisystem disorder, that leads to gradual deterioration of pulmonary function. Advancements in therapy of CF-related lung disease have delayed its progression. However, lung transplantation remains the only therapeutic option for majority of such patients. Aim of the study was to assess qualification process and outcome of lung transplantation as a treatment of CF patients qualified in a single center between 2011 and 2018. Material/Methods This retrospective study assessed 41 patients who were qualified to be treated by means of lung transplantation due to CF in Lung Transplant Program of Silesian Center for Heart Diseases between 2011 and 2018. Analysis of patients during qualification process and after lung transplantation was performed. Lung recipients were observed during 1-year follow-up by means of pulmonary function tests. Results 1-year survival was noted among 80% of the patients; 3-year survival and 5-year survival were noted among 70% of the recipients. Mean forced expiratory volume in 1 second (FEV1) increased after lung transplantation: 21.19% at qualification; and 76.67% at 12 months after lung transplantation. Mean forced vital capacity (FVC) results also improved: 34.18% at qualification and 78.34% at 12 months after lung transplantation. The 6-minute walk test (6MWT) before and after treatment noted an increase of 175.55 m. Conclusions Lung transplantation improves respiratory capacity of CF patients and prolongs their life.