Lung transplantation in cystic fibrosis: a regional Center experience

Key words: Cystic fibrosis, Lung transplantation, Survival, Pulmonary function, Quality of life

Background - Cystic fibrosis (CF) is a rare genetic disease that progressively involves the pancreatic and pulmonary functions: as respiratory failure develops, lung transplantation is the only therapeutic option available nowadays.
Objective - The purpose of the paper is to describe the efficacy and the safety of bilateral lung transplantation in patients with cystic fibrosis.
Materials and Methods - The clinical records of the patients followed by the Regional Centre of Diagnosis and Treatment of CF in Trieste (Italy) who underwent lung transplantation between 1970 and June 2015 were analysed. Survival rate, respiratory, metabolic and nutritional parameters, quality of life and complications after transplantation were evaluated.
Results - Ten (12%) out of 83 patients underwent bilateral lung transplantation (average age 28.3 ± 9.5 years old, M:F=5:5) and 2 (20%) were in paediatric age (11 and 18 years). Five-year survival rate was 80%: one patient (10%) died because of a primary graft dysfunction and one patient (10%) died of Burkholderia Cepacia sepsis (10%). Indication for lung transplantation was chronic respiratory failure in 80% of the patients and acute respiratory failure in 20%. The mean time spent in the waiting list was 18.8 ± 13.2 months; the mean post-transplantation follow-up time was 4.5 ± 4 years. After transplantation, mean FEV1%, FVC%, FEF25-75% and oxygen saturation significantly improved (p value < 0.05) and no patient needed NIV or oxygen supplementation. Weight and BMI also improved (p value=0.3). The most frequent complications after transplantation were CMV infections, arterial hypertension, acute rejection, neutropoenia, dyslipidemia, gastro-intestinal problems and renal dysfunction. Scores in the Cystic Fibrosis Quality of Life questionnaire significantly improved in all the 12 fields explored.
Conclusions - Lung transplantation is the only life-saving option available for end-stage pulmonary disease in patients with CF: it can improve the pulmonary function, the metabolic and nutritional profile and the quality of life, even if monitoring for post-transplantation complications is required.