Introduction

The disease

Cystic fibrosis (CF) is an inherited disease characterized by malabsorption, bronchopulmonary sepsis and a high sweat sodium concentration. The inheritance is by a Mendelian recessive gene. The abnormal CF gene primarily controls abnormalities of sodium and chloride transport. A functionally defective chloride channel in the apical membrane of the epithelial cell leads to loss in luminal salt and water in the airways. This predisposes to lung infection and bronchiectasis. Secondary bacterial infection can stimulate the host immunological response and there is an excess production of mediators from neutrophils, macrophages and lymphocytes. This all stimulates mucus production and further damages the airway [1]. Respiratory disease is the major cause of morbidity and mortality in CF [2]. When the disease was first described in l938, 80% of babies died within the first year of life [3] but now 50% of patients in the UK survive to 31.5 years [4]. Survival in North America is similar [5]. One of the best predictors of death in CF patients is a forced expiratory volume in 1 s (FEV1) [6]. Although the survival for patients with CF has improved significantly in recent decades, many young people are still dying of end-stage respiratory failure and it is for them that the advent of lung transplantation has been a very significant advance.

Special challenges of CF for the transplant team

Patients with CF are often malnourished due to malabsorption. They often have coexisting liver disease, may be diabetic and may have infection present in the upper respiratory tract.