There are an estimated 70000 people with cystic fibrosis (CF) globally, with the vast majority of cases in Caucasian populations. Over the past 30 years, CF has been transformed from a predominantly fatal disease in children and young adults to one in which its greatest impact is borne in adult life [1,2]. In many countries, CF adults now outnumber CF children as a result of improved therapies for children and increased life expectancy of adults, resulting in the need to re-structure CF care systems and to develop adult specialist care centers [3,4].
The median survival of patients with CF in most parts of the world is close to 40 years of age, and even those patients with advanced airflow obstruction (percent of predicted forced expiratory volume in 1s <30%), which was previously thought to forebode a high risk of death within 2 years, now generally have prolonged survival . For those patients with end-stage lung disease and their families, lung transplantation provides the opportunity and hope of enhanced survival . Despite such developments, the vast majority of patients with CF will die as a result of progressive respiratory failure and the complications of suppurative lung disease .