Paediatric origins of adult lung disease

Cystic fibrosis (CF) remains an incurable life limiting condition which is the most common inherited lethal condition in most Western countries. Advances in the care of this condition, particularly advances in respiratory management, have seen the focus of this review become a reality. In the 1940s adult consequences of CF were minimal as most patients died during infancy and early childhood. Today, the life expectancy of patients with CF has advanced well into adult life and has prompted the development of specific adult CF care centres in many major hospitals as well as publications addressing adult issues relating to this disease. Future projections suggest that, within the next decade, most patients with CF in many countries will, in fact, be adults rather than children. While debate continues between proponents of newborn screening for CF and those against it, the drive of CF care for the paediatric age group is aimed at providing the patient with a high quality life for as long as possible. The underlying element to achieving this is minimisation of lung disease. While nutritional factors—together with endocrinological factors, psychological factors, and sexual factors—are vitally important in providing a good quality of life to the patient with CF, the basic determinant of longevity and quality of life in most CF patients is the degree of lung disease and its rate of progression with increasing age.