Cystic Fibrosis

Cystic fibrosis (CF) has traditionally been considered a disease for pediatricians and pediatric pulmonologists owing to the short life expectancy for patients suffering from this disease in the recent past. Advances in the maintenance of nutritional status, as discussed by Drs. Matel and Milla, and aggressive health maintenance and early recognition of and improved treatments for disease complications, presented in the article by O’Sullivan and Flume, have all led to substantial increases in life expectancy. The fact that two adult pulmonologists served as editors for this issue is a great testament to decades of scientific rigor and advancements in patient care by our pediatric colleagues in that survival to adulthood is now the norm rather than the exception.

Prolongation of survival presents a new series of challenges to CF providers. Dr. Cruz and colleagues discuss anxiety and depression and their impacts on disease outcomes and adherence to therapy, CF-related diabetes, which differs from types 1 and 2 diabetes, and bone disease such as osteopenia and osteoporosis are more prevalent in CF patients than in the general population. Their implications and therapy are discussed by Dr. Curran and colleagues. Dr. Lynch discusses infection with resistant pathogens, specifically Burkholderia cepacia complex and the combination therapies currently in use. The atypical organisms nontuberculous mycobacteria and fungi and their role in CF are discussed by Drs. Whittaker and Teneback.

Drs. Bendiak and Ratjen present new strategies that are evolving for the management of Pseudomonas aeruginosa and Drs. Morton and Glanville explore the appropriate use of lung transplantation. Drs. Truitt and Aris describe advancements in the understanding of the genetics of CF and alternative pathways for ion transport, which have contributed to a burgeoning pharmaceutical pipeline as presented by Dr. Ashlock and colleagues. Drs. Kraynack and McBride describe similar advances that have taken place in the recognition of opportunities to improve the quality of care delivery and the formalization of quality improvement initiatives.

We are grateful for the opportunity to edit this issue and appreciate the hard work of our panel of authors whose contributions elegantly explore the advances of the recent past and those on the horizon.