Cystic fibrosis and pulmonary involvement from multiple perspectives

Abstract

The pulmonary involvement in cystic fibrosis (CF) is discussed from several perspectives including historical, current treatment, and possible future therapies. The clinical realities that have limited and frustrated the successful control of the pulmonary progression are reviewed. A variety of pending new therapies are briefly presented including antiinflammatory agents, protease inhibitors, ion transport inhibitors, antiviscosity agents, chloride channel modifiers, and immunomodifiers. An appeal is made for more effective down-regulation of the overstimulated local and systemic immune responses to the chronic inflammatory processes. More attention needs to be paid to improvement of current methods of aerosol administration that will be of critical importance for living cell/viral/gene/liposome delivery. Other means of buying additional survival time are urgently needed such as improvements in lung transplantation and better treatment of cor pulmonale. The explosion of molecular genetics progress in CF basic research is an exciting and encouraging dynamic that may lead to a cure for patients with CF in spite of the more than 200 known genetic variations known to date. The survival of CF patients has increased 1 year for 1 year during the past 10 years and now is 29.4 years in 1991. If this progress can be maintained, the hope is that advances in molecular genetics and cell electrophysiology can soon be brought to clinical application in an effective fashion. A magnificent choice of new treatment directions is becoming available, leading to very realistic hope and optimism for the future for patients with CF.