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Published ahead of print on August 26, 2005, doi:10.1164/rccm.200505-840OE

Am. J. Respir. Crit. Care Med., Volume 173, Number 5, March 2006, 475-482

A more recent version of this article appeared on March 1, 2006
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Submitted on May 31, 2005
Accepted on August 24, 2005

Cystic Fibrosis Since 1938

Pamela B Davis*

* To whom correspondence should be addressed. E-mail: Pamela.davis{at}case.edu.

Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic diagnosis, life expectancy was ~6 months, and the autosomal recessive disease was thought to arise from abnormal mucus plugging exocrine ducts. Death often occurred from lung infection. Discovery of the sweat electrolyte defect in 1953 and standardization of the sweat test in 1959 allowed identification of milder cases, and CF was no longer considered only a disorder of mucus. In 1955, establishment of centers with programs of aggressive, comprehensive care initiated striking improvement in longevity. The pillars of care established then (attention to nutrition, airway clearance, treatment of lung infection) remain today. In 1983, chloride transport was identified as the basic physiologic CF defect, accompanied by increased sodium reabsorption. In 1980 we learned that inflammation contributes independently to lung disease and constitutes an independent therapeutic target. In 1989, the discovery of the CF gene demonstrated the basic defect to be in a cAMP-regulated chloride channel. This afforded new diagnostic tests, opportunities for research, and prospects for using the gene as therapy. Since then, substantial advances in basic and clinical research catalyzed therapeutic improvements: median survival age now exceeds 30 years. The Cystic Fibrosis Foundation Center network provides not only opportunity to conduct clinical trials, but also means to disseminate new therapies. In the future, treatments directed at the basic defect can be expected, with concomitant improvements in morbidity and mortality.
Key words: cystic fibrosis, Pseudomonas aeruginosa, sweat test




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