Treatment consists of dietary adjustment (low fat–high calorie) and the administration of vitamins , pancreatin, and antibiotics to ward off secondary infections. Special measures are necessary to decrease the viscosity of pulmonary secretions aerosol application of recombinant human DNase, an enzyme that digests the sticky extracellular DNA that helps form these viscous secretions, was approved in 1993. In some cases lung transplantation (see transplantation, medical ) is helpful. The identification of the abnormal gene (1989) paved the way for gene therapy aimed at altering the genetic structure by transferring to the patient cells with normal CFTR genes.
Identification of the genes has also made genetic testing and diagnosis possible. Evolutionary biologists have suggested that the gene, which must be inherited from both parents to cause the disorder, affords carriers some protection against cholera , a disease that kills through profound loss of fluids.
See P. Davis, Cystic Fibrosis (1993) M. E. Hodson and D. M. Geddes, Cystic Fibrosis (1994).
The Columbia Electronic Encyclopedia, 6th ed. Copyright © 2012, Columbia University Press. All rights reserved.
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