A GENETIC, CHRONIC, AND LIFE-THREATENING DISEASE THAT CAUSES THICK, STICKY MUCUS TO BUILD UP IN THE LUNGS, DIGESTIVE TRACT, AND OTHER AREAS OF THE BODY. Cystic Fibrosis:

Effect on the Respiratory System Cystic Fibrosis clogs the lungs and bronchi with a thick, sticky mucous. The mucous secretions plug up tubes, ducts, and passageways within the lungs and especially the alveoli.

Cause of Cystic Fibrosis It is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). This gene is required to regulate the components of sweat, digestive juices, and mucus. Normally, such secretions are thin and slippery, but in cystic fibrosis, this defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions clog the lungs.

Therapy for Cystic Fibrosis Intravenous, inhaled, and oral antibiotics are used to treat chronic and acute infections Mechanical devices, such as inflatable vests, vibrate to loosen and clear mucous Manually clapping with cupped hands on the front and back of the chest with the person's head over the edge of the bed allows gravity to help clear the secretions Lung transplantation and gene therapy attempt to cure some of the effects of cystic fibrosis

Pharmacology Antibiotics to prevent further lung infections include ciprofloxacin [Cipro] and tobramycin [TOBI] Mucous-thinning drugs include Dnase, such as Pulmozyme The bronchodilator drug Albuterol, delivered through an inhaler, helps keep airways open by relaxing the muscles around your bronchial tubes Corticosteroids, such as Prednisone or Medrol, reduce inflammation

Progression of the Cystic Fibrosis It is a chronic, life-threatening disease People diagnosed with Cystic Fibrosis often develop additional lung infections such as pneumonia Cystic Fibrosis can also affect the stomach, intestines, liver, and pancreas

Expected Outcome for Recovery Since there is no cure for cystic fibrosis, there is no recovery from cystic fibrosis It is chronic and fatal