Cystic fibrosis (CF) is an inherited, multisystem disease of exocrine gland function that is primarily characterized by diffuse obstruction and chronic infection of the airways and poor digestion resulting from exocrine pancreatic insufficiency. CF affects a cell protein called CFTR (cystic fibrosis transmembrane regulator). CFTR controls the flow of water and certain salts in and out of the body’s cells. As the movement of salt and water in and out of cells is changed, the mucus that many cells normally make gets thicker. People with CF get a defective gene from both parents. People who have one defective gene from one parent do not have the disease but are called carriers.
Respiratory Therapists play an important role in the treatment of patients with Cystic Fibrosis. This course provides an overview of the disease, discusses the use of NIV with CF, covers the use of ultrasound in diagnosing the severity of the disease, and reviews the importance of drying the nebulizer equipment between patients in order to provide good infection control.