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Cystic Fibrosis

Cystic fibrosis is a common, hereditary chronic disease affecting the body. It is caused by a defective gene, which causes proteins in the body to produce sticky and unusually thick mucus in the lungs and digestive system. These mucus secretions act as clogging agents to the lungs, pancreas, and other organs. Continuous mucus build-up can lead to lung infections and digestive issues, making cystic fibrosis a life-threatening disease. Cystic fibrosis is also known as: fibrocystic disease of the pancreas, mucoviscidosis, and pancreatic cystic fibrosis.

As a hereditary disease, cystic fibrosis is primarily diagnosed through prenatal amniocentesis and chorionic villus screening, or newborn blood and genetic testing. Other common tests available for children and adults are: sweat tests, chest x-ray, sinus x-ray, lung function test, and a sputum culture (saliva testing). Cystic fibrosis commonly affects Caucasians of northern European decent, and it is typically diagnosed in children and young adults. In the United States, approximately 1,000 new cases of cystic fibrosis are diagnosed each year. Currently, there are about 30,000 cystic fibrosis patients in the U.S. and 70,000 worldwide.

There are more than 1,000 genetic mutations of cystic fibrosis, so the disease presents a varied range of symptoms from one person to another. Some of the most common respiratory symptoms include:

  • Fatigue
  • Frequent lung & sinus infections
  • Persistent cough
  • Recurrent pneumonia
  • Wheezing or shortness of breath

Digestive symptoms of cystic fibrosis include:

  • Belly pain or swelling
  • Constipation or intestinal blockage
  • Foul-smelling or greasy stool
  • Poor growth or poor weight gain in spite of a generally good appetite
  • Salty-tasting skin

There are numerous respiratory and digestive system complications, from chronic infections of the lung to diabetes. However, there are a number of treatments available for cystic fibrosis that can help manage the disease and prevent such life threatening complications. Some of these therapy options include: prescribed courses of antibiotics, inhaled medicines, pancreatic enzymes, the use of mechanical devices such as a chest clapper, an inflatable vest, and other breathing devices.

Stat RX Pharmacy can provide a number of medications to help treat cystic fibrosis, including a wide range of antibiotics, mucus thinning drugs, and bronchodilators. Our specialty pharmacy currently stocks Acetylcysteine, Pulmozyme, and TOBI specifically for the treatment of CF. A cure for cystic fibrosis has yet to be developed, however, through proper practice of therapy regimens and the maintenance of good nutrition, cystic fibrosis patients can improve outcomes and quality of life.