What is Cystic Fibrosis?

Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. This mucus also obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. Although symptoms and treatments vary from person to person many CF patients must undergo rigorous physical therapy sessions to clear mucus from the lungs as well as take enzymes to aid in food digestion. In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

How is it treated?

As stated above, symptoms and ailments can vary from person to person but the treatments generally remain the same. Individuals with frequent lung infections will usually undergo numerous physical therapy sessions daily to clear their airways of the thick mucus. Until recently individuals would need another person to physically pound on their back, chest and underarm areas to shake the mucus free. Although many patients still prefer this method, technology has allowed CF'ers to perform these therapy sessions on their own using devices such as The Vest. Exercize is also often used as an airway clearance technique. Individuals with severe pancreatic problems usually will take enzymes with meals to help properly digest and absorb food.

If an infection in the lungs becomes to severe CF patients must combat this using antibiotics administered through an IV. Usually this will lead to an extended hospital stay until the infection is under control.