What is Cystic Fibrosis?

Cystic fibrosis (CF) is the most common type of chronic lung disease in children and young adults. In people with CF, a thick, sticky fluid called mucus builds up in the lungs and digestive tract. This can lead to fatal lung infections and cause serious digestion problems.

At Keck Hospital of USC, we have physicians and scientists who specialize in the study and treatment of CF. If you or a loved one has the disease, or if you suspect someone has CF, our doctors may be able to help.

Symptoms of Cystic Fibrosis

The symptoms of CF can vary from person to person, but some of the most common symptoms are:

• Very salty-tasting skin
• Persistent coughing or wheezing, shortness of breath
• Frequent respiratory infections, including pneumonia
• Weight loss, or a failure to gain weight normally, even when the appetite is good
• Difficulty having a bowel movement, or frequent, abnormal bowel movements
• Clubbing (enlargement of the fingertips and toes)

The Prognosis for Cystic Fibrosis

Just 50 years ago, children with CF seldom lived long enough to attend elementary school. Today, thanks to advances in diagnosis and treatment, the median age of survival for people with CF is 37. In fact, since 1988, the number of adults with CF has grown by 97.1%.

Cystic Fibrosis Glossary

• Azithromycin: An antibiotic shown to modify CF lung disease.
• Bone Density or DEXA scan: A test that measures the strength and density of bones, it is used to diagnose osteoporosis, a condition in which bones become thin and fragile. CF patients are at a higher risk for osteoporosis.
• Burkholderia-Cepacia (B. cepacia, for short): A group of bacteria to which people with CF are susceptible.
• Clubbing: This is when the tips of the fingers and toes get bigger and rounder. Clubbing is common in CF patients.
• Complete Blood Count (CBC): A CBC is a test that examines components of the blood including the number of red blood cells, white blood cells and platelets.
• Cystic Fibrosis-Related Diabetes (CFRD): About 15% of patients over 35 have CFRD. Adults who have CF are at risk for developing CFRD and insulin-therapy is the treatment of choice. An oral glucose tolerance test is the gold standard for diagnosis of CFRD.
• Exacerbation: A change in respiratory symptoms that requires treatment with antibiotics and aggressive airway clearance. Signs and symptoms of an exacerbation can include increased cough, sputum (phlegm or mucus that’s coughed up) and increased breathing, weight loss, fever, fatigue, malaise and/or shortness of breath.
• G-Tube: A gastric feeding tube, or "G-tube,” is a tube inserted through a small incision in the abdomen into the stomach. It is used when CF patients cannot take enough food by mouth to maintain their weight.
• Hypertonic Saline: This is extra-salty, sterile water that has been found to help clear the thick mucus from the lungs of people with CF
• Malabsorption: When the body cannot absorb necessary nutrients from food. In CF, mucus can clog the ducts of digestive organs so that the enzymes that break down food and help with the absorption cannot do their job.
• Nebulizer: The mouthpiece device used to deliver important respiratory medication (TOBI, Pulmozyme, Albuterol, Hypertonic Saline, etc.).
• Oral Glucose Tolerance Test (OGTT): This is a test to diagnose CFRD. This test requires fasting of 8-10 hours, then after a fasting glucose level is drawn you will be asked to drink a high-glucose beverage.
• Osteoporosis: A decrease in bone mass and bone density that causes an increased risk and/or incidence of fracture.
• Pancreatic enzyme replacement therapy (PERT): Pancreatic enzyme replacements are capsules/beads, taken with food, to help CF patients absorb necessary nutrients and fat.
• Pancreatic insufficiency: People with CF have mucus that either partially or fully obstruct their pancreatic ducts, keeping important digestive enzymes from reaching the small intestine to aid in the absorption of necessary fat and other nutrients.
• Portacath: A catheter surgically implanted into a major blood vessel to allow easier access fro drug administration.
• Pulmonary Function Test (PFT): A test used to measure lung function. It measures airflow and lung volume.
• Pseudomonas aeruginosa: A type of bacteria often found in the lungs of people with CF and the most common cause of lung infections.
• Pulmozyme®: A mucus-thinning drug shown to reduce the number of lung infections and improve lung function.
• SED Rate: The ESR (erythrocyte sedimentation rate) is a one-hour screening test that detects and monitors inflammation.
• Sputum culture: A lab test done quarterly to identify microorganisms growing in the airways. Results from this test are used to determine what antibiotics will be best to treat the infection.
• Sterility: Approximately 98 percent of men with CF are sterile secondary to the absence or abnormalities of the Vas Deferens.
• TOBI®: This stands for tobramycin solution for inhalation. TOBI is an antibiotic in aerosol form used for chronic maintenance and suppression of pseudomonas colonization in the airways.