Nasal Polyp
An excessive growth of tissue lining the nose. Nasal polyps are common in patients with CF. Nasal Potential Difference (NPD)
A test of salt transport in the cells lining the nose. Transport of ions such as sodium and chloride creates an electrical potential difference across the epithelium (or lining) of the airway. This nasal potential difference (NPD) can be measured in patients by placing an electrode on the lining of the nose. The NPD can be used as a diagnostic test for CF. Nebulizer
A device used to deliver inhaled medications to a patient. The nebulizer uses pressurized air or oxygen and water with the medication to create an aerosol that can be inhaled into the lungs via a hand-held device or face mask. The nebulizer is attached to a compressor, which generates the air flow to aerosolize medications. Newborn Screening
Testing for genetic diseases like CF in newborn infants. The goal of newborn screening is to make the diagnosis of CF before the infant has any symptoms so that preventive therapies can be started as early as possible. Osteopenia
A condition which causes reduced bone strength due to decreased calcium in bones, leading to decreased bone density. CF patients are at higher risk for osteopenia due to decreased intestinal absorption of calcium and vitamin D, reduced physical activity, pulmonary disease, and use of steroids. This causes an increased risk of traumatic fractures among CF patients. Osteopenia is common in CF patients at any age, and accelerates during adolescence and early adulthood. Preventive measures include maintaining good nutrition, encouraging physical activity, and minimizing long-term use of oral steroids. Osteoporosis is a severe form of osteopenia. Bone density can be measured by a non-invasive test called a DEXA scan. Pancreas
An organ in the digestive system of the body that is responsible for production of many of the enzymes needed for the proper digestion and absorption of food nutrients and also for the production of insulin. In cystic fibrosis, pancreatic insufficiency leads to intestinal malabsorption of fats, proteins, and to a lesser extent, carbohydrates (sugars). This in turn leads to increased unused fat and proteins in the stool, and malnutrition. Most patients with CF have pancreatic insufficiency that requires life-long replacement therapy with pancreatic enzyme supplements and fat-soluble vitamins (A, D, E, and K). Pancreatic insufficiency
Patients with pancreatic insufficiency (which occurs in 85-90 percent of patients with CF) need to take daily medications that include replacement enzymes for the proper absorption of dietary fats, proteins and carbohydrates. The amount of enzyme taken with each meal is usually based on a patient’s weight and symptoms. Abdominal distension and discomfort, poor weight gain, decreased fat and muscle tissue, and frequent passage of pale, odorous and often oily stools can result from lack of enzyme replacement. PICC (Peripherally Inserted Central Catheter)
An intravenous (IV) line that is placed into a large (central) vein in the body. This type of IV is usually inserted into the patient’s arm by a specially trained nurse. A PICC can stay in place for several weeks. Pilocarpine Iontophoresis
This is the technical name for a sweat test. Port-a-cath®
An intravenous (IV) line placed permanently under the skin that goes directly into a large vein in the body. The IV line (or catheter) is used by medical personnel to administer medications that are given on a frequent basis, or when IV access has been difficult in a patient in the past. Medications are infused through a port, or small receptacle under the skin, by placing a small needle through the skin into the port. This is called “accessing” the port. Once the medications are no longer needed, the needle is withdrawn from the port, which remains under the skin.
Pseudomonas aeruginosa
Pseudomonas aeruginosa
(P. aeruginosa) is a bacteria that can chronically infect, or colonize, the lungs of CF patients. P. aeruginosa is resistant to many antibiotics and they are capable of surviving in conditions that few other organisms can tolerate. P. aeruginosa is often encountered in hospital and clinical settings because it is a major cause of hospital acquired (nosocomial) infections, but it can also be found in many other places that are readily accessible to patients. P. aeruginosa thrives in moist places. The bacteria rarely causes infection in healthy individuals, but in CF patients it can cause increased cough and sputum production, weight loss, decreased lung function, and fatigue or malaise. Nearly 80 percent of patients with CF are chronically infected with P. aeruginosa bacteria. Antibiotic therapy is usually directed at reducing the amount of chronic infection. Pulmonary Function Testing (PFT)
Evaluation of lung function by measuring the amount of air a patient is able to inhale and exhale. The patient is asked to perform several sequences of maximal inspiration and expiration, and a computer measures the amount of air going into and out of the lungs. The computer can then estimate the capacity and function of the lungs as compared to healthy individuals of the same size. | 
