Glossary

A 

Airway clearance techniques: A variety of methods for cleaning mucus from the lungs. Airway clearance techniques include coughing and huffing, chest physical therapy, and active cycle breathing techniques. 

Anabolic steroids: A synthetic hormone that promotes weight gain.  

Antibiotics: A type of drug used to fight bacterial infections. 

 

B 

Bronchiectasis: The permanent weakening and reshaping of the airway.  

Bronchodilator: A type of treatment that widens airways so that other treatments such as antibiotics can go further into the lungs. 

Bronchoscopy: A procedure that uses a thin, lighted tube to look at the airways.  

 

C 

Carrier: If someone only has one copy of a CFTR gene mutation, but they don’t have CF, then they are a carrier. 

Cystic Fibrosis Related Diabetes (CFRD): a type of diabetes that is caused by cystic fibrosis due to pancreatic damage. It has characteristics of Type 1 and Type 2 diabetes. CFRD develops with age. 

Compressor: A compressor is the electric pump that is used to change liquid medicine into a fine mist that can be inhaled. The nebuliser is the mouthpiece or mask that is used to breathe in the medicine. 

Creon®: An enzyme that is taken with food to help you absorb nutrients and vitamins. 

Cross infection: The transmission of bacteria and infections from one person to another. Cross-infection is the reason why people with CF are discouraged from spending time together.  

Cystic fibrosis (CF): Cystic fibrosis (also called CF) is the most common, life-limiting genetic condition affecting Australians. CF causes an abnormal build-up of thick and sticky mucus in the lungs, airways and digestive system. 

Cystic fibrosis transmembrane conductance regulator (CFTR) gene: This is the gene that controls the production of CFTR protein. The CFTR protein creates channels on the cell surface to allow the movement of chloride in and out of the cell. When the CFTR protein is not working properly, the balance of chloride and fluids is affected. 

CFTR modulators: A type of drug for people with CF, modulators work by restoring the function of the faulty CFTR protein made by the CFTR gene and treating the cause of CF. 

 

E 

Enzyme: A protein that speeds up biological reactions. 

 

F 

F508del: The most common genotype among people with CF. 

FEV1: A key measure of lung function, FEV1 refers to forced expiratory volume in 1 second.  

 

G 

Gene: A piece of DNA that contains all the necessary information to manufacture a protein 

Gastroesophageal reflux disease (GERD): A condition where stomach acid flows back into the esophagus. Also known as acid reflux, acid indigestion, or heartburn. 

 

H 

Hospital in the Home (HITH): Provides hospital care for people in their own home.  

 

I 

Infertility: Often defined as the inability to get pregnant after 12 months of trying. CF can cause fertility issues for women and men.  

Immune suppressed: Someone who has a lower resistance to infection due to having a compromised immune system.    

 

K  

Kalydeco®: The first CFTR modulator approved for use in Australia. The active ingredient in Kalydeco® is called ivacaftor. Ivacaftor binds to the defective CFTR protein at the cell surface and helps to open the channel so that chloride can flow in and out of the cells. This helps the balance of chloride and fluids at the surface of the cells and helps to thin mucus in the lungs and other organs. 

 

L

Lung function: A critical measure of overall health for people with CF. 

Lung transplant: A major surgery that replaces both lungs in a person with CF with healthy lungs from a donor. 

 

M 

Malabsorption: The failure to absorb nutrients. 

Mucolytic: A treatment or technique that thins mucus which then makes it easier to expel.  

 

N 

Nasal polyp: A growth in the nose or sinuses. 

Nebuliser: A device used to deliver inhaled medicines to the lungs. A nebuliser is the mouthpiece or mask that is used alongside a compressor machine. The compressor changes liquid medicine into a fine mist, which is then inhaled through the nebuliser. 

 

O 

Osteoporosis: A condition where bones become weak and brittle. Osteoporosis increases the risk of fracture. 

Orkambi®: A combination therapy, Orkambi has two active ingredients: ivacaftor and lumacaftor. Lumacaftor helps the F508del-CFTR protein change to its correct shape, move to the surface of the cell, and stay there longer. Ivacaftor then helps to open the channel so that chloride can flow in and out of the cells. 

 

P 

Pancreas: A gland that sits behind the stomach and secretes digestive enzymes into the upper bowel and insulin into the blood. 

Pancreatic sufficient: A person who has enough pancreatic function to absorb fat normally. 

Pathogen: A microscopic organism that causes disease. 

PICC line: A peripherally inserted central catheter (PICC line) is a type of intravenous access that can be used for extended periods of time.  

Polyp: A growth of tissue that protrudes from a surface in the body, often from a mucous membrane such as the lining of nose or bowel. 

Port-a-Cath (Port): A small medical appliance that is inserted beneath the skin. It is attached to a catheter. A port-a-cath or port is used to draw blood, provide fluids and drugs intravenously. A port may stay in place for months or years.  

Pseudomonas aeruginosa: A common bacteria, and a major cause of lung infections in people with CF. 

Pancreatic enzyme replacement therapy (PERT): Enzymes taken with every meal and snack to help with digestion. 

Pancreatitis: Inflammation of the pancreas. 

Pneumothorax: A collapsed lung caused by air between the lungs and the chest wall.  

Pulmonary function test: Non-invasive tests that measure how well the lungs are working. Tests commonly measure lung volume, capacity, rates of flow, and gas exchange. 

Pulmonologist: A doctor who specialises in lung and respiratory diseases. 

 

S 

Spirometer: An instrument for measuring the amount of air inhaled and exhaled.  

Sputum test: A test that is used to find and identify bacteria that infect the lungs or airways 

Staphylococcus aureus: A common germ that infects the airways of people with CF 

Sweat test: A test that measures the salt content of a baby’s sweat. This test is used to confirm a diagnosis of CF. 

Symdeko®: a combination therapy. It had two active ingredients, ivacaftor and tezacaftor. Tezacaftor works in a similar way to lumacaftor in Orkambi®. It helps the CFTR protein change to its correct shape, move to the surface of the cell and stay there longer. Ivacaftor then helps to open the channel so that chloride can flow in and out of the cells. 

 

T 

Trikafta®: a triple combination therapy. It has three active ingredients, elexacaftor, tezacaftor and ivacaftor. Elexacaftor and tezacaftor help the F508del-CFTR protein change to the correct shape, move to the surface of the cell and stay there longer. Ivacaftor then helps to open the channel so that chloride can flow in and out of the cells. 

 

V 

Vans deferens: The tube that moves sperm from the testicle to the urethra. For a large percentage of males with CF, the vans deferens are blocked.