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.  

BMI: Body Mass Index

 

C 

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

Cervix: The lowest part of the uterus.

Cervical Screening Test: A test where cells are collected from the cervix and looked at under the microscope for changes of cancer or pre-cancer.

Chlamydia: A type of sexually transmitted disease that can result in infertility for a woman.

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. 

 

D

Diaphragm: The muscle that separates the chest from the abdomen.
It is used to breathe in which explains why the tummy moves in and out with  breathing. Also, a form of contraception where a diaphragm, or artificial  barrier, is inserted into the vagina to sit over the cervix to prevent sperm from passing.

Dietitian: A health professional specialised in the nutritional contents of food and special diets.

 

E 

Enzyme: A protein that speeds up biological reactions. 

Endocrinologist: A doctor who specialises in the treatment of hormone problems.

Estrogen: A female hormone that is responsible for most of the changes that occur with puberty in females.

 

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.  

Fertility: The ability to contribute to the conception of a baby. For a male this means the ability to produce sperm and ejaculate; for a woman, this means the ability to produce eggs which can be fertilised and then implanted in the uterus.

 

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. 

Gastrostomy: A tube that goes through the skin into the stomach, used to give extra feeds.

GP (General Practitioner): Also known as a family doctor, local doctor.

Genetic counselling: Discussion with a health professional expert in the understanding of diseases transmitted from parents to children

Genital warts: A type of sexually transmitted disease that may come and go but can’t be cured. It still needs to be treated.

Gonorrhoea: A type of sexually transmitted disease that can lead to  infertility in a woman if untreated.

Growth spurt: A period of rapid, accelerated growth caused by hormone changes around the time of puberty.

Gynaecologist: A doctor who specialises in the conditions affecting the female reproductive tract.

 

H 

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

Herpes: A type of sexually transmitted disease that once caught, may come, and go, but cannot be cured.

HIV (Human Immunodeficiency Virus): A virus transmitted by sharing body fluids (blood, semen, and others), that is, by unprotected sex, sharing needles, or blood transfusion (which is a rare means of transmission now that donors and blood can be checked). This virus causes AIDS.

 

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.    

IUD (Intra-Uterine Device): A method of contraception where a small copper device is implanted into the uterus to prevent implantation of a fertilised ovum.

IVF (In-vitro Fertilisation): Literally ‘in glass’ fertilisation. A technique of becoming pregnant where an egg is fertilised with sperm in a test tube and then transferred into the woman.

 

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. 

Menarche: The onset of the first ever period.

Menstruation (Also known as a ‘period’ and many other nicknames): With cycles of female hormones, the lining of the uterus becomes thick and juicy ready for a fertilised egg to implant. If fertilisation
does not occur, the lining of the uterus is shed, coming out as a period.

MESA (Microscopic Epididymal Sperm Aspiration): Technique of collecting sperm with a needle to bypass blocked or absent transport tubes.

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. 

Nocturnal emissions: Sometimes men release semen while asleep. These events are commonly called wet dreams

 

O 

Obstetrician: A doctor who specialises in the care of pregnant women.

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. 

Ovaries (Singular: ovary): The organs where eggs (ova) are stored and released (ovulation) one-by-one with the cycles in female hormone levels.

Ovulation: The release of an egg (‘ovum’) ready to be fertilised.

Ovum (Plural: ova): A human egg, which is stored in the female’s ovaries. Ova contain genetic information from the mother to join with genetic information from the father to form the new individual

 

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. 

Pelvic floor muscles: Muscles in the pelvis that help to keep pelvic contents in the right place.

Physiotherapy (Also known as ‘airway clearance’): Physiotherapy for cystic fibrosis care involves clearance of sputum from the lungs using physical means such as huffing, positive pressure manoeuvres and exercise, and  training in better use of muscles, movement, and posture. Physiotherapy can also address urinary incontinence issues. 

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

Primary amenorrhea: The absence of periods, but before they have actually started. That is, delayed menarche.

Progesterone: A female hormone responsible for the preparation of the uterus for the implantation and development of a fertilised ovum.

Psychiatrist: A doctor specialised in the assessment and treatment of mental health.

Psychologist: A non-doctor health professional specialised in the assessment and treatment of mental health.

Puberty: A period of rapid change caused by changes in hormones. The end result of puberty is adult sexual maturity

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 

Secondary amenorrhea: The absence of periods, but after menarche has occurred.

Seminal vesicles: Parts of the male anatomy where semen is stored prior to release.

Sex hormones: Estrogen and progesterone for women and testosterone for men.

Sexual development: For women, sexual development includes growth of breasts, changes in the vagina and uterus, onset of periods and development of body hair. For men it includes growth of testes, changes in the penis and development of body hair. 

Sperm: Tadpole-like cells made in the testes which carry genetic information from the father. At fertilisation, the sperm joins with the egg (ovum) from the mother, leading to the eventual development of a new individual.

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.

STIs: Sexually transmitted infections.

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. 

Syphilis: A sexually transmitted disease which is now uncommon. This disease is treatable with antibiotics but if untreated can eventually lead to severe complications

 

T 

Testosterone: A male hormone responsible for growth of testes, changes in the penis, development of body hair, sexual drive, aggression, and muscle development. Females have a small amount of testosterone, which leads to the development of body hair.

The pill: An oral contraceptive. This is the most effective form of  contraception taken by women to prevent pregnancy, but it does not prevent STIs.

Thrush: (Also known as ‘candida’, ‘fungal vaginitis’.) A type of infection caused by overgrowth of yeasts. This occurs commonly when antibiotics disturb the normal balance of yeasts that live in the body.

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. 

 

U

Urinary incontinence (Also known as stress incontinence): Leakage of urine inappropriately which can occur with raised abdominal pressure such as with laughing, coughing.

Uterus: The female organs which sit in the pelvis. This is where the fertilised egg implants and where the foetus grows and develops for nine months.

 

V 

Vasectomy: A surgical procedure that prevents sperm leaving the penis during ejaculation.

Vas 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.