What is cystic fibrosis?
Cystic fibrosis or “CF” is a genetic condition which affects each individual differently. People with CF are missing an essential protein from their cells which results in mucus in the body becoming thick and sticky, building up in organs such as the lungs and the pancreas.
The mucus causes impairment of the pancreas and traps bacteria in the lungs causing recurrent infections which lead to irreversible damage.
There is currently no cure.
How do you get cystic fibrosis?
CF is an inherited genetic condition. In the Australian population, 1 out of 25 people carry the CF gene. If both parents each carry a CF gene, they have a 1 in 4 chance of having a child with CF with every pregnancy. There are over 1800 different gene changes that causes CF. Find out more about CF and options for carrier screening from Dr Sean Fabri.
How is cystic fibrosis diagnosed?
Since the year 2000, all babies born in Australia are tested for the most common gene changes that cause CF, using the Guthrie Heel Prick blood test. If the test comes back positive then a sweat test will be done to measure the amount of salt in the sweat. Most babies born with CF in Australia will be diagnosed within the first 2 months of life.
To find out if you are a carrier of the CF gene, have a chat with your GP, obstetrician or contact the genetic services in your state. You can also find out more on the Cystic Fibrosis Community Care website.
What are the symptoms?
People who have CF can be affected differently by the condition, however some common symptoms are:
- frequent respiratory infections
- bowel issues
- poor appetite and weight loss
- shortness of breath
- tiredness and lethargy
- increased risk of dehydration
How does cystic fibrosis affect the body?
The build-up of thick and sticky mucus occurs in the airways of the lungs and can lead to infection and inflammation, causing airway and lung damage over time. Regular physiotherapy using airway clearance devices and/or nebulisers, exercise and oral antibiotics are used to prevent and manage infections in the lungs.
The Digestive System:
85% of people who have CF suffer from malabsorption (difficulty in digesting food) due to mucus blocking the pancreas. This can lead to low levels of vitamins being absorbed, poor weight gain and compromised lung function.
Enzyme replacement therapy (either in capsule or granule form), vitamins and a high energy diet of 20% to 50% more calories, is often recommended to assist in counteracting the effects of malabsorption.
People with CF lose between two to five times more salt in their sweat than average. The salt loss can lead to dehydration, which can have serious consequences. People with CF need to take salt supplements as well as consume extra salt in their diet. Even more salt supplements and fluids are required in summer and in warmer climates.
Cystic fibrosis can also cause issues with:
- the vas deferens
- pelvic floor
- bone density
- CF related diabetes (CFRD)
- increased risk of depression and or anxiety
Due to great advances in research and treatment, people with CF are living much longer than ever before. People with CF are now travelling, working full or part time and raising families. It is vital that young people with CF have access to all educational opportunities available so that they can prepare for their lives as adults.
CFSmart resources for schools and the community
To improve understanding of cystic fibrosis (CF) within schools and the community we have developed a range of resources which include teachers guides, student health plans, CF Fact Sheets and free CF e-learning modules.