Cystic Fibrosis usually called "CF" is the most common life-threatening, genetic condition affecting more than 3000 Australian children, adolescents and adults. CF affects about one in every 2,500 babies born in Australia which means on average one baby is born with CF every 4 days. About one person in every 25, or approximately one million Australians unknowingly carry the faulty gene for CF. Carriers are healthy and do not experience symptoms of CF. The birth of a child with CF often comes as a total surprise to a family - most will have no previous family history of CF.
CF mainly affects the lungs, digestion and sweat glands, but can affect other parts of the body including the liver, nose & sinuses, and reproductive organs. CF is a complex disease caused by glands which produce fluid secretions in the body such as mucus, enzymes & sweat. The secretions are normally thin & slippery but in CF they are thick & sticky.
For people with CF the thick & sticky mucus in the lungs is difficult to clear, bacteria is often trapped in the small airways, leading to infection and inflammation.
Treatments involve inhalations via a nebulizer and up to 2 hours of chest physiotherapy every day to clear the thick mucus from the lungs. Oral and inhaled antibiotics are frequently used to treat infections. Regular visits to CF clinics and hospitalization are common - a chest infection usually requires a 2 week admission in hospital for IV antibiotics & intensive physiotherapy, we call them ‘tune ups’.
The thick & sticky mucus also causes blockages in the ducts of the pancreas, preventing digestive enzymes from reaching the small intestine where they would normally break down and digest food, resulting in incomplete digestion, poor absorption of vitamins and nutrients leading to malnutrition. This is called pancreatic insufficiency and occurs in about 85% of people with CF. Most people with CF need to take approximately 30 to 40 tablets a day: enzymes to help digest their food, vitamin supplements, salt tablets, antibiotics etc. Good nutrition for people with CF is vitally important in the management of their condition and they need to consume more calories than you or I just to maintain their weight, increase their chance of absorbing nutrients and to help with fighting off infections.
People with CF can also develop nasal polyps (small growths in the nose), CF related diabetes, osteoporosis, and cirrhosis or scarring of the liver which usually develops slowly & is stable over many years.
People with CF have sweat that is 5 times more saltier than people without CF. Activities like exercise that lead to sweating, and the hot summer weather we have in tropical NQ means there is always a risk of salt depletion and dehydration, which is why salt replacement with salt tablets, glucolyte, sports drinks, salty snacks & maintaining a good fluid intake is so important in CF.
Currently there is no cure for CF, and treatments are aimed at slowing the progression of the disease. The average life expectancy is just 37 years.