Cystic fibrosis

Cystic fibrosis

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About cystic fibrosis

Children with cystic fibrosis have too much salt in their sweat. They also produce thick, sticky mucus that blocks their lungs, clogs their airways and is difficult to cough up. These blockages trap bacteria, which leads to lots of infections and lung damage.

Because of this, children with cystic fibrosis might cough a lot, have difficulty breathing and keep getting chest infections.

Also, the thick mucus caused by cystic fibrosis can clog the pancreas, blocking the flow of pancreatic juices into the gut to digest food. Because their food hasn't been digested properly, children with cystic fibrosis can suffer from malnutrition, weight loss and diarrhoea.

Cystic fibrosis can lead to many long-term problems, like repeated chest infections, poor growth and diabetes.

Men with cystic fibrosis might be infertile, meaning they have trouble having children naturally.

Although many children with cystic fibrosis now survive well into adulthood, their condition means they might not live as long as other people.

Causes of cystic fibrosis

Cystic fibrosis is a genetic condition caused by a defect or abnormality in the gene that controls how much salt and water go in and out of the body's cells.

A baby might be born with cystic fibrosis if both parents are carriers of this gene. Most carriers of the gene are healthy without any symptoms.

Parents who have a child with cystic fibrosis have a one in four chance of having another child with the condition.

Diagnosis of cystic fibrosis

If you have cystic fibrosis in your family, it's a good idea to have genetic counselling and testing for cystic fibrosis. People who carry the gene can be identified with a simple blood test.

If you're pregnant and there's a possibility of your child inheriting the gene for cystic fibrosis, you can have further tests during pregnancy to work out whether your child is affected.

If you give your consent, your baby will be tested for cystic fibrosis as part of standard newborn screening in Australia. This is a simple test that involves taking blood from your baby's heel.

If this test shows that your baby has a high level of an enzyme called immunoreactive trypsin (IRT), genetic testing for common gene mutations can show whether your child has cystic fibrosis. If it's needed, this testing also happens as part of the newborn screening program.

A sweat test, which involves stimulating a small area of skin to produce sweat, is also used to diagnose cystic fibrosis.

Support and treatment for children with cystic fibrosis

There's no cure for cystic fibrosis.

If your child has cystic fibrosis, she'll probably be treated in a specialist unit by a specialist multidisciplinary cystic fibrosis team.

Treatments for cystic fibrosis can be intensive and time consuming. Children need to go to the specialist unit regularly, and some children need to go to hospital often to manage chest infections.

Physiotherapy and various other treatments can support children's health and improve the quality of life for children with the condition. Children usually need special treatments to clear their lungs two times a day. These treatments are usually administered by a parent or carer.

Daily antibiotics are usually necessary. Children with cystic fibrosis might also need nutritional supplements and steroids to treat lung infections. Doctors sometimes advise enzyme replacement therapy to help improve children's nutrition, as well as a high-energy diet, with salt and vitamin supplements.

If your child has cystic fibrosis, you and your child might work with any or all of the following health professionals:

  • genetic counsellor
  • paediatrician
  • respiratory physician
  • dietitian
  • physiotherapist.

Living with cystic fibrosis

Staying active, exercising and having chest physiotherapy can help children with cystic fibrosis keep their lungs healthy.

A child with cystic fibrosis might miss a lot of school because of frequent hospital visits. If your child has cystic fibrosis, it's important to let teachers know about his condition and to communicate with them regularly.

These days, with good treatment, people with cystic fibrosis can live productive lives well into middle age.