How CF is treated

Management and treatment of CF is lifelong and ongoing. The severity of CF symptoms can vary from person to person, so treatments are tailored to each individual.

Treatment plans are prepared by specialist CF physicians in consultation with the person with CF and their family. People with CF generally visit a hospital CF clinic several times each year so their progress can be monitored.

Daily routines for people with CF will vary, but the most common treatments are:

  • Intensive daily chest physiotherapy, and other airway clearance techniques, to loosen mucus from the lungs
  • Taking enzyme replacement capsules with food to aid digestion
  • Antibiotic therapy to treat lung infections
  • Aerosol mist inhalations via a nebuliser to help open the airways
  • Salt and vitamin supplements
  • A nutritious diet that’s also high calorie, high salt and high fat
  • Exercise which is important to help clear the airways and build core strength

In recent years, a new type of medication known as CFTR modulators has become available in Australia. These drugs work by correcting the malfunctioning protein that causes CF. In doing so, these medications directly address the cause of CF rather than just the symptoms. They are not a cure but they help the body’s cells to function more normally.

CFTR modulators Orkambi and Kalydeco are now available on the Pharmaceutical Benefits Scheme (PBS) meaning that the Australian Government subsidises most of their cost. Another modulator drug, Symdeko, is currently being assessed for the PBS.

Hospital CF clinics assess people’s suitability to be prescribed these modulator drugs. Unfortunately these medications do not work for everyone with CF, but advances in pharmaceuticals and other treatments such as gene therapy mean that treatment options become greater every year.