Cystic fibrosis (CF)

Need to know

• What are the symptoms of cystic fibrosis? icon plus

  Most cases of cystic fibrosis are picked up at birth. Common symptoms include:
  In the lungs

  • coughing
  • wheezing and shortness of breath
  • recurring chest infections due to mucus build-up in the lungs, an ideal breeding ground for bacteria

  Liver-related

  • an enlarged liver (hepatomegaly)
  • an enlarged spleen (splenomegaly) that develops when there is scarring in the liver
  • fluid build-up in the abdomen (ascites)
  • bleeding (bruising, nosebleeds and visible veins on the abdominal wall)
  • poor growth and muscle wasting
  • jaundice

  People sometimes develop other conditions towards the end of childhood, or in adulthood, such as:

  • delayed puberty
  • reduced fertility
  • diabetes
  • bone disease (weak bones)

• How is cystic fibrosis diagnosed? icon plus

  All newborns are screened for Cystic fibrosis as part of the newborn blood spot test. If your baby is suspected of having CF, two additional tests to confirm a diagnosis are needed:

  • The first is a sweat test to measure the amount of salt in sweat, which will be abnormally high in those with CF
  • The second is a genetic test where a sample of blood is checked for the gene that causes CF .

  These tests can also be used to diagnose cystic fibrosis in older children and adults who didn't undergo the newborn screening. Another reason why a person with CF was not diagnosed earlier is that the condition can vary widely in severity and symptoms, and can mimic other lung diseases such as asthma.

• Treatment options for cystic fibrosis icon plus

  There's no cure for cystic fibrosis but the condition can be managed with the following treatments:

  • Antibiotics, steroids and medicines to clear the air passageways
  • Regular exercise and physiotherapy
  • Active Cycle of Breathing Techniques (ACBT) – a cycle of deep breathing, huffing, coughing and relaxed breathing to shift the mucus and clear your airways
  • Dietary and nutritional advice from a dietician
  • In severe cases, a lung transplant when your lung has failed