Symptoms, Diagnosis and Treatment

What are the Symptoms of Cystic Fibrosis?

The most common symptoms of CF are:

  • Very salty-tasting skin
  • Persistent coughing, wheezing or shortness of breath
  • Excessive appetite but poor weight gain
  • Greasy, bulky stools

How is Cystic Fibrosis Detected?

CF is diagnosed through the sweat test, which measures the amount of salt in the sweat. A high salt level indicates that a person has CF.
CF also can be identified before birth through prenatal screening and after birth through newborn screening.

How is Cystic Fibrosis Treated?

Treatment for CF depends on the stage of the disease and the organs involved. It may include:

Airway clearance techniques to clear mucus from the lungs
One technique is called postural drainage and percussion. The person with CF sits, stands or lies in a position that helps free up mucus. The chest and back are pounded and clapped to loosen the mucus. A person with CF may use a mechanical vest or blow into a device that shakes the mucus loose.

Inhaled medicines
These may be taken with a nebulizer, a machine that changes liquid medicine into a fine mist which makes it easier to inhale deep into the lungs. Some medicines can also be breathed in through a metered dose inhaler (MDI). Inhaled medicines for CF include:

  • TOBI, an aerosolized antibiotic used to treat lung infections
  • Pulmozyme, a mucus-thinning drug shown to reduce the number of lung infections and improve lung function
  • Bronchodilator medicines to help open the airways
  • Hypertonic saline to help draw more water into the airways to thin mucus

Other treatments for CF

  • Azithromycin, an antibiotic that fights bacteria in the lungs
  • Anti-inflammatory medicines such as ibuprofen to help reduce swelling in the airways

Lung transplantation may be an option in some severe cases of CF.