Cystic fibrosis is an inherited disorder that primarily affects the lungs, but also affects the digestive system and other organs. Individuals with cystic fibrosis have a build-up of mucus in the lungs that leads to symptoms such as recurrent cough, lung infections, shortness of breath, and chronic difficulty breathing. Cystic fibrosis is progressive and can eventually lead to respiratory failure.
Rapid worsening of symptoms in individuals with cystic fibrosis are called pulmonary exacerbations (PEx).
Symptoms of PEx may include:
- increased cough
- shortness of breath
- increase in mucus (called sputum)
- chest pain
- loss of appetite or weight lost
Individuals with CF who have more episodes of PEx have been shown to have worse long term health outcomes. Currently there are no approved therapeutics or management guidelines specific to pulmonary exacerbations.