Cystic Fibrosis and the Respiratory System
How does cystic fibrosis affect the respiratory system?
Cystic fibrosis (CF) is an inherited disease that causes problems in the cells of
the body that make salt, water, and mucus. There is no cure for CF. It is a disease
that gets worse over time. But improved treatments can help people with CF live longer
and healthier lives. Most people with CF live into their late 30s, and many into their
50s. Some people with CF now live into their 70s.
CF causes thick mucus to build up and clog certain parts of the body like the lung.
The buildup is caused by an abnormal gene called the cystic fibrosis transmembrane
regulator (CFTR). CFTR controls the flow of water and salt in and out of the body's
cells. Changes cause mucus to become thickened and sticky. This first affects the
small airways in the lungs. Over time, it can affect all airways.
Children with CF may develop lung infections. This is because bacteria that are normally
cleared from the lungs remain in the thickened mucus. Many of these lung infections
are long-term (chronic).
Children with CF also can have problems in their upper respiratory tract. They can
develop nasal polyps. These are small growths of tissue from the lining of the nose
that extend into the nasal cavity. Sometimes these polyps must be taken out by a healthcare
provider. Children with CF also often have sinus infections.
A child with CF may have these symptoms, because of how CF affects the respiratory
Coughing up blood
A rounding and enlargement of the fingers and toes (clubbing)
Frequent lung infections with thick phlegm
Shortness of breath
Inflamed nasal sinuses (sinusitis)