Cystic fibrosis (CF) is an
inherited disease. It causes problems in the body's cells that make salt, water, and
mucus. There is no cure for CF. It is a disease that gets worse over time. But better
treatments can now help people with CF live longer and healthier lives. Most people
CF live into their late 30s, and many into their 50s. Some people with CF now live
CF causes thick mucus to build up
and clog certain parts of the body such as the lung. The buildup is caused by an
abnormal gene called CFTR (cystic fibrosis transmembrane regulator). CFTR controls
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,
can affect all airways.
Children with CF may have lung
infections. This is because bacteria that are normally cleared from the lungs stay
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 have nasal polyps. These are small
growths of tissue from the lining of the nose. They go into the air-filled space above
and behind the nose (nasal cavity). Sometimes these polyps must be taken out by a
healthcare provider. Children with CF also often have sinus infections.
CF affects the respiratory system.
So a child with CF may have these symptoms:
Coughing up blood
A rounding and enlargement of
the tips of the fingers and toes (clubbing)
Frequent lung infections with thick phlegm
Shortness of breath
Inflamed nasal sinuses