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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 system: 

  • Chronic cough

  • Coughing up blood

  • Collapsed lung

  • A rounding and enlargement of the fingers and toes (clubbing)

  • Frequent lung infections with thick phlegm

  • Heart enlargement

  • Nasal polyps

  • Shortness of breath

  • Inflamed nasal sinuses (sinusitis)

Online Medical Reviewer: Blaivas, Allen J., DO
Online Medical Reviewer: Holloway, Beth Greenblatt, RN, M.Ed.
Date Last Reviewed: 11/1/2016
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