Adult CF: The Respiratory System

Cystic fibrosis (CF) is an inherited disease that causes problems in the body's cells that make salt, water, and mucus.

CF is caused by a change in the CFTR gene. CFTR stands for cystic fibrosis transmembrane conductance regulator. This change, or mutation, causes a problem in the CFTR protein that controls the flow of water and salt in and out of the body's cells. If you have CF, this protein doesn’t work right. So mucus and other secretions become thick and sticky.

Improved treatments can help people with CF live longer and healthier lives. Many people with CF live past age 30. Life expectancy is improving with continued development of new treatments.

Some people who have advanced CF may be considered for lung transplant. But this doesn't cure CF or stop it from harming other parts of the body.

You can also have problems in your upper respiratory tract. You 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 health care provider. You may also have sinus infections.

CF’s effects on the respiratory system also can cause these symptoms:

• 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

• Shortness of breath

• Fever

• Loss of appetite

• Weight loss

• Wheezing

• Inflamed nasal sinuses (sinusitis)