Bronchiectasis (brong-kee-EK-tuh-sis) is a type of lung damage that is common in people with CF. It occurs when the bronchi (walls of the airways) that carry air in and out of the lungs thicken and widen, then become flabby and scarred. Bronchiectasis often results from repeated lung infections.
These less flexible airways, combined with the thick mucus of CF, make it harder for the lungs to clear pollen, bacteria, viruses, and fungi from the lungs as they should. When mucus cannot be cleared, it creates an environment where germs stay too long in the lungs, leading to more serious lung infections. This leads to a cycle of more lung infections and even more damage to the airways (more severe bronchiectasis).
Bronchiectasis is irreversible; therefore, it is important to treat lung infections aggressively.
Causes of bronchiectasis
CF causes almost 50% of the cases of bronchiectasis in the U.S.1 One study reported that almost a third of children with CF showed signs of bronchiectasis by age 3, along with mucus obstruction and inflammation on CT scan.1,2
In children, the types of lung infections that can lead to bronchiectasis include:
- Haemophilus influenzae (flu)
- Staphylococcus aureus (staph infection)
- Pneumonia
- Fungal infections
- Whooping cough, measles
- Pseudomonas aeruginosa
- Tuberculosis2
As the disease progresses, children and adults with CF become more susceptible to other bacteria that target the lungs, including Pseudomonas aeruginosa, Stenotrophomonas maltophilia and Achromobacter, Burkholderia cenocepacia, mycobacterium abscessus and mycobacterium avium-intracellulare. These more serious infections may need to be treated with intravenous antibiotics, which may mean a hospital stay.1
Symptoms of bronchiectasis
Signs and symptoms of bronchiectasis become apparent after repeated lung infections. The most common symptoms are much like other symptoms of CF-related respiratory infections because both infections and bronchiectasis lower blood oxygen levels. These include:
- Daily, frequent cough
- Large amounts of mucus (sputum) coughed up daily
- Shortness of breath or wheezing
- Chest pain
- Clubbing of the fingers and toes
Diagnosis of bronchiectasis
A CT scan of the chest is the most common test used to diagnose bronchiectasis. CT stands for computed tomography.
Other tests that your doctor may order include a chest X-ray, blood tests, sputum culture, and lung function tests or PFT.
Treatment for bronchiectasis
Treatment for bronchiectasis includes treating the lung infection with medicine, removing mucus through airway clearance techniques, and staying hydrated. Hydration helps reduce the thickness of the mucus.
The drugs used to treat a lung infection include antibiotics (if your infection is caused by bacteria), mucus thinners, bronchodilators, expectorants, or inhaled steroids.
If treatments do not seem to be working, your doctor may order a bronchoscopy. This test is used to look inside the airways. This thin tube with a light on the end is inserted through the mouth or nose and travels into the airways to see if there is something blocking the airways or if there is bleeding.3
If the bronchiectasis is more severe and causing respiratory failure, oxygen therapy may be recommended. Surgery may help if only one part of the lung is impacted or there is bleeding that needs to be stopped.3
Each infection damages the airways. Over time, this prevents enough oxygen from reaching the other vital organs, which can lead to respiratory failure and heart failure. Some people with bronchiectasis may need a lung transplant in order to survive.1,3
Living with bronchiectasis
Many people living with CF are also living with bronchiectasis. To prevent more lung damage, it is important to practice infection control techniques. Washing your hands and getting the flu and pneumonia vaccines are important first steps in staying as healthy as possible. Other tips include staying six feet from other people with CF and avoiding second-hand smoke, dusty or pollen-filled environments, indoor and outdoor fires, and buildings under construction.