What is Bronchiectasis?

Cystic fibrosis is a chronic condition in which a mutation of the cystic fibrosis trans-membrane regulator (CFTR) protein causes the body to produce abnormally thick, sticky mucus. When this mucus becomes lodged in the respiratory passages, infection is likely to result. Blockage and subsequent infection can cause the airways to become weak and stretched out, scarred, and otherwise deformed. This condition is called bronchiectasis.

Nearly 50% of all reported instances of bronchiectasis are associated with the inflammation and infections associated with cystic fibrosis. Sometimes, the diagnosis of bronchiectasis is made prior to a formal diagnosis of cystic fibrosis. Bronchiectasis can also develop in patients who have allergic bronchopulmonary aspergilliosis (ABPA).

The severity of bronchiectasis is related to which of three types of the condition are present.

1) Fusiform (Cylindrical)

2) Varicose

3) (Saccular) Cystic

Cylindrical forms are often caused by acute bronchitis or other lung infection. In this form, the respiratory passages are only slightly widened, similar to how a garden hose would expand when filled with water. This type of bronchiectasis can be reversed, as there are no collapsed sections of the bronchial wall. Varicose bronchiectasis, is a more serious form, characterized by extended and collapsed portions of the bronchial wall. The most serious and irreversible form is cystic bronchiectasis, in which the bronchi are greatly ballooned.

Bronchiectasis is usually identified during a routine chest x-ray as part of a cystic fibrosis patient’s annual lab tests. Further imaging such as a CT scan as well as pulmonary function tests and a sputum cultures will help the doctor determine the underlying cause, extent and severity of lung damage.

Bronchodilators such as albuterol, are prescribed to help open the airways. Fluticason and ipatropium are useful in controlling inflammation. Percussive respiratory therapy and postural drainage designed to dislodge the mucus is also used. Patients with cystic fibrosis will often use a combination of respiratory therapy including nebulized antibiotics to help control infection, bronchodilators, mucolytics and a vibrating vest such as the InCourage or ThAIRapy vest. Each of these therapies is important not only to control infection, but to slow the rate of declining lung function. Bronchiectasis is less likely to develop if infections are treated early.

In 2005 the FDA approved an inhalation powder called Bronchitol. Phase II clinical studies demonstrated adequate safety and effectiveness in patients at risk for developing bronchiectasis. The medication works by stimulating mucus hydration, enabling the usually thick, sticky mucus to be cleared more easily. Nebulized hypertonic saline produces a similar effect.